Intestinal adenocarcinoma arising in a mature cystic teratoma of the ovary: a case report

Gastrointestinal adenocarcinoma arising in mature cystic teratomas of the ovary is extremely rare. We report a case of well-differentiated intestinal adenocarcinoma arising in a mature cystic teratoma of the ovary in a 77-year-old woman, presenting as acute abdomen with ovarian torsion. An immunohistochemical study revealed expression of CK20 and CK7, and the tumor was also positive for MUC2. The patient had no evidence of disease after 12 months of follow-up.     

Primary squamous carcinoma of the ovary likely arising from a monodermal cystic mucinous teratoma

We present a 58-year-old woman with primary squamous carcinoma of the ovary likely arising from a monodermal cystic mucinous teratoma. Noninvolved ovary showed no Brenner tumor, endometriosis, transitional carcinoma, endometrioid adenocarcinoma, or typical multigerm layer classic mature teratoma. Moreover, no other primary site was possible because there were no prior or concomitant squamous carcinomas, or history of cervical intraepithelial neoplasia. The tumor showed strong positivity for p63 and CK5/6, reactivity that also extended from the squamous carcinoma into the basal-cell lining of the mucinous cyst of a likely monodermal teratoma. This basal-cell pattern was absent in a series of conventional benign and borderline cystic mucinous cystadenomas of the ovary, but clearly present in the mucinous cysts part of mature teratomas. We present this as a unique case of squamous carcinoma likely arising from a monodermal cystic mucinous teratoma. Moreover, we submit that the p63 and CK5/6 staining pattern may help to differentiate monodermal cystic mucinous teratoma from conventional cystic mucinous tumors.     

Meningioma in mature cystic teratoma of the ovary

A case of meningioma arising in a mature cystic teratoma of the ovary in a 60-year-old woman is described. The tumor was located in the right ovary, and salpingo-oophorectomy was performed. The right ovary was 10 x 10 x 8 cm in size and contained an unilocular cyst. In the wall, a solid nodule measuring 3 x 3 x 2 cm was noted. Histologically, the cyst wall was composed of typical mature cystic teratoma. In contrast, the mural nodule was composed of the proliferating spindle- and polygonal-shaped cells showing positive staining for epithelial membrane antigen and microcystic change was prominent. These findings were consistent with microcystic meningioma. The arachnoidal cells around mature brain tissue may be the origin of this unusual tumor. To the best of our knowledge, this is the first case of mature cystic teratoma with meningioma of the ovary reported in English medical literature. This case may further indicate the totipotential nature of mature cystic teratoma.     

Adenocarcinoma arising from mature cystic teratoma of the ovary

An extremely rare adenocarcinoma arising from a mature cystic teratoma is reported. A 58-year-old woman underwent bilateral salpingo-oophorectomy because of a tumor in each ovary. The right ovarian tumor (solid, 9.6 × 9.6 × 6.3 cm) was a benign thecoma. Histology revealed the left ovarian cystic tumor (multilocular, 6.4 × 4.8 × 2.8 cm) was a mature cystic teratoma containing skin, fatty tissue and respiratory epithelial tissue. In addition, there was a small focal adenocarcinomatous lesion contiguous to the teratomatous ciliated columnar epithelium without stromal invasion (so-called adenocarcinoma in situ ) that was suggestive of respiratory epithelium origin. However, goblet cells were present in the glandular structures of the lesion and immunohistochemical staining was segmentally strongly positive for CK20 and uniformly negative for CK7. These results suggested that the adenocarcinomatous lesion had a mucin secretory gastrointestinal phenotype. Further investigation and the collection of more cases is necessary to determine the origin and growth mechanism of adenocarcinoma arising from mature cystic teratoma of the ovary.     

