bluuebunny/crossref_metadata_2025 huggingface dataset:
Gastric Carcinoma with Lymphoid Stroma: A Rare Case Report with EBV Negativity
Authors: Alqassim E , Taherian M , Huang Y
Published: October 2020

Introduction/Objective

Gastric carcinoma with lymphoid stroma (GCLS) is a distinct and rare histologic subtype of gastric adenocarcinoma that is characterized by undifferentiated carcinoma mixed with prominent lymphoid cell infiltration. Around 80% of GCLS cases are associated with Epstein-Barr virus (EBV) infection. Here we report a case of EBV-negative GCLS.

Methods

The patient was a 79-year-old female with a recent history of abdominal distention, weight loss, and gastrointestinal bleeding who presented for evaluation. Endoscopy found a 4.6 mm lobulated lesion at the incisura of the stomach with endosonographic evidence of invasion through the submucosa. Biopsy was consistent with intramucosal diffuse neoplasm, favoring adenocarcinoma. The decision was made to do subtotal gastrectomy.

Results

The patient underwent distal gastrectomy with Billroth 2 reconstruction. Gross examination of the stomach revealed a firm 1.4 cm nodule in the background of diffuse mucosal granularity in the adjacent stomach.

Microscopically, the tumor invaded the submucosa without lymph nodes involvement.

Staining showed the tumor diffusely positive for CAM5.2, focally and weakly positive for pancytokeratin and CK7, while negative for CK20. Mismatch repair (MMR) proteins staining demonstrated a loss of nuclear expression of MLH1 and PMS2. Her2/Neu FISH was non-amplified. The tumor was found to be EBV negative by EBER ISH while PD-L1 was expressed. The histopathological findings confirmed the final diagnosis of gastric carcinoma with lymphoid stroma (GCLS).

Conclusion

We report a case of GCLS that was EBV negative with non-amplified HER2, while MMR-deficient, and PD-L1 positive. These results suggest that this tumor subtype may be less responsive to targeted therapy, but show susceptibility to immune checkpoint inhibitors.
Segmental Diffuse Inflammatory Pseudopolyps Associated With Diverticulitis
Authors: Aghighi M , Rashidbaigi A , Kang Y
Published: October 2020

Introduction/Objective

Inflammatory pseudopolyps (IPs) often develop in response to chronic active inflammation in patients with inflammatory bowel disease (IBD). IPs can be diffuse and numerous in severe IBD. IPs can also occur from other chronic active injuries, such as ulcers, persistent infection, ischemic colitis, anastomotic site and diverticulitis. IPs arising from diverticulitis is uncommon and generally few in numbers.

We report a rare case of a patient with segmental diffuse IPs associated with diverticulitis in the absence of IBD.

Methods

A 59-year-old male patient with a history of sigmoid diverticulitis presented with abdominal pain and was treated with antibiotics. Three months later he had severe recurrent diverticulitis. CT scan showed wall thickening of the sigmoid colon and multiple sigmoid diverticula. Colonoscopy showed extremely severe inflammatory colitis in the area of diverticulitis between 25.0 and 35.0 cm from the anal verge. Biopsies revealed cryptitis, crypt abscesses, crypt architecture distortion and Paneth cell metaplasia. The patient underwent laparoscopic left colectomy.

Results

The rectosigmoid colon specimen is 18.0 cm in length and 6.0 cm in diameter. There was a 7.5 cm segment with numerous polyps. Multiple diverticula with diverticulitis were also identified in this region. The rest of the colon was unremarkable. Microscopic examination revealed numerous IPs and multiple diverticulitis. No evidence of IBD was identified.

Conclusion

To our knowledge, this is the first time that segmental and diffuse IPs are described with associated diverticulitis in the absence of IBD. The pathogenesis of IPs in non-inflammatory bowel disease is not well described. Previously, it was reported that diverticulosis and subsequent diverticulitis has no significant impact on the development of IPs, and IPs may arise independent of location and time of diverticulitis. Sporadic IPs may also appear without any underlying pathology. In the current case, there were multiple diverticula and diffuse diverticulitis, which may have contributed to the diffuse IPs. Further clinical inquiries revealed no clinical signs and symptoms of IBD.
Hepatoblastoma With Rubinstein-Taybi Syndrome: A Rare Association
Authors: Arshi J , Allison D , Rao D
Published: October 2020

Introduction/Objective

Hepatoblastoma is a malignant neoplasm of liver, exclusively seen in pediatric population. It has been known to occur with various syndromes like Li-fraumeni, Beckwith-Wiedemann, and Simpson-Golabi- Behmel among others. Small undifferentiated cells and macro-trabecular pattern with increased mitotic activity and AFP>100 are associated with unfavorable prognosis. Rubinstein-Taybi syndrome is a rare genetic disease. Even though there has been intensive research on the genetic and epigenetic function of the CREBBP and EP300 genes in animal models, the etiology of this rare and devastating syndrome is largely unknown. Hepatoblastoma with Rubinstein-Taybi syndrome is an extremely rare occurrence.

Methods

Here we present an incidental case of Hepatoblastoma with Rubinstein-Taybi syndrome

Results

A 17-month-old Caucasian female with Rubinstein-Taybi syndrome, was found to have an incidental 1.1 cm nodule in the right lobe of liver. Her AFP was elevated (244.9ng/ml). Hepatic resection revealed a well-circumscribed tan white mass, measuring 3.5 cm in the greatest dimension. On microscopy, uniform round to cuboidal cells resembling fetal hepatocytes, with finely granular cytoplasm, small round nuclei, and indistinct nucleoli were seen. The cells were arranged in solid sheets and scant trabecular arrangement separated by narrow sinusoids. The tumor displayed predominantly fetal histology, with rare embryonal and small undifferentiated cell nests. The diagnosis of Hepatoblastoma was made. The patient underwent chemotherapy and was doing well at 5-years follow-up.

