bluuebunny/crossref_metadata_2025_split huggingface dataset:
The efficiency of hypogammaglobulinemia reflex immunofixation electrophoresis in identifying monoclonal proteins
Authors: Zanfagnin V , Petersen P , Ikoma S , Chambliss A 
Published: October 2021

Abstract

Introduction/Objective
Hypogammaglobulinemia can be a common occurrence in disorders with monoclonal gammopathies. Because hypogammaglobulinemia may mask a monoclonal protein on serum protein electrophoresis (sPEP), its presence in the absence of a discernible M-spike is often the basis of reflexive testing by immunofixation electrophoresis (IFE). At our Institution, reflex IFE has historically been performed in cases where the gamma fraction on sPEP is &lt;0.6 g/dL. The aim of this study was to test the predictive performance of hypogammaglobulinemia in identifying abnormal bands on IFE in newly screened patients.

Methods/Case Report
All patients that underwent sPEP testing from November 2020 to May 2021 at our Institution were identified. Among them, patients with gamma fraction &lt;0.6 g/dL and no previous sPEP testing were included for analysis. Reflex IFE results were reviewed for identification of abnormal bands.

Results (if a Case Study enter NA)
Out of a total number of 1,374 patients tested for sPEP in the study period, 72 had serum gamma fraction &lt;0.6 g/dL (5.2%). Among them, 36 patients had no previous sPEP testing, and their reflex IFE were reviewed. In 38.8% of the cases, the IFE showed one or more abnormal (monoclonal) bands. When considering a new threshold for hypogammaglobulinemia IFE reflex of &lt;0.4 g/dL, the diagnostic yield for finding abnormal bands increased to 62.5%. Moreover, a percentage reduction of 64.2% was observed in the number of reflex IFE performed.

Conclusion
Although these data must be confirmed using a larger sample population, a lower threshold for hypogammaglobulinemia may be proposed at our Institution to reduce labor and costs and to improve efficiency of monoclonal protein detection.
Evaluation of Roche Hemolysis Index for Reporting of Plasma Hemoglobin, and Considerations Regarding Hemolysis Detection in Use of Ventricular-Assist Devices
Authors: Alqabbani R , Stickle D 
Published: October 2021

Abstract

Introduction/Objective
Our cardiac intensive care unit (CICU) asked the laboratory to provide plasma hemoglobin testing to monitor effects of ventricular-assist devices (VAD). Roche hemolysis index (HI) has been reported to be suitable for this purpose. Use of HI as a reportable test, however, must be validated in-house as a laboratory- developed test (LDT), according to CLIA regulations. We describe results of our evaluation, and caveats for intended use.

Methods/Case Report
Concentrated hemolysate was produced by osmotic lysis of packed red blood cells in water. Hemolysis in plasma was by dilution of concentrated hemolysate. Verification of HI as a measure of hemoglobin (mg/dL) used the Sigma-Aldrich spectrophotometric hemoglobin assay as a gold standard. Linearity, linear range, sensitivity, were investigated by dilution measurements; interferences by admixture experiments; reproducibility (precision, intra- and inter-assay) in each case by replicate measurements (n = 20).

Results (if a Case Study enter NA)
HI was confirmed to correspond to hemoglobin in mg/dL. Linearity was between 2-1000 mg/dL (r2 &gt;0.99). Intra-assay precisions were &lt;=2.5% (hemoglobin = 74,148 mg/dL); inter-assay precisions were &lt;=4.9% (hemoglobin = 69,139 mg/dL). HI variability was ±2 at very low values (0-10 mg/dL). There were no interferences observed among common therapeutic drugs. Hyperlipemia (evaluated up to lipemic index (LI) = 225) and hyperbilirubinemia (evaluated up to icteric index (II) = 7) demonstrated no significant interference.

Conclusion
Analytically, HI exhibited acceptable performance for reporting of plasma hemoglobin. Deployment would require establishment of quality control (QC) and proficiency testing procedures for HI. Clinically, a caveat for use is that HI cannot distinguish between hemolysis in circulation vs. that due to sample collection. For the CICU, HI &gt;10 mg/dL is characteristic of more than 20% of samples drawn in general, irrespective of use of VAD. Temporal patterns of HI must therefore be carefully evaluated in parallel with haptoglobin measurements to assist in VAD management.
The Utility of PathElective.com as a Curricular Adjunct in Laboratory Medicine Education
Authors: Lilley C M , Marin M J , Wiencek J , Fu L , Wolniak K , Kanakis C E , Hermelin D , Scordino T , Thomas C , Mirza K M , Demas C 
Published: October 2021

Abstract

Introduction/Objective
PathElective.com was founded as a means of combating stagnating resident and medical student education due to halting of in-person educational activities during the COVID-19 pandemic. The site has since grown to be included in medical student and resident training programs at numerous institutions across the world, serving as a unique means of delivering high-quality and trackable laboratory medicine education.

