Joanne L. Rutgers

Ovarian mullerian mucinous papillary cystadenomas of borderline malignancy. A clinicopathologic analysis

Ovarian mucinous cystadenomas of borderline malignancy that contain foci of intestinal differentiation are well recognized. Borderline tumors lined by mucinous epithelium of endocervical type and characterized by papillae architecturally similar to those of serous borderline tumors, however, have not been described in the literature. We have studied 30 tumors of this type. The patients averaged 34 years of age. Forty percent of the tumors were synchronously bilateral and 30% were associated with endometriosis. Four tumors were complicated by peritoneal implants, one by both peritoneal implants and lymph node metastasis and one by lymph node metastasis alone. No patient had pseudomyxoma peritonei. Follow‐up information was available on all the patients for a mean interval of 3.7 years. In two cases tumors developed in the conserved contralateral ovary; no deaths occurred. These tumors have important clinical and pathologic differences from mucinous borderline tumors with intestinal differentiation, but have many similarities to mixed‐epithelial borderline tumors of Mullerian type.

The Androgen Insensitivity Syndrome (testicular Feminization): A Clinicopathologic Study of 43 Cases

Summary:Forty-three patients with the androgen insensitivity syndrome (AIS), ages 14 to 83 (average 27) years, were studied. Forty patients had complete AIS and three patients had incomplete AIS. Microscopic examination of the testes revealed immature tubules, which contained rare spermatogonia in 28% of the cases. Prominent Leydig cells and a spindle-cell stroma resembling ovarian stroma were found in a majority of cases. The organization of the testicular parenchyma could be classified into one of four patterns: diffuse tubulostromal, lobular tubulostromal, mixed tubulostromal, or stromalpredominant. Hamartomas were present in 63% and Sertoli cell adenomas in 23% of the cases. Malignant tumors developed in 9% of the patients and comprised two seminomas, one intratubular germ cell neoplasm with early stromal invasion, and a malignant sex cord tumor. At least one fallopian tube was present in 35% of the cases.

The testicular tumor of the adrenogenital syndrome: a report of six cases and review of the literature on testicular masses in patients with adrenocortical disorders

The clinical and pathological features of 40 cases in which testicular masses developed in patients with the adrenogenital syndrome are reviewed; this study was based on six personally observed cases and 34 other cases in the literature. The adrenal disorder was of the salt-losing form in two-thirds of the cases and the non-salt-losing form in the other third. Although the clinical diagnosis of the adrenogenital syndrome had been established prior to the discovery of the testicular lesion in most of the patients, in 18% of them the diagnosis was not made until or after the development of a testicular mass. Two-thirds of the masses were palpable (up to 10 cm); these cases were usually discovered in early adult life (average, 22.5 years). The remaining one-third were small (under 2 cm) and were usually found in children either at autopsy or on testicular biopsy. Eighty-three percent of the masses were bilateral. Eighty-six percent of the small lesions were located in the hilus. The larger lesions involved the testicular parenchyma in all but one case. They formed well-demarcated but unencapsulated brown-green masses, typically separated into lobules by prominent bands of fibrous tissue. Microscopical examination revealed sheets, nests, and (rarely) cords of cells with abundant eosinophilic cytoplasm separated by bands of fibrous tissue. Lipochrome pigment was identified in the cytoplasm in many cases, but crystals of Reinke were uniformly absent. The major pathological differential diagnosis is Leydig cell tumor; the associated clinical and laboratory features—including the high frequency of bilaterality and a decrease in the size of the tumor with corticosteroid therapy—are diagnostic of a testicular “tumor” of the adrenogenital syndrome. Although a variety of origins have been suggested for these lesions, in our opinion an origin from hilar pluripotential cells, which proliferate as a result of the elevated level of adrenocorticotropic hormone, is most likely.

Significance of comprehensive surgical staging in noninvasive papillary serous carcinoma of the endometrium.

OBJECTIVE To evaluate the biological behavior of noninvasive papillary serous carcinoma of the endometrium. METHODS; From 1990 to 2001, all women with noninvasive uterine papillary serous carcinoma (UPSC) at three Southern California hospitals were identified from tumor registry databases. Data for analysis were collected from hospital charts, office records, and tumor registry files. RESULTS Of the 100 patients diagnosed with UPSC, 16 had noninvasive lesions. Twelve underwent a comprehensive surgical staging procedure with omental resection. Six of these 12 women were found to have disease beyond the uterine corpus, including 4 with adnexal involvement, 3 with omental disease, 2 with cervical extension, 1 with pelvic lymph node involvement, and 3 with positive washings. Three women were found to have positive cytology and metastases in more than one location. Of the 12 patients, 1 of the 6 with stage IA disease had distant recurrence and 4 of the 6 with stage II-IV disease recurred. Of the remaining 4 patients who underwent a staging procedure without pathologic omental assessment, 1 was found to have cervical extension. In these 4 women, 1 with stage IA disease recurred. CONCLUSION The typical patterns of spread and prognostic factors for endometrioid carcinoma of the uterus do not apply to UPSC. In our series, omental assessment was necessary to detect the 25% of patients with stage IVB disease due to omental involvement. Thus, women with noninvasive UPSC should undergo a comprehensive staging procedure including omental sampling to determine the extent of disease.

