bluuebunny/crossref_metadata_2025 huggingface dataset:
Spontaneous Complete Regression of Testicular Germ Cell Tumor: A Case Report
Authors: Liu S Shawn , Hernandez Sara Avalos , Zotto Valeria Dal , Herrera Guillermo
Published: September 2019

Testicular germ cell tumors (TGCTs) may spontaneously regress, making it a diagnostic challenge. We present here a case of 39-year-old African American male with constant abdominal pain radiating into the back. Computed tomography identified bulky retroperitoneal lymphadenopathy. There was no history of testicular trauma. Ultrasonography of right testicle revealed multiple microcalcifications, and a laboratory test indicated elevated beta-hCG and LDH. Patient underwent right orchiectomy, which revealed a grossly well-demarcated white oval lesion (3.1 cm). Histologic examination identified a fibrotic lesion in a background of atrophic parenchyma consistent with scar tissue. The specimen was completely submitted for microscopic evaluation, but neoplasia was not identified. A placental alkaline phosphatase (PLAP) immunohistochemical stain failed to reveal residual germ cell neoplasm in situ. Biopsy of retroperitoneal lymph node identified malignant neoplastic cells expressing PLAP, CD117, CD30, and cytokeratin AE1/AE3. The morphology and immunophenotype were consistent with metastatic germ cell tumor. Spontaneous regression of TGCT is defined as partial or complete tumor disappearance without any treatment. The clinical presentation could be nonspecific. Histologically, tumor regression is indicated by formation of fibrous scar, testicular atrophy, microcalcification, lymphoplasmacytic infiltration, and hemosiderin-phagocytosed macrophages, all of which were present in the current case. However, similar histological features could be identified in testicular ischemia or infraction. Therefore, the diagnosis of complete regression of TGCT is challenging and frequently relies on the present of extragonadal germ cell tumor. In the current case, we reported a spontaneous complete regression of TGCT, supported by a spectrum of histological features and metastasis to retroperitoneal lymph node. Spontaneous regression of TGCT is rare, with less than 150 cases reported in the literature. Since there is a lack of definitive histologic criteria to diagnose complete spontaneous regression of TGCT, when scarring is seen in the testis, additional clinical and imaging evaluation should be followed.
Common Etiologies and Clinical Significance of Gastric Intraepithelial Lymphocytosis
Authors: Ahmed Sunjida , Xu Ruliang
Published: September 2019

Background

Increased intraepithelial lymphocytes or intraepithelial lymphocytosis (IEL) in the upper gastrointestinal (GI) tract is a response to various mucosal injury. However, the frequency of gastric IEL in GI tract biopsy is not well documented, and the etiologies of gastric IEL are yet to be defined.

Methods

Cases with a diagnosis of “intraepithelial lymphocytosis” and “intraepithelial lymphocytes” were retrieved from 25,074 GI biopsies and 8,921 partial gastrectomies in our departmental database (Powerpath) for a 1-year period. The diagnosis of IEL was confirmed by the report and/or slide review. Possible etiology or causes of gastric IEL were investigated by correlation with clinical information from LIS (EPIC).

Results

A total of 694 cases with IEL were identified from 33,995 GI tract specimens (biopsy and resection). Among 694 cases, 34 (4.89%) were gastric biopsy and resection cases with IEL, whereas 561 (80.8%), 37 (5.3%), and 62 (8.9%) were duodenal, esophageal, and colonic specimens, respectively. Thirty-four gastric cases with IEL were closely associated with morbid obesity (8, 23.5%), H pylori infection (8, 23.5%), celiac disease (5, 14.7%), lymphocytic gastritis (5, 14.7%), nonspecific gastritis (4, 11.4%), inflammatory bowel disease (3, 8.8%), and gastroesophageal reflux disease (1, 1.9%). Seventeen of 34 (50%) cases had IEL in both gastric and duodenal mucosa. Those 17 cases with gastroduodenal IEL had morbid obesity (n = 5), celiac disease (n = 5), lymphocytic colitis (n = 2), inflammatory bowel disease (n = 2), H pylori gastritis (n = 2), and nonspecific gastritis (n = 1). Five patients with a diagnosis of lymphocytic gastritis were treated with a protein pump inhibitor after pathologic diagnosis. Among them, 4 had a followed-up endoscopy in 12 months, and 3 of them showed persistent IEL in a follow-up biopsy.

Conclusion

Gastric IEL is less common than duodenal IEL. It is associated with a broad differential diagnosis. Follow-up biopsy may be necessary for some types of gastric IEL. Persistent IEL in follow-up biopsy may be suggestive of a different etiology or requires different treatment strategy.
A Diagnosis to Wrestle With: Intraventricular Angiomatoid Fibrous Histiocytoma
Authors: Canevari Margaux , Keenan Bryan , Horkayne-Szakaly Iren , Miettinen Markku
Published: September 2019