Conclusion

Extensive screening of patients in recent times has revealed mutations in CREBBP and EP300 in only around 50% of cases. The genetic and epigenetic associations of the syndrome in the remaining half of cases remains to be identified. Hepatoblastoma with Rubinstein-Taybi Syndrome is an extremely rare occurrence and might shed some light on other genes that could be involved in this syndrome. Further studies to review the mutation spectrum to understand the causative molecular mechanisms are deemed essential.
Unusual Case of Multiple Synchronous Gastrointestinal Stromal Tumors of the Jejunum Presenting as an Arteriovenous Malformation
Authors: Murzabdillaeva A , Taha H , Hausner R J
Published: October 2020

Introduction/Objective

Gastrointestinal stromal tumor (GIST) arises from the interstitial cell of Cajal. 30% of GISTs arise in the jejunum. GISTS are generally solitary although multiple GISTs may be synchronous or metachronous. There are a few reports of a GIST of the jejunum mimicking a vascular malformation/arteriovenous malformation (AVM) prior to surgical excision.

Methods

Herein, we report a case of four synchronous GISTs of jejunum. One GIST, highly vascularized, presented with lower gastrointestinal bleeding. The patient was a 40-year-old female presenting with epigastric pain and melena.

Results

She was found to have an ulcerated lesion in the distal jejunum by capsule enteroscopy and double balloon enteroscopy, interpreted as an arteriovenous malformation and tattooed. Several other non-ulcerated “polyps” were described. Gross examination of the ensuing segmental resection of jejunum demonstrated four bosselated tumors ranging from 3.1 cm to 6.5 cm. Microscopically, three tumors did not extend to the surface of the jejunum and were predominantly composed of spindle cells. One tumor (identified by tattoo) extended to the surface with ulceration. This tumor was dominated by wide vascular channels with a spindle cell component between the channels. The four tumors were each positive for CD117, DOG1, and Succinate dehydrogenase B (SDH), identifying the four tumors as non-SDH-deficient GISTs. Ki-67 proliferation index was less than 5% in all four masses.

Conclusion

Multiple GISTs are rare, classified as either sporadic or familial. Familial GEISTs are described in neurofibromatosis type 1, Carney’s triad and Carney-Stratakis syndrome. Pediatric GISTs, with clinical and genetic features often differing from typical adult tumors may also be multiple. Our patient’s four GISTs are considered multiple synchronous sporadic neoplasms. This case serves as an important reminder for pathologists consider GIST in the evaluation of a highly vascular gastrointestinal proliferation and keep in mind the possibility of multiplicity.
Case Report: Malakoplakia Developing in the Graft Colon Post Intestinal Transplant
Authors: Dimopoulos Y , Zeck J , Kallakury B
Published: October 2020

Casestudy

Malakoplakia is a rare chronic granulomatous disease thought to be caused by a macrophage bactericidal defect and most commonly affecting the genitourinary tract. Most cases are associated with chronic infections or immunosuppression, with most transplant related cases observed after renal transplantation.

Results

A male in his thirties developed short gut syndrome secondary to injuries and subsequently underwent a small and large bowel transplant and was treated with immunosuppressive medications.

He developed multiple episodes of acute rejection and multiple infectious foci, including a peri-rectal fluid accumulation growing Escherichia Coli, bacteremia with Klebisella oxytica, and Epstein Barr Virus viremia. Functional imaging revealed increased activity in mesenteric lymph nodes and in the peri-rectal fluid accumulation. On endoscopy, a rectal ulcer associated with mucosal protuberance was noted and tissue was obtained for pathologic diagnosis.

Histologic examination revealed mucosal infiltration by numerous macrophages with abundant granular cytoplasm. Michaelis-Gutmann bodies (considered pathognomonic for malakoplakia) were identified showing PAS and Von- Kossa positivity. Acid fast stain was negative. These findings supported the diagnosis of malakoplakia.

Other differential diagnoses were considered, including post-transplant lymphoproliferative disorder, neoplasia, infection with mycobacterial or fungal species, and secondary hemophagocytic lymphohistiocytosis syndrome. These were excluded based on the characteristic histologic findings.

Given the diagnosis, treatment with oral antibiotics was attempted. Follow up biopsies revealed disease persistence and the patient was transitioned to a prolonged course of intravenous antibiotics and bethanechol, with eventual histologic response. Unfortunately, due to stenosis and adhesions that developed secondary to the episodes of acute rejection, the patient eventually underwent graft explanation.

Conclusion

Our case highlights the risk factor milieu encountered in intestinal transplant patients and the prolonged treatment often required to treat malakoplakia. Additionally, malakoplakia constitutes an unusual consideration in intestinal transplant patients and prompt diagnosis is required to direct proper management of these patients.
Checkpoint Inhibitor-Induced Colitis: A Case Series
Authors: Avalos Hernandez S R , Anderson S A , Dal Zotto V
Published: October 2020

Introduction/Objective

Checkpoint inhibitors are novel immune-stimulating antibodies that have revolutionized the management and prognosis of several malignancies. The primary targets are cytotoxic T-lymphocyte–associated antigen-4 (anti-CTLA-4; e.g. pembrolizumab) and programmed cell death-1 receptor (anti-PD-1; e.g. ipilimumab and nivolumab). In spite of the significant advantages, many immune-related adverse effects have been identified. One of which is checkpoint inhibitor-induced colitis (CIC). Although there is awareness of the histopathologic features of anti- CTLA-4 induced colitis, there is much to be discovered about the pathologic features of anti-PD-1 colitis.

