Granulosa cell tumor and mucinous cystadenoma arising in a mature cystic teratoma of the ovary: A unique case report and review of literature

Mature cystic teratoma of the ovary, though it contains derivatives of all three embryonic germ cell layers, rarely presents together with ovarian epithelial or sex cord-stromal tumors. Only a few cases of ovarian cystic teratoma in association with granulosa cell tumor have been reported in the literature, and simultaneous occurrence of mucinous cystadenoma and granulosa cell tumor is even rarer. To our knowledge, there has never been a report of mature cystic teratoma of ovary coexisting with granulosa cell tumor and mucinous cystadenoma. We report a case of mature cystic teratoma, mucinous cystadenoma, and granulosa cell tumor in the ovary of a 40-year-old woman. The involved ovary, massively enlarged by a multiloculated cyst, showed a hair-sprouting mass with a yellow-tan nodule embedded in the cyst wall. Microscopically, three tumor types were revealed: mature cystic teratoma, mucinous cystadenoma, and granulosa cell tumor.     

Clear cell carcinoma arising from a uterus‐like mass

A uterus‐like mass is an extrauterine mass with a cavity lined by endometrial tissue and a smooth muscle layer resembling the uterine corpus. It is a rare condition of unknown histogenesis. Herein, we describe a case of clear cell carcinoma arising from a uterus‐like mass located in the retroperitoneal space. The patient, a 67‐year old nulliparous woman, had been followed with the diagnosis of an ovarian endometriotic cyst for 14 years until ultrasonography and magnetic resonance imaging (MRI) demonstrated an enlargement of the cystic mass with a thickened irregular wall. Suspicion of malignant transformation prompted us to excise the lesion. At laparotomy, the uterus and right ovary appeared normal, and a mass measuring 8 cm was identified in the retroperitoneal space without any connection to the uterus. Grossly, the removed mass was composed of a cyst filled with blackish‐brownish fluid and a thick wall resembling uterine myometrium. Microscopically, endometrial tissue inside the cyst, which was diffusely lined by clear cell carcinoma, was identified. Although the histogenesis of a uterus‐like mass remains unclear, this case indicates that malignant tumors may occur from a uterus‐like mass through the pathway similar to the carcinogenesis of endometriosis‐related ovarian neoplasms.     

Carcinosarcoma arising in a dermoid cyst of the ovary.

A case of carcinosarcoma arising within an otherwise benign cystic teratoma is reported. The patient, a 78 year old nulliparous woman, presented with right sided abdominal pain of short duration and subsequently underwent a bilateral salphingo-oophorectomy. Slicing of the left ovary revealed a unilocular cyst containing hair admixed with soft yellow material with a thin wall apart from a solid area at one pole. Extensive areas of necrosis and cystic degeneration were present within this mass. Histologically, the large cyst was a typical mature cystic teratoma, containing carcinomatous and sarcomatous elements. Mature cystic teratomas have been reported in association with a variety of malignant ovarian tumours such as mucinous cystadenocarcinoma and malignant germ cell neoplasms. Secondary malignant transformation within a dermoid cyst is a much rarer occurrence, estimated as less than 2% of all such lesions. Adenocarcinomas are the second most common malignancies arising within dermoid cysts. Sarcomas alone or in combination with squamous carcinoma have been described arising in a mature cystic teratoma. To the best of our knowledge, no case of sarcoma arising in association with adenocarcinoma has been described before.     

Coexistence of a mature teratoma and mucinous cystadenocarcinoma in the same ovary--a case report

A case of right sided abdominal lump with pain in a 45 years female was presented. Radiological investigation revealed a multiloculated cystic mass with a few mural nodules arising from right ovary. The tumor was removed by exploratory laparotomy and histopathological examination showed coexistence of dermoid cyst with mucinous cystadenocarcinoma in the same ovary. Left ovary and uterus was healthy and there was no tumor extension or omental deposits. Postoperative period was uneventful.     