We report a case of a 15-year-old boy who presented with an intraventricular angiomatoid fibrous histiocytoma. The patient suffered a concussion during a wrestling match and underwent a head and spine CT, which prompted an MRI. MRI revealed an enhancing 2.3-cm mass in the posterior horn of the right lateral ventricle with no hydrocephalus. The patient subsequently admitted to having had headaches with associated appetite suppression but no visual problems or weakness. The tumor was resected. It was composed of sheets and cords of histiocytoid cells with focal pleomorphism. Interspersed throughout was a robust lymphoplasmacytic infiltration with Russell bodies and lymphoid follicles. The histiocytoid neoplastic cells were desmin, EMA, and sparsely S100 protein positive. They were negative for CD31, CD34, Cam 5.2, CD163, CD1a, HMB45, Melan-A, progesterone, and ERG. The abundant plasma cell population was confirmed by CD138 reactivity and was polyclonal by kappa and lambda immunohistochemistry. Fluorescence in situ hybridization revealed an EWSR gene rearrangement. The patient was radiographically free of disease 3 years after surgery with headaches as residual sequela. The differential diagnosis for this case included a lymphoplasmacyte-rich meningioma or a plasmacytoma. The EWSR rearrangement made both of these unlikely. The polyclonal nature of the plasma cell component and the age of the patient also argued against plasmacytoma. A literature search revealed one other ventricular-based angiomatoid fibrous histiocytoma in a pediatric patient.
Fecaloma Complicating Colostomy for Hirschprung Disease
Authors: Ohayi Samuel , Onyishi Nnaemeka , Chukwubuike Kevin
Published: September 2019

Background

Hirschprung disease (HD) is a congenital disorder characterized by absence of ganglion cells in the distal portion of the large intestine with resultant obstruction. It commonly manifests shortly after birth, hence its usual diagnosis in the first year of life. A defunctioning colostomy precedes definitive treatment. Prolonged obstruction following untreated HD or any condition may progressively lead to fecaloma, a mass of inspissated feces commonly in the rectosigmoid that is much harder than impacted feces. If prolonged, affected intestine looks like a tumor. We present a case of fecaloma of the transverse colon complicating colostomy done for Hirschprung disease.

Case Report

A 6-year-old boy with a 6-year history of difficulty with passing stool with progressive abdominal swelling had Swenson’s procedure (ie, proctectomy) and prolapsing of ganglionic colon to the anus with left hemicolectomy. He had presented to the pediatric surgery unit of our hospital 4 years previously with these symptoms with a defunctioning colostomy performed for suspected HD. He reportedly passed meconium a day after birth and subsequently passed stool once in 7 to 14 days spontaneously or after digital manipulations. Stooling frequency progressively reduced but no vomiting. Following administration of herbs, he developed diarrhea, and with worsening symptoms, he presented to our hospital. Three years postcolostomy, rectal biopsy was performed with a histology confirming aganglionosis of the rectum (HD). Barium enema had shown long-segment HD with dilated rectosigmoid colon but no fecaloma. Surgical specimen received in the histopathology laboratory consisted of colon segment comprising a colostomy stump with a ring of skin (colostomy stoma) and a proximal markedly dilated portion filled with fecaloma followed by a hypertrophied proximal portion. Histology showed presence of ganglion cells in these segments.

Conclusion

Fecaloma should be suspected in a protracted colostomy with abdominal mass proximal to the stoma.
Rare Testicular Neoplasm: Melanotic Neuroectodermal Tumor of Infancy
Authors: Canevari Margaux , Wang Larry , Rossi Christopher
Published: September 2019

We report the case of an 18-month-old boy who presented with a several-week history of left testicular swelling. On physical examination, the testicle was firm, enlarged, and nontender to palpation. The patient’s urinary habits and family history were unremarkable. An ultrasound was performed that revealed an enlarged left testis (4.7 cm) with a hypervascular heterogeneous appearance and scattered echogenic foci that raised concern for a neoplastic process. Microlithiasis was identified in the right testis (1.6 cm). AFP and hCG were within normal limits, and LDH was mildly elevated. An orchiectomy was performed and gross dissection revealed a 4.5 × 3.5 × 3.0-cm mass. The tumor was biphasic and composed of a population of poorly differentiated small round blue cells (neuroblastoma-like) and a separate component of nested epithelioid cells that had abundant cytoplasm with melanotic granules. Mitotic and karyorrhectic figures were scattered throughout. Epididymis infiltration was present. The epithelioid cells were pan-cytokeratin, EMA, and HMB-45 positive. The small round blue cells were positive for synaptophysin, vimentin, and CD117. A subpopulation was also moderately positive for CD99. A Phox2B and TDT were negative in the tumor cells, which helped to exclude neuroblastoma and lymphoma. The combined features of this case were diagnostic for melanotic neuroectodermal tumor of infancy (MNTI). This is a rare tumor with a strong predilection for the head and neck region with testicular/paratesticular localization accounting for less than 5% of MNTI cases. MNTIs are classically seen in infants under 1 year of age and typically behave in a benign fashion, although metastases to retroperitoneal lymph nodes and lymphatics have been documented. It is important to remember the differential of pediatric testicular neoplasms as it can mimic other small round blue cell tumors.
Mixed Adenoneuroendocrine Carcinoma: Is It a Collision Tumor or a Dedifferentiated Carcinoma?
Authors: Nazir Bushra , Ross Howard , Mittal Juhi , Taraif Suad
Published: September 2019

