Cases reported "Leydig Cell Tumor"

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1/6. A testicular tumor resembling the sex cord with annular tubules in a case of the androgen insensitivity syndrome.

    sex cord tumor with annular tubules (SCTAT) is a distinctive neoplasm with indifferent cells of sex cord derivation in a characteristic arrangement of ring-like tubules. Much attention has been drawn to its association with the peutz-jeghers syndrome (PJS) with reported occurrence of the tumor in the testis of a boy with PJS. The authors present two cases of the androgen insensitivity syndrome (AIS), one of the cases being distinctive in having a large multicystic tumor resembling the SCTAT in the immature gonad. Additionally, the focal areas of the tumor, the large sertoli cells lining the tubules, resembled those of a large cell calcifying sertoli cell tumor (LCCSCT) although no calcific areas were discernible. Although the occurrence of neoplasms like germinomas and tubular adenomas is well known in the AIS, SCTAT has hitherto not been reported in a gonad of the AIS. SCTAT has been placed under an "unclassified sex cord-stromal" category in the world health organization (WHO) classification, yet, opinions are divided as to its origin from a granulosa or Sertoli cell, although an overlap in the histologic features of the two cell categories is to be anticipated in view of their homologous nature. In the case presented, the close resemblance of the tumor cells to the sertoli cells of the uninvolved gonad would further support the concept of a Sertoli line of differentiation of the SCTAT.
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2/6. Ovarian stromal tumors with minor sex cord elements: a report of seven cases.

    Seven ovarian tumors that were predominantly stromal, but contained, in addition, a minor component of sex cord elements, were encountered in patients ranging from 16 to 65 years of age. The tumors varied from 1 to 10 cm in diameter and resembled grossly fibromas or thecomas. On microscopical examination they were predominantly fibromatous, but also contained small nests or tubules composed of cells resembling granulosa cells, sertoli cells, or indifferent cells of sex cord type. Two tumors also had cells of steroid-hormone-cell type; these cells contained crystalloids of Reinke in one case. These two tumors were classified as luteinized thecoma and stromal-leydig cell tumor with minor sex cord elements. The other five tumors were designated fibromas with minor sex cord elements. Five-year follow-up, available in three cases, revealed no evidence of recurrence after operative removal. Although the presence of sex cord components has generally resulted in the classification of an ovarian tumor in either the granulosa cell or Sertoli-Leydig cell category, we propose that tumors with only minor sex cord components be placed in a separate category.
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3/6. Ovarian Sertoli-Leydig cell tumors with a retiform pattern: a problem in histopathologic diagnosis. A report of 25 cases.

    A review of 232 cases of ovarian Sertoli-Leydig cell tumors yielded 25 that contained a prominent component with a retiform pattern. A lack of awareness of this pattern, which has not been described adequately in the literature, resulted in frequent initial misinterpretation of these tumors as neoplasms of other types, including endodermal sinus tumor (seven cases) and serous adenocarcinoma (four cases). The patients ranged in age from 2 to 39 years. Three of them (12%) presented because of androgenic manifestations; most of the remainder complained of abdominal swelling or pain, but one of them was amenorrheic and hirsute and three had amenorrhea, suggesting androgen secretion by the tumor. All the tumors were Stage I; one was bilateral. Their average maximum dimension was 16 cm and they were typically cystic or both cystic and solid. In eight tumors the cysts were lined by papillae, which were edematous in five cases. On microscopic examination the retiform pattern, which predominated in 14 of the 25 tumors, was characterized by an irregular network of elongated, often slit-like tubules and cysts, which often contained papillae and resembled the rete testis. Follow-up of 21 patients from 6 months to 17 years (average 4 years) revealed that 16 of them were alive and free of disease; five had died of tumor from 6 months to 17 years postoperatively.
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4/6. Sertoli cell and sertoli-Leydig cell tumors of the ovary. A report of three cases with ultrastructural findings.

    Three tumors of the ovary containing sertoli cells were studied by light and electron microscopy. Two of these tumors were well-differentiated neoplasms with epithelial cells often forming tubules. These cells were cylindrically shaped, contained round to oval nuclei and stood on a thin basement membrane. The cytoplasm was fibrillary and showed rough and smooth endoplasmic reticulum, lipid droplets and secretory granules. At the luminal borders the cells were often irregular and displayed apocrine-like activity. Having compared our data with results of studies from the literature of normal sertoli cells, Sertoli cell adenomas of the testis and cells from other parts of the male reproductive system and those of normal ovarian stroma, we conclude that the Sertoli cell is most probably the cell of origin of these tumors. The third tumor was undifferentiated with a sarcomatoid appearance and contained islands of cartilage, which we consider to be metaplastic.
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5/6. Ovarian endometrioid tumors mimicking Sertoli and Sertoli-Leydig cell tumors: Sertoliform variant of endometrioid carcinoma.

    We have encountered four cases of an unusual variant of well-differentiated endometrioid carcinoma that was predominantly composed of tubules, solid or hollow, as well as cord-like areas histologically mimicking Sertoli and Sertoli-Leydig cell tumors. The two features most helpful in differential diagnosis were the presence of areas of tumor with the typical confluent pattern of endometrioid carcinoma, and the presence of mucin at the apical borders of the tumor cells and/or within glandular lumina. Other features that were helpful if present, but were observed only in one case each, were foci of squamous metaplasia or the presence of ciliated epithelium. In two cases, ultrastructural studies showed well developed microvilli and perinuclear microfilaments confirming the endometrioid nature of the neoplasm. The patients varied from 22-74 years in age. All tumors were confined to a single ovary, and no tumor is known to have recurred or metastasized. One of the patients died at age 80, six years following operation, presumably without evidence of recurrent neoplasm or metastases. Two other patients are living and well, one and 14 years after diagnosis. In one patient follow-up is short. The clinicopathologic features of this variant of endometrioid carcinoma are reviewed with emphasis on differential morphologic features.
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6/6. Somatic and germinal cells' interrelationship in the course of seminiferous tubule maturation in man.

    Certain successive phases of seminiferous tubule maturation were observed in a transsection of a Leydig cell adenoma-bearing testis of a boy with precocious puberty. Massively accumulated leydig cells may stimulate the maturation of sertoli cells, as indicated by progressive replacement of Sertoli cell precursors by mature sertoli cells at a distance closer to the adenoma. On the other hand, tubules less advanced in maturation contained a higher number of somatic cells than those more advanced in maturation. Leydig-cell-dependent maturation of sertoli cells may be in competition with Certoli cell multiplication, or numerous undifferentiated somatic cells may undergo a natural elimination in the course of tubular maturation. An inverse relation between the number of Sertoli cell precursors and the number of meiotic spermatocytes suggests that quantitative reduction of Sertoli cell precursors may be important for the intratubular milieu necessary for the onset of the first meiosis in man.
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