Ovarian signet-ring stromal tumor: a potential diagnostic pitfall

Signet-ring stromal tumor is a rare ovarian neoplasm with only 10 reported cases in the literature. We report an unusual case of ovarian signet-ring stromal tumor in a 69-year-old woman who presented with right adnexal mass and underwent total abdominal hysterectomy and bilateral salpingo-oophorectomy. The diagnosis was based on histological, histochemical, immunohistochemical, and electron microscopy characteristics. The main significance is to differentiate this benign tumor from the highly malignant Krukenberg tumor, and this differential diagnosis is discussed.     

Ovarian sex cord tumour with annular tubules diagnosed by fine needle aspiration cytology--a case report

Sex cord tumor with annular tubules (SCTAT) is a rare ovarian neoplasm with distinctive morphological features whose cytological features have rarely been described. We report a case of SCTAT diagnosed on fine needle aspiration cytology (FNAC) with a brief review of literature. A 35 year old female presented with recurrent abdominal mass. Fine needle aspiration was done under ultra sound guidance. Cytology smears showed three dimensional tubular structures, metachomatic hyaline masses and uniform neoplastic cells dispersed and in cohesive clusters. Cell block study showed the characteristic simple and complex tubules leading to the diagnosis. The cytological findings of a rare ovarian neoplasm are described, which is only the third such report in English literature. The cytologic features of ovarian SCTAT are as distinctive as its histologicfeatures. Cell block study brings out the distinctive architecture of the tumor and helps in differentiating it from other closely related tumors.     

Solid pseudopapillary tumor: an invasive case report of primary ovarian origin and review of the literature

Solid pseudopapillary neoplasm occurring as a primary tumor outside the pancreas is a rare event. We report a case of an ovarian primary occurring with an ill-defined cystic mass in a 39-year-old woman. The morphologic and immunohistochemical features of the ovarian neoplasm described in this report are compatible with those of solid pseudopapillary neoplasm of the pancreas. Histologically, the tumor cells of the case we report infiltrate into the ovarian parenchyma. Because of the diagnosis is not clear before surgery, the patient had a reoccurrence two months after the operation in which laparoscopic simple ovarian cystectomy and part ovarian tissue removal, followed by the right salpingo-oophorectomy. The case herein confirms that solid pseudopapillary neoplasm of the ovary belongs to the class of low-grade malignant tumor with certain invasiveness. The diagnosis should be taken into serious consideration in order to avoid missed diagnosis and delay treatment. Through this case we have a better understanding of the biological behavior of solid pseudopapillary neoplasm of the ovary.     

Unilateral malignant struma ovarii in a case of bilateral ovarian teratoma with raised CA-125 level: a rare case with treatment dilemmas

Struma ovarii are specialized form of mature ovarian teratoma comprised predominantly of thyroid tissue (>50%). Most of the struma ovarii are benign; rarely can they undergo malignant transformation. Elevated CA-125 levels with benign struma ovarii have been seen in only 5 cases in literature. The association of malignant struma ovarii and high CA-125 levels with pseudo-Meig syndrome has been reported in only 2 cases in English literature. We describe a case of a 46-year-old multigravida who presented with an abdominal mass and raised CA-125 levels. Radiological investigations revealed bilateral cystic adnexal masses with ossified elements on left side suggesting a teratoma. Intraoperative frozen section and final pathology revealed bilateral teratoma with follicular variant of papillary thyroid carcinoma arising in the left ovary. To the best of our knowledge, this is the first case of malignant struma ovarii in combination with bilateral teratoma. The dilemmas related to preoperative diagnoses with elevated CA-125 levels, mimicking an epithelial ovarian neoplasm; intraoperative frozen section consultation; management and follow-up issues in this rare malignancy are discussed.     

Ovarian angioleiomyoma: a case report

Angioleiomyoma (ALM) is a rare and painful, benign neoplasm that is referred to an uncommon type of leiomyoma originating from smooth muscle cells of arterial and venous walls. ALM is very rarely found in the female genital organs such as uterus or ovary. Herein, we present the case of a huge primary ovarian ALM in a 35 year-old woman, clinically and radiologically mimicking an ovarian fibroma, which histologically has diffuse myxoid degeneration. This is the third case of ovarian ALM and the first case of ovarian ALM with diffuse degeneration reported in English literatures, to the best of our knowledge.     