Methods

We herein report three cases of CIC. There were two women and 1 male (age range, 50 to 73-years-old, mean 64-years-old) who presented with diarrhea and/or hematochezia after multiple cycles of pembrolizumab or ipilimumab/ nivolumab combination therapy. Endoscopic examination was abnormal in all of these cases.

Results

The histologic features were similar in two cases, with moderate active chronic colitis and one case with focal active colitis. Two of the three patient were given steroids in addition to their regular medications with symptom improvement. One patient was removed off all medications and enrolled into hospice due to disease progression. CIC has been an increasingly recognized immune-related adverse effect that has a wide spectrum of clinical presentations ranging from mild diarrhea and abdominal pain to severe colitis and intestinal perforation. However, it is thought to be underestimated. Histologically, CIC can mimic inflammatory bowel disease, microscopic colitis and active colitis. Our cases showed histopathologic features mimicking those of ulcerative colitis.

Conclusion

Awareness of CIC is crucial for the multidisciplinary management essential for these patients. The histopathologic pattern coupled with the clinical history can allow pathologists to confirm the diagnosis of CIC and facilitate timely diagnosis and treatment.
Collision Tumor of the Kidney with Clear Cell Renal Cell Carcinoma and Papillary Renal Cell Carcinoma Components: Report of a Rare Case
Authors: Avalos Hernandez S R , Liu S , Sameeta F , Mneimneh W
Published: October 2020

Introduction/Objective

Collision tumors refer to the phenomenon where distinct, well-defined tumor subtypes are present within a single lesion. While this phenomenon has been described in different organs, it has been rarely encountered in renal tumors outside the context of rare tumor susceptibility syndromes. Collision tumors of the kidney may encompass any known benign or malignant renal tumor types. However, a collision tumor of two different renal cell carcinoma (RCC) subtypes is remarkably rare.

Methods

A 43-year-old male was referred to our institution for the management of a right lower pole renal mass. The right radical nephrectomy specimen revealed a 6.5 x 5.0 x 4.0 cm well circumscribed, yellow-orange and hemorrhagic cortical mass abutting the renal capsule and extending into the renal sinus fat. A grossly distinct 1.5 x 1.4 x 1.2 cm pale, nodular area was demonstrated at the periphery of the tumor.

Results

Microscopically, the tumor displayed two distinct, neoplastic components within the same mass, without transitional morphology. The majority of the tumor consisted of a nuclear-grade-2 conventional clear cell RCC, while the peripheral nodule represented a type-2 papillary RCC component. Immunostains further supported these findings: The papillary component was strongly and diffusely positive for CK7 and P504S/AMACR with variable EMA and vimentin expression, and negative for CAIX, while the clear cell component was positive for CAIX, EMA and vimentin with only focal and weak staining for CK7 and faint nonspecific P5O4S/AMACR staining. TFE3 Mart1 and HMB45 immunostains were negative in the tumor.

Conclusion

Collision tumor of the kidney with two distinct RCC subtypes is an exceedingly rare finding. Careful gross examination may be the first clue to identify such lesions, and sampling of all grossly distinct tumor areas is crucial.

The identification of collision tumors may have important therapeutic implications, given the difference in pathophysiology and outcome between RCC subtypes.
Calretinin-Positive Mucosal Innervation (C-mi) as a Potential Biomarker to Predict Fibrosis in Crohn’s Disease (CD): A Pilot Study
Authors: Arslan M , Li H , Lee H , Mikula M , Ahn S
Published: October 2020

Introduction/Objective

There is no reliable biomarker to predict the degree of fibrosis in CD. Quantification of C-mi has been used as a surrogate for enteric neural structures in Hirschsprung disease. The quantity of C-mi at the proximal margin may correlate with the degree of fibrosis in resected CD.

Methods

Ileocolonic resection cases for 20 CD and 3 trauma (control) were retrieved. Cases with severe mucosal inflammation at the margins were excluded. The proximal and distal margin sections were subjected to calretinin immunohistochemistry. Random mucosal images were captured from scanned slides (x200, JPEG), submucosa was edited out, and C-mi was calculated by image processing and analysis. rC-mi was defined as the mean C-mi of proximal margin normalized by that of distal margin. Cases with rC-mi less than the mean rC-mi + 2 SD of the controls were excluded. The maximum thicknesses of submucosa and muscularis propria were measured at the most stenotic site, microscopically. Pearson’s correlation test and Student’s t-test were performed to correlate the parameters and compare the means, respectively.

Results

A total of 216 images were captured (mean 9.3 images per case; range 7 to 10). The mean rC-mi of CD (1.26) was greater than the controls (mean 0.43) (p<0.05). The mean rC-mi + 2 SD of the control group was 0.91, thus 2 CD cases were excluded from final analysis. The rC-mi of CD showed an inverse relationship with maximum submucosal thickness (mean 3.4 mm, range 0.2 to 5.9) at the site of stenosis (r=-0.47; p<0.05), but not with muscularis propria.