KRAS mutation positive mucinous adenocarcinoma originating in mature ovarian teratoma: Case report and review of literature

Mature ovarian teratomas rarely undergo transformation into malignancy. Carcinomas, mostly squamous cell carcinoma, are the most common malignancy arising in mature cystic teratoma. In the present report we describe a 13‐year‐old girl who developed a large mass in her ovary. Fine needle biopsy identified intestinal type mucinous adenocarcinoma, which was also identified in the full surgical specimen. Extensive sampling of the surgical specimen also identified areas of mature cystic teratoma. Interestingly, molecular analysis of DNA extracted from various components of the lesion identified KRAS mutation in the carcinoma, borderline mucinous tumor and benign intestinal‐type epithelium but not in the epidermal component of the teratoma. To the best of our knowledge this is the first report of KRAS mutation in mucinous carcinoma originating in mature cystic teratoma. We discuss the importance of extensive tissue sampling to differentiate between carcinoma originating in teratoma and metastatic colorectal carcinoma to the ovary. Additionally, the identification of KRAS mutation in the morphologically benign intestinal‐type epithelium indicated that it is an early event in the carcinogenic sequence and that the molecular pathway of carcinogenesis in teratoma is similar to that in the carcinogenic process of somatic tissue.     

Intestinal metastases in a squamous cell carcinoma arising in mature cystic teratoma of the ovary: a case report

Metastatic spread to the intestines by squamous cell carcinoma (SCC) arising in mature cystic teratoma (MCT) or dermoid cyst is a very rare event. A case of a 40-year-old cachectic woman is presented with an MCT of the left ovary found at autopsy. Focal mural thickening of the cyst wall harbored a moderately differentiated SCC with single cell keratinization. Intramural metastases were noted in the jejunum, ileum, colon transversum, and left colonic flexure. In addition, liver metastases were detected. The tumor was staged as FIGO IV. The peritoneum was covered diffusely by fibrinous and suppurative exudate. In this unusual case, metastasizing SCC of the ovary arising in an MCT led to suppurative peritonitis and consequently to death of the patient. Follow-up procedures regarding intestinal spread could be useful in patients with SCC in MCT.     

Mature cystic teratoma of the ovary associated with complete colonic wall and mucinous cystadenoma

Mature cystic teratomas of the ovary frequently contain intestinal type epithelium, but they are rarely associated with complete intestinal wall. The association of mature cystic teratoma with mucinous cystadenoma is not unusual. However, the pathogenetic relationship between these two lesions remains unanswered. We report a mature cystic teratoma of the ovary in a 16-yr old female that contained a complete colonic wall in continuity with an endocervical-type mucinous cystadenoma. Both the mucinous cystadenoma and the colonic wall showed the typical histopathological and immunohistochemical patterns of classical mucinous cystadenoma (positive for CK7, negative for CK20) and normal colonic wall (positive for CK20, negative for CK7), respectively. The microscopic and immunohistochemical patterns of the epithelium from the transitional zone between colonic wall-like structure and mucinous cystadenoma demonstrated features of both types of epithelium, positive for both CK7 and CK20, and focally positive for a neuroendocrine marker, chromogranin, which is normally present in colonic mucosa. These results suggest that the mucinous cystadenoma originated from the colonic epithelium of the mature cystic teratoma.     

Bowel loop in an ovarian tumor: Grossly visible, completely developed intestinal loop in mature cystic teratoma of malignant mixed germ cell tumor

Although a gastrointestinal-type epithelium is observed in 7–13% of mature cystic teratoma cases, the occurrence of a grossly visible, organized gastrointestinal loop formation is very rare. Presented here is the case of a 14-year-old girl with malignant mixed germ cell tumor in the ovaries. In her left ovary a grossly visible, intestinal loop, 9 cm long, with hanging mesentery attached to the cystic wall of a mature cystic teratoma associated with a yolk sac tumor was observed, and in her right ovary another mature cystic teratoma was observed. Microscopy of the intestinal loop indicated a well-organized, intact layer of small intestinal wall. The yolk sac tumor predominantly had a polyvitelline pattern. Previously, gastrointestinal wall or epithelium that was identified on microscopy has been reported. To the authors' knowledge this is the first case report of the formation of a grossly visible, completely developed intestinal loop in a malignant mixed germ cell tumor.     

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.     

Combined microcystic adnexal carcinoma and squamous cell carcinoma arising in the ovarian cystic teratoma--a brief case report.

