Large cell calcifying Sertoli cell tumors (LCCSCTs) are extremely rare, with less than 100 tumors being described to date. Most of the tumors are benign with a few malignant cases, and aggressive behavior is infrequent. These tumors are a type of Sertoli cell tumor, and these tumors comprise less than 0.3% of all testis tumors in Saudi Arabia. They usually occur in boys and young adults and can affect one or both testicles in multifocal form causing microcalcifications. A 28-year-old male visited our hospital with left testis pain. Physical examination of the scrotum revealed that both testicles were normal sized with no palpable mass. Ultrasonography evaluation revealed grade 3 left varicocele and an incidental 9 mm calcified mass in the right testicle, which was further confirmed by MRI. Partial orchiectomy was performed. Clinical data, radiological studies, and morphological and immunohistochemical characteristics were analyzed.
Background
Sinonasal renal cell-like adenocarcinoma (SNRCLA) is a rare entity that was introduced to head and neck tumors classification on 2017 as an emerging entity. Clinically, it could be asymptomatic or show non-specific symptoms like epistaxis, nasal obstruction, or hyposmia. Diagnosis of the lesion is challenging, especially in small biopsies, and requires utilization of clinical, radiological, histopathological and ancillary tests for accurate characterization of the lesion. There is around 20 cases reported in the literature with 3 cases associated with VHL syndrome.
Case Presentation
A 41 years old female with a nasal mass for two years ago that presented initially as frequent epistaxis from the right side. Histopathological examination revealed proliferation of clear cells associated with hemorrhagic background forming follicular and glandular structure and dense eosinophilic secretion. Tumor cells were diffusely positive for CK7, EMA, and Inhibin while were negative for CK20, P63, CK 5/6, CD10, RCC, TTF1, PAX8, CEA, and GATA3. Proliferation index (KI67) was less than 5% (Figure.3). The diagnosis was consistent with sinonasal renal cell-like adenocarcinoma. The patient has no recurrence and no symptoms after 1 year.
Conclusion:
Our study report a rare case of SNRCLA with discussion of the histological features and its association with VHL syndrome.
Sinonasal renal cell-like adenocarcinoma (SNRCLA) is a rare malignant sinonasal tumor with relatively indolent clinical course. Clinically, it could be asymptomatic or show non-specific symptoms such as epistaxis, nasal obstruction, or hyposmia. Diagnosis of the lesion is challenging, especially in small biopsies, and requires clinical, radiological, histopathological, and ancillary tests to characterize the lesion accurately. We herein report a case of a 41-year-old female with a nasal mass noted two years ago, which presented initially as frequent epistaxis from the right side. Histopathological examination revealed proliferation of clear cells associated with hemorrhagic background forming follicular and glandular structure and dense eosinophilic secretion. Tumor cells were diffusely positive for CK7, EMA, and inhibin, while they were negative for CK20, P63, CK 5/6, CD10, renal cell carcinoma (RCC), TTF1, PAX8, CEA, and GATA3. The proliferation index (KI67) was less than 5%. The diagnosis was consistent with SNRCLA. The patient has no recurrence and no symptoms after one year. Thus, our study reports a rare case of SNRCLA with a discussion of the histological features and its association with von Hippel Lindau syndrome.
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