Papillary Endothelial Hyperplasia of the Adrenal Gland: A Case Report and Review of the Literature
In this report we present our case in the context of the literature and review differential diagnosis of PEH in the adrenal gland. (Source: Pathology Case Reviews)
Source: Pathology Case Reviews - January 1, 2022 Category: Pathology Tags: Case Reviews Source Type: research

Differential and Ancillary Testing in the Diagnosis of Perinephric Myxoid Pseudotumor of Fat: A Case Report and Review of the Literature
We report the 14th case of perinephric myxoid pseudotumor of fat. A 70-year-old woman with a medical history of rheumatoid arthritis, diabetes mellitus type 2, hypertension, hyperlipidemia, and hypothyroidism presented with a long-standing retroperitoneal mass and worsening abdominal distension and pain. Computed tomography scan showed a large 21-cm right retroperitoneal mass and gallstones. Histologic sections show a lipomatous lesion with fat necrosis, areas of myxoid change, and lymphoplasmacytic infiltrate. No atypical stromal cells were identified. Well-differentiated liposarcoma and IgG4-related disease were consider...
Source: Pathology Case Reviews - January 1, 2022 Category: Pathology Tags: Case Reviews Source Type: research

Primary Sclerosing Epithelioid Fibrosarcoma of the Kidney: A Case Report and Review of the Literature
We present a case of SEF that arose in the kidney. Histological examination revealed densely hyalinized epithelioid tumor suggestive of SEF. The diffuse immunohistochemical staining of MUC4 by neoplastic cells and the presence of EWSR1 gene rearrangement by fluorescence in situ hybridization analysis confirmed the histological diagnosis. (Source: Pathology Case Reviews)
Source: Pathology Case Reviews - January 1, 2022 Category: Pathology Tags: Case Reviews Source Type: research

Two Gastric Cancers With Uncommon ALK Fusion Diagnosed With Comprehensive Panel Sequencing and Confirmed With Companion Diagnostic Assay
ALK gene rearrangement is clinically significant due to the ALK tyrosine kinase inhibitor, important in several types of ALK-rearranged malignancies, most prominently in non–small cell lung cancers. ALK fusion in the gastrointestinal tract is rare. Herein, we report 2 cases of gastric cancer, in which ALK is fused with two uncommon partners, SLMAP and STRN. Two cases of ALK fusion were identified by comprehensive panel sequencing of 231 clinical gastric cancer samples. The results were confirmed using the Ventana ALK (D5F3) CDx assay. Both were advanced stage and were signet ring cell carcinomas with histological feature...
Source: Pathology Case Reviews - January 1, 2022 Category: Pathology Tags: Case Reviews Source Type: research

Primary Appendiceal Gastrointestinal Stromal Tumor
We report the incidental primary gastrointestinal stromal tumor of the appendix and discuss its biological behavior. (Source: Pathology Case Reviews)
Source: Pathology Case Reviews - January 1, 2022 Category: Pathology Tags: Case Reviews Source Type: research

Metastatic Hepatobiliary Cystadenocarcinoma Mimicking a Gynecologic Malignancy
We report a case of hepatobiliary cystadenocarcinoma that subsequently presented as metastatic disease mimicking a primary gynecological malignancy. The 30-year-old patient had a history of a completely resected hepatobiliary cystadenocarcinoma 2 years prior to presentation, without adjuvant therapy. She presented to her gynecologist with pelvic pain and vaginal bleeding. Physical examination revealed diffuse nodularity along the anterior vaginal wall, and imaging showed a 5.8 × 4.1 cm pelvic mass with diffuse metastatic disease. Biopsies of the vaginal wall and an inguinal lymph node showed nests of pleomorphic cells wit...
Source: Pathology Case Reviews - January 1, 2022 Category: Pathology Tags: Case Reviews Source Type: research

Exceptional and Difficult: When to Think Outside the Box
No abstract available (Source: Pathology Case Reviews)
Source: Pathology Case Reviews - January 1, 2022 Category: Pathology Tags: Editorial Source Type: research

Sclerosing Microcystic Adenocarcinoma: A Case Report and Review of Literature
Sclerosing microcystic adenocarcinoma is an extremely rare tumor that was described by Mills et al in 2016 and occurs in the head and neck region. There are 8 cases published with this terminology in the English language literature. Here, we present a 59-year-old female patient with sclerosing microcystic adenocarcinoma in the anterolateral tongue and review the previously reported 8 cases. Most of the reported cases are in females and localized to the tongue. This tumor mimics microcystic adnexal carcinoma, which is a cutaneous-based malignant tumor. The tumor in the present case was composed of cells that were arranged i...
Source: Pathology Case Reviews - October 28, 2021 Category: Pathology Tags: Case Reviews Source Type: research