Malignant transformation of mature cystic teratoma of the ovary can develop with an incidence of 1-2%. Squamous cell carcinoma is the most common malignant tumor arising in benign cystic teratoma. The authors have recently experienced a case of combined microcystic adnexal carcinoma and squamous cell carcinoma arising in a benign cystic teratoma of the ovary in a 72-year-old Korean woman. The right ovarian cystic mass had been ruptured and firmly adhered with salpinx and omental fat tissue on operation. Thickened cystic wall with yellowish white solid infiltrative lesion was noted grossly, and two different malignant tumors of microcystic adnexal carcinoma exhibiting both eccrine and hair follicular differentiation and squamous cell carcinoma were observed microscopically. PAS and CEA positivities suggested eccrine differentiation in areas of microcystic adnexal carcinoma.     

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.     

Mixed angiosarcoma, clear cell adenocarcinoma and mature teratoma elements in an ovarian tumor: a case report and literature review

Malignant transformation of a mature teratoma in the ovary is a rare event, with an approximate rate of only 1-2%. Here, we report an ovarian tumor with a unique combination of epithelial and non-epithelial malignant components, including mature teratoma elements. A 59 year-old postmenopusal woman underwent total hysterectomy and bilateral salpingo-oophorectomy to remove a huge solid mass of the right ovary. The ovarian tumor was 16 × 12 × 4.5 cm in dimensions, composed of red-brown and greyish-white tissue with several cystic areas. Microscopically, atypical cells immunopositive for both CD31 and CD34 formed irregular ectatic vascular patterns with a high MIB-1 labeling index in red-brown areas. In contrast, tubule-cystic and papillary structures were lined by HNF-1β-immunopositive atypical cuboidal and hobnail cells with clear cytoplasm in greyish-white areas. In addition, normal-looking epithelial and stromal components, including mature squamous, cuboidal and ciliated epithelial cells, and adipose tissues, were observed in red-brown areas, suggesting an ovarian tumor combining angiosarcoma, clear cell adenocarcinoma, and mature teratoma features. We could demonstrate identical X-chromosome inactivation patterns among all three components by human androgen receptor gene (HUMARA) assays, pointing to complex inter-relationships regarding their pathogenesis. These observations suggest that a malignant tumor composed of two characteristic phenotypes arose in mature teratoma.     

Mature cystic teratoma of the ovary with a small ganglioneuroma

A case of ganglioneuroma arising within a cystic mature teratoma of the ovary in a 34-year-old woman is reported. Patient underwent right adnexectomy. The ovary was completely replaced by a bilocular cystic lesion, measuring 8 cm in diameter and filled with adipose tissue and pilosebaceous material. Microscopically the cyst was composed by a mature cystic teratoma containing skin with dermal appendages, fatty tissue and bronchial epithelium. Furthermore a nodule (0.5 cm in size) composed of mature ganglion cells, axons and Schwann cells, was identified. Ganglion cells were positive for NSE and synaptophysin, while Schwann cells stained positively with S100 protein and GFAP. To the best of our knowledgment this is the first reported cases of ganglioneuroma arisen within a cystic mature teratoma of the ovary.     

Mucinous cystadenocarcinoma of the breast with a basal-like immunophenotype

Mucinous cystadenocarcinoma (MCA) of the breast is extremely rare and was only recently described as a distinct variant of invasive ductal carcinoma of the breast. A case of MCA is reported in a 41-year-old woman. Mammographic and ultrasonographic examinations showed an irregularly shaped 10.0 × 8.0 × 5.5 cm lesion with patching calcification in the upper outer quadrant of the left breast. The gross examination revealed that the tumor has a well-circumscribed edge with a gelatinous cut surface and hemorrhage and necrosis were also noticed in the mass. Microscopically, the mass resembled mucinous cystic neoplasm of the ovary and pancreas closely, with cystic areas lined by columnar mucinous cells and associated with abundant extracellular and intracellular mucin, which is distinctively different from mucinous carcinoma with typically nests of low grade neoplastic cells floating in the mucin pool. The tumor cells were positive for CK7, CK20 and CDX2 were negative and displayed a typical immunophenotype of basal-like breast cancer (ER, PR, HER2 were negative, CK5/6 and EGFR were positive). Metastatic carcinoma was identified in three of 14 axillary lymph nodes. We describe here a very unusual case of breast MCA with basal-like immunophenotype.     

Ovarian mucinous cystadenoma with mural nodule of anaplastic carcinoma.

