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.     

卵巢Sertoli细胞瘤临床病理特征分析

目的 探讨2例卵巢Sertoli细胞瘤的临床病理特征、免疫表型、诊断、鉴别诊断及预后。方法 回顾性分析2012年1月~2018年12月于我院手术切除的2例卵巢Sertoli细胞瘤的临床特征、组织学特点及免疫表型,并复习相关文献。结果 病例1,女性,54岁,超声检查发现左附件区12.5 cm×10.9 cm×10.3 cm囊实混合性肿物。病例2,女性,22岁,超声检查示右卵巢5.5 cm×4.5 cm×4.0 cm肿物。镜下肿瘤细胞排列成实性片状、条索状、中空和实性小管状结构,瘤细胞圆形、卵圆形或梭形,胞质丰富淡染,无明显异型性,核分裂像罕见,未见Leydig细胞。免疫组化示α-inhibin、WT1阳性,CR、CK灶状阳性,CD99、EMA、Syn、CgA阴性。2例患者随访时间分别为22个月及19个月,一般状态良好,未见复发和转移。结论 卵巢Sertoli细胞瘤罕见,通常为良性生物学行为,结合临床病史、组织学形态及免疫组化染色可做出病理诊断。     

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.     

Primary retroperitoneal mucinous cystadenocarcinoma in a male patient

Primary retroperitoneal mucinous cystadenocarcinomas (PRMCs) are rare. This is the first reported case in the literature in English of PRMC in a man. The 64-year-old man presented with a large retroperitoneal cystic tumour measuring 24 x 20 x 16 cm3, which was removed intact. Areas ranging from a benign mucinous cyst to borderline mucinous tumour to mucinous cystadenocarcinoma were observed on microscopy. Strong patchy staining for cytokeratins 7 and 20 and strong diffuse staining for MUC2 and MUC5AC core peptides, similar to staining patterns in ovarian mucinous tumours, were shown in the benign and atypical epithelium. Staining for CA19.9 and carcinoembryonic antigen was also shown by both components. The theory of its origin from the mucinous metaplasia of peritoneal (mesothelial) inclusion cysts, rather than from ectopic ovarian tissue or ovarian teratomas, is supported by the occurrence of such a tumour in a male patient.     

Neuroendocrine cells in cystic mucinous tumours of the ovary

This histogenesis of cystic mucinous ovarian tumours is still controversial. It has been proposed that these neoplasms may arise from metaplastic ovarian surface epithelium. Others have suggested that these tumours represent monophyletic (intestinal) types of teratoma. Against this background we have studied the presence of different types of neuroendocrine cells in a series of cystic mucinous ovarian tumours. Argyrophil neuroendocrine cells were found almost exclusively in tumours which were histologically classified as borderline or low-grade mucinous carcinomas, whereas these cells were very rare in mucinous cystadenomas and in grade III and IV carcinomas. Several gut peptide hormones could be demonstrated in these cells, but only in borderline tumours and low-grade mucinous carcinomas. Mucin histochemistry did not reveal characteristic patterns in these neoplasms. The results confirm that with regard to the presence of endocrine cells the epithelium of borderline mucinous cystadenomas and mucinous cystadenocarcinomas bears strong resemblance to intestinal epithelium. These findings do not rule out the possibility that these tumours arise by metaplasia from ovarian germinal epithelium but are equally compatible with a teratomatous origin. The epithelium of most benign mucinous cystadenomas resembles that of ovarian inclusion cysts.     

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.     

Sertoli Leydig cell tumor with heterologous gastrointestinal epithelium: a case report

Sertoli Leydig cell tumour (SLCT) of the ovary with the presence of heterologous elements is very unusual. A case of SLCT with virilizing symptoms and androgenic endocrinological profile is presented. The tumor showed presence of heterologous element in the form of mucinous epithelium of the gastrointestinal type.     

Immunohistochemical and ultrastructural analysis of a poorly differentiated pediatric age Sertoli-Leydig cell tumor

Ovarian Sertoli-Leydig cell tumors (SLCT) are rare in young women. They are divided into six categories based on the degree of differentiation and the presence of heterologous elements. Less than 15% of these tumors are poorly differentiated. A 14-year-old obese African-American girl presented with amenorrhea, progressive abdominal pain, and increasing abdominal girth. Pelvic CT revealed a 10 x 9 x 9 cm right adnexal mass which was resected successfully. The gross appearance was dark tan and red with central hemorrhage and necrosis. Microscopically, this was poorly differentiated with compact aggregates of moderate size oval to elongated cells separated by zones of edematous stroma containing scattered spindle shape cells. Areas of ill-formed tubules and primitive cords were present. Clusters of Leydig cells were observed. The oval and spindle cells showed multiple mitoses and were diffusely positive for inhibin and patchy but strong positivity for calretinin. Both preoperative and postoperative studies revealed no metastases. Serum alpha-fetal protein (AFP), androgen, and dihydroepiandrosterone sulfate (DHEA-S) were elevated.     

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.     

Detection of calretinin expression in abnormal immature Sertoli cells in non-obstructive azoospermia

The current study identified for the first time calretinin expression in abnormal Sertoli cells of azoospermic men who underwent testicular biopsy for sperm recovery and application of the retrieved sperm by in vitro fertilization techniques. Testicular biopsies with various spermatogenic impairments were evaluated immunohistochemically for the expression of the calretinin calcium-binding protein and the marker for immaturity of Sertoli cells, cytokeratin-18 (CK-18). Distribution of the markers was assessed in testes demonstrating a histological phenotype of mixed atrophy, Sertoli cell-only, or normal spermatogenesis (obstructive-azoospermia) and in men carrying a deletion in the azoospermia factor region located on the Y chromosome. Calretinin-immunopositive immature Sertoli cells revealed by co-localization of both markers, calretinin and CK-18, were identified in the mixed atrophy group in seminiferous tubules demonstrating spermatogenic failure. Sertoli cells expressing both markers were rarely detected in all other groups. Leydig cells in all the assessed biopsies expressed calretinin and served as a built-in control for immunoreactivity. This pattern of calretinin-selective expression in immature Sertoli cells suggests a functional relationship between calretinin expression and the degree of Sertoli cell differentiation. Disorders of Sertoli cell differentiation as indicated by calretinin and/or CK-18 expression contribute to the multifactorial mechanisms underlying spermatogenic failure.     