Embryonic implantation in carcinoma of the endometrium

We recently encountered a patient in whom placental implantation occurred directly in carcinoma of the endometrium. At the time of surgery for a presumed tubal pregnancy, the 33-year-old patient was discovered to have bilateral ovarian tumors that were histologically identical to the endometrial neoplasm.     

Bilateral ovarian carcinoma metastatic from the ampulla of Vater: a rare Krukenberg tumor.

Carcinoma of the ampulla of Vater is a relatively rare neoplasm and its longterm survival rate is considerably high. However, because of differences in tumor pathologic features and local invasiveness, a 5-year survival rate differ widely. We present a case of metastatic carcinoma of the ampulla of Vater presenting as a Krukenberg tumor in a 59-year-old woman. Eight months earlier, she had been diagnosed as well-differentiated adenocarcinoma of the ampulla of Vater. Abdominal examination revealed a hard mass with mild tenderness in the RLQ area. The laboratory findings were unremarkable except for mild anemia. CT scan of the abdomen revealed enlargement of both ovaries. An exploratory laparotomy disclosed bilateral ovarian masses, 18 x 12 x 8 cm and 8 x 5.5 x 4 cm in size, respectively. Histologic findings of the both ovarian masses were consistent with metastatic adenocarcinoma from the ampulla of Vater.     

Solid pseudopapillary neoplasm, pancreas type, presenting as a primary ovarian neoplasm

Solid pseudopapillary neoplasm has historically been associated with the pancreas, categorized as a tumor of low malignancy. Recently, solid pseudopapillary neoplasm was reported to arise as a primary ovarian tumor in 3 women. We report a fourth case identified in a 48 year-old woman with an 8-cm left ovarian mass. A left salpingo-oophorectomy was performed. Microscopic examination demonstrated a predominately cystic neoplasm comprised of solid nests of cells with an epithelioid to plasmacytoid appearance, associated with blood vessels, hemorrhage, and degenerative changes, that is, pseudopapillary structures. The tumor cells stained focally for pancytokeratin, progesterone receptor, and CD57 with diffuse nuclear expression of β-catenin. Ki-67 was 5% to 10%. Synaptophysin, inhibin, and E-cadherin stains were negative. Clinical and radiologic follow-up of our patient demonstrated no pancreatic lesions. This is a rare report of a primary ovarian solid pseudopapillary neoplasm. Prolonged follow-up is needed to determine how this case will fare clinically.     

Hepatoblastoma in an adult with metastasis to the ovaries

A 19-year-old female had ascites, an enlarged liver, pelvic masses, and an alpha-fetoprotein level of 397,000 micrograms/L with a normal beta-human chorionic gonadotropin serum level. Abdominal exploration revealed a large solitary liver mass with bilateral ovarian masses, and bilateral salpingo-oophorectomy and wedge liver biopsy were performed. The tumor was composed of cords, nests, and pseudorosettes of polyhedral cells with eosinophilic cytoplasm and nuclei with prominent nucleoli. The clinical, light microscopic, and immunocytochemical features indicate that the primary liver neoplasm and ovarian metastases were a purely epithelial hepatoblastoma. To the authors' knowledge, this is the first reported case of this type.     

Sclerosing stromal tumor of the ovary in a 4-year-old girl with characteristics of an ovarian signet-ring stromal tumor

Ovarian sclerosing stromal tumor (OSST) is an extremely rare neoplasm that primarily affects young women. Signet-ring stromal tumor is another rare non-functioning benign ovarian stromal neoplasm. We report a case of a right OSST with prominent characteristics of signet-ring stromal tumor in a 4-year-old girl with symptoms of premature thelarche. We describe the clinical, histopathological, and immunohistochemical findings and review the literature. To our knowledge, the 4-year-old patient presented here is the youngest case of OSST reported in premenarchal children. The presence of non-mucin/non-lipid obvious signet-ring-like cells in this case suggests a possible relationship between OSST and signet-ring stromal tumor of the ovary.     

Primary mucinous carcinoma of the skin. A case report

Primary mucinous carcinoma of the skin is a rare sweat-gland neoplasm with a high recurrence rate. We report a new case of a primary recurrent mucinous carcinoma of the face in a 59-year-old man. Histopathologic examination of the neoplasm showed epithelial islands floating in mucoid material compartmentalized by fibrous septa. Cytokeratin 7, protein S100, estrogen and progesterone receptors were detected at immunohistochemical study, while cytokeratin 20 and actin were undetectable. Histologically, mucinous carcinoma of the skin can be mistaken for a metastasis from extracutaneous sites, particularly the breast or the gastrointestinal tract. Mucinous carcinoma of the skin has a relatively good prognosis with rare distant metastases, but high recurrence rate.     