Composite mucinous and granulosa cell tumor of the ovary

A composite mucinous and granulosa cell tumor of the ovary in a 76-year-old woman is herein reported. At laparotomy this tumor proved to be a solid and cystic mass measuring 10 cm in greatest diameter. Many of the cysts were lined with a benign mucinous epithelium of the endocervical type, and solid areas contained a proliferation of granulosa cells. These two disparate components were intimately mixed. A theca cell component was also present in areas adjacent to the mucinous epithelium. The coexistence of mucinous and granulosa cell tumor is extremely rare and only four such cases have previously been reported in the literature, and the histogenesis of this tumor has not yet been elucidated. In the present case it is suggested that the granulosa cell element commenced as a reactive stromal hyperplasia in the wall of the pre-existing mucinous neoplasm and thereafter progressed to the point of producing a tumor-like mass or neoplastic changes.     

Primary leiomyoma of the ovary co-existing with serous cystadenofibroma

A 48-year-old woman with a 14 cm left ovarian mass had total abdominal hysterectomy and bilateral salpingo-oophorectomy. On cut section the tumor had a solid and a cystic component. There was a 2.5 cm nodule attached to the cyst wall. Sections taken from the solid component revealed a tumor composed of interlacing bundles of fusiform cells, resembling a leiomyoma. Smooth muscle actin positivity confirmed the diagnosis. Sections taken from the nodule in the cystic part revealed an adenofibroma. There was an SMA positive area in the cyst wall which we think is the possible origin of the leiomyoma. As far as we know, this is the first case of a co-existing leiomyoma and serous cystadenofibroma in the ovary, and we think it will broaden the histological spectrum of primary ovarian leiomyomas.     

Clinicopathological and immunohistochemical features of a sebaceous carcinoma arising within a benign dermoid cyst of the ovary

Pure sebaceous neoplasms arising in dermoid cysts of the ovary are exceedingly rare. A 63-year-old female with abdominal swelling and pain underwent a right salpingo-oophorectomy that showed a unilocular cyst weighing 830 g and measuring 15×12×10 cm, filled with sebaceous material containing a few hair shafts. The cyst wall exhibited plaques protruding into the cavity of the cyst. Microscopy revealed a dermoid cyst with nests and lobules of atypical and infiltrating sebaceous cells surrounded by basaloid cells. The tumor cells stained diffusely for high-molecular-weight cytokeratins and focally for cytokeratin 7, cytokeratin 19, epithelial membrane antigen and carcinoembryonic antigen in the immunohistochemistry study. Low-molecular-weight cytokeratins, cytokeratin 20, vimentin, S100, p63, estrogen receptor, progesterone receptor, p53 and c-erbB-2 were negative in tumoral cells. The proliferative labeling index (Ki67 and proliferating cell nuclear antigen) was low. Basal cell carcinoma with sebaceous differentiation and sebaceoma must be considered in the differential diagnosis. However, the presence of obvious malignant sebaceous differentiation in nearly every tumor nest and lack of peripheral palisading and peri-tumoral myxoid stroma excluded these diagnoses. Some histogenetic concepts relevant to this case are discussed along with a brief review of this neoplasm. To our knowledge, this is the sixth case report of a sebaceous carcinoma arising in a mature cystic teratoma of the ovary.     

A retroperitoneal bronchogenic cyst with malignant change

A unique case of adenocarcinoma arising in a retroperitoneal bronchogenic cyst is presented. A 55-year-old woman presented with lower abdominal discomfort. Computed tomography revealed a retroperitoneal cystic mass attached to the ascending colon. The resected cyst was unilocular and filled with milky white mucus and hemorrhagic debris. Histologically, most of the cyst wall was of well-differentiated papillary adenocarcinoma with no cyst wall invasion. Other small areas of the cyst were lined with variably atypical dysplastic/metaplastic cuboidal to pseudostratified columnar epithelium. The cyst wall was mostly hyalinized, but there was apparent thickened subepithelial basement membrane, elastosis, and a single layer of smooth muscle that suggested bronchial wall structures. A mucin staining study with O-acylated sialic acid, which is used for the demonstration of gastrointestinal, cholecystic and uterine cervical mucins, was negative for the mucin-producing epithelial cells of the cyst. Thus, to our knowledge, this is the first reported case of adenocarcinoma arising in a retroperitoneal bronchogenic cyst.     

Ultrastructure and immunohistochemistry of a fetal-type Leydig cell tumor.