The occurrence of malignant mural nodule in benign cystic common epithelial tumor of the ovary have been reported in only three cases; the case one was mucinous cystadenoma with a mural nodule of fibrosarcoma and the others were of carcinomas. Our case was another rare case of ovarian mucinous cystadenoma with mural nodule of anaplastic carcinoma in a 42-year-old woman. The cystadenoma had an unilocular cystic cavity and a mural nodule with thick multinodular solid wall. The internal cystic wall was lined with mucinous cystadenoma without any malignant features. The mural nodule showed anaplastic carcinomatous differentiation and its nature was confirmed by immunohistochemistry and electron microscopy. This tumor had metastasized to the right salpinx, uterus, cul-de-sac, periureter and mesentery.     

Squamous cell carcinoma arising in mature cystic teratoma of the ovary: an immunohistochemical analysis of its tumorigenesis

AIMS: Squamous cell carcinoma (SCC) is the most common form of malignant transformation in mature cystic teratoma (MCT) of the ovary. Some investigators have suggested the possibility of origin from columnar epithelium. The aim of this study was to analyse such tumours immunohistochemically to elucidate their histogenesis. METHODS AND RESULTS: The expression of cytokeratin (CK) 10 and CK18 was examined in 21 samples of SCC arising in MCT. The expression of CK10 and CK18 was also assessed in SCCs arising in different organs (skin, vulva, lung and uterine cervix) for the purpose of comparison. SCC in MCT expressed CK10 in 7/21 cases [33.3%, 95% confidence interval (CI) 0.12-0.53] and CK18 in 14/21 cases (66.7%, 95% CI 0.46-0.87). SCC in MCT expressed CK10 less frequently, but CK18 more frequently, as is the case in SCCs of the uterine cervix (CK10, 20%; CK18, 70%) and the lung (CK10, 5%; CK18, 90%), both of which are derived from columnar epithelium by squamous metaplasia. CONCLUSIONS: SCC in MCT may be derived from metaplastic squamous epithelium.     

Adenocarcinoma of small intestinal type in retroperitoneal mature teratoma

We report a case of small intestinal type adenocarcinoma arising in retroperitoneal mature cystic teratoma in a young male. The patient initially experienced intermittent abdominal pain, and radiographic workup revealed an encapsulated retroperitoneal mass. A laparotomy was performed and the resected specimen represented a cystic lesion, the inner surface of which was mostly covered by sebaceous debris with hairs. Microscopically, mature derivatives of all three germ layers as well as completely developed gastrointestinal tract were identified. Adenocarcinoma without stromal invasion was observed adjacent to the small intestinal mucosa. Immunohistochemistry of the adenocarcinoma tissue revealed p53 overexpression and high Ki‐67 labeling index as well as positive staining for CD10, cytokeratin 7, and cytokeratin 20. Therefore, the diagnosis of small intestinal adenocarcinoma was made. To our knowledge, this is the first case of small intestinal adenocarcinoma arising in retroperitoneal mature cystic teratoma. A unique feature of this case is that malignant transformation in retroperitoneal mature teratoma arose even in the fully developed intestine. Favorable prognosis due to detection in the ‘early stage’ is also discussed.     

Müllerian adenosarcoma with a neuroectodermal component associated with an endometriotic cyst of the ovary: a case report

Reported here is a case of Müllerian adenosarcoma of the ovary which contained a primitive neuroectodermal tissue component within the stroma. The adenosarcoma coexisted with clear cell adenocarcinoma in an endometriotic cyst. The patient was a 33-year-old woman with a large unilocular endometriotic cyst of the right ovary. On the internal wall of the cyst, both a plaque-like protrusion with a papillary surface and a dome-like mural nodule were noted. The former exhibited features of Müllerian adenosarcoma, and the latter showed those of clear cell adenocarcinoma. In the deeper portion of adenosarcoma, teratoma-like tissue which contained various tissue components including primitive neuroectodermal tissue was found. The presence of primitive neuroectodermal tissue in the stroma of adenosarcoma suggested the diagnosis of 'adenosarcoma with neuroectodermal differentiation' ('teratoid adenosarcoma'), although the possibility of the incidental occurrence of an immature teratoma could not be completely excluded.