Epithelioid bilateral ovarian leiomyosarcoma: a study

Primary ovarian leiomyosarcoma is extremely rare and generally affects post menopausal women. It is usually unilateral and more than 10 cm in diameter. Its histogenesis is not clear its prognosis seems to be improved by radical surgery and adjuvant therapy. We report a case of a 54 year-old, nulliparous, post menopausal woman who had abdominal distention. Physical examination revealed a large pelvi-abdominal mass. The excision of tumor with bilateral salpingo-oophorectomy and hysterectomy was carried out. A diagnosis of bilateral ovarian epithelioid leiomyosarcoma was made on pathological examination with immunohistochemistry. Adjuvant chemotherapy was given. After 3 months of follow up, abdominal sonography revealed a peritoneal recurrent tumor with hepatic metastasis. Our objectives are to discuss the histopathological features and the prognosis of this tumor.     

CASE REPORT: Recurrent maternal virilization during pregnancy associated with polycystic ovarian syndrome: a case report and review of the literature

Maternal virilization in pregnancy is associated, in most benigncases, with luteoma of pregnancy and hyperreactio luteinalis.Only a few reports relate this phenomenon to hyperthecosis orpolycystic ovarian syndrome (PCOS). A case of recurrent maternalvirilization during two consecutive pregnancies in a patientwith PCOS is presented. In both pregnancies, the deepening ofher voice, facial hirsutism and scalp hair loss began at theend of the first trimester and regressed 3–4 months post-partum.The patient underwent ovarian venous catheterization, and androgensecretion from both ovaries was found to be markedly high butsimilar, therefore ruling out an ovarian androgen-secretingtumour. Reviewing the English literature of similar cases, wefound reports of only seven cases of maternal virilization duringpregnancy associated with PCOS. Here, we present a case of recurrentmaternal virilization in pregnancy associated with PCOS.     

Ovarian strumal carcinoid in association with multiple endocrine neoplasia, type IIA.

Strumal carcinoid is an unusual form of monodermal ovarian teratoma with thyroid-like follicles admixed with typical carcinoid tumor patterns. We encountered a case of this neoplasm in a patient with multiple endocrine neoplasia, type IIA (Sipple's syndrome), including a medullary thyroid carcinoma diagnosed 24 years previously. During evaluation of bilateral adrenal pheochromocytomas, a unilateral left ovarian strumal carcinoid was discovered. Subsequently, the patient had a parathyroid adenoma excised. The ovarian tumor was immunohistochemically reactive for neuron-specific enolase, chromogranin, synaptophysin, and serotonin, but did not stain for calcitonin. The follicular structures stained for thyroglobulin. This unusual case shows that ovarian strumal carcinoid, like carcinoid tumors at other sites, may arise in association with multiple endocrine neoplasia.     

Ovarian sertoliform endometrioid carcinoma

Sertoliform endometrioid carcinoma (SEC) is a rare ovarian neoplasm occurring almost exclusively in post-menopausal patients. We studied a 71-year-old patient who underwent a total hysterectomy with bilateral salpingo-oophorectomy for a right ovarian mass measuring 25 cm in its maximal dimension. Histology revealed an SEC, featuring foci of typical endometrioid carcinoma and areas of clear cell differentiation. This particular type of ovarian neoplasm, already described in 21 reported cases in the literature, must be distinguished from Sertoli cell tumours and Sertoli-Leydig cell tumours which are encountered at a younger age. We discuss the elements of the differential diagnosis and insist upon the value of anti-epithelial membrane antigen in identifying an SEC.     

Ovarian granulosa cell 'tumorlet' and mature follicles with ectopic decidua in pregnancy--a case report

Non-neoplastic, tumor like lesions are encountered in the ovaries during pregnancy. Of these pregnancy luteoma is the most common lesion mimicking an ovarian neoplasm. Ovarian granulosa cell proliferations are also reported in the ovaries removed during pregnancy as an incidental finding. The granulosa cell proliferation occurs due to the follicular stimulating hormone (FSH) like activity of human chorionic gonadotropin (hCG). We report a case of second gravida who developed uterine atonia, necessitating emergency hysterectomy. Right ovary showed extensive ectopic decidua, numerous well-formed follicles and granulosa cell tumor-like proliferation. Usually follicles are not formed in the ovaries during pregnancy. The same FSH like activity of hCG might have been responsible for the formation and maturation of follicles in this case.     