A symptomless scrotal mass was removed from a 34-year-old man. The lesion was 7 cm in diameter and it was grossly a hemorrhagic cyst with indurated walls. By light microscopy tumor cell clusters and cords were seen infiltrating the testicle, tunica albuginea, and paratesticular tissue. In the immunohistochemical analysis the tumor cells were immunoreactive with anti-S-100 protein and anticarcinoembryonic antigen, but they did not express cytokeratin or alpha-fetoprotein as tested with paraffin sections. Tumor cell clusters were enveloped by a laminin-positive basement membrane. Electron microscopy revealed abundant smooth endoplasmic reticulum, lipid droplets, and membranous whorls in the cytoplasm. Lamellar whorled bodies were also seen in mitochondria, which contained tubulovesicular cristae. The presence of a well-developed, often multilayered basement membrane was confirmed at ultrastructural level. The activity of 3-beta-hydroxysteroid dehydrogenase suggested that the tumor cells were capable of androgen synthesis. The morphological features are reminiscent of fetal-type Leydig cells and are distinctly different from the Leydig cell tumors described so far.     

Extracutaneous seborrheic inclusion cyst: an unusual presentation

Seborrheic inclusion cyst is an unusual variant of epidermal cyst characterized by parietal histology similar to seborrheic keratosis. Cysts with such changes have been called "seborrheic keratosis-like changes in epidermal cyst" or "epidermoid cyst with seborrheic verruca-like cyst wall" or simply "seborrheic cyst". To date, this lesion has been described exclusively in cutaneous sites. We describe the first case of an extracutaneous seborrheic inclusion cyst arising from round ligament. A 30-year-old female was referred to our institution for abdominal pain. Ultrasonography showed a hypoechoic heterogeneous, round mass adjacent to the lower extremity of the left ovary, measuring 4.5 cm in maximum diameter. Contrast-enhanced computed tomography of the pelvis in the venous phase showed a round (4.5 cm in diameter) cystic lesion with inhomogeneous fluid content in the side of the left large ligament and anterior to the homolateral adnexa. Laparoscopic resection of the mass was performed. Intraoperatively, an extraperitoneal glistening pelvic mass was discovered: the lesion was attached to the intrapelvic 1/3 middle portion of the left round ligament. Macroscopically, the mass measured 6 cm x 6 cm x 3.5 cm and exhibited a smooth and glistening external surface. On cut sections, the mass was an unilocular cyst filled with soft, yellow, amorphous material. Histologically, the cystic wall was lined by a stratified squamous epithelium with a granular cell layer. The cavity contained keratin-like material. The cystic wall showed numerous areas with close-set basaloid cells and pseudohorn cysts. The latter aspect consisted of cystic invaginations of the epithelium filled with surface keratin, which in a given microscopic section may be cut in cross-section, thereby appeared as "cysts" within the involved epithelium. Parietal rupture was present, accompanied by granulomatous inflammation. There were no postoperative complications, and the patient was discharged 3 days after the procedure. The present case is unique in that it is the first reported case of an extracutaneous seborrheic inclusion cyst arising from a very unusual site, namely the round ligament. The site of origin of the lesion and its cystic nature were established by computed tomography findings. Conservative treatment with enbloc resection was possible. Histological examination confirmed computed tomography findings. The present report described a lesion typically found in dermatopathology practice, but which had arisen in an extracutaneous site.     

Malignant Sertoli cell tumor of the ovary metastatic to the lung mimicking neuroendocrine carcinoma: report of a case.

A Sertoli cell carcinoma of the ovary with lung metastases mimicking neuroendocrine carcinoma is presented. Lung metastases frequently occur. Primary and secondary tumors may exhibit similar growth patterns and differentiating primary from secondary tumors may be troublesome. This process may be more difficult when metastases occur from a tumor in which metastases are uncommon and morphologically resemble only a small portion of the primary tumor. We report the case of a 52-year-old woman who underwent resection of a 4,550-g Sertoli cell tumor of the ovary. Histologically, in addition to the characteristic tubular pattern of growth, 5% of the tumor consisted of poorly differentiated areas with tumor cells in sheets, a high mitotic rate, and areas of necrosis. Eleven months after this surgery she presented at a different institution with multiple pulmonary nodules. Microscopic examination of a subsequently resected lung nodule showed histologic findings similar to those of the poorly differentiated areas of the ovarian tumor and initial immunohistochemical studies showed positive staining for cytokeratin, neuron-specific enolase, and focal positivity for synaptophysin. Without knowledge of the ovarian tumor the lung lesion was interpreted as large-cell neuroendocrine carcinoma. On review of the clinical history and comparison with the previous surgical material, however, both tumors showed similar light microscopy and immunohistochemical reactivity, and a final diagnosis of metastatic Sertoli cell tumor was made. Immunohistochemical staining for inhibin revealed weak positivity in the poorly differentiated areas of the ovarian tumor but not in the lung metastasis. This is one of the rare reports of ovarian Sertoli cell tumor metastasizing to the lungs and it emphasizes the importance of complete clinical histories, ancillary studies, appropriate sampling, and review of archival material in such unusual cases.     