Epstein-Barr Virus Negative Lymphoepithelioma-Like Cholangiocarcinoma in a Patient of European Descent: A Report of a Case
We present the first case report of Epstein-Barr virus–negative lymphoepithelioma-like carcinoma–associated cholangiocarcinoma occurring in a patient of European descent. The 62-year-old woman described here presented with nonspecific symptoms of lethargy, low-grade pyrexia, and raised white blood cell count. Subsequent investigations revealed a large hepatic mass with subsequent hepatectomy yielding a large tumor containing poorly differentiated adenosquamous cholangiocarcinoma alongside a rich infiltrate of lymphocytes and lymphoplasmacytic inflammatory infiltrate. Epstein-Barr virus in situ hybridization was negativ...
Source: Pathology Case Reviews - October 28, 2021 Category: Pathology Tags: Case Reviews Source Type: research

Too Young for Clear Cell Sarcoma of the Kidney? A Case Report With Review of Differential Considerations
Clear cell sarcoma of the kidney (CCSK) comprises 3% of all childhood renal cancers. Accurate diagnosis is vital for appropriate therapy, which results in a 70% to 90% overall survival rate in this previously lethal tumor. Renowned for its ability to mimic and be mimicked by every other pediatric renal tumor, and even some extrarenal retroperitoneal tumors, CCSK has a unique metastatic pattern and molecular aberrations, as well as a generally consistent clinical presentation which is of great utility in the differential. A case of CCSK in a 4-month-old boy is presented, only the fifth case reported in a patient younger tha...
Source: Pathology Case Reviews - October 28, 2021 Category: Pathology Tags: Case Reviews Source Type: research

Composite Renal Cell Carcinoma and Angiomyolipoma: A Rare Case and Possible Diagnostic Pitfalls
Angiomyolipomas are benign renal neoplasms commonly associated with tuberous sclerosis complex (Semin Diagn Pathol 1998;15(1):21–40). However, angiomyolipoma with concurrent renal cell neoplasia is very uncommon, with fewer than 50 reported cases. Even less common is direct admixture of renal cell carcinoma (RCC) and angiomyolipoma within 1 tumor mass, with only rare possible cases reported in the literature. A multi-institutional study of 36 cases of synchronous renal cell neoplasm and angiomyolipoma found that clear cell RCC was the most common concurrent cancer in patients with sporadic or tuberous sclerosis-associate...
Source: Pathology Case Reviews - October 28, 2021 Category: Pathology Tags: Case Reviews Source Type: research

Primary Peritoneal Ependymoma: A Case Report and Literature Review
We report such an example in a 58-year-old woman who presented with abdominal pain, bloating, and history of a 70-lb weight loss over the prior year. Computed tomography scan showed a 20-cm pelvic mass extending into the abdomen and a 3.5-cm peritoneal-based right hepatic lobe/diaphragm mass. Histologic examination of the pelvic mass revealed an ovarian mucinous cystadenoma. The peritoneal mass showed a highly cellular solid neoplasm containing perivascular pseudorosettes and true ependymal rosettes. Tumor cell nuclei were rounded, oval, and elongated with coarse, granular chromatin and occasional small nucleoli. Mitoses w...
Source: Pathology Case Reviews - October 28, 2021 Category: Pathology Tags: Case Reviews Source Type: research

Mucinous Cystic Neoplasm of the Liver With Associated Neuroendocrine Carcinoma: A Case Report
We present the case of a 63-year-old woman with a history of choledochal cyst, who now presented with abdominal pain. Computed tomography showed a multilocular cystic lesion in the liver. The cyst was surgically enucleated, and the histological examination revealed an MCN with low-grade dysplasia, with an associated invasive large cell NEC. To our knowledge, this is the first report in the literature of a biliary MCN with associated NEC. (Source: Pathology Case Reviews)
Source: Pathology Case Reviews - October 28, 2021 Category: Pathology Tags: Case Reviews Source Type: research

Uterine Sarcoma With a Novel WWOX-NTRK2 Fusion in a Postmenopausal Woman With Li-Fraumeni–Like Syndrome: A Case That Expands the Spectrum of NTRK-Rearranged Uterine Tumors
NTRK-rearranged tumors are being increasingly recognized and targeted with TRK inhibitor therapies. A novel NTRK2 fusion–positive uterine sarcoma arising in a patient with Li-Fraumeni–like syndrome is described in this article. (Source: Pathology Case Reviews)
Source: Pathology Case Reviews - October 28, 2021 Category: Pathology Tags: Case Reviews Source Type: research

Sneddon Syndrome: Case Report and Literature Review
Sneddon syndrome (SS) is a rare disorder that has a variety of pathologic associations, often described as the first cutaneous manifestation of antiphospholipid syndrome. Sneddon syndrome is characterized by livedo racemosa (LR), cerebrovascular symptoms, and microangiopathic disease in the deep vessels on skin biopsy. We discuss a case of a 55-year-old woman with a complex history of arterio-occlusive disease who presented with a violaceous and painful rash on her thigh. Skin biopsy showed thrombotic vasculopathy. Her clinical and cutaneous manifestations led to the question and the diagnosis of SS. Our case is one of the...
Source: Pathology Case Reviews - October 28, 2021 Category: Pathology Tags: Case Reviews Source Type: research