Extrapulmonary small cell carcinomas (ESCCs) are poorly differentiated neuroendocrine tumors that are characterized by an aggressive course and poor survival rates. While these tumors can be found anywhere in the body, presentations of lesions in the orbit are exceedingly rare. We present the case of a 47-year-old man who presented with blurry vision, lacrimation, and tenderness of his right eye, as well as a small but palpable temporal mass. Upon workup, he was diagnosed with ESCC in the orbit as well as lesions in the liver and spine. He received systemic chemotherapy but unfortunately proceeded to have rapid spread of his disease and succumbed to this cancer only a year after presentation. This patient illustrates the importance of developing optimal treatment strategies, which have yet to be delineated, and especially the impact of newer immunotherapy agents remains to be seen.
Well-differentiated papillary mesothelioma is an uncommon benign, although potentially recurrent mesothelial lesion that occurs in the peritoneum of women over a wide age range. We recently encountered an incidental case sent for frozen section at the time of hysterectomy for endometrial carcinoma. Familiarity with this lesion will help prevent overinterpretation as a serous carcinoma.
Primary cardiac tumors are rare entity, and the most common benign cardiac neoplasms include myxomas, lipomas and papillary fibroelastomas (PFE) with PFE accounting for 8% of the tumors. A 53-year-old female presented with chronic chest pain and exertional shortness of breath. She first sought evaluation four years ago, when her pulmonologist referred her to thoracic surgery after an incidental finding of a 3.9 cm anterior mediastinal mass on CT-scan (Figure 1A-B). The patient described the pain as a constant pressure and squeezing sensation in her chest and worsening shortness of breath while laying on her sides, relieved only when a pillow was propped up under her chest. The pain did not significantly reduce her mobility or capacity to work. Initial evaluation, including transthoracic echocardiogram, stress testing, carotid ultrasound, and pulmonary function testing - was performed and found to be unremarkable. Further evaluations revealed a hiatal hernia, leading her symptoms to be attributed to GERD as causing her persistent non-exertional chest pain. The patient remained in good health despite her symptoms persisting. She later re-sought attention for chest pain on exertion and hoarseness three years later. She again underwent a full cardiopulmonary workup, yielding benign findings on EKG, troponins, and echocardiogram. Due to no alarming symptoms at the time, she was subsequently discharged. The patient continued to endorse chest pain and a follow-up MRI revealed a now 5 cm anterior mediastinal mass, thought to be a thymic cyst. Over the next few months, the patient continued to endorse worsening chest pain and exertional shortness of breath. She followed up with her cardiologist and a subsequent TEE was significant for a pulmonic valvular mass. She was admitted to the hospital for further investigation. During her hospital stay, the patient underwent surgery to resect the anterior mediastinal mass likely to be a thymic cyst and the pulmonary artery mass which was determined to be a pulmonary valve papillary fibroelastoma in the histopathology exam (Figure 2). The surgery was successful with no complications postoperatively. The patient was discharged with no further chest pain and with improvement of the exertional shortness of breath.
Introduction/Objective Epithelioid sarcoma (ES) is a rare, malignant mesenchymal neoplasm that has a known tendency for local recurrence, regional lymph node involvement, and distant metastases. Two histologic variants have been recognized: classic ES also known as the distal type, and proximal-type ES (PES). The classic ES is common in young adults. It occurs more frequently in the distal upper extremities followed by the distal lower limbs and has a male prevalence of 2:1. Conversely, PES commonly involves deep tissues in the pelvic region, including the genital area. It tends to occur in older patients and follows a more aggressive clinical course. In the female genital tract, PES occurs most frequently in the vulva. The incidence of primary sarcoma of the vulva accounts for 1.5-5% of all malignant tumors, making PES a very rare incidence. Methods/Case Report Here we report a 60-year-old female diagnosed with vulvar epithelioid sarcoma treated with a right radical vulvectomy and bilateral inguinal lymph node dissection in 2008. In 2017, further surgery and adjuvant radiation were given for local recurrence. In 2020, the patient developed left hip pain and was found to have an expansile lytic lesion in the left proximal femur. Extensive resection was performed. Grossly the vulvar lesion was nodular with diffuse hemorrhage, degeneration, and necrosis. Microscopically, the tumor cells had large vesicular nuclei, prominent nucleoli, and abundant eosinophilic cytoplasm. Histologically, the morphology of the tumor cells are similar for the primary vulvar specimen and the bone metastases. Immunohistochemically, the tumor cells are positive for vimentin, GATA, FLI-1, SMA, SMHC, partially positive for CAM5.2, AE1/AE3, CD31, and CD163. Immunohistochemistry was negative for CDX2, CD56, S-100, TTF-1, CK5/6, CK20, P40, mammoglobin, MOC31, ER, CK7, CK903, HMB45, PAX8. A Ki-67 proliferative index was around 30-40%. NGS molecular testing detected a SMARCB1 mutation with loss of exons 1-3 and exons 7-9 supporting the diagnosis of epithelioid sarcoma. Results (if a Case Study enter NA) NA Conclusion In summary, we report a case of PES of the vulva in a 60 year old female. Grossly, the lesion was nodular with histology showing large vesicular nuclei, prominent nucleoli, and abundant eosinophilic cytoplasm. It showed loss of INI1/SMARCB1 nuclear expression. The patient is receiving further adjuvant treatment and shows no new metastases.
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