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Pathologic quiz case: a papillary ovarian tumor in a 4-year-old girl. (Residents' Pages).

A 4-year-old girl presented with a large abdominal mass that had been noticed approximately 1 month previously by her mother. Other than asthma, the child's previous medical history was unremarkable. Physical examination revealed a large, freely moveable abdominal mass. The abdomen was not tender, and the girl had no signs of endocrine abnormalities. A computed tomographic scan confirmed the presence of the mass. An exploratory laparotomy was subsequently performed. During the operation, a large left ovarian tumor was detected, and it was resected along with the left fallopian tube and omentum. There was no significant lymphadenopathy of the pelvic and retroperitoneal lymph nodes. Gross examination of the specimen showed an encapsulated mass of 12 X 11.5 X 6 cm that weighed 500 g. On cut sections the mass appeared partially cystic, with polypoid or grapelike nodules protruding into the cystic spaces (Figure 1). The more solid areas were soft and tan to brown. Areas of hemorrhage were also noted. Microscopic examination revealed a blue cell tumor, which lacked significant mitotic activity (<1 mitosis per 10 high-power fields). The cells had elongated nuclei with moderate pleomorphism and little to moderate amount of cytoplasm. Slitlike glandular spaces and cysts of varying sizes, reminiscent of rete testis, formed the majority of the tumor (Figure 2, hematoxylin-eosin, original magnification X100). The cysts contained papillary projections lined by primitive-appearing cuboidal to columnar cells (Figure 3, hematoxylin-eosin, original magnification X400). Some of the papillae had hyalinized cores. Other areas of the tumor showed cords and columns of primitive cells in a slightly edematous stroma (Figure 4, hematoxylin-eosin, original magnification X400). In some areas, cells with large, bizarre nuclei were identified; however, they did not show any mitotic activity.

[FIGURES 1-4 OMITTED]

What is your diagnosis?

Pathologic Diagnosis: Sertoli-Leydig Cell Tumor, Retiform Type

Sertoli-Leydig cell tumors (SLCT) are rare neoplasms accounting for less than 0.5% of all ovarian tumors. (1) Initially, these tumors were called "arrhenoblastoma" or "androblastoma." Because many of them are nonfunctioning and some are estrogenic, the morphologic designation "Sertoli-Leydig cell tumor" is more appropriate. (2) The average age of patients with SLCT is 25 years. Seventy-five percent of patients are 30 years of age or younger, and approximately 10% are older than 50 years of age. (3) The well-differentiated tumors generally occur in older patients, whereas tumors with a retiform pattern are mostly seen in younger patients in their first or second decade. (3) Fifty percent of patients with SLCT have no endocrine manifestations, and they usually present with abdominal swelling or pain. (1) One third of the patients present with hyperandrogenic symptoms, such as oligomenorrhea, amenorrhea, loss of female secondary sex characteristics, hirsutism, and other signs of virilization. A small percentage of patients have symptoms related to excess estrogen production, such as menometrorrhagia or postmenopausal bleeding. (1) The gross appearance of these tumors is not specific; however, the retiform type may show polypoid and papillary structures simulating serous papillary cystic tumors or a hydatidiform mole. (1) The World Health Organization classification divides these tumors into 5 types: well-differentiated, of intermediate differentiation, poorly differentiated, retiform, and mixed. (3) A well-differentiated tumor contains an easily identifiable component of Leydig cells and Sertoli cells arranged in a tubular pattern. (2) Tumors of intermediate differentiation show immature Sertoli cells arranged diffusely, in islands, or in cords resembling testicular embryonic sex cords; well-defined tubules may be present, and mature Leydig cells are usually present. (2) Histologically, the poorly differentiated tumors resemble the indifferent gonad; these tumors may have a sarcomatous appearance. (2) In one study, only 18 out of 27 poorly differentiated SLCTs had Leydig cells. (2) Intermediate and poorly differentiated tumors may contain heterologous elements (cell types foreign to the developing gonad, such as mucus-filled epithelial cells, argentaffin cells, cartilage, and skeletal muscle). Retiform types are composed of irregularly branching, elongated, and narrow tubules and cysts, which may contain papillary structures. Columns or ribbons of immature Sertoli cells are also frequently present. Very large and bizarre cells without significant mitotic activity are regarded as degenerative in nature. A retiform component is present in only 15% of the SLCTs and is usually present in association with intermediate or poorly differentiated tumors. (3) This pattern is not described in well-differentiated tumors. (1) Retiform SLCTs must be mainly differentiated from yolk sac tumors and serous borderline tumors. The papillae of the yolk sac tumor are usually lined by poorly differentiated cells. The presence of Schiller-Duval bodies and positive immunohistochemical stain for [alpha]-fetoprotein also support the diagnosis of yolk sac tumor. Serous borderline tumors occur in older patients and lack the columns and ribbons of immature cells. Granulosa cell tumor can also mimic SLCT. Although they can occur in young patients, a retiform pattern has never been described in granulosa cell tumors. The prognosis of these tumors depends on the stage, the degree of differentiation, and the presence or absence of tumor rupture. In one study, none of the well-differentiated tumors, 11% of those of intermediate differentiation, 59% of poorly differentiated tumors, and 19% of tumors with heterologous elements were clinically malignant. (2) Tumors with a retiform pattern are thought to have a worse prognosis than are tumors without a retiform component. (1) When the tumor recurs, it does so in the pelvis or abdomen. Distant metastases to the lung, liver, skin, and supraclavicular lymph nodes have also been described. (1)

References

(1.) Scully RE, Young RH, Clement PB. Sertoli-stromal cell, mixed and unclassified sex cord-stromal tumors. In: Tumors of the Ovary, Maldeveloped Gonads, Fallopian Tube, and Broad Ligament. Washington, DC: Armed Forces Institute of Pathology; 1998:203-224. Atlas of Tumor Pathology; 3rd series, fascicle 23.

(2.) Young RH, Scully RE. Ovarian Sertoli-Leydig cell tumors: a clinico-pathological analysis of 207 cases. Am J Surg Pathol. 1985;9:543-569.

(3.) Young RH. Ovarian tumors other than those of surface epithelial-stromal type. Hum Pathol. 1991;22:763-775.

Accepted for publication August 3, 2001.

From the Department of Pathology, Loyola University Medical Center, Maywood, Ill. Dr Atilla Omeroglu is currently a surgical pathology fellow at Memorial Sloan-Kettering Cancer Center, New York, NY.

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Author:Omeroglu, Atilla; Husain, Aliya N.; Siziopikou, Kalliopi
Publication:Archives of Pathology & Laboratory Medicine
Geographic Code:1USA
Date:Mar 1, 2002
Words:1017
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