Methods/Case Report
The PathElective website was developed using Squarespace, video lectures were recorded by each professor or sourced from openly available web resources incorporating virtual slides, reading assignments, or relevant podcasts at the course director’s discretion. Monthly website traffic data were obtained through Squarespace analytics for the first 11 months of the website being available (May 1, 2020, to April 22, 2021). Geographic and source data were obtained through deidentified IP address analysis built-in to Squarespace analytics. Students who registered to take the online courses were assessed before and after interacting with the course materials using a dual form crossover quiz design to prevent memorization of questions and assess comprehension. Quiz data were all anonymous and improvement was determined using a paired t-test. Comparisons between courses were made via percentage improvements.

Results (if a Case Study enter NA)
Over the tracking period, PathElective.com received 352,012 page views, 73,550 visits, 33,225 unique visitors, and accrued 5,815 registered users. Most of the visitors (72.8%) arrived at the site via a direct URL input, 14.9% through a search engine, and 10.6% through social media. Most visitors were from the US (43.8%) or India (11.9%). With a total of 1598 test pairs being analyzed from all clinical pathology modules, the average increase in score was 14.4% (95%, CI=10.3-18.6, p=0.0052). All courses experienced a statistically significant increase in scores except for Clinical Chemistry lesson 3.2 (7%, -1.8-15.9% CI, p=0.12). Courses were well received with a median satisfaction score was very satisfied in all six assessment categories.

Conclusion
PathElective.com is a free and effective means of enhancing clinical pathology training in medical education. Students liked the online format, the quality of the lectures, and course faculty, and felt they could get help if needed. An area of improvement for this platform would be the interaction with students on social media.
Cytologic Analysis of a Glomus Tumor in the Left Second Toe: Case Report
Authors: Hwang J , McDowell S , Cole B , Huber A R , Reyes C 
Published: October 2021

Abstract

Introduction/Objective
Glomus bodies reside in the stratum reticularis of the dermis as well as in visceral organs. Their functions involve temperature and blood pressure regulation. The incidence of glomus tumors is approximately 1.5%, occur more frequently in women, and generally manifest during the third to fifth decade of life. A majority of glomus tumors are diagnosed by biopsy and excision. At least 19 case reports exist in the literature where glomus tumors are diagnosed by fine-needle aspiration (FNA). We add to this growing literature by discussing a case report involving the cytologic findings of an FNA-diagnosed glomus tumor.

Methods/Case Report
A 66-year-old female presented with left second toe pain for 41 years but worsening in the past several months. Physical exam revealed 5/5 muscle strength in her toes without loss of sensation. There was no edema, erythema, nor ecchymosis. Pain was notably out of proportion when palpating her second nailbed. Magnetic resonance imaging (MRI) with contrast was performed revealing a 1.1 x 1.0 x 0.9 cm circumscribed, ovoid mass involving the dorsal aspect of the second toe distal phalanx along its dorsal margin (Figure 1). The patient underwent fine needle aspiration and biopsy. Cytologic findings included clusters of uniform cells with round to oval nuclei and scant cytoplasm. There was spindling of cells noted in some of the clusters. Cells were surrounded by thick wisps of magenta colored myxoid material reminiscent of a pleomorphic adenoma (Figure 2). Biopsy showed uniform cells surrounding capillaries. Immunohistochemistry performed on the biopsy showed that lesional cells were positive for alpha-smooth muscle actin (SMA). A diagnosis of glomus tumor was made. Amputation was performed with clear margins.

Results (if a Case Study enter NA)
NA

Conclusion
Glomus tumor is a rare tumor that is usually diagnosed on biopsy; however, it has distinct cytologic features that can aid in its diagnosis on fine needle aspirations.
Fine-needle aspiration (FNA) of an abdominal wall mass malakoplakia mimicking malignancy: a case report
Authors: Alkashash A M , Segura S 
Published: October 2021

Abstract

Introduction/Objective
Malakoplakia is a granulomatous disease of infectious etiology that affects immunosuppressed individuals. It commonly involves the genitourinary tract, but the involvement of other systems and organs has been reported. Gram-negative bacterial infections are mostly the responsible agent, with Escherichia coli found in approximately 80% of cases. Malakoplakia can present clinically and radiologically as a malignancy.