Mixed adenoneuroendocrine carcinoma (MANEC) of the gastrointestinal tract is uncommon with about 100 cases reported involving esophagus, stomach, colon, rectum, and biliary tree. The aggressive behavior of these tumors is usually dictated by the high-grade neuroendocrine carcinoma (NEC) component, which raises the possibility that these may represent areas of dedifferentiation. We here report an 88-year-old male presenting with rectal bleeding. Colonoscopy identified a fungating, villous, and partially circumferential cecal mass. Biopsy revealed tubular adenoma with focal high-grade dysplasia. However, excision was recommended given the impression of a mass. A right hemicolectomy revealed an 8.7-cm friable and ulcerated mass in the cecum. Initially, eight sections (one per cm) of the tumor were examined and revealed intramucosal adenocarcinoma. However, one of the lymph nodes was replaced by a high-grade poorly differentiated tumor, immunophenotypically consistent with NEC. In search for a primary, the rest of the mass was submitted and subsequent histologic examination revealed areas of transition from villous adenoma to intramucosal and muscle invasive adenocarcinoma in addition to deeply invasive NEC showing marked cytological atypia, brisk mitotic activity (Ki-67 >90%), and lymphovascular invasion. Three lymph nodes had metastatic adenocarcinoma and NEC along with several tumor deposits. Following an uneventful postoperative course, the patient received adjuvant chemotherapy. Our case recapitulates the classic description of MANEC where NEC is usually associated with a differentiated adenocarcinoma, commonly arising from an adenoma. The currently proposed pathogenesis is a collision tumor (previously known as mixed exocrine/endocrine carcinomas). However, we propose viewing MANEC as one tumor rather than two colliding tumors utilizing the concept of dedifferentiation, which has been extensively studied in the GYN literature. In a prior study, we found neuroendocrine expression in 41% of undifferentiated endometrial carcinomas. We therefore propose revisiting larger case series of MANEC with this concept in mind.
Signet Ring Cell Carcinoma of the Extrahepatic Bile Duct: A Case Report
Authors: Hameed Nadia , Marbut Stephen
Published: September 2019

Introduction

Signet ring cell carcinoma (SRCC) of the biliary system is extremely rare and its clinicopathological features and impact on prognosis are not well known. We report the extremely rare case of SRCC originating from the extrahepatic bile duct.

Case Report

We present a 76-year-old female who had elevated liver enzymes noted on routine laboratory test. Abdominal imaging revealed a polypoid mass within the central bile duct, located above the level of pancreatic head with proximal intrahepatic duct dilatation with no significant lymphadenopathy or ascites. Total bilirubin was normal, alanine transaminase level was 163 IU/L, and aspartate aminotransferase was 55 IU/L. The patient underwent common bile duct excision with cholecystectomy. The specimen showed a 3.3-cm centrally located tan polypoid lesion in the common bile duct with adjacent firm lymph node. Microscopic examination of the lesion revealed sheets of tumors cells with signet ring cell morphology underlying benign biliary epithelium. The lymph node was completely replaced by neoplastic cells. The neoplastic cells were diffusely positive for CDX2 and focally positive for CK7 and CK20. The histomorphology and immunophenotype are consistent with poorly differentiated adenocarcinoma with signet ring cell type.

Conclusion

SRCC of the extrahepatic bile duct is a histologically distinct entity classified by the World Health Organization, but it is very rare. To best of our knowledge, only five cases of SRCC of the extrahepatic bile duct have been reported in the English literature.
A Case of Immune-Mediated Necrotizing Myopathy With Associated Skeletal Muscle Involvement by Sarcoid Granulomata: A Rare Association
Authors: Akbar Shalla , Dasaraju Sandhya , Elkadi Osama
Published: September 2019

Skeletal muscle involvement by noncaseating granulomata occurs in a variety of conditions, including sarcoidosis, infections, and rarely in association with primary inflammatory myopathies such as inclusion body myositis (IBM) and dermatomyositis (DM). Sarcoid myopathy is typically asymptomatic; however, a picture of acute myositis with proximal muscle weakness has been described. Immune-mediated necrotizing myopathy (IMNM) is a subgroup of inflammatory myopathies typically presenting with proximal muscle weakness and markedly elevated muscle enzymes, mostly occurring in the setting of statin treatment. IMNM is associated with positive autoantibodies, but a subset of cases is antibody negative. Here we describe a case of myopathy occurring in association with sarcoidosis with combined features of granulomatous and necrotizing myopathy.

The patient was a 54-year-old African American male with medical history significant for statin use 3 years ago, which was discontinued due to myalgia and elevated muscle enzymes and biopsy-proven sarcoidosis diagnosed on a pulmonary lymph node biopsy. He presented with progressive worsening of bilateral proximal weakness involving the upper and lower extremities. Electromyogram showed features of active myopathy with no evidence of peripheral neuropathy. Myositis panel was negative for the following antibodies: anti-Jo1, Mi-2, anti-Ku, PL-7, PL-12, OJ, EJ, and SRP. However, there was elevation of aldolase, CRP, and CK-MB. Biopsy of thigh and deltoid muscle showed necrotic muscle fibers, myophagocytosis with associated minimal inflammation, and multiple well-formed nonnecrotizing granulomas with multinucleated giant cells. Myopathic features include increased internalized nuclei, round atrophic fibers, and scattered split fibers. Specific features of IBM or DM were not present.

Conclusion

Myopathies developing or worsening after discontinuation of statin are rare. The association of necrotizing myopathy with sarcoidosis is not well described in the literature. Additional studies are warranted to elucidate this association.
Prostatic Ductal Adenocarcinoma Masquerading as a Rectal Mass: A Case Report
Authors: Sahni Suhalika , Mehta Vikas
Published: September 2019

Objective

The approach to a patient presenting with a rectal mass who has a history of bright red blood per rectum (BRBPR) renders a wide range of differential diagnoses. The clinical reasoning to suspect prostatic ductal adenocarcinoma (PDA) in a patient with BRBPR without a classical clinical presentation is highly unlikely. The incidence of PDA is rare with only 0.4% to 0.8% in pure ductal origin.

Methods

A 71-year-old African American male presented with 3-month history of watery, bloody diarrhea and a recent history of BRBPR with unintentional weight loss. Patient underwent colonoscopy, which showed a large polyp 10 cm from the anal verge, measuring 7 cm. The unclear history of this patient contributed to an initial impression of a colorectal cancer leading to a biopsy of the colonic mass.

