Paragangliomas are rare neuroendocrine tumors that infrequently localize to the porta hepatis, where they create significant diagnostic challenges by mimicking hepatocellular carcinoma or distinct mesenchymal neoplasms. We report the case of a 64‐year‐old woman presenting with vague abdominal pain and an enhancing porta hepatis mass compressing the inferior vena cava. An initial core needle biopsy, which suggested a benign mesenchymal tumor, underscores the potential for misdiagnosis in such atypical presentations. During surgery, the patient developed paroxysmal hypertension suggestive of occult catecholamine release. Subsequent histopathological examination revealed a classic zellballen pattern, with immunohistochemical positivity for Chromogranin A, Synaptophysin, and S100 definitively confirming a paraganglioma. The patient remains disease‐free at a 2‐year follow‐up. This case highlights the importance of considering paraganglioma in the differential diagnosis of porta hepatis masses and emphasizes the value of histopathology and immunohistochemistry for accurate diagnosis, particularly in patients who lack preoperative adrenergic symptoms.
Epithelioid hemangioendothelioma (EHE) is a rare vascular tumor with variable presentation and malignancy. These highly uncommon tumors may arise from the liver, soft tissues, bones, and, more infrequently, from the lungs. Given its rarity, treatment approach is not unanimous, with ongoing debate regarding the relative benefits of surgical resection and radiation therapy. In support of growing efforts to standardize treatment of these tumors, we present our findings of a rare pulmonary EHE, incidentally detected in the left lung of a 65-year-old, nonsmoking female patient with a history of diabetes, hypertension, and renal cell carcinoma (RCC) in remission for 8 years after surgical resection. A surgical route was recommended, and left robot-assisted thoracoscopic wedge resections were performed. Final pathological assessment confirmed two pulmonary EHE tumors, one resected from the left upper lobe (LUL) and another from the left lower lobe (LLL) of the lung. Her postoperative course was uneventful, and at 13 months postresection, there has been no evidence of recurrence.
A case of calcifying pseudoneoplasm of the neuraxis (CAPNON) that arose as an intradural mass of the thoracic spinal cord of a 53-year-old man, a motorcycle mechanic, is reported. The mass showed regrowth after the subtotal removal and was resected again 8 years later. Both primary and regrowth lesions consisted of calcified round nodules of the myxochondroid matrix rimmed by polygonal or short spindle cells. Fibrous tissue showing chronic inflammatory cell infiltration intervened between the nodules. Lamellar or woven bone tissue was also observed. In the regrowth lesion, the myxochondroid matrix was reduced in amount and partly replaced by fibrous tissue. Cells rimming the myxochondroid matrix were immunoreactive to epithelial membrane antigen and somatostatin receptor Type 2A, supporting the meningothelial origin. The pathogenesis of CAPNON remains obscure, but it is a nonneoplastic, probably reactive disorder to some unknown causes, and its close histopathological resemblance to tumoral calcinosis of somatic soft tissue suggests that both disorders share a common pathogenesis. Meningothelial cells surrounding myxochondroid nodules in the present lesions probably represent reactive meningothelial hyperplasia and reflect the locoregional specificity of the lesions. In the present case, some chronological changes of histopathological findings could be observed by comparing the specimens of two operations. Occupational mechanical overload to the vertebral column for a long term might have contributed to the pathogenesis of CAPNON.
Plasmablastic lymphoma is a rare, aggressive large B cell lymphoma characterized by plasmablastic morphology, terminal B cell (plasma cell) differentiation, frequent loss of conventional B cell markers, and poor clinical outcomes. Although classically associated with immunodeficiency and Epstein–Barr virus (EBV) infection, plasmablastic lymphoma has also been described as an uncommon mechanism of relapse after CD19-directed chimeric antigen receptor T cell (CAR-T) therapy. We report a 69-year-old man with a remote history of chronic lymphocytic leukemia/small lymphocytic lymphoma and subsequent refractory diffuse large B cell lymphoma (DLBCL) who developed rapidly progressive, CD19-negative plasmablastic lymphoma approximately 1 month after CD19-directed CAR-T therapy. Although DLBCL was of a nongerminal-center phenotype with expression of pan-B markers including CD19 and lack of CD138, the post-CAR-T transformed lymphoma demonstrated acquisition of plasmablastic morphology and CD138 expression, loss of pan-B markers and negative for EBV by in situ hybridization and HHV8 by immunostaining. Fluorescence in situ hybridization (FISH) on the plasmablastic lymphoma showed a MYC::IGH fusion and low-level MYC copy gain that were absent in the antecedent DLBCL. Disease dissemination was later confirmed on left pleural fluid cytology a few days before the patient passed away. This case highlights EBV and HHV8 negative plasmablastic transformation as a rare but important post-CAR-T relapse pattern and underscores the need for repeat biopsy, comprehensive paired immunophenotyping, and molecular studies in patients with atypical progression after cellular therapy.
Danon disease is a rare, X-linked dominant inherited lysosomal storage disorder that involves a mutation in the lysosomal-associated membrane Protein 2 gene. Mutation in this gene results in severe disease clinical manifestations that may include skeletal and cardiac myopathies, retinopathies, and intellectual disabilities. This case report explores postmortem findings observed in a 25-year-old male donor who succumbed to heart failure secondary to Danon disease. With no transplant history, postmortem findings include hypertrophy, extensive fibrosis, and vacuolization in cardiomyocytes compared with expected anatomical findings in an unaffected patient. This case report is aimed at deepening the understanding of the clinical manifestations of Danon disease, raise awareness, and explore diagnostic approaches and differentials to help identify additional treatment options that may improve patient outcomes.