Conclusion

The rC-mi of CD was inversely correlated with submucosal fibrosis. Altered stromal integrity, impaired intercellular signaling, progressive reduction and loss of telocytes induced by submucosal fibrosis may reduce the regenerative capacity of enteric neural structures in CD. Therefore, a decreasing trend in rC-mi may predict fibrosis progression in CD.
Intraductal Papillary Adenocarcinoma Of The Bile Duct With Associated Invasive Neuroendocrine Component: Case Report
Authors: Alhussain A , Abraham A , Grunes D , Subramony C
Published: October 2020

Casestudy

Intraductal papillary neoplasm of the bile duct (IPNB) is a variant of bile duct carcinoma that is characterized by intraductal growth and better outcomes compared with common cholangiocarcinoma. IPNBs are mainly found in patients from Far Eastern countries, where hepatolithiasis and clonorchiasis are endemic.

Approximately 40%-80% of IPNBs are associated with invasive carcinoma. The invasive carcinomas are most often tubular or mucinous type. Invasive neuroendocrine component has not been previously described.

We present a case of 69 year-old African American male who presented with worsening abdominal pain and jaundice over few months. Computed tomography showed 4 cm mass near the porta hepatis with massive ductal dilatation. A fine needle aspiration of the mass was performed and a diagnosis of adenocarcinoma was made. Subsequently, the patient underwent Right Trisegmentectomy.

Conclusion

Histologic sections revealed intraductal papillary neoplasm in the bile duct lumina. The papillae were lined by columnar epithelial cells with delicate fibrovascular cores. The tumor cell nuclei were slightly elongated, hyperchromatic and basally located without significant multilayering. The cytoplasm was eosinophilic with occasional intestinal differentiation. Frank invasion of the stalk and underlying periductular tissues was identified. Interestingly; the invasive component had different cytological features than the intraductal component, it was composed of small to medium sized cells with granular cytoplasm. The nuclei were uniform and round with “salt and pepper” chromatin. The invasive component stained positively for the neuroendocrine markers (Synaptophysin, Chromogranin and CD56), whereas the intraductal component stained positively for CK7, CK20 and CDX2 and negative for neuroendocrine markers.

Three years later, a recurrent tumor was found in the left lobe on imaging. Biopsy of the lesion showed metastatic neuroendocrine carcinoma. The intraductal papillary neoplasm with invasive neuroendocrine component is very unusual tumor, we think more studies are warranted to best evaluate the treatment options and the prognosis of such neoplasms.
Identification Of Strn-Ntrk2 Rearrangement In A High Grade Sarcoma, With Good Clinical Response To Firstline Larotrectinib Therapy
Authors: Lin R , Wang Z , Jiang W , Basu-Mallick A
Published: October 2020

Casestudy

Gene fusions involving tropomyosin receptor kinase genes, NTRK (NTRK1-3), are important in tumorigenesis. Larotrectinib, a selective NTRK inhibitor, is recently approved to treat NTRK fusion positive solid tumors. We herein report a case of soft tissue sarcoma harboring two STRN-NTRK2 gene fusions, with good clinical response to firstline larotrectinib treatment.

Results

A 35 year-old female presented with pain in the right gluteal region, and a large solid mass without overlying erythema, edema and induration was identified. Initial MRI study showed a heterogenous, vascular and partially necrotic mass (16.5 x 12.9 x 10.4 cm) centered in the right gluteus medius and maximus muscles. A core biopsy of the mass showed a cellular mesenchymal neoplasm with round/ovoid cells, high mitosis (21 per 10 HPFs) and focal staghorn type vessels, reminiscent of solitary fibrous tumor. However, STAT6 immunostaining was negative.

Additional immunostains show no specific lineage. Our in-house NGS fusion panel showed two in-frame STRNNTRK2 fusions, containing the same 5’ partner sequence (exon 1-3) of STRN, with the 3’ fusion partner starting from either the exon 15 or the exon 16 of NTRK2. Due to the large size and location of the tumor, larotrectinib was initiated as firstline therapy. The patient noticed a quick amelioration of tumor related pain, and a significant shrinkage of the size of tumor following the initial 7-day treatment. On post-treatment day 52, MRI showed the tumor significantly decreased in size to 7.7 x 7.4 x 6.6 cm with satisfactory symptomatic relief.

Conclusion

NTRK2 fusions are relatively rare when compared with NTRK1 and NTRK3, especially in sarcoma. Of note, the only other report in the literature of NRTK2 fusion- positive sarcoma also showed SFT-like morphology, and the patient responded well to larotrectinib as second line therapy.
Tactile Corpuscle-like Bodies in Rectal Mucosa - A Mysterious Misleading Lesion
Authors: Anderson S A , Dal Zotto V
Published: October 2020

Introduction/Objective

Tactile corpuscle-like bodies (TCLB) are specialized mechanoreceptors derived from Schwannian structures, resembling Wagner-Meissner corpuscles which are often found in the skin and within schwannomas, neurofibromas, and melanocytic nevi. However, their presence in gastrointestinal mucosa is a rare phenomenon. To our knowledge, only 27 cases have been reported in the English literature.

Methods

We present two cases showing this rare entity in rectal mucosa. The first case is in a 34-year-old female was a past medical history significant for Ehlers-Danlos syndrome. The second case was submitted in consultation, of a 64-year-old male. In both cases, rectal polyps were identified on endoscopy.

Results

Histologically, clusters of unencapsulated, lamellated spindle cells in the lamina propria were identified. Immunoperoxidase stains using antibodies against CD68 and S100 protein were performed on both specimens. Additionally, on the first case synaptophysin was also performed. The laminated clusters were strongly positive for S100 and negative for CD68. The first case showed focally positive synatophysin. Although the etiology of TCLBs is still unclear, they are considered to be benign, incidental neural proliferations. The histological differential diagnosis includes: schwann cell hamartomas, neurofibromas, ganglioneuromas, mucosal neuromas, nodular amyloid deposition, or granulomas. However, those in question can be differentiated with the use of S100 protein and histiocytic markers.