Muzinöse Ovarialtumoren

Mucinous ovarian neoplasms represent the second largest group of epithelial ovarian tumors after serous neoplasms, of which benign cystadenomas constitute more than 80?%. Mucinous cystadenomas and carcinomas cannot be distinguished by the clinical features or the mean age of onset of the disease. They typically occur unilaterally, are confined to the adnexae (FIGO stage I) and clinically present with non-specific abdominal symptoms or are diagnosed by chance. The mean age of disease onset is around 50 years old. The prognosis is excellent. Implants, peritoneal metastases and bilateral occurrence of ovarian mucinous neoplasms should lead to the suspicion of metastasis particularly from a gastrointestinal tumor. Neither microinvasion defined as a maximum extent of invasion of 5 mm, nor intraepithelial carcinoma characterized by high grade atypia without invasion, affect the prognosis of mucinous borderline tumors. Mucinous carcinomas typically show confluent glandular, expansile growth that leads to a labyrinth-like pattern. A destructive infiltrative or nodular growth pattern, however, should lead to the consideration of metastasis. Mural nodules that may reveal a spindle cell sarcomatous or anaplastic carcinomatous pattern occur infrequently in mucinous and do not affect the prognosis. Pax8 positivity is indicative of a primary ovarian neoplasm. In this case, however, mucinous tumors associated with teratomas may show the colonic immunoreaction pattern (CK7?/CK20+/CDX2+). The rare mucinous tumors with endocervical differentiation are now designated as seromucinous tumors and consist of two or more distinct cell types, are frequently associated with endometriosis and seem to show a molecular genetic relationship to endometrioid neoplasms.     

Case 6: A Spindle Cell Breast Lesion in a 54-lfear-Old Woman

The endometrioid-like variant of ovarian yolk sac tumor is a rare neoplasm composed partially or completely of glandular tissue resembling endometrioid carcinoma, but identifiable histologically and immunohistochemically as an extraembryonic endodermally differentiated germ cell tumor. Only nine examples of this neoplasm have been published previously, and none was studied by electron microscopy. One of these rare neoplasms, the first documented to have foci of carcinoid, has recently been observed and studied ultrastructurally. Electron microscopic observations showed that the main component of the neoplasm had glandular epithelial cells with nuclear and cytoplasmic features difficult to distinguish from the cells of Müllerian endometrioid and endometrial adenocarcinoma, but a minor component of mucinous adenocarcinoma was confirmed as intestinal by the epithelial cells having characteristic microvilli with filamentous cores and rootlets. In addition, neuroendocrine cells present in the glands and in nests were readily identified ultrastructurally by their dense-core granules. Electron microscopy may be useful in supplementing light microscopic and immunohistochemical examination of this variant of yolk sac tumor.     

Bilateral virilizing sclerosing stromal tumours of the ovary in a pregnant woman with Gorlin''s syndrome: implications for pathogenesis of ovarian stromal neoplasms

A woman with Gorlin's syndrome who had become pregnant following clomiphene therapy presented in early pregnancy with bilateral ovarian sclerosing stromal tumours which were associated with profound virilization. Pre- and post-operative hormone profiles indicated androgen production by the tumours. Ovarian sclerosing stromal tumours have not previously been reported as occurring bilaterally or in association with Gorlin's syndrome. We discuss the questions raised by this unusual case regarding the pathogenesis of ovarian stromal neoplasms.     

Bilateral massive ovarian leiomyomata in a young woman: a case report with review of the literature.

We report a case of ovarian leiomyomata, bilateral and massive, in a 21-yr-old woman. Primary leiomyoma of the ovary is a very rare tumor and is usually small, unilateral, and concomitant with uterine leiomyomata. To our knowledge, this is the first report in the English literature of bilateral ovarian leiomyomata. We document the smooth muscle origin of the tumors with immunohistochemical studies that show appropriate staining with antibodies to vimentin, muscle specific actin, desmin, smooth muscle actin, and collagen type IV. The available literature is reviewed. The characteristics of both typical and atypical ovarian leiomyoma and theories of its origin are discussed.