Sinonasal hamartomas are uncommon lesions of nasal and sinus cavities. Based on indigenous cellular components and characteristic histologic features, they are further classified into four entities: respiratory epithelial adenomatoid hamartoma (REAH), seromucinous hamartoma (SH), chondro-osseous and respiratory epithelial hamartoma (CORE), and nasal chondromesenchymal hamartoma (NCH). REAH, SH, and CORE are seen in adult patients, while NCH predominantly occurs in newborns and infants. Morphologically REAH and SH are composed of respiratory epithelium and seromucinous glands, CORE is related to REAH but with additional feature of chondroid and/or osseous tissue, and NCH is composed of chondroid and stromal elements but devoid of epithelial component. All four lesions can present as sinonasal mass lesions and with associated obstructive symptoms. Given the rarity of these lesions, diagnosis can be challenging, especially in unusual clinical scenario. In this study, we report six cases of sinonasal hamartoma, including one case of NCH, one case of CORE, two cases of SH, and two cases of REAH. All cases were from adult patients including four men and two women. We also review the literature of the clinical and pathologic features of these rare lesions.
BCOR-CCNB3 sarcoma (BCS) is a rare recently defined undifferentiated sarcoma that predominantly affects children and young adults. The diagnosis of this tumor is difficult due to the highly variable morphology and nonspecific immunophenotype. Emerging data suggest that patients with BCS show response to Ewing sarcoma-based treatment regimen, thus correct diagnosis is of clinical relevance. In this study, we report a case of BCS arising from the big toe of a 15-year-old male patient. The tumor had a prominent population of rhabdoid cells with bright eosinophilic cytoplasm mimicking rhabdomyosarcoma. The tumor cells were focally positive for desmin and myogenin, and negative for CD99. Next-generation sequencing showed the presence of BCOR-CCNB3 gene fusion. BCS with prominent rhabdoid cells has not been described before. This study further expands the morphologic spectrum of BCS.
Papillary carcinoma in the male breast is uncommon. Here, we report a case of a large encapsulated papillary carcinoma (EPC) in a 62-year-old male. The patient presented with a left breast mass of 1-year duration and bloody nipple discharge for several days. Mammography and breast ultrasonography showed a large left breast mass. The initial biopsy demonstrated fat necrosis with acute and chronic inflammation only. Due to clinical suspicion, a repeat biopsy was performed and revealed scant fragments of papillary carcinoma in a background of inflammation. The patient underwent left total mastectomy. Grossly, the breast contained a 9.0 cm entirely cystic lesion lined by a hemorrhagic thick fibrotic wall. No solid area was identified in the cyst. The entire cyst wall was examined under microscopy; only a few sections with papillary carcinoma were identified. The lesion was confined to the cyst wall; so, a diagnosis of EPC was made. Compared to the previously reported EPC cases of male breast, the lesion of this case was unusually cystic, which making the diagnosis challenging. Therefore, awareness of this unusual feature, repeat biopsy when the pathology result is discordant, and extensive sampling of the lesion are essential for making the correct diagnosis and guiding patient management.
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