Results

An erroneous diagnosis of tubulovillous adenoma was rendered initially. Further workup showed a large mass originating from the prostate area that was seen penetrating the colorectal region on an abdominal CT scan. The patient’s serum prostate-specific antigen (PSA) level was alarmingly high (4,800 ng/mL) and a subsequent prostate biopsy showed PDA. The colonic biopsy was reviewed again by a genitourinary pathologist and a correct diagnosis was rendered.

Conclusion

The role of pathology in diagnosing PDA is crucial as patients with PDA can have normal digital rectal examination (DRE) as well as a normal serum PSA level (less than 4.0 ng/mL). Majority of PDAs originate from periurethral prostatic ducts. The unique presentation of primary prostate pathology offers appreciation into the approach and workups to diagnose PDA. Diagnosing PDA can be challenging due to its more aggressive nature of PDA compared to prostatic acinar adenocarcinoma. The clinical reasoning and pathological findings from this rare case can aid clinicians and pathologists to establish the diagnosis of PDA in a timely and efficient manner.
Multiple Subcutaneous Nodules Leading to the Diagnosis of Colon Cancer
Authors: Madabhavi Irappa , Modi Mitul , Sarkar Malay , K S Sandeep , Shah Mansi , Mallikarjuna Vinay Sakaleshpura
Published: September 2019

Cutaneous metastasis refers to the growth of cancer cells in the skin originating from an internal cancer. The incidence of subcutaneous metastasis from a visceral malignancy, excluding malignant melanoma, lymphoma, and leukemia, is rare with an incidence of 5.3%. Skin involvement, as a presenting sign of a silent malignancy, is a rare event occurring in approximately 0.8%. Here, we report an interesting case of a 39-year-old man who presented with multiple subcutaneous nodules over anterior chest and forehead for the past 3 months. Physical examination showed nontender, firm to hard multiple subcutaneous nodules ranging from 1 to 2 cm in size. The patient also reported unintentional weight loss and altered bowel habits. Complete blood count showed hemoglobin of 8.0 g/dL, with mean corpuscular volume (MCV) of 97 pg/mL and carcinoembryonic antigen (CEA) of 480 IU/mL. Fine-needle aspiration of the cutaneous nodules revealed malignant cells arranged in a glandular pattern with intraglandular mucin, as well as hyperchromatic and vesicular nuclei with prominent nucleoli. Imaging of the abdomen revealed asymmetrical circumferential thickening with luminal narrowing of cecum and ascending colon and mild ascites with no evidence of liver metastasis. Microscopic examination of the ascending colonic mass showed malignant cells with similar microscopic features. Patient was managed with multiple blood transfusions, and palliative right-sided hemicolectomy was performed in view of intermittent constipation. Sixteen cycles of postoperative chemotherapy were given, after which the subcutaneous lesions regressed and the CEA came down to 56 IU/mL. Skin metastases from colorectal cancer are extremely rare and further analysis of the biology of this mode of metastases may shed light on mechanisms of metastases to specific organs and hopefully provide guidelines for novel targeted therapeutic interventions.
Incidental Gallbladder Cancer: A Clinicopathological Study
Authors: Dhorajiya Poojaben , Mannan Rifat , Sharabi Abdelsalam
Published: September 2019

Objectives

Incidental gallbladder cancer (iGBC) is defined as gallbladder cancer (GBC) diagnosed at the time of or after cholecystectomy, performed for unsuspected benign gallbladder disease. Being rare, it is found in 0.2% to 2.9% cases of cholecystectomies performed for cholecystitis and represents approximately 40% of all GBCs. Here, we present the clinical and pathological features of iGBC diagnosed at our institute over a 10-year period.

Methods

The pathology reports of GBC in patients who underwent simple cholecystectomies due to sole complain of cholecystitis between 2008 and 2018 were retrieved from electronic medical records. Cases with dysplasia (without invasive component), clinically suspected gallbladder malignancy, polyp (nonincidental), or cholecystectomies performed as a part of any other procedure were excluded.

Results

Twenty-two cases of iGBC were diagnosed during the study period. Average patient age was 69.59 (range; 48-91) years, with a slight female (n = 12, 54.5%) predominance. Most common site was body (n = 7), followed by fundus (n = 4), neck (n = 2), and unknown (n = 9). Mean tumor size was 1.6 cm, where measurement of tumor size was possible (n = 16). Histopathological diagnoses were adenocarcinoma (n = 21) and carcinosarcoma (n = 1). The AJCC tumor staging was pT1a (n = 3), pT1b (n = 3), pT2 (n = 12), pT3 (n = 3), and unidentifiable (n = 1). Four cases were identified with N1 lymph node status. Cystic duct margin was positive in two cases. Perineural invasion was identified in three cases.

