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.     

Intrarenal mature cystic teratoma associated with renal dysplasia: case report and literature review

We report a case of intrarenal teratoma in a 6-year-old boy. Two years before his operation, multicystic masses had been found in the left side of his abdomen. In the operation, three main cystic masses were located in the upper and lower poles of the left kidney, which were removed in pieces. Histologically, the cyst wall was lined mainly with keratinizing squamous epithelium with hair follicles, shafts and sebaceous glands. The adjacent renal parenchyma showed atrophy, with partially dysplastic and angiomyolipoma-like lesions. Based on these findings, the lesion was diagnosed as mature cystic teratoma of dermoid cyst type. Extragonadal teratoma occurs predominantly along the median line of the body. Intrarenal teratoma is extremely rare; however, it should be distinguished from teratoid Wilms' tumor and other renal cystic lesions.     

Primary carcinoid tumor arising in a mature teratoma of the kidney: a case report and review of the literature

Primary carcinoid tumor, especially that arising in a mature teratoma of the kidney, is extremely rare; only 3 cases have been reported in the world literature to date. Because of the rarity of the lesion, its histogenesis and prognosis are unknown. We report a case of primary renal carcinoid tumor occurring in a mature teratoma in a 30-year-old woman. A computed tomographic scan of the abdomen revealed a mass in the left kidney containing dense calcification with minimal contrast enhancement. Histologically, the tumor was composed of trabecular and anastomosing ribbonlike nests, identical to the features of carcinoid tumors of other sites. Immunohistochemical stainings were positive for cytokeratin, neuron-specific enolase, and chromogranin. In addition, there were mature teratoid tissues, such as columnar epithelium, smooth muscle, and bone. The carcinoid tumor was under and closely apposed to the lining of the cysts. The patient did not have clinical manifestations of the carcinoid syndrome and had an uneventful recovery.     

A case of small cell carcinoma arising in a mature cystic teratoma of the ovary

Malignant transformation of ovarian mature cystic teratomas is reported to occur in about 1% of cases. The most common malignant evolution is that of squamous cell carcinoma and one of the least common is that of small cell carcinoma. We report a case of distant metastasis from a small cell carcinoma arising in a mature cystic teratoma. A 28-year-old nulliparous female presented with signs of supraclavicutar lymph node metastasis. This Is the first known case report of lymph node metastasis as an Initial sign of cancer developing in a pre-existing mature cystic teratoma. Histologically, the tumor cells had microscopic features similar to those of pulmonary small cell carcinoma. This report gives a literature review, and the histological, Immunohistochemical, flow cytometric and ultrastructural features are described.     

A case of ovarian mature cystic teratoma presenting as a pedunculated ileal tumor

We report on a 34-year-old Japanese woman who presented with a pedunculated ileal tumor and who was finally diagnosed as having a right ovarian mature cystic teratoma penetrating and protruding into the ileum. She had undergone laparoscopic left ovarian cystectomy, whose specimen was diagnosed as dermoid cyst when she was 27 years old. The colon fiberscope revealed an ileal polyp, diagnosed as mature teratoma. Because of adhesion to the necrotic nodule between the tumor and the right ovary, ileocecal resection and right ovarian cystectomy were performed. The ileal tumor contained tissues of skin, neuroglia, ganglion, choroid, retina, smooth muscle, as well as fibrous and adipose tissues, cartilage, bone, mucous epithelium, and bronchial structures with bronchial glands. The necrotic nodule showed abscess, granulation tissue, foreign body reaction, hairs, normal ileal epithelium, and the ovary with ovums.     

Ovarian teratoma showing a predominant hemangiomatous element with stromal luteinization: report of a case and review of the literature

A solid well-circumscribed ovarian tumor in a 63-year-old Japanese woman is reported. Histologically, the tumor consisted predominantly of a hemangiomatous component with a small cyst lined by a respiratory ciliated epithelium. The remaining ovarian stroma showed a marked proliferation of luteinized cells that were strongly positive for inhibin immunohistochemically. Neither dermoid nor other teratomatous components were found, even in the serial sections of the tumor. To date, vascular tumors of the ovary are uncommon and some cases of ovarian hemangioma have been reported. However, their origin, whether a monodermal teratoma or a true neoplasm arising in the ovarian stroma, has not yet been elucidated. When a hemangiomatous proliferation is found in an ovarian tumor, a thorough microscopic search for teratomatous components is necessary before diagnosing the tumor as a pure hemangioma.     