Ovarian mixed‐epithelial papillary cystadenomas of borderline malignancy of mullerian type: A clinicopathologic analysis

Borderline tumors with papillae that are architecturally similar to those of serous tumors but with a lining of more than one mullerian cell type have not been well characterized in the literature. We have studied 36 such tumors. The patients averaged 35 years of age. Twenty‐two percent of the tumors were bilateral; all were confined to the ovaries as confirmed at operation. Fifty‐three percent were associated with endometriosis. Follow‐up information was available on 34 patients for a mean interval of 4.8 years. A tumor developed in the contralateral ovary in one patient 2 years after unilateral salpingo‐oophorectomy. Three patients had pelvic recurrences between 7 months and 3 years, but all of them were successfully treated and none have died. These tumors differ clinically and pathologically from intestinal‐type mucinous borderline tumors, but they have many similarities with mullerian mucinous borderline tumors and, to a lesser extent, with serous borderline tumors.

Mural nodules in common epithelial tumors of the ovary.

The occurrence of mural nodules in cystic common epithelial tumors of the ovary is well established, but differences in terminology and difficulties in histopathologic interpretation have hampered adequate understanding of their differential diagnoses and prognoses. Using immunohistochemistry and ultrastructural analyses supplementally, we studied two cases, one a serous cystadenocarcinoma with nodules of undifferentiated sarcoma, the other a mucinous cystadenocarcinoma with mural nodules of anaplastic carcinoma. Based on these cases and a review of 48 cases in the literature, we propose a standardized terminology. Mural nodules may be either reactive or neoplastic. Neoplastic mural nodules may be composed of benign elements, carcinoma, carcinoma with reactive elements, sarcoma, or an admixture of carcinoma and sarcoma; the latter category has some similarities to malignant mixed mesodermal tumors. The prognosis of patients with malignant mural nodules is poor, with 50% mortality. Strict morphologic criteria supplemented by immunohistochemistry aids in the sometimes difficult differential diagnosis among these types of mural nodules.

Ovarian yolk sac tumor arising from an endometrioid carcinoma

A yolk sac tumor that arose within an ovarian endometrioid adenocarcinoma in a 50-year-old woman is described. The tumor had typical microscopic features, stained immunohistochemically for alpha-fetoprotein, and was associated with an elevated serum alpha-fetoprotein level when metastases appeared. This is the fourth case in which a yolk sac tumor has been reported to develop from a somatic carcinoma and the second in which this tumor has been associated with epithelium of endometrioid type. Tumor heterogeneity or neometaplasia may account for the origin of a tumor of germ cell type from a carcinoma of somatic origin.

Cysts (Cystadenomas) and Tumors of the Rete Ovarii

SummaryCysts and tumors of the rete ovarii have been described only rarely and little is known of their pathology. We describe herein 16 cases of cysts (cystadenomas), 2 solid adenomas, and 1 adenocarcinoma of rete ovarii origin. The patients with the cysts were 23–80 years of age (mean age, 59 years). The cysts averaged 8.7 cm in diameter and were characterized by a location in the hilus; a flat, cuboidal, or columnar, usually nonciliated-lining epithelium; crevices along the inner surfaces; and a fibromuscular wall that often contained hyperplastic hilus cells. The cysts showed many similarities to microscopic dilatations of the rete. The rete adenomas were incidental microscopic findings in two women 45 and 68 years of age. They were located in the hilus and were composed of cords and tubules lined by cells resembling closely those of the normal rete. The rete adenocarcinoma occurred in a 52-year-old woman, was bilateral, and could be partly localized to the hilus. Microscopic examination revealed retiform spaces containing papillae and nests of transitional-like epithelium. Criteria for distinguishing cysts and tumors of rete origin from other ovarian tumors are discussed.

DNA Mismatch Repair Deficiency in Endometrial Carcinoma

Microsatellite instability (MSI) is the hallmark of a molecular pathway to carcinogenesis due to sporadic or inherited abnormalities of DNA mismatch repair genes. Inherited mutations are seen in hereditary nonpolyposis colorectal cancer syndrome. Endometrial carcinoma shows as high an incidence of MSI as does colorectal carcinoma. This review provides a framework for the gynecologic pathologist to understand the complexities of MSI in endometrial carcinoma, by discussing the basic mechanisms of mismatch repair and carcinogenesis, testing, the morphologic features of MSI endometrial cancer and the contradictory data regarding prognosis.

Extrauterine lesions of intermediate trophoblast

Lesions of intermediate trophoblast arising in the uterus include exaggerated placental site, placental site nodule, placental site trophoblastic tumor (PSTT), and epithelioid trophoblastic tumor. Only 12 examples of extrauterine lesions of intermediate trophoblast (ELIT) have been previously reported; 7 new cases are described herein. Six lesions were located in the fallopian tube or paratubal region and one in the ovary. The patients were 25 to 47 (average 36) years of age. The lesions ranged from 0.6 to 4 cm in diameter; four were cystic. The four placental site nodules (three tubal, one paratubal) were composed of small, sharply circumscribed nodules of intermediate trophoblast with no mitotic activity. The three PSTTs (two tubal, one ovarian) exhibited irregular stromal infiltration by intermediate trophoblast, mitotic activity, and necrosis. Chronic salpingitis was seen in the six tubal/paratubal cases, and endometriosis was seen in four cases. Immunoreactivity for human placental lactogen, human chorionic gonadotropin, and cytokeratin in two cases was consistent with an origin from intermediate trophoblast. ELITs presumably arise from previous ectopic pregnancies, a history of which was present in two of these patients. Follow-up, available on two of the three patients with PSTT, was uneventful at 6 and 12 years, but study of additional cases is necessary to reliably determine the behavior of extrauterine PSTTs.