Faculty Bibliography

Background: Clear cell carcinoma of ovary (CCCO) accounts for 5-25% of ovarian carcinomas. Considered a high-grade malignancy by default, the role of histological grading for assessing clinical outcome is not established in CCCO. We aimed to evaluate histopathological features predictive of clinical outcome in patients with CCCO.
Design(s): Pathology database was searched after IRB approval. A total of 69 cases of CCCO were studied. Slides from primary tumor resection were reviewed blinded to outcome. The original diagnosis of CCCO was confirmed and the following histopathologic features were recorded: percentage of solid component, degree of nuclear atypia, mitotic activity, intratumoral inflammation, presence of small tumor nests (intratumoral single cells or clusters of <5 cells in non-hyalinized stroma, Figure 1A-B), tumor budding (peritumoral single cells or clusters of <5 cells), lymph node involvement and endometriosis. Information regarding age at diagnosis, clinical stage, treatment and followup was obtained from medical charts. Kaplan-Meier survival curves with log rank test, Fisher's exact test and Mann-Whitney test were used for statistical analysis.
Result(s): Median patient age was 52 years (range 26-75 years). Forty-nine (71%) tumors were associated with endometriosis and 37 (53%) presented at stage I. Ten (15%) patients died of disease, 14 (20%) were alive with active disease and 45 (65%) had no evidence of disease at last follow-up (median follow-up: 34.2 months, range 1.2 - 230.6). Advanced stage, positive lymph nodes and presence of small tumor nests were significantly associated with shorter overall survival (p=0.006, p<0.001, p=0.004, respectively; Figure 2A-C) and recurrence/progression despite treatment (p<0.0001, p=0.0011, p=0.0003, respectively; table 1). Also, within the cohort of low stage patients (stage I and II), presence of small tumor nests was associated with recurrence/disease progression (p=0.0014; table 1). None of the other studied features reached statistical significance for assessment of prognosis.
Conclusion(s): Besides the classic prognostic factors of stage and lymph node status, presence of small tumor nests seems to be associated with poorer outcome in patients with CCCO. Specifically, in patients with early stage disease, evaluation of small tumor nests may help to better determine prognosis. These findings should be further evaluated in larger studies

Fine-needle aspiration biopsies (FNABs) are a common modality used in the evaluation of salivary gland neoplasms. We present the cytologic and histologic features of a rare case of lymphoepithelioma-like carcinoma (LELC) in a 40-year-old Hispanic male with a 1.0-cm painless well-circumscribed parotid mass that had been present for 8 years. FNAB smears showed cohesive groups of intermediate-sized basaloid cells with vesicular nuclei, occasional pleomorphic nuclei and prominent nucleoli, and spindled morphology. Mature lymphocytes were seen in the background, either adjacent to the atypical epithelial cells or dispersed in the background. This lymphoid background raises considerations of salivary gland neoplasms that can have prominent lymphocytic backgrounds, such as acinic cell carcinoma and, more commonly, Warthin's tumor or metastasis involving intraparotid lymph node. Surgical resection of the parotid showed syncytial sheets of predominantly undifferentiated cells with spindled to epithelioid morphology and occasional prominent nucleoli and focal areas of squamous differentiation. The background showed dense areas of lymphocytes with germinal center formation. Immunohistochemical (IHC) stains showed positive reactivity for p63, p40, and EBV in situ hybridization (EBV ISH) in the tumor cells and negative reactivity for p16. The findings were supportive of LELC if a metastasis from the nasopharynx was excluded. A subsequent nasopharyngeal biopsy was benign. Although histologic features of LELC are well established, we identified rare case reports describing the cytomorphology in the literature. Cytopathologists should be aware of this lesion as another salivary gland neoplasm that can show lymphocytes admixed with the tumor cells and a distinct lymphoid background. The basaloid appearance and cytologic atypia should distinguish it from acinic cell carcinoma and Warthin's tumor. However, metastatic lesions should also be considered with a distinct lymphoid background and need to be clinically excluded before establishing the diagnosis of LELC

Microcystic/reticular schwannoma is a recently described variant of schwannoma with a predilection for the gastrointestinal tract, rarely involving the head/neck region. This is the first reported case involving the submandibular gland. We present a case in a 34 year old man with 4.5 cm submandibular mass. Fine needle aspiration suggested a spindle cell lesion. Frozen section evaluation raised the possibility of mucoepidermoid carcinoma. Resection showed a well circumscribed mass with a mucoid appearance. Histologic findings include a lobular architecture with fibrous septa, a lympho-plasmacytic infiltrate, and scattered lymphoid aggregates at the periphery. There are two distinct histologic patterns with solid areas of spindle cells and areas of spindle/ovoid cells with a microcystic pattern in a myxoid background. The tumor has a pushing border, with extension into adipose and adjacent parenchyma, without cytologic atypia or necrosis. Immunohistochemical stains are positive for S-100 and CD34, and negative for calponin, mammoglobin, ALK1, p63, ER, GFAP, SMA, desmin, cytokeratin 7, cytokeratin AE1/AE3, and C-Kit. Mucicarmine stain is negative. Recognition of this benign unusual variant of schwannoma is paramount for appropriate conservative treatment due to the morphologic and immunohistochemical overlap with primary salivary gland carcinomas.

Undifferentiated pleomorphic sarcoma (UPS), previously known as malignant fibrous histiocytoma, is a neoplasm that occurs most often in the extremities, trunk, and retroperitoneum. Rarely, UPS can occur in the cardiac chambers and great vessels. The diagnosis of UPS is difficult to establish with noninvasive imaging techniques, and these tumors may be mistaken for benign neoplasms preoperatively. Surgical excision is the standard therapy, although the extent and location of the tumor may limit the ability to perform a complete resection. Adjuvant chemotherapy and/or radiation are often used for incomplete resections. We report the case of a 57-year-old woman with a large right ventricular UPS who presented with signs of right-sided heart failure. Preoperative imaging was suggestive of a myxoma; however, histopathologic evaluation of the specimen confirmed a diagnosis of UPS. Microscopic margins of the specimen were positive, and adjuvant chemotherapy was given. We discuss diagnostic and treatment considerations for this unusual cardiac tumor. <Learning objective: The diagnosis and treatment of primary cardiac tumors are challenging. Noninvasive imaging often lacks the sensitivity and specificity to differentiate between benign and malignant neoplasms. In addition, the extent and location of tumor involvement may limit the ability to perform a complete resection. The diagnosis and treatment of right ventricular undifferentiated pleomorphic sarcoma are discussed.>.