Methods/Case Report
We present a case of a 16-year-old male with a history of acute liver failure requiring liver transplantation due to autoimmune hepatitis. He was admitted with concerns for rejection, and he was found to have an abdominal wall mass. MRI studies showed a lobulated, solid, and cystic mass measuring 11.0 x 4.9 x 1.9 cm in the right abdominal wall. The mass was extending through all the layers of the abdominal wall and protruding into the abdominal cavity with mass effect on the liver, encasing the common bile duct, hepatic artery and main portal vein, concerning for malignancy, versus a possible infectious process. Ultrasound-guided FNA and biopsy were performed. The Diff-Quik and Papanicolaou-stained smears showed abundant foamy macrophages with numerous granular intra- and extracellular slightly refractile inclusions (4-10 um), rare multinucleated giant cells, and fibroblasts. A Von Kossa stain highlighted the targetoid inclusions consistent with Michaelis-Gutman bodies. Additional stains were performed on the core needle biopsy, and the histiocytes were positive for CD68 and CD163. Stains for keratins, CD45, HSA, CD1A, and Langerin were negative. No acid-fast or fungal microorganisms were identified on AFB and GMS stains, respectively. The final diagnosis was malakoplakia, with no evidence of malignancy.

Results (if a Case Study enter NA)
NA

Conclusion
Fine-needle aspiration cytology is useful in establishing a fast diagnosis of malakoplakia and excluding malignancies.
Cytomorphologic findings of thyroid carcinoma showing thymus-like (CASTLE) differentiation: A case report
Authors: Abu-Salah A K , Segura S , Mesa H , Cramer H M 
Published: October 2021

Abstract

Introduction/Objective
Thyroid carcinoma showing thymus-like differentiation also known as intrathyroidal thymic carcinoma is a rare thyroid neoplasm arising from either an ectopic intrathyroidal thymic tissue or from remnants of thymobranchial pouches. On fine needle aspiration (FNA) its diagnosis can be challenging due to overlapping morphologic features with other aggressive thyroid carcinomas.

Methods/Case Report
A 31-year-old female consulted for 6-month history of neck swelling and tenderness. Thyroid ultrasound demonstrated a 2.6 x 2.5 x 2.1 cm nodule in the right lobe with punctate calcifications. FNA showed cellular smears composed of loosely cohesive and single basaloid neoplastic cells exhibiting significant cellular and nuclear pleomorphism. Focal squamous differentiation with keratinization was noted on the cell block sections. Immunocytochemical stains showed that the tumor was positive for cytokeratin AE1/AE3, p40, p63, CD117 and CD5 consistent with intrathyroidal thymic carcinoma. The Ki67 proliferative index was approximately 40%. A right thyroidectomy with central neck dissection confirmed the diagnosis and showed a 3.0 cm tumor with invasion into skeletal muscle, lymphovascular invasion and positive lymph nodes. The patient completed adjuvant radiotherapy and remain in remission at 3-months follow-up.

Results (if a Case Study enter NA)
NA

Conclusion
Intrathyroidal thymic carcinoma is a rare thyroid neoplasm that frequently shows squamous differentiation and therefore overlaps with papillary thyroid carcinoma with squamous morulae, squamous cell carcinoma and anaplastic carcinoma. The coexpression of squamous markers together with CD5 and CD117 allows the recognition of CASTLE on FNA samples.
Microsatellite unstable esophageal adenocarcinoma masquerading as a conventional esophageal primary
Authors: Jayaratne N , Reese A , Jiang K 
Published: October 2021

Abstract

Introduction/Objective
Patients with microsatellite instability (MSI) are susceptible to developing epithelial malignancies in multiple organs and systems, especially along the gastrointestinal tract. It’s important to know that MSI-tumors may present with an intriguing non-classical, “non-MSI-appearing”, histomorphology that deviates from the well-known MSI or Lynch syndrome spectrum. Pathologists should thus be prepared for unusual histopathological presentations in order to detect MSI and related therapeutic and prognostic targets. Ultimately, a timely and accurate classification is a crucial step in guiding clinical course.

Methods/Case Report
An 80-year-old male presented to an outside facility due to worsening dysphagia. Imaging and endoscopy studies confirmed a near-obstructing distal esophageal mass. Subsequently, an esophagogastrostomy was performed, which removed a moderately differentiated adenocarcinoma (pT3N1). The patient was managed with conventional chemotherapy after the surgery; however, due to progressive metastasis involving the liver, he was referred to our cancer center.