Conclusion

Awareness of TCLBs is important. Although they are benign neural proliferations, it is important to differentiate from histologic mimics often associated with inherited syndromes.
Identification Of Liver Fluke Eggs In Bile Duct Brush Cytology In An 82 Year Old Man With A Hepatic Mass: Report Of A Case
Authors: Chen F , Aguero-Rosenfeld M , Simsir A , Brandler T C
Published: October 2020

Introduction/Objective

Clonorchis sinensis infects the liver, gallbladder, and bile ducts in humans. It is acquired via consumption of raw/undercooked fish, crabs, or crayfish in endemic areas. Though majority are asymptomatic, long lasting infections may cause severe disease. Without treatment, human infections may persist for the parasite lifespan (25–30 years). Diagnosis in cytology is challenging with a potentially acellular sample with miniscule eggs that can be overlooked as an artifact. Here we reported a rare case of liver fluke eggs diagnosed in bile duct brush cytology.

Methods

An 82 year old man who emigrated from China 20 years prior presented to the hospital with worsening jaundice for 2 weeks. Abdominal CT revealed a 4.8 x 2.9 cm central right hepatic lobe mass extending to the hilum, obstructing the bile ducts, resulting in severe intrahepatic ductal dilatation and stricture and focal occlusion of the right anterior portal vein. His CBC revealed eosinophilia. Bile duct brushing was submitted for cytology.

Results

ThinPrep and cell block showed oval-shaped parasite eggs measuring 10-24 µm. There was no other cellular matter. Eggs had a convex operculum resting on “shoulders” at the smaller end of the egg. At the opposite (abopercular) end, a small knob or hook-like protrusion was often visible. Wet mounts revealed visible miracidium inside the eggs. These findings were compatible with liver fluke eggs.

Conclusion

Cytologic microbiologic features of fluke eggs confirmed the findings. The most important features are the shape and size of the eggs; The seated operculum with its prominent “shoulders”, a small knob at the abopercular end, which can distinguish them from acellular artifact or a human cell. The cytologic and microbiologic features combined with the travel history and clinical presentation can aid in achieving the diagnosis.
Single Institutional Experience on Cutaneous Manifestation of Adult T-cell Leukemia/Lymphoma - Potential Diagnostic Pitfall
Authors: Bridgelall S , Michalski J , Zhang X , Sokol L , Dong N , Zhang L
Published: October 2020

Introduction/Objective

Adult T-cell Leukemia/Lymphoma (ATLL) is an aggressive peripheral T-cell neoplasm caused by the human T-cell lymphotropic virus-1 (HTLV1). Approximately half of the patients diagnosed with ATLL have heterogeneous cutaneous manifestations and 1/3 of those patients have skin changes e.g. rashes, papules, and nodules at initial presentation. There is clinical and morphologic overlap between ATLL and other cutaneous T-cell neoplasms such as Cutaneous T-Cell Lymphoma/mycosis fungoides (CTCL/MF) which could pose a potential diagnostic challenge.

Methods

A retrospective study was conducted using PathNet system to search for HTLV1 positive ATLL patients. Clinicopathologic features of the patients with cutaneous involvement were analyzed.

Results

Total 31 patients with ATLL were identified. Nine patients (29%, median 54.5 years, range 47-67 years, male: female ratio 2:7) showed skin manifestations, and the cutaneous involvement with ATLL was confirmed by skin biopsy. Five (55.5%, 5/9) cases were initially misdiagnosed as CTCL/MF. Among the 5 patients, 2 presented with skin rash or diffuse erythematous patch/plaque before developing generalized lymphadenopathy or overt circulating atypical lymphocytosis; 2 developed severe pruritic rash with erythematous skin changes resembling Sezary syndrome; and 1 patient had folliculotrophic MF diagnosed 12 years before. Notably, atypical lymphocytosis (0.46- 41.19/µL) occurred in 3 of the 4 remaining cases. In addition, eight of the 9 patients displayed a variable level of CD3+/CD4+/ CD25strong+ abnormal T-cells on flow cytometry. A low-level bone marrow involvement (2-10%) was found in 8 of 9 cases. Elevated calcium levels were identified in 3 of 9 cases (33%). There were 7 of 8 patients (87.5%) who developed generalized lymphadenopathy when diagnosis of ATLL was rendered.

Conclusion

In patients with cutaneous manifestations, features including hypercalcemia, atypical lymphocytosis, lymphadenopathy, CD3/CD4/strong CD25 coexpression, and bone marrow involvement should prompt a test for HTLV1. Early diagnosis of ATLL can initiate proper treatment and improve patient clinical outcomes.
An Unusual Case of Basal Cell Carcinoma with Myoepithelial Differentiation
Authors: Myers D , Smith N
Published: October 2020

Introduction/Objective

Basal cell carcinoma (BCC) is a commonly encountered dermatologic entity by general surgical pathologists and dermatopatholgists alike and usually does not pose a diagnostic dilemma. However, BCC can take on a variety of histopathologic morphologies, sometimes mimicking more malignant entities. We present an unusual case of BCC with a rare morphology.

Methods

A 56-year-old male with history of non-melanoma skin cancer presented to his dermatologist with a 5mm pink, pearly, papule on his left forearm. A biopsy was performed, and histology and immunohistochemistry was utilized to evaluate and characterize the neoplasm.