Multilocular peritoneal inclusion cyst with extensive xanthogranulomatous stromal changes: a differential diagnosis of cystic pelvic tumors in women

This report presents an unusual case of multilocular peritoneal inclusion cyst with extensive xanthogranulomatous changes in the cyst stroma occurring in a 21-year-old woman. The multicystic tumor was found attached to the mesentery of the terminal ileum. Microscopically, the cystic spaces were lined by flattened to cuboidal cells, which displayed immunoreactivity for cytokeratin but not for factor VIII-related antigen. Large stromal areas contained cholesterol clefts, multinucleated foreign body giant cells, and accumulations of foam cells. The pathogenesis of this multilocular peritoneal inclusion cyst remains obscure. The patient is disease-free 10 years after surgery. The most important differential diagnosis to this case is mature cystic teratoma of the ovary with xanthogranulomatous changes.     

Mucinous cystadenoma coexisting with stromal tumor with minor sex-cord elements of the ovary: a case report

Mucinous neoplasms occur rarely in association with cystic teratoma, Sertoli-Leydig cell tumor, granulosa cell tumor or carcinoid tumor. Several cases of an ovarian stromal tumor with minor sex-cord elements have been reported in the literatures. However, there has been no report about an ovarian mucinous neoplasm coexisting with a stromal tumor with sex-cord elements yet. We report a case of an ovarian neoplasm composed of both mucinous cystadenoma and stromal tumor with minor sex-cord elements in a 58-yr-old female. The ovary including the mass measured 5 cm in size. On section, it revealed an unilocular cyst (4.5 cm in diameter) filled with mucinous fluid. There was a round, yellow, solid nodule, 1.5 cm in diameter within the wall. Microscopically, the cyst was lined by a single layer of endocervical mucinous epithelium and the nodule was composed of spindle cells showing an intersecting and whorled arrangement. There were cell nests showing polygonal shape with abundant cytoplasm among the spindle cells. They showed immunoreactivity for inhibin and did not have any connection with the adjacent mucinous epithelium. Therefore, we interpret the mucinous cystadenoma as having arisen de novo.     

Immunoelectronmicroscopic demonstration of surface tumor antigen Ca 125

Tissue from a serous cystadenocarcinoma of the ovary was incubated with a monoclonal antibody, OC 125, which reacts with a surface tumor antigen in serous, endometrioid, and some clear cell carcinomas of the ovary. Electron microscopic preparation demonstrated the surface nature of the antigen. It is seen as a linear electron-dense deposit that extends onto villous surfaces. It is in some areas focally discontinuous. No other cell structures reacted with the antibody.     

Benign cystic mesothelioma of the peritoneum--a case report

A case of benign cystic mesothelioma in a 53-year-old woman is presented. The patient had abdominal pain and a palpable mass for 4 days. This mass was noticed incidentally by an obstetrician. A computed tomographic scan of the abdomen and pelvic sonography showed a cystic mass, 6 x 4 cm, in the right lower quadrant close to the cecum and suggested cystadenocarcinoma of the ovary. Operation showed that the mass involved peritoneum in region of the appendix. Complete removal of the mass was done. Grossly it consisted of multilocular cyst containing clear, serous, gelatinous fluid. The light microscopic examination revealed that this lesion consisted of cystic spaces of various size and intervening connective tissue stroma. The cells lined the cysts varied from flattened to cuboidal with occasionally a picket-fence or hobnailed appearance in areas. Brush borders were seen on the luminal surface of some cells. Electron microscopic examination confirmed that the cells were mesothelial origin. This lesion mimics cystic lymphangioma of the abdomen grossly and light microscopically, from which differential features are discussed.     