Squamous cell carcinoma arising in a mature cystic ovarian teratoma with bladder invasion: a case report

Background

Malignant transformation in a mature cystic ovarian teratoma is rare. Except in cases with high index of suspicion or overt metastasis, oophorectomy is the mainstay of treatment for ovarian teratoma.

Method

A 46-year-old perimenopausal woman who had salpingo-oophorectomy following a clinical diagnosis of benign ovarian tumour that was subsequently reported histologically as mature cystic ovarian teratoma with malignant transformation is presented.

Results

She was referred to our facility based on the histopathology report and haematuria two weeks after surgery. Cystoscopic biopsy done was reported as metastatic squamous cell carcinoma most probably from the ovary. Patient was thereafter referred for radiotherapy but was lost to follow-up after the first course.

Conclusion

Adequate evaluation prior to surgery in suspected ovarian teratoma with malignant transformation is critical to determine extent of surgery and adjuvant therapy. Prognosis in advanced disease condition such as the case presented is generally poor although radical pelvic surgery with resection of the adjacent involved bladder before radiotherapy would probably have improved her prognosis.     

Mixed epithelial and stromal tumor of the kidney: report of a rare case and review of literature

Mixed epithelial and stromal tumor of the kidney (MESTK) is a rare benign tumor composed of epithelial and stromal cells. We report a rare male case with detailed clinicopathological data and follow-up information. The patient presented with gross hematuria. Computed tomography (CT) and magnetic resonance imaging study showed a 60 mm×40 mm cystic lesion with thickened septa and minimal contrast enhancement at the lower pole of the right kidney. The patient underwent nephron sparing surgery (NSS). Intraoperative frozen section showed benign histological features without significant cytologic atypia and mitosis. By additional immunohistochemistry investigations, the epithelial component was positive for cytokeratin-7, high molecular weight cytokeratin, and PAX-8. The stromal component showed strong positivity for vimentin and smooth-muscle actin. This case emphasizes that it is difficult to establish a precise diagnosis of MESTK preoperatively due to lack of any typical radiological features. Thus, intraoperative frozen section is of great clinical significance for NSS with preservation of kidney function. Additionally, regular follow-up is necessary for the MESTK with malignant potential.     

Carcinoid tumor in the female urethral orifice: rare case report and a review of the literature

We experienced an extremely rare tumor in the female urethral orifice in a 57-year-old Japanese woman. To our knowledge, only two cases of primary urethral carcinoid tumor have been reported. The previous reports of urethral carcinoid tumor were recognized in the male middle urethra and penile urethra. The present case was resected, and diagnosed as a carcinoid tumor by histological, immunohistochemical and ultrastructural findings. The tumor cells were stained by chromogranin A, synaptophsin and neuron-specific enolase, and neurosecretory granules were confirmed with electron microscopy. The patient did not complain of any symptoms until 5 years after the resection of the tumor. Therefore, the case we describe here is the first known report of carcinoid tumor in the Japanese female urethra.     

Fine-needle aspiration cytology of metastatic carcinoid tumor: report of a case and review of the literature

While carcinoid tumor is a relatively common neoplasm in surgical pathology, fine-needle aspiration (FNA) cytology as a method of primary diagnosis has only been reported in the literature a few times. We report on the case of a 42-yr-old female with multiple large metastatic tumor deposits in her liver, pelvic adnexae, bones, and lungs, with an unknown primary. FNA was performed on one of the liver masses, and was diagnosed using routine histochemical and immunohistochemical stains as carcinoid tumor. No follow-up tissue diagnosis has been obtained. However, the patient is still alive with her tumor 1 yr later. Fine-needle aspiration cytology can be a useful and safe tool in the diagnosis of metastatic carcinoid tumors, avoiding the need for surgery which would not otherwise be indicated for treatment.     

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.     

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.     