Results

H&E stained histopathologic sections demonstrated an unusual epithelioid neoplasm with infiltrative growth, peripheral palisading of atypical basaloid cells, and areas of prominent rhabdoid/plasmacytoid cytology reminiscent of myoepithelial cells. The neoplasm was diffusely positive for Ber-Ep4 while SMA highlighted the areas with rhabdoid/plasmacytoid appearance. Mart-1 and SOX-10 were positive in only a few bland intralesional melanocytes.

Conclusion

The histological appearance was concerning for melanoma with rhabdoid morphology or an unusual variant of basal cell carcinoma. Positive Ber-Ep4 staining supported the histological impression of an unusual basal cell carcinoma variant while staining for SMA supported the impression of myoepithelial differentiation. Myoepithelial differentiation is extremely rare in BCC with only 16 prior cases described in the literature. To our knowledge, this is the first reported case of BCC with myoepithelial differentiation presenting outside the face. Consequently, this is an important diagnostic differential to consider when evaluating epithelial neoplasms with rhabdoid or plasmacytoid morphology.
Primary Conjunctival Kaposi Sarcoma; A Rare And Unheard Entity
Authors: Hussain F , Laharwani H , Sonani H , Adams K
Published: October 2020

Introduction/Objective

Kaposi’s sarcoma (KS) is the most common malignant vascular tumor in human immunodeficiency virus (HIV) patients caused by human herpesvirus 8. It can affect the vascular endothelium of any part of the body however ocular KS as an initial manifestation has been reported only in 3 cases so far. It affects the vascular endothelium of the lacrimal gland, eyelids, orbit, palpebral and bulbar conjunctiva. It presents as a violaceous fleshy nodular mass.

Methods

A 23-year-old male with a past medical history of HIV presented with bilateral eye erythema and significant upper and lower lid edema. The patient was initially admitted for facial swelling which led to a conjunctival biopsy and a diagnosis of KS was made and doxorubicin along with chemotherapy was started. Four months later after the initiation of chemotherapy, the patient experienced worsening of facial swelling, and an area on the left side of his face got very firm. His physical examination was positive for a fever, drooling, facial swelling with trouble swallowing, and change of voice along with shortness of breath.

Results

CT of the orbit revealed symmetric enhancement swelling of the preseptal periorbital soft tissues which was thought to be nonspecific and a differential diagnosis included lymphedema, acute sinusitis, and orbital cellulitis was considered. A chest x-ray revealed bilateral pleural effusions and CT head revealed diffuse scalp and facial cutaneous and subcutaneous edema. Lab findings were significant for a low CD4: CD8 ratio. Cytological examination of the pleural fluid revealed predominantly blood, reactive mesothelial cells, macrophages, neutrophils, and lymphocytes, and appropriately controlled immunostain was positive for ERG and HHV8 with a possibility that Kaposi’s sarcoma cannot be completely excluded. Unfortunately, the patient passed away during the same time of pleural fluid analysis.

Autopsy findings revealed multiple metastatic foci in the mediastinal soft tissue, within the right land left lung, scalp soft tissue, retroperitoneal soft tissue, and hilar lymph node which were positive for CD34, CD31, and HHV8 and consistent with metastatic KS.

Conclusion

Thus, it is important to keep in mind that isolated conjunctival KP with later on systemic metastasis is rare with conjunctiva being an unusual site for the initial presentation, and a possibility of primary conjunctival KS should be considered in HIV positive patients.
Diagnostic Approach To Oligomonocytic Chronic Myelomonocytic Leukemia
Authors: Muldoon J , Czader M
Published: October 2020

Casestudy: Pathologic diagnosis of chronic myelomonocytic leukemia (CMML) is typically straightforward with the majority of patients presenting with persistent monocytosis (>1x109/L, >=10%) and bone marrow dysplasia. The diagnosis may be challenging in patients with unusual features such as lack pf peripheral blood monocytosis and non-diagnostic bone marrow morphology. In this abstract, we present a 67-year-old female with a 5-year history of anemia of unclear etiology. At the time of initial presentation, the laboratory work-up of normocytic anemia was non- contributory, the bone marrow was reported as normocellular with maturing trilineage hematopoiesis and no significant dysplasia. Over the course of the disease, the peripheral blood monocyte count fluctuated from 11% to 18% with absolute monocyte count ranging from 0.4 to 0.7x109/L. The most recent bone marrow was markedly hypercellular with increased trilineage hematopoiesis with left shift, dysgranulopoiesis and dysmegakaryopoiesis. Blasts (including promonocytes) constituted 10% of the differential count and were immunophenotypically abnormal with uniform expression of CD117, dim to negative CD13, and partial CD15. Monocytes were elevated at 12% and were strongly positive for CD64 and partially for CD14. They were negative for CD16 consistent with classical monocytes, and showed partial loss of CD13. The karyotype was normal. Molecular testing revealed TET2, RELN and SRSF2 mutations at high allelic frequencies. This case illustrates a value of flow cytometric immunophenotyping and molecular genetic studies in diagnosing challenging cases of CMML. While the patient’s absolute monocyte count remained below the diagnostic threshold of 1x109/L throughout the course of the disease, peripheral blood and bone marrow monocytes showed skewed classical immunophenotype, immunophenotypic abnormalities of myeloid series and high allelic frequency mutations. These findings should raise a differential diagnosis of oligomonocytic CMML, even when morphologic abnormalities and monocyte count threshold are not diagnostic.
Simplification of Clinical Flow Cytometry by Adoption of Premixed Antibody Panels
Authors: Polski J M
Published: October 2020

Introduction/Objective

Multiparameter flow cytometry (MFC) is the standard of care for the diagnosis and monitoring of hematopoietic and lymphoid neoplasms. While MFC is a mature technology, new innovations are brought to the market. Such an innovation is the ClearLLab10C, FDA-cleared, 4-tube, 10-color, dry, premixed antibody panels (Beckman Coulter Diagnostics, Brea, California). Our laboratory decided to implement this new reagent system combined with 10-color clinical flow cytometer, Navios EX from the same vendor. This paper describes the clinical validation results for leukemia/lymphoma (LL) evaluation as well as logistical and labor savings in the laboratory.