Conclusion

iGBC after a cholecystectomy for clinically benign condition is a rare finding. Although cholecystitis is much more frequent in female, iGBC had only a slight predominance for female population in our study. It is more common in people over 60 years of the age, which is comparable to the literature. This entity still remains a diagnostic and therapeutic challenge, and management is often more complicated in cases with advanced stage.
Secretory Carcinoma Ex Pleomorphic Adenoma: A Case Report
Authors: Egal Erika , Souza Reydson , Scarini João , Martins Antonio , Casarini Andre , Altemani Albina , Mariano Fernanda
Published: September 2019

The objective of this study is to report a case of carcinoma ex pleomorphic adenoma subclassified as secretory carcinoma ex pleomorphic adenoma frankly invasive. A 60-year-old male patient attended our service for cervical injury assessment, painless with slow and progressive growth for about 3 years. At the clinical examination, an increase in volume in the submandibular region and a lesion in the region of the nasolabial sulcus were observed. A biopsy of the lesion was performed and the morphological characteristics were compatible with secretory carcinoma of the salivary gland. The patient underwent a radical cervical emptying, excision of the left submandibular gland, and removal of the lesion in the left nasogastric sulcus. At the macroscopic examination, the submandibular gland presented a hardened, heterogeneous nodule, poorly delimited, with gross calcification at the center, measuring 2.0 × 1.5 × 1.5 cm. At the histopathological examination, eosinophilic cells containing intracytoplasmic vacuoles, with a ductal formation and solid arrangement and dystrophic calcification, were observed corresponding to the old hyalinized areas of residual PA. In view of these findings, the diagnosis was secretory carcinoma ex adenoma pleomorphic frankly invasive of the submandibular gland with extension to the nasogenian sulcus. Complementary immunohistochemical examination was carried out evidencing positivity for mammaglobin, S100, and CK7 in tumor cells and negativity for calponin, 1A4, and p63.

Conclusion: Although CXPA is a rare lesion and is more common in parotid glands, it may affect other locations. Several histopathological phenotypes may arise from the malignant transformation of the PA, but to the best of our understanding, this is the first case of secretory carcinoma ex pleomorphic adenoma reported in the literature.
Quality Assurance of the Gallbladder and Relationship Between Gallstones and Abdominal Panniculus Thickness in Adult Hospital Autopsies
Authors: Kaur Gagandeep , Williams Kaitlyn , Vidhun Ramapriya , Dodge Jessica
Published: September 2019

Objectives

Inconsistency in the autopsy report can be a liability issue. We studied the discordance between the presence of the gallbladder and a corresponding abdominal scar and suggest how to improve this error. To our knowledge, the relationship between abdominal panniculus thickness in adult hospital autopsies and the presence of gallstones has not been reported in the literature. We report the correlation between age, sex, average panniculus thickness, and gallstones.

Methods

The laboratory information system was searched for autopsies performed between 1/1/09 and 12/21/18. Patients <18 years old and partial, nonabdominal autopsies were excluded. Autopsy reports were reviewed, and the following data were recorded: age, sex, presence or absence of gallbladder, abdominal scars, abdominal panniculus thickness, and gallbladder pathology.

Results

Out of 385 autopsies reviewed, 48 (12.5%) had cholecystectomies. Of these 48 patients, 6 (12.5%) abdominal scars were not documented. The presence of the gallbladder was not mentioned in 6 (1.6%) reports. The most common pathology was gallstones, 78 (20.3%); cholesterolosis, 9 (2.3%); and cholecystitis, 6 (1.6%). The average age of females and males with gallstones was 68.9 and 66.4 years, respectively. The average abdominal panniculus thickness in males with and without gallstones and in females without gallstones was 3.3 cm. In contrast, the average abdominal panniculus thickness in females with gallstones was 4.0 cm.

Conclusion

Our study demonstrated that 12.5% of our autopsy reports failed to document the presence of abdominal scars in patients who surgically lacked a gallbladder. There is a need to improve documentation of abdominal scars and the presence or absence of the gallbladder. This can be achieved by creating mandatory fields (eg, gallbladder present/surgically absent) in the autopsy report template. Also, our study found that females with gallstones have a thicker abdominal panniculus than females without gallstones and males with or without gallstones.
Dysphagia Lusoria: A Rare Entity and Underrecognized Cause of Dysphagia
Authors: Estrella Melanie , Chauhan Suman
Published: September 2019

Management of dysphagia lusoria (DL), dysphagia caused by esophageal compression by an aberrant right subclavian artery (ARSA), is a clinical challenge. Sixty percent of patients with ARSA develop an aneurysm in the aberrant artery, which increases the risk of developing worsening dysphagia, tracheal compression, aortic dissections, and aneurysmal ruptures. Surgical management of ARSA has been studied, but no definitive standards have been established. As a result, most patients elect for surgery only when compressive symptoms become too severe.

We present the case of a 68-year-old male found to have worsening dysphagia from an enlarging ARSA aneurysm. A bilateral carotid-subclavian bypass with embolization of both subclavian arteries and aortic endograft placement was performed. Despite imaging showing successful bypass of the aneurysm, the patient’s compressive symptoms worsened. The patient had a prolonged postoperative course that was complicated by pneumonia and tracheal compression, requiring stent placement. On postoperative day 45, the patient went into cardiac arrest and expired.

Postmortem examination revealed an ARSA aneurysm measuring 7.8 × 4.5 × 4.3 cm. All grafts and embolization plugs were in place, without evidence of failure or migration. The aneurysmal sac contained a firm, well-organized clot, occupying 80% of the lumen. Diffuse ulcerative atherosclerotic disease was noted in the aorta. The lungs had gross and histologic findings consistent with pneumonia. Final cause of death was determined to be acute pneumonia with mass effect of the ARSA aneurysm as a contributing factor. Although the cause of the aneurysmal clot formation could not be identified, it raised the question of whether the degree of aortic atherosclerosis and aneurysmal size should be considered while developing indications for surgery.