Fat-forming solitary fibrous tumor of the kidney: a case report and literature review

Fat-forming solitary fibrous tumor (SFT) is a rare soft tissue tumor. Herein, we reported a 30-year-old woman was found to have a solid mass measuring 60×45 mm in the right kidney on an abdominal computed tomography scan. The tumor was well-circumscribed and composed of cellular nodules with the classic SFT admixed with clusters and lobules of mature adipocytes. Immunohistochemistry staining showed that the tumor cells were diffusely and strongly positive for CD34 and Bcl-2, focally and weakly positive for CD99 and EMA. Mature adipocytes were positive for S-100 protein. Ki-67 expression was found in approximately 2% of tumor cells. However, tumor cells were negative for cytokeratin, S-100 protein, HMB-45, Melan-A, SMA, and CD117. We made the pathological diagnosis of fat-forming SFT of the right kidney. The differential diagnosis includes angiomyolipoma, liposarcoma, spindle cell lipoma, sarcomatoid renal cell carcinoma, synovial sarcoma, and gastrointestinal stromal tumor. The patient was alive and well without evidence of recurrence or metastasis at 19 months after tumor resection.     

Carcinoid tumor associated with adjacent dysplastic columnar epithelium in the renal pelvis: A case report and literature review

Carcinoid tumors are well documented in the pulmonary and gastrointestinal systems, but very rare in the urinary tract, especially in the renal pelvis. We report on a 60‐year‐old female patient who presented with left flank pain and fever. Abdominal computed tomography demonstrated a heterogeneously enhancing mass in the left renal pelvis and a stone at the left proximal ureter. Multiple parenchymal lesions were also observed, which were identified as uneven caliectasis displaying air‐fluid levels and renal parenchymal atrophy. The patient underwent simple nephro‐ureterectomy. Macroscopically, a polypoid mass was observed in the renal pelvis. Microscopically, the tumor revealed acinar, tubular, and solid pattern and was composed of small, monotonous and hyperchromatic cells. Lining epithelia in renal pelvis and ureter revealed columnar epithelia with dysplastic change. The tumor cells were positive for chromogranin A, synaptophysin, CD56, and focally positive for cytokeratin. Immunohistochemical staining of synaptophysin and chromogranin A highlighted the neuroendocrine cells in the columnar epithelium. Ki‐67 (1:50; MIB‐1) labeling index was less than 1% in the area with highest uptake. We report here a case of carcinoid tumor of the renal pelvis that was associated with adjacent dysplastic columnar epithelium.     

Coexisting Brenner tumor and struma ovarii in the right ovary: case report and review of the literature

A bilateral ovarian tumor composed of mixed Brenner tumor and struma ovarii in the right ovary and mature cystic teratoma in the left ovary, is described. Mixed Brenner tumor and struma ovarii is rare; eight cases are reviewed. In this case, in addition to the typical Brenner tumor and struma ovarii, some nests composed of both Brenner tumor and struma ovarii in one nest were found in the right ovarian tumor. Immunohistochemically, the struma ovarii is stained for thyroglobulin, and Brenner nests showed various degrees of positive stain for thyroglobulin, which is a specific finding. Brenner tumor, in this case, may produce thyroglobulin or have a receptor to thyroglobulin or analog of thyroglobulin. The origin of mixed Brenner tumor and struma ovarii may be germ cell, as described in some literature, or the Brenner tumor may be of a metaplastic nature, although the Brenner tumor is fourfold the size of struma ovarii in the case presented.     

Malignant rhabdoid tumor arising in a mixed epithelial,stromal tumor of kidney: report of a male case,review of the literature

Mixed epithelial and stromal tumor (MEST) of the kidney is a rare biphasic tumor composed of both stromal and epithelial components, the latter showing a variable proportion of solid to cystic areas. These tumors show a marked female predominance, commonly occur in perimenopausal age groups, and often have an ovarian-type stroma with ER and PR positivity, suggesting steroids may play a role in pathogenesis. Although typically benign, rare cases showing malignant transformation have been reported. We present a case of a 42-year-old man with a 10 cm right kidney mass located in the renal pelvis. Histologically, the majority of the tumor was composed of a diffuse, sheet-like growth of malignant cells demonstrating a rhabdoid morphology with large nuclei, prominent nucleoli, and eosinophilic eccentric cytoplasm. Brisk mitotic activity and coagulative type necrosis was also noted. Intimately associated with this malignant rhabdoid component was a much smaller portion of tumor featuring variably sized bland epithelial tubules embedded within a stroma composed of bland spindle cells and areas of hyalinization, diagnostic of MEST. By immunohistochemistry, the malignant rhabdoid tumor portion of the neoplasm showed complete loss