Methods

Thirty specimens submitted for LL evaluations were tested by our existing 5-color flow cytometer with custom antibody combinations as well as by the Navios EX instrument with the ClearLLab10C product supplemented by additional antibody combinations for intracellular analysis. The validation cases included normal and abnormal cases, representing bone marrows, blood, lymph nodes, and a few other specimens.

Results

There was a complete agreement in the qualitative results.

The new simplified premixed reagents allow for noticeable simplification of inventory and saving in labor. The existing LL antibody cocktails required stocking 41 antibodies. The new platform requires only 4 LL reagents for surface immunophenotyping and we elected to stock additional 8 antibodies for optional intracellular evaluation. The amount of manual pipetting is greatly reduced. One additional advantage of utilizing the new ClearLLab10C is the available electronic and book publication from the vendor illustrating typical results for 24 normal and abnormal specimens analyzed using ClearLLab10C. This resource together with a user-friendly analysis software Kaluza C greatly facilitate interpretation of 10-color results by pathologists, medical technologists, and especially by the pathologists in training.

Conclusion

ClearLLab10C product is a cost-effective and user-friendly solution for laboratories seeking a streamlined MFC and a great reduction of inventory and labor.
Mesenteric Cysts: A Retrospective Review. Its Not All That Simple
Authors: Bourgeau M , Avadhani V
Published: October 2020

Introduction/Objective

Mesenteric cysts are rare intra-abdominal lesions in adults. However, with the advanced imaging techniques and laparoscopic techniques, they are more often being identified and resected when clinically significant. There is a lack of detailed information in histopathology (except as case reports) since mesentery is generally neglected in our organ-based textbooks. The aim of our study is to highlight the importance of identifying and classifying mesenteric cystic lesions; they are not all that simple.

Methods

We performed a retrospective search on all mesenteric cysts submitted as excisions in our electronic database from 2013-2019. We classified them as per the de Perrot (PMID: 11053936) classification with modification.

Results

Our search showed: A. Lymphatic origin-11 (lymphangioma-10, Lymphangioma hamartomatous-1, associated with LAM-0), B. Mesothelial origin-68 (Benign mesothelial cysts-57, multilocular mesothelial cyst-11), C. Enteric origin- 3, D. Urogenital origin (Urachal cyst, mullerian inclusion cyst)-9, E. Mature cystic teratom-2, F. Pseudocyst-12, G. Epithelial cyst (not urogenital)- 11 (a/w LAMN-3, MCN-4, Mucinous cystadenoma-4), H. Associated with carcinoma-2.

Case illustration: A 61-year-old male presented with worsening dysphagia, emesis and hiccups. A CT scan showed a 21.2 cm cystic mass with at least one septation (Fig 1). The cyst was resected. On gross pathological examination, the cyst measured 18 cm in greatest dimension with a thick, rough, tan-brown capsule. Microscopic examination showed a fibrous capsule, and cyst wall composed of numerous lymphatic vessels (CD31 positive) and prominent smooth muscle proliferation (Desmin positive). Scattered lymphoid aggregates were also present throughout the cyst wall. No definite epithelial lining was identified and was suspected to have been denuded. HMB-45 immunostain was negative, ruling out association with LAM. The final diagnosis of a Lymphangiomyoma, hamartomatous was rendered.

Conclusion

Though most of the mesenteric cysts are benign, some of them are significantly important such as Lymphangiomyoma (esp secondary to LAM), MCN, those associated with LAMN etc. and identifying and differentiating from their mimics has distinct clinical implications.
Status of Histopathology Services In Ethiopia
Authors: Yeabyo G O
Published: October 2020

Introduction/Objective

Cancer is one of a great public health problem in developing countries like Ethiopia and scarcity of histopathology laboratory is another burden for diagnosis and follow up of cancer diseases. The main purpose of this study is to evaluate the accessibility of histopathology services in Ethiopia.

Methods

Cross sectional study design were used to analyze the status of histopathology services in Ethiopia.

Results

There are 13 histopathology laboratories in the nation and a survey was conducted at these institutions.

Out of these 13 Histopathology services seven are located in the capital, Addis Ababa and six found out of the capital located in four different regions. About 40,239,100 habitants have no histopathology service around their catchment area.

Conclusion

There are very limited histopathology laboratories, as a result diagnosis and prevention of cancer diseases are inadequate throughout the country.
Reducing Inappropriate Overutilization of the Massive Transfusion Protocol
Authors: Nguyen P T , Boral L I , O’Brien J , Zhang D , Gao Y , Bernard A
Published: October 2020

Introduction/Objective

Massive transfusion protocols (MTPs) make it possible to deliver blood products to exsanguinating patients in an efficient, sustained, and immediate manner. However, overutilization of MTPs continues to remain a significant problem for hospital blood banks. The study aims to address the characteristics of possibly inappropriate MTP activations during the first phase (cooler) at a level 1 trauma academic medical center.