With the increased detection of ARSA, this case suggests the degree of aortic atherosclerosis and size of the aneurysm should be considered in the establishment of surgical guidelines.
Clinically Unsuspected Invasive Fungal Infections in the Setting of Malignancy
Authors: Hendrix Joshua , Prochilo Gina , Serei Virian , Brister Kathriel , Fyfe Billie , Minerowicz Christine
Published: September 2019

Background

Hospital autopsy rates are currently less than 5% of all nonmedicolegal deaths, significantly decreased from a peak of 40% to 60% in the 1960s. Studies repeatedly demonstrate that advanced imaging and laboratory tests cannot substitute for postmortem examination. We present three cases of clinically unsuspected invasive fungal infections in the setting of malignancy.

Methods and Materials

We performed clinical chart reviews, postmortem examination with microbiology cultures and special stains, and literature review of three adult autopsy cases.

Results

Case 1: 55-year-old man with multiple myeloma 4 days after autologous stem cell transplant who presented with rapidly deteriorating clinical status despite successful treatment for MRSA sepsis; autopsy confirmed cause of death as disseminated Rhizopus infection.

Case 2: 65-year-old woman with myelodysplastic syndrome with myeloproliferative overlap and neutropenic fever on antimicrobial prophylaxis, including fluconazole. Cause of fever was presumed to be noninfectious etiology; autopsy confirmed cause of death as disseminated Candida krusei, an opportunistic invasive fungus resistant to fluconazole.

Case 3: 28-year-old woman with metastatic high-grade pancreatic neuroendocrine carcinoma. An autopsy was performed for the purposes of tissue retrieval; postmortem examination demonstrated a primary pancreatic head mass with oligometastasis to the liver and disseminated Aspergillus infection.

Discussion

Studies show discordance between the clinical cause of death suggested by the primary care team and the autopsy findings in up to 19% of cases. Additionally, autopsy confirmed the clinical diagnosis only 29% of the time and found that nearly 5% of antemortem diagnoses were completely refuted. Fungal infections are a frequent cause of missed diagnoses. A multi-institutional study examining 192,095 autopsies reported a 9% prevalence of fungal infections contributing to death, half of which were missed clinically. The most common risk factor was leukemia and the most common pathogen was Aspergillus species.
Abbott ARCHITECT STAT Contemporary Troponin I Outlier Detection and Prevention of Erroneous Outlier Result Reporting Using an Immediate Repeat Testing Protocol: A 1-Year Summary
Authors: Morgan Bradley , Droes Dawn
Published: September 2019

Objectives

The laboratory sought to monitor and eliminate reported patient cardiac troponin outlier results using the Abbott ARCHITECT STAT Contemporary Troponin-I test.

Methods

The laboratory implemented an immediate repeat test for all patient samples yielding an Abbott ARCHITECT STAT contemporary Troponin I concentration greater than the combined male and female 99th percentile of 28 ng/L. The lower troponin concentration of the paired testing was reported. Samples with reference range troponin concentration were reported without repeat testing. Blood collected in Becton Dickinson Vacutainer PST Gel and Lithium Heparin tubes was centrifuged using a HemoCue StatSpin Express 4 centrifuge 4,000 g × 5 minutes and immediately tested on the primary tube. Troponin outliers were defined as paired samples showing a percentage change based on the low value of the pair exceeding the significant change threshold of 4.7x%CVanalytical for brackets of troponin concentrations (P0.0005), representing a significant change value for serial tests of 1.41xZxCVa%, where Z is 3.3. Critical troponin outliers were sample pairs with one result exceeding and one result within the 99th percentile reference range. Turnaround time was defined as time of receipt to time of verification.

Results

The outlier rate, based on 4,575 troponin tests performed on 3,797 patient samples, was 0.48% (N = 22) of troponin determinations, 0.58% of test samples, and critical outliers 0.13% (N = 6) of troponin determinations, 0.16% of test samples. The significant change threshold at 28 ng/L was 37%. The worst outlier pair was 5,978 ng/L, true value 3,184 ng/L; critical outlier pair 146 ng/L, true value of 14 ng/L. Twenty-one percent of test samples were tested in duplicate; 777 samples had troponin concentration >28 ng/L. Median and mean turnaround time were 32 minutes; 90% of orders completed in 54 minutes.

Conclusion

The laboratory detected and prevented reporting erroneously high outlier troponin results in 0.58% and critical outlier troponin results in 0.16% of test samples using an immediate repeat testing protocol.
Calling Critical Troponin Values Using Significant Delta Rises in Serial Draws Instead of a Hard Cutoff to Help Identify Non-ST-Elevated Myocardial Infarction
Authors: Sheehy Patrick , Skelton Tim
Published: September 2019

Introduction

Non-ST-elevated myocardial infarction diagnoses rely more heavily on lab values. In this study, we examined the effectiveness of an algorithm calculating delta rise in troponins to identify critical values. Using this algorithm in combination with a system to directly call physicians for critical values would improve treatment time for these patients.

Methods

Troponin values were measured using Abbott Architect Stat TnI assay. Reports for 09/01/17 to 11/30/17 were generated using Beaker module of our Epic EHR.

Results

A total of 11,233 troponin values were evaluated for critical values. A total of 108 patients were identified as NSTEMI patients (troponin ordered as well as dual antiplatelet and/or high-dose heparin treatment). In total, 106 NSTEMI patients, or 98.14%, had a critical value using a 0.3-ng/mL cutoff. Eighty-six patients, or 79.63%, had critical values using the algorithm. Of the 106 criticals from a 0.3-ng/mL cutoff, only 76 would be called before treatment was ordered compared to 66 of the 86 criticals using the algorithm. Over 3 months, the algorithm would have called 485 total critical results while a 0.3-ng/mL cutoff would have called 1,913.