Results (if a Case Study enter NA)
Upon review of outside pathology materials, the tumor displayed irregularly arranged bizarre glands with eosinophilic cytoplasm and unusually large nuclei within the abundant fibrotic stroma. No intra-tumoral lymphocytes or “Crohn’s-like” pushing borders were identified. However, ancillary tests performed on acquired outside material showed loss of MLH1 and PMS2 with retained MSH2 and MSH6 in the tumor. Subsequent mutational analysis revealed additional MSI-related carcinogenic mutations. Accordingly, the previous regimen was switched to immunotherapy-based management. The patient remains disease-free 10-months later.

Conclusion
This unusual MSI esophageal adenocarcinoma exemplifies that such tumors may present with non- MSI/non-lynch histomorphology and that ancillary analyses should be carried out without reservation to evaluate microsatellite status. Thus, optimizing patient management and outcome.
Invasive Large Duct Type Pancreatic Adenocarcinoma: A Diagnostically Challenging Entity
Authors: Al-Attar M M , Khedr S M 
Published: October 2021

Abstract

Introduction/Objective
Large duct type invasive pancreatic adenocarcinoma is a morphological variant of pancreatic ductal adenocarcinoma that poses a radiologic and pathologic diagnostic challenge as it closely mimics other non- invasive neoplasms, including mucinous cystic neoplasms and intraductal papillary mucinous neoplasms (IPMN). We report a case of large duct type invasive adenocarcinoma that on radiology closely mimics an IPMN.

Methods/Case Report
The case id that of a 73-year-old male presenting with weight loss and chronic diarrhea. Abdominal imaging revealed features suggestive of main duct IPMN with possible side branch extension into the pancreatic head. Endoscopic retrograde cholangiopancreatography showed a bile duct stricture that was stented and biopsied, revealing mucinous material with neoplastic cells. A Whipple procedure was performed, revealing a 3.3 cm, tank-pink, papillary lesion in the pancreatic head, that is partially cystic with mucinous contents, and displaying exophytic extension into the main pancreatic duct.
Microscopically, the lesion consists of a high-grade IPMN involving the main pancreatic duct with side branch extension, and associated with invasive adenocarcinoma, the majority of which is of the large duct type, showing irregularly distributed large ducts with jagged edges and surrounding desmoplasia, with intra-luminal mucinous material and neutrophils. The lining cells show focal pseudo-stratification and bland cytology. Foci of conventional type invasive adenocarcinoma, clusters of invasive tumor cells, and peri-neural invasion are also identified. Post- procedure, the patient underwent chemotherapy with marked symptomatic improvement.

Results (if a Case Study enter NA)
NA

Conclusion
Large duct type invasive adenocarcinoma of the pancreas can closely mimic non-invasive pancreatic neoplasms, posing diagnostic challenges that require identification of subtle features to achieve the correct diagnosis and guide treatment.
Iso-chromosome 12p Analysis by Fluorescent In-Situ Hybridization: An Academic Institutional Experience
Authors: Patwardhan P P , Satturwar S , Dhir R , Quiroga-Garza G M 
Published: October 2021

Abstract

Introduction/Objective
Chromosome 12 abnormalities like iso-chromosome 12p (i12p) and amplification of 12p are seen in majority (89%) of the primary and metastatic testicular germ cell tumors (TGCTs). i12p can be detected by karyotyping, fluorescent in-situ hybridization (FISH) or reverse transcriptase polymerase chain reaction. The aim of this study was to review i12p FISH data at our institution and assess the clinical utility.

Methods/Case Report
Laboratory information system was queried over a period of 15 years to search for cases where i12p FISH test was requested. FISH test was performed using TelVysion 12p telomeric probe and CEP 12 centromere probe on paraffin-embedded tissue or cell blocks. A ratio of 12ptel/CEP12 signal of 1.4 or greater was considered as positive. Patient demographics, clinical presentation, pathologic findings, and follow-up data were documented and correlated.

Results (if a Case Study enter NA)
Total 58 cases were identified with an age range of 14 to 76 years. Majority were male (M=52, F=6). Of these cases, 15 were testicular and 43 extra-testicular cases that included resection (n=35), biopsy (n=20) and cell-blocks (n=3). i12p was detected in 8 out of 15 testicular cases while i12p was detected in 16 out of the 43 extra-testicular cases. The extra- testicular cases included 17 retroperitoneal lesions, 8 lesions from the mediastinum, 6 lymph nodes from other sites and 12 miscellaneous lesions. Using pathology diagnosis with immunohistochemistry as gold standard, overall sensitivity was 60% and specificity was 86%. There were 3 false positive cases [Benign testicular parenchyma (n=1), suspicious for germ cell neoplasia in-situ (n=1) and undifferentiated epithelioid neoplasm (n=1)].