Methods

This was a retrospective review of 328 phase 1 MTPs out of 696 total MTPs performed during a two-year period (Jan 1, 2017 and Dec 31, 2018) and divided into three categories: trauma, non-trauma surgery, and medicine services. We assumed that a possibly inappropriate MTP for trauma cases would show an Assessment of Blood Component (ABC) score of 1 or less and physicians used clinical discretion. For possibly inappropriate MTP non- trauma surgical and medicine cases, we devised a new system where we looked at only whether the blood pressure was equal to or less than 90 mm Hg and/or the pulse was greater than or equal to 120 beats per minute. If there was a score of 0 where there were neither vital sign criteria, then the MTP was considered possibly inappropriate.

Results

For trauma service, there were 43/168 (25.6%) cases that had an ABC score of 0, 49/168 (29.2%) cases that had an ABC score of 1, 53/168 (31.5%) cases that had an ABC score of 2, 21/168 (12.5%) cases that had an ABC score of 3, and only 2/168 (1.2%) case that had an ABC score of 4. Non-trauma surgery service had 31/51 (60.8%) cases with a new score of 0, 20/51 (39.2%) cases with a new score of 1, and 0/51 (0%) cases with a new score of 2. Medicine services had 57/109 (52.3%) patients with a new score of 0, 47/109 (43.1%) patients with a new score of 1, and 5/109 (4.6%) patients with a new score of 2. Non-trauma surgery service had the greatest possibly inappropriate MTP activations [31/51 cases (60.8%)], followed by trauma [92/168 (54.8%) cases] and medicine [57/109 (52.3%) cases].

Conclusion

Our study shows greater overutilization with MTPs using clinical judgment than with using the ABC criteria. Non-trauma surgery service had the greatest number of possibly inappropriate MTP activations at our hospital without having a significantly abnormal pulse or blood pressure. While ABC criteria is already liberal for activating an MTP, physician discretion appears to be more liberal in activating a possibly inappropriate MTP.
Curating Archival Anatomic Pathology Material For Machine Learning Algorithm Development
Authors: Collins A , Norgan A , Garcia J J
Published: October 2020

Introduction/Objective

Advances in whole slide imaging have enabled the application of machine learning algorithms to anatomic pathology. In the current state, the development of accurate algorithms requires robust training data with correctly assigned diagnostic and classification labels. Increasingly, institutions have looked to their archival slides as a source of “ground truth” for algorithm development. However, the curation and use of archival data poses several challenges. Here, we share lessons learned from reviewing head and neck pathology consult cases spanning a 10- year period at Mayo Clinic Rochester.

Methods

Archived surgical pathology slides from 2,590 consult cases were reviewed. Clinical and demographic information was recorded for each case, including surgical date, surgical procedure, anatomic site, age, gender and diagnosis. Cases were excluded from the curated archive if there was insufficient volume or quality of tissue to render a specific diagnosis (141 cases, 5.6%). Slides with a range of tissue size and quality, from numerable laboratories were included in the curated archive. Selected cases were collated by anatomic site: ear, gnathic, larynx, nasopharynx, neck, oral cavity, oropharynx, salivary gland and sinonasal tract.

Results

Common diagnostic reconciliations (115 cases, 4.4%) fell within the following categories: (1) novel entities (59 cases, 2.3%), including biphenotypic sinonasal sarcoma and clear cell carcinoma; (2) novel classifications (21 cases, 0.8%), as seen in HPV-related oropharyngeal squamous cell carcinoma and polymorphous adenocarcinoma; and (3) novel grading schema (35 cases, 1.4%), as seen in keratinizing dysplasia and oropharyngeal malignancies.

Conclusion

Several nuances emerged in the process of reviewing slides, highlighting the need for continual amendment of any machine learning dataset over time. Curating anatomic pathology cases for machine learning algorithm development requires the recognition of emerging entities, with re-classification and re-grading as needed.
Community Health Screening And Education Through Laboratory Science Workshop: Participant Evaluation Of The 2018 Pilot Program In Aco, Peru
Authors: Ellis J R , Vreeland J , Jaimes F
Published: October 2020

Introduction/Objective

Public health outreach initiatives underutilize laboratory medicine students and professionals. This poster will discuss a novel bilingual community health screening and education (CHS & E) through laboratory science service-learning (SL) study abroad (SA) program that could be utilized to improve community engagement and understanding of public health issues from a lab perspective.

Methods

In collaboration with The Foundation for International Medical Relief of Children (FIMRC), Texas State University faculty member, Joanna Ellis, and five CLS senior-level students traveled to Huancayo, Peru in June of 2018 for a CHS & E through lab science SL-SA program. Utilizing the self-powered Lab-in-a-Suitcase from International Aid, the group conducted four CHS & E through lab science events in four different communities. We conducted hemoglobin, urinalysis, glucose, cholesterol, and STI screenings on more than 160 people. The CUY Project was the most involved activity with six interactive stations for the families to learn about anemia, parasites, and nutrition through lab tests. Parents and children learned more about their conditions through interactive lab science activities and tests. Following the workshop, parent participants were given an anonymous survey about their knowledge before and after the workshop as well as their suggestions for its improvement.

Results

The lab-science based activities illustrated the biological impact of their lifestyle and dietary choices in a new and impactful way. Participants self-assessed that they learned significantly more about anemia and diabetes during the workshop. Participants learned more about hygiene than they knew before the workshop; however, the increase was not statistically significant. Parent participants surveyed (100%) stated that the workshop would help them make decisions in caring for their children and would help prevent future health problems.

Conclusion

Although the newest iteration of the workshop is entitled Health Education Advances with Lab Science (HEALS) this poster will describe the preparation, implementation, and first stage of evaluation of the CHS & E through laboratory science workshop in Peru. This poster session is an opportunity to discuss how laboratory professionals can interact with their community and showcase the value of the field in public health education initiatives.