Conclusions

A 0.3-ng/mL cutoff had a higher percentage of critical results for NSTEMI patients, but similar numbers of clinically actionable critical results were identified by both methods. Upon further chart review of the 10 discrepant criticals identified by the cutoff but not the algorithm, 2 were identified as false-positive NSTEMI and 5 could be caught with minor algorithm adjustments. The algorithm was able to identify NSTEMI within a clinically actionable timeframe as effective as a 0.3-ng/mL cutoff with 75% fewer criticals called over a 3-month period, allowing criticals called directly to treating physicians to improve time to treatment.
Incidence Rates of Critical Low Glucoses (<40 mg/dL) Among Inpatients Before and After Adoption of a Policy for Laboratory Call-Back Notification of Clinically Significant Hypoglycemia (<54 mg/dL)
Authors: Castillo Jesus Rico , Stickle Douglas
Published: September 2019

Objectives/Background

Beginning in 2017, the American Diabetes Association defined clinically significant hypoglycemia (CSH) as glucose <54 mg/dL. In January 2018, our hospital initiated a call-back policy for inpatient CSH results made in the central laboratory, along with a new nursing policy for CSH response to measurements made either by the central laboratory or by point-of-care testing. Our objective was to determine whether these new policies had affected the incidence rate of low critical values for glucose (CRITICAL, <40 mg/dL) among inpatients at our institution, a 950-bed academic medical center hospital.

Methods

Glucose results for two 12-week intervals before (PRE) and after (POST) adoption of the CSH call-back policy were obtained retrospectively for both central laboratory (C) and point-of-care (P) measurements. Critical incidence rate (R) was defined as number of critical results per total number of measurements of glucose in the interval.

Results

There were four data sets: C-PRE (n = 44,142; CRITICAL = 35), C-POST (n = 46,341; CRITICAL = 66), P-PRE (n = 76,745; CRITICAL = 169), and P-POST (n = 78,838; CRITICAL = 218). For C, R increased in POST by 80% (PRE = 0.79E-3; POST = 1.42E-3). For P, R increased in POST by 26% (PRE = 2.20E-3; POST = 2.77E-3). Combining C and P data, overall R increased in POST by 34% (PRE = 1.69E-3; POST = 2.27E-3). These calculations for R did not, however, exclude possible repeated measurements on individual patients. Counting first or only CRITICAL results from unique individual patients, obtained either from C or P, a revised overall R was still significantly increased in POST, by 22% (PRE = 0.87E-3; POST = 1.06E-3) (P < .01).

Conclusion

After CSH policy changes, the rate of identification of new patients having critical low glucose increased substantially (+22%). This increase was unanticipated, and the exact cause was unknown. Potentially, the increase may reflect an increased awareness of CSH by clinical staff, leading to a higher preselection rate for measurement of CRITICALS and identification of a greater number of patient cases.
Cystic Fibrosis–Related Liver Disease in a 12-Year-Old: Important Questions of Portal Hypertension Etiology That Start With Pathology: A Case Presentation
Authors: Burkholder Ryan , Ozluk Ekin , Cruz Nestor E Dula
Published: September 2019

Introduction

Cystic fibrosis liver disease (CFLD) has long been postulated to be secondary to dysfunctional cystic fibrosis transmembrane conductance regulator in the apical biliary epithelium, leading to bile stasis and eventually cirrhosis with portal hypertension (PH). We present a considerably young patient with signs of esophageal varices and liver biopsy indicating CFLD with significant obliterative portal venopathy as a result of long-standing portal hypertension.

Case Presentation

A 12-year-old with CF (diagnosed at 1 year) and pancreatic insufficiency was noted to have chronically elevated liver enzymes with decreasing platelet counts (130,000 K/ µL). These findings prompted an ultrasound, which showed splenomegaly. Upper endoscopy showed grade 1 esophageal varices, and liver needle biopsy performed at this time demonstrated focal neutrophilic lobular inflammation with marked pseudoacinar transformation and biliary metaplasia. Noted was grade 1 macrovesicular steatosis, focal dense fibrosis also known as “focal biliary cirrhosis,” and obliterative portal venopathy.

Discussion

CF patients with liver fibrosis have significant risk of future morbidity. Of specific interest is identification of PH, and obliterative portal venopathy is a very important feature to distinguish on biopsy as it predicts clinical course. Two recent studies in young adults described obliterative portal venopathy (OPV) and noncirrhotic portal hypertension (NCPH) as the predominant pathophysiology in young adults (median, 22 years) with CFLD. One study found cirrhosis present in only 27% of patients with portal hypertension. In addition, it appears that portal hypertension can precede cirrhotic changes. This unique case in a very young patient with CFLD demonstrates that the underlying pathogenesis of noncirrhotic portal hypertension and CFLD remains to be fully solved. These observations may have important consequences for CFLD management, including opting away from “cholestasis-targeted” treatments and more often considering portosystemic shunting procedures instead of transplant, as liver function remains preserved in numerous CFLD cases.
An Interesting Case of EBV-Associated Autoimmune Hemolytic Anemia
Authors: Desai Sapna , West Jeffrey , Sena Salvador
Published: September 2019