Conclusion
Our results show that although the sensitivity was limited, FISH test for i12p demonstrated high specificity(86%) for diagnosis of primary or metastatic TGCTs. As an adjunct test, i12p FISH can help identify and further characterize a significant number of GCTs with unusual morphology or clinical presentation.
Malignant Glomus Tumor of the Kidney: A Case Report and Review of the Literature
Authors: Nwanze J , Shih J , Rolf N , Halat S K 
Published: October 2021

Abstract

Introduction/Objective
Glomus tumors are rare neoplasms arising from the glomus body which is a specialized arteriovenous anastomosis that has a role in temperature regulation of the skin. They account for less than 2% of soft tissue tumors and typically manifest as painful nodules in the extremities, most commonly in the subungual region of the fingers and dermis of the wrist, forearm, and foot. They rarely arise in visceral organs as they lack glomus bodies. There are rare case reports of benign glomus tumors arising in the kidney, and malignant renal glomus tumors are exceedingly rare.

Methods/Case Report
We present a case of a 32-year-old man who was found to have a right renal mass incidentally after being involved in a motor vehicle accident. Computed tomography of the abdomen revealed a 5.3 cm enhancing mass in the posterior aspect of the inferior pole of the right kidney. Histological examination revealed a solid neoplasm composed of a pleomorphic population of cells with densely eosinophilic to pale cytoplasm, centrally located round to oval nuclei with fine nuclear chromatin and relatively inconspicuous nucleoli. Prominent lymphovascular invasion and 1 mitotic figure per 10 high power fields were also present. Areas composed of necrosis, sclerosis, hemosiderin deposition and calcification were identified. Immunohistochemical stains revealed the neoplastic cells to be positive for CD34, vimentin, smooth muscle actin, caldesmon, with focal reticulin staining around the tumor cells. Cells were negative for desmin, high and low molecular weight cytokeratins, synaptophysin, chromogranin, PAX8, and GATA3. The morphology and immunoprofile of the mass are consistent with the diagnosis of glomus tumor.

Results (if a Case Study enter NA)
NA

Conclusion
Although very rare, the presence of prominent vascular invasion, increased mitotic activity as well as necrosis are consistent with malignant behavior and support the classification as a malignant glomus tumor.
Adenoid cystic carcinoma of the female genital tract – Case series with review of literature
Authors: Bhele S , Crisi G , Hunt J 
Published: October 2021

Abstract

Introduction/Objective
Adenoid cystic carcinoma (ACC) of the female genital tract is rare and the published literature is sparse. In the vulva it is considered to arise from Bartholin’s gland whereas, in cervix it arises from the reserve cells. Although morphologically ACC of female genital tract is similar to its counterparts in salivary glands, the clinical behavior is different.

Methods/Case Report
We describe the clinicopathologic and immunohistochemical features of three cases of adenoid cystic carcinoma of female genital tract with review of literature. The age of our patients ranged from 48 to 75 years with tumors ranging from 1.9 to 3.9 cm. The tumor sites were vulva (2 cases) and cervix (1 case). Two of the 3 patients presented with mass lesions and one patient presented with postmenopausal bleeding. Microscopically, all tumors showed classic morphologic features with cribriform, tubular and solid patterns. On immunohistochemistry, CD117 was consistently reactive in all 3 cases. In addition, the ductal and myoepithelial cell population were highlighted by keratin cocktail and p63 respectively. P16 was diffuse and block-like positive in two cases (one vulvar and cervical ACC). High risk HPV (HPV 33) was detected in one case of vulvar ACC. Two patients underwent surgical excision while one patient was deemed unfit for surgical treatment. All 3 patients received radiation therapy. On follow- up that ranged from 17 to 76 months, one patient is alive without evidence of disease. One patient died of disease with local recurrence and lung metastasis. The other patient died of complications (radiation proctocolitis with colonic perforation).

Results (if a Case Study enter NA)
NA

Conclusion
Our cases highlight the aggressive behavior of adenoid cystic carcinoma in the female genital tract and the need for close follow-up. Also high risk HPV has been demonstrated in cervical ACC previously, but our series demonstrates high risk HPV association with vulvar ACC.