The ultrastructure of the cyst wall of a murine Sarcocystis

Summary Electron microscope studies of the cyst wall of a murine Sarcocystis-like organism of Mus musculus indicate that the sarcocyst wall is a degenerate muscle cell. The degenerate muscle cell contains nuclei, mitochondria, ribosomes, and myofibrils in disarray with the Z-bands thickened. Surrounding the outside of the muscle cell there is a layer of fibrous material, and occasional fibroblasts are seen. The parasites are found within a parasitophorous vacuole in the muscle cell. There are short finger-like projections extending into the vacuole from the muscle cell. The ground substance of the vacuole is fairly homogeneous, but somewhat more electron dense between the finger-like projections.     

Prolactin-producing pituitary adenoma associated with prolactin cell hyperplasia

A 24-yr-old woman with amenorrhea, galactorrhea, hyperprolactinemia, and sellar mass underwent transsphenoidal surgery. Histologic, immunohistochemical, and electron microscopic investigation revealed a well-differentiated, sparsely granulated prolactin (PRL) cell adenoma of the pituitary showing conclusive PRL immunoreactivity. In the nontumorous adenohypophysis PRL cell hyperplasia was noted. Marked differences were evident between the neoplastic and hyperplastic areas. The tumor consisted of sparsely granulated PRL cells immunoreactive only for PRL. As demonstrated by immunoelectron microscopy, the hyperplastic are a comprised monohormonal sparsely granulated PRL cells as well as bihormonal mammosomatotrophs immunoreactive for both PRL and growth hormone. The MIB-1 index was higher whereas microvessel density was lower in the adenoma as compared with the hyperplastic area. In addition, the nontumorous area showed lymphocytic infiltration whereas inflammatory reaction was not seen in the adenoma. This case represents a rare association of a PRL cell adenoma and PRL cell hyperplasia. The fact that these two lesions were contiguous in the surgically removed material raises the possibility that hyperplasia can precede and transform into adenoma.     

Sertoli-Leydig cell tumor of ovary with heterologous element: a case report

Sertoli-Leydig cell tumors are uncommon tumors of ovary accounting for about 1% of sex cord stromal tumors. They constitute between 0.1% to 0.5% of all the primary ovarian neoplasms. The majority of reported cases are moderately or poorly differentiated combined Sertoli-Leydig cell tumors. A rare case of Sertoli-Leydig cell tumor with heterologous element in a 14 year girl is described. The heterologous component comprised smooth muscle, an uncommon element, that was seen in 60 to 70% of the tumor area. The epithelial element of the tumor consisted of solid, tubular and foci of retiform pattern. Both these features imply a poor prognosis.     

肝脏原发粒层细胞瘤伴海绵状血管瘤1例报道并文献复习

目的探讨肝脏原发粒层细胞瘤(granulosa cell tumor)伴海绵状血管瘤的临床病理学特点。方法对1例肝脏原发粒层细胞瘤伴海绵状血管瘤进行组织学观察和免疫组化染色,观察其超微结构及临床影像学特点,并复习相关文献。结果患者女性,CT及MRI提示肝脏右叶可见一巨大实性肿物,低密度。肉眼见肿物突向肝脏下方,切面呈实性;另外,在肝脏膈面被膜下见一灰红色出血区,呈疏松海绵状。镜下巨大肿物中肿瘤细胞椭圆形或梭形,呈微滤泡状、小梁状排列,部分区域呈实性弥漫状分布,类似于卵巢粒层细胞;部分区域呈束状排列,间质中可见较多胶原纤维瘢痕形成,似孤立性纤维性肿瘤。肿瘤细胞核内可见小核仁,并可见纵形核沟。免疫组化结果示肿瘤细胞CD99、α-inhibin、CK8、CK18、SMA和BCL-2均(+),Ki-67增殖指数<1%。超微结构显示肿瘤细胞可见各种形式的核皱叠形成,部分细胞呈Call-Exner小体样结构排列,偶见桥粒结构,提示该肿瘤与卵巢粒层细胞瘤极其相似。结论粒层细胞瘤原发于肝脏且合并海绵状血管瘤,非常罕见,目前其临床生物学行为难以确定,需对患者进行长期随访。