A rare case of primary gastric plasmacytoma: An unforeseen surprise

Primary plasmacytoma of the gastrointestinal tract is a rare entity. We report a case of a primary gastric plasmacytoma in a 57-year-old man who presented with upper-gastrointestinal bleeding. Endoscopy showed a nodular gastric mass with central umblication. Histological examination of the gastrectomy specimen revealed a monoclonal lambda-chain extramedullary plasmacytoma. Further staging was found to be negative for multiple myeloma. As other more common pathologic processes at this site may also be endowed with numerous plasma cells, awareness of this entity and distinction using immunohistochemistry are extremely crucial. Because systemic disease ultimately develops in many patients with localized plasmacytoma, such patients should be followed closely for the appearance of clinical, biochemical, and roentgenologic evidence of multiple myeloma

A rare case of primary gastric plasmacytoma: An unforeseen surprise

Primary plasmacytoma of the gastrointestinal tract is a rare entity. We report a case of a primary gastric plasmacytoma in a 57-year-old man who presented with upper-gastrointestinal bleeding. Endoscopy showed a nodular gastric mass with central umblication. Histological examination of the gastrectomy specimen revealed a monoclonal lambda-chain extramedullary plasmacytoma. Further staging was found to be negative for multiple myeloma. As other more common pathologic processes at this site may also be endowed with numerous plasma cells, awareness of this entity and distinction using immunohistochemistry are extremely crucial. Because systemic disease ultimately develops in many patients with localized plasmacytoma, such patients should be followed closely for the appearance of clinical, biochemical, and roentgenologic evidence of multiple myeloma

Synchronous Squamous Cell Carcinoma of Tongue and Unicentric Cervical Castleman’s Disease Clinically Mimicking a Stage IV Disease: A Rare Association or Coincidence?

Malignancy in a setting of hyaline vascular type of Castleman’s disease (HVCD) is an exceptional occurrence. Herein, we report an extremely rare case of synchronous unicentric cervical HVCD and squamous cell carcinoma (SCC) of tongue mimicking stage IV disease. A 32-year-old gentleman presented with an ulcerated mass on the right tongue border and ipsilateral cervical nodal mass. As the clinical stage was IVB (T1N3M0), an anterior two-third glossectomy with bilateral modified neck dissection was performed. On gross examination, an ulcerated mass on the right lateral border was identified. In addition, an 8 cm large nodal mass at right level III–V was seen. Microscopy from the ulcerated growth in the tongue revealed an invasive well differentiated squamous cell carcinoma. However, the right cervical nodal mass yielded surprise histology of Castleman’s disease, hyaline-vascular type. Final tumor pathological staging was revised to pT1N0M0. This case reveals that HVCD can rarely be associated with an epithelial malignancy wherein it can clinically mimic nodal metastasis. Whether such a phenomenon occurs due to underlying immune aberrations or is a rare co-incidence remains unclear

NIPBL::NACC1 Fusion Hepatic Carcinoma

Several reports describing a rare primary liver tumor with histologic features reminiscent of follicular thyroid neoplasms have been published under a variety of descriptive terms including thyroid-like, solid tubulocystic, and cholangioblastic cholangiocarcinoma. Although these tumors are considered to represent histologic variants, they lack classic features of cholangiocarcinoma and have unique characteristics, namely immunoreactivity for inhibin and NIPBL::NACC1 fusions. The purpose of this study is to present clinicopathologic and molecular data for a large series of these tumors to better understand their pathogenesis. We identified 11 hepatic tumors with these features. Immunohistochemical and NACC1 and NIPBL fluorescence in situ hybridization assays were performed on all cases. Four cases had available material for whole-genome sequencing (WGS) analysis. Most patients were adult women (mean age: 42 y) who presented with abdominal pain and large hepatic masses (mean size: 14 cm). Ten patients had no known liver disease. Of the patients with follow-up information, 3/9 (33%) pursued aggressive behavior. All tumors were composed of bland cuboidal cells with follicular and solid/trabecular growth patterns in various combinations, were immunoreactive for inhibin, showed albumin mRNA by in situ hybridization, and harbored the NIPBL::NACC1 fusion by fluorescence in situ hybridization. WGS corroborated the presence of the fusion in all 4 tested cases, high tumor mutational burden in 2 cases, and over 30 structural variants per case in 3 sequenced tumors. The cases lacked mutations typical of conventional intrahepatic cholangiocarcinoma. In this report, we describe the largest series of primary inhibin-positive hepatic neoplasms harboring a NIPBL::NACC1 fusion and the first WGS analysis of these tumors. We propose to name this neoplasm NIPBL:NACC1 fusion hepatic carcinoma