ABSTRACT
Ovarian sex cord stromal tumor with annular tubules (SCTAT) is a distinctive, rare subtype of sex cord stromal tumor of the ovary, predominant component of which has morphological features intermediate between that of granulosa cell and sertoli cell. The majority of ovarian SCTAT are benign. So far, malignant behavior in SCTAT has been reported only in sporadic cases. We have presented a case of SCTAT in a 40 year old lady with no association of Peutz-Jegher (P-J) syndrome. The patient’s chief complaints were post-menopausal bleeding for 1 year on and off along with menorrhagia. MRI abdomen was suggestive of intensely enhancing solid tissue mass lesion in the right adnexa, features suggestive of ovarian mass. Panhysterectomy was done. Grossly uterus and left adnexa appeared to be normal. Right ovary showed mass measuring 17x11x9cm3 in size, on cut section, solid, homogenous lobulated, yellowish areas identified. Microscopic and Immunohistochemistry findings confirmed the diagnosis of sex cord stromal tumor with annular tubules of granulosa cell type. PAS stain supported the diagnosis.
ABSTRACT
Sex cord tumors with annular tubules are known to originate from the sex cord of embryonic gonads that synthesize Sertoli cells, Leydig cells, granulosa cells, and theca cells of the ovarian stroma, while ovarian small cell carcinoma of the hypercalcemic type is a type of neuroendocrine tumor. Both these tumors are uncommon, potentially malignant neoplasms in children. We report the case of a sex cord tumor with annular tubules in an 11-year-old girl and a case of small cell carcinoma of the hypercalcemic type in a 10-year-old girl. We also discuss the prognosis and management of these tumors.
Subject(s)
Child , Female , Humans , Male , Carcinoma, Small Cell , Gonads , Granulosa Cells , Hypercalcemia , Leydig Cells , Neuroendocrine Tumors , Ovary , Prognosis , Sertoli Cells , Theca CellsABSTRACT
Sex cord tumor with annular tubules (SCTAT) is a rare ovarian neoplasm which usually occurs in two forms. In patients associated with Peutz-Jeghers syndrome, the tumors are usually small, bilateral or multifocal, and show benign clinical course. However, tumors from patients without the syndrome are often large, usually unilateral, and rarely show malignant behavior. Serous surface papillary carcinoma (SSPC) is an aggressive neoplasm which involves peritoneal linings, including ovarian surface. Recently, we encountered a case of an unusual combination of SCTAT and SSPC in the ovary of a 55-year-old Korean woman presented with abdominal distention for one year. Systemic review and physical examination were within normal limit, except for abdominal discomfort and distention. There was no stigmata of Peutz-Jeghers syndrome in all diagnostic examinations, including gastroscopy and colonoscopy. Pelvic computed tomography showed adnexal mass with multiple peritoneal nodules. Exploration revealed uterine and ovarian surfaces covered with multiple, yellow-white papillary nodules. However, the sizes of both ovaries were within normal limit. Typical serous papillary carcinomas were identified in nodules from peritoneum and ovarian surfaces. Well-circumscribed columnar epithelial cell nests composed of ring-shaped tubules encircling hyalinized basement membrane-like materials were found in the ovary away from serous surface papillary carcinoma.
Subject(s)
Female , Humans , Middle Aged , Carcinoma, Papillary , Christianity , Colonoscopy , Epithelial Cells , Gastroscopy , Hyalin , Ovarian Neoplasms , Ovary , Peritoneum , Peutz-Jeghers Syndrome , Physical ExaminationABSTRACT
The sex cord tumor with annular tubules(SCTAT) is a rare ovarian neoplasm, which charateristically shows simple and complex annular tubules with central acidophilic hyaline bodies. This tumor has been considered as a tumor of low-grade malignancy with late recurrence. We presented a brief case report of metastatic SCTAT of ovary in pleural fluid from ovary with cytopathologic and clinical features. The cytologic features of differential diagnosis are discussed.
Subject(s)
Female , Diagnosis, Differential , Hyalin , Ovarian Neoplasms , Ovary , Pleural Effusion , RecurrenceABSTRACT
An ovarian sex cord tumor with annular tubules(SCTAT) in an 18-year-old woman who had secondary amenorrhea but not Peutz-Jeghers syndrome was studied by light and electron microscopy. Her clinical features were consistent with those of Turner's syndrome, i.e, shortness of stature, webbing of the neck, scanty or absent axillary and pubic hairs, lower hair line with streak gonad. Chromosomal analysis revealed 45, X/46, XX karyotype. The patient has been well and disease free for a period of 2 years after surgical removal of tumor. Histologically, the tumor was composed of nests of cells arranged in simple and complex annular tubules with central acidophilic hyaline bodies. Ultrastructurally, the cells had deeply indented nuclei and the cells were joined by specialized intercellular junctions along their lateral adjacent borders. Central acidophilic hyaline body consisted of concentric lamellae of basal lamina, but Charcot-Boettcher filaments were not observed.
Subject(s)
Female , HumansABSTRACT
The sex cord tumor with annular tubules (SCTAT) is a distinctive ovarian neoplasm of which predominant component has intermediate morphologic features between the granulosa cell tumor and the Sertoli cell tumor; focal differentiation into either granulosa cell or Sertoli cell tumor may occur. We presented a 24-year-old woman with SCTAT metastatic to the mesentery root. The origin of the primary was the left ovary, and the tumor was diagnosed nine years ago. This report dealt with clinical presentation and prognosis.