Perspectives on Low-grade Sarcomas: The Extraordinary Contributions of Sharon W. Weiss, MD.

Soft-tissue pathology encompasses a wide spectrum of neoplasms that represent some of the most challenging and problematic tumors in surgical pathology. Owing to the intensive work of dedicated pathologists, this once esoteric field has become increasingly well defined. In this review, Dr Sharon Weiss' monumental contributions to low-grade sarcomas, including low-grade fibromyxoid sarcoma/so-called hyalinizing spindle cell tumor, atypical lipomatous tumor/well-differentiated liposarcoma and dedifferentiated liposarcoma, epithelioid hemangioendothelioma, and dermatofibrosarcoma protuberans with fibrosarcomatous transformation will be discussed.

A rare case of a non-neural granular cell tumor presenting as an enlarging right chest wall nodule in a 4-year-old male and review of the literature.

Non-neural granular cell tumor was first described in 1991 as an unusual primitive, polypoid variant of the conventional granular cell tumor. To date, this neoplasm remains a rare entity and the cell of origin is uncertain. While the histological features are similar to the conventional granular cell tumor, it represents a distinct entity that is negative for S100 and lacks true nerve sheath differentiation. Here, we describe a case of a 4-year-old male who presented with a painless, soft nodule on his right chest wall that was slowly increasing in size. The mass was excised and sent for pathologic analysis. Microscopic examination reveals spindle and epithelioid cells with vesicular nuclei and prominent granular eosinophilic cytoplasm. Immunohistochemical analysis shows negative staining for S100 and AE1/AE3/PCK26 but is positive for CD68. A diagnosis of a non-neural granular cell tumor was made. We report a rare and diagnostically challenging case in a pediatric patient.

Neuroendocrine Neoplasia in Extranodal Marginal Zone Lymphoma of Mucosa-Associated Lymphoid Tissue (MALT Lymphoma) of the Lung: A Case Report and Immunohistochemistry Analysis of Eight Pulmonary MALT Lymphomas.

Carcinoid tumorlets are peribronchiolar proliferations of neuroendocrine cells often associated with lung scars. Extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma) is a non-Hodgkin's lymphoma that frequently involves the gastrointestinal tract but less commonly is described in the lung. Simultaneous occurrence of neuroendocrine neoplasms and MALT lymphoma is extraordinarily rare and has predominately been reported in the gastrointestinal tract. In this article, we describe the case of a 73-year-old female with coexisting pulmonary MALT lymphoma and carcinoid tumorlets of the right middle lobe. Retrospective series of 8 pulmonary MALT lymphomas are evaluated for neuroendocrine neoplasia by immunohistochemistry. No correlation between MALT lymphoma and neuroendocrine neoplasia was identified in this case series. While the concurrence of these distinctive neoplasms is most likely coincidental, the presence of a common risk factor, or one neoplasm as a risk factor for the other, deserves study of a larger group of pulmonary MALT lymphomas.