We report a case of a 28-year-old male with no significant past medical history who presented with a weeklong history of flu-like symptoms, dark urine, and cold-induced pain and discoloration in his fingers. Upon presentation, he was noted to be significantly jaundiced. Abdominal ultrasound showed splenomegaly. Laboratory test results returned as follows: hematocrit, 26.4%; hemoglobin, 9.0 g/dL; WBC count, 9.8 × 109/L; and automated differential, 0.42 segmented neutrophils, 0.44 lymphocytes, and 0.08 monocytes. Manual differential showed 0.26 atypical lymphocytes, 0.21 lymphocytes, 0.10 monocytes, 0.03 metamyelocytes, and 0.05 segmented neutrophils. He was found to have a normochromic macrocytic anemia with absolute lymphocytosis, monocytosis, and thrombocytopenia. Peripheral blood smear revealed normochromic macrocytic RBCs with anisocytosis. Downey type II cells, plasmacytoid lymphocytes, large activated lymphocytes, and monocytes were present in addition to a neutrophilic left shift up to metamyelocytes. Liver function tests showed elevated levels of total bilirubin (8.4 mg/dL), direct bilirubin (2.6 mg/dL), aspartate aminotransferase (193 U/L), alanine aminotransferase (102 U/L), and alkaline phosphatase (132 U/L). Urinalysis was positive for urobilinogen. Serology was nonreactive for anti–hepatitis A IgM, hepatitis B surface antigen, and anti–hepatitis C antibody. Positive results were obtained for a rapid monoscreening test and Epstein-Barr virus viral capsid antigen IgM. Direct antiglobulin testing showed positivity for IgG, and complement and cold autoantibody were detected. He was transfused and treated with prednisone, IVIG, and external warmth. After improvement, the patient was discharged with recommendations for follow-up. Hemolytic anemia is a relatively rare complication of patients with infectious mononucleosis and occurs in approximately 1% to 3% of these patients. The pathogenesis of EBV-related autoimmune hemolytic anemia is unknown, with a suspicion for inadequate B-cell function after infection. Appropriate laboratory testing and analysis are critical for rapid diagnosis in these patients to ensure adequate treatment with no long-term sequelae.
CSF Autoantibody Panel Test Utilization in Patients Presenting With Neurologic Alterations: A 3-Year Retrospective Study
Authors: Salazar Cristo Guardado , Carayannopoulos Mary
Published: September 2019

Introduction

When patients present with a range of neurological symptoms, it can be difficult to identify the cause. CSF evaluation of patients who present with a subacute neurological disorder of undetermined etiology is very helpful, especially in patients with known risk factors for autoimmune neurologic disease or cancer. The goal of this study was to review the results of CSF autoantibody panels ordered over the last 3 years and determine the percentage of panels that were positive.

Methods

Results of CSF autoantibody panels ordered over the last 3 years were reviewed and the percentage of samples that tested positive and the most common autoantibody was calculated.

Conclusion

At our institution, pathology residents review orders for CSF autoantibody panel evaluation and approve testing based on the clinical history and neurologic evaluation in order to prevent inappropriate test utilization. Of the 192 CSF evaluations ordered between 2015 and 2018 (5 dementia, 72 encephalopathy, and 115 paraneoplastic CSF-autoimmune evaluation), 19.8% of the tests were positive. The most common autoantibodies detected were the NMDA (4.68%) and VGKC (4.68%) receptors.
Concordance Between the TIRADS Ultrasound Scoring Criteria, Fine-Needle Aspiration Cytology, and Thyroid Final Resection Diagnosis
Authors: Kaseb Hatem , Charifa Ahmad , Abi-Raad Rita , Cai Guoping , Hammers Lynwood , Prasad Manju , Adeniran Adebowale
Published: September 2019

Objectives

Thyroid imaging reporting and data system (TIRADS) criteria were recently introduced in our institution to aid in predicting diagnosis for various thyroid lesions. We evaluated the association of TIRADS imaging score and fine-needle aspiration (FNA) cytology with thyroid lesions that had a confirmed diagnosis at resection, with a focus on understanding the predictability of this diagnostic tool in malignancy prediction.

Methods

We assessed the concordance of TIRADS criteria and FNA diagnosis to the final anatomical diagnosis in the assessment of thyroid lesions. We retrieved the cases from the archives of the Yale pathology department between June 2017 and January 2018. Our inclusion criteria included patients who had a TIRADS score, cytology diagnosis, and final surgical resection diagnosis. A total of 65 subjects with an age range of 11 to 88 years were identified.

Results

The majority of the patients were females, 65% (42/65). Cases with TIRADS score 1-2 (likely benign) and Bethesda I/II nondiagnostic/benign were few since most of these cases did not go for surgical resection. The mildly suspicious TIRAD score 3 and FLUS category showed similar trends, 68% and 67%, respectively, in predicting malignant lesions. The TIRADS score 4 when compared to cytology (IV)/(V) demonstrated similar consistent results in malignancy prediction, both being high at 89% and 87%, respectively. The TIRADS score 5 demonstrated a 95% malignancy prediction. The overall sensitivity and specificity of TIRADS score in our cohort were 66% and 77%, respectively. The positive and negative predictive values of TIRADS score in our cohort were 89% and 39%, respectively. In comparison, the overall sensitivity and specificity of cytology assessment in our cohort were 91% and 44%, respectively. The positive and negative predictive values of cytology assessment in our cohort were 85% and 57%, respectively.

Conclusion

Our results demonstrated that both cytology and TIRAD score had similar trends in malignancy prediction. Cytological assessment had higher sensitivity but lower specificity compared to TIRADS score. While both techniques showed concordant high predictability of malignant lesions (approximately 91%), the use of both modalities adjunctively will be very useful in triaging cases for surgery. Overall, utilizing TIRADS score with cytology will help reduce the risk of unnecessary invasive procedures in patients with a low probability of malignant thyroid disease.