Histogenetic considerations on mucinous cystomas of the ovary based on histochemical and immunohistochemical findings

Mucinous cystomas of the ovary, according to a new proposed classification (I.A.P., Dublin 1988), are classified in three types: endocervical, intestinal and mixed. Their histogenesis is still controversial, thus requiring further investigations. There are two main theories on this matter: a teratomatous theory based on the assumption that the mucinous cystoma is allegedly a teratoma having a monophyletic development where only the endodermal gastrointestinal component remains. The second theory, currently the most widely accepted one, maintains that mucinous cystomas derive from Muller duct residues or, more generally, from introflections of the coelomic epithelial lining through a Muller-type metaplastic process. Some authors also accept both theories. A group of 117 mucinous cystomas were investigated by histochemical methods (PB/KOH/PAS; PAT/KOH/Bh/PAS), to demonstrate the presence of O-acetylated sialomucin variants in goblet cells of intestinal type component. Endocervical type mucinous cystomas have always presented as PB/KOH/PAS negative, whereas mixed type mucinous cystomas presented as positive according to the following percentage: benign forms, 31%; borderline, 67%; malignant, 50%. These data should confirm the hypothesis that intestinal type cystomas may derive from the surface coelomic epithelium of the ovary, through a gastrointestinal metaplastic process. This hypothesis is further supported by the data obtained from the observation on two cases of intestinal metaplasia of endocervical glands, kindly supplied by Dr. Trowell. In one of them, a weak O-acetylated sialomucin secretion was identified, in addition to the presence of argentaffin cells. Furthermore, out of 38 adenocarcinomas of the endometrium and 15 adenocarcinomas of the endocervix, one case of endocervical adenocarcinoma was found, characterized by a mucous secretion rich in O-acetylated sialomucins. Moreover, immunohistochemically, by means of anti-chromogranin A monoclonal antibodies, endocrine cells were found in benign, borderline and malignant mucinous cystomas of mixed type. These data do not seem to confirm the assumed correlation between neuroendocrine cell presence and biologic behaviour of the neoplasm nor do they clarify tumor histogenesis. Another immunohistochemical study with BD5 monoclonal antibody demonstrated that this marker was present in the intestinal type epithelium of mixed mucinous cystomas. The histogenetic teratomatous hypothesis of ovarian mucinous cystomas was confirmed by reviewing 100 ovarian teratomas, in which O-acetylated sialomucins were found in the epithelial component of one mucinous carcinoid and in the intestinal type epithelium of 9 mature cystic teratomas.(ABSTRACT TRUNCATED AT 400 WORDS)     

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.     

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.     

Paraganglioma: report of a rare case with ovarian involvement

We report a well-documented case of paraganglioma involving right ovary, which was initially misdiagnosed as a Sertoli-Leydig cell tumor and recurred one year later. The right ovarian tumor measured 105x90x60 mm and was associated to a subdiaphragmatic tumor measuring 80x60x35 mm, a peritoneal and a preureteral nodules measuring 10 mm either. Microscopically, tumor cells were arranged in trabeculae and cords separated by a delicate stroma. Their cytoplasm was abundant granular and eosinophilic. Their nuclei were enlarged and regular in size with coarse chromatine and a large nucleolus. The tumor expressed neuroendocrine markers (chromogranin, synaptophysin) epithelial membrane antigen and focally cytokeratin 7 and E-cadherin. Pathological ovarian paraganglioma diagnosis could be difficult but one should be aware of its bona fide existence. The clinical course is favourable in most of the cases.     

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.     

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.     

Gynandroblastoma of the ovary: A case report with an immunohistochemical and ultrastructural study

An ovarian gynandroblastoma in a 60-year-old woman is described. The cut-surface of the right ovary showed multiple macrofollicles separated by white fibrous tissues and multiple ill-defined yellowish nodules. The tumour consisted of substantial amount of a granulosa cell element and a Sertoli cell element with intermingled Leydig cells. Immunohistochemically, the tumour cells in both the granulosa cell and Sertoli cell elements were positive for cytokeratin CAM5.2. The granulòsa cell element showed strong membrane staining of Ewing's sarcoma antigen 013 and the Sertoli cell element was focally positive. Vimentin was observed in both the Sertoli cell element and the granulosa cells. Both elements and the Leydig cells were uniformly negative for epithelial membrane antigen, muscle specific actin, CD31 and CD34. The tumour was aneuploid by flow cytometry. The patient was well with no evidence of tumour five months after surgery.     

Clear cell carcinoma of the ovary arising in a mucinous cystadenoma

A 57 year old woman presented complaining of increasing abdominal swelling of six months duration. A mixed solid cystic left ovarian tumour measuring 24 cm in diameter was excised. Histology showed numerous cysts lined by benign mucinous epithelium blending imperceptibly into borderline clear cell and mucinous areas that in turn merged with an invasive clear cell carcinoma. To the best of our knowledge, this is the first reported case of clear cell carcinoma arising in a mucinous cystadenoma. The implications for the previously postulated pathogenesis of these tumours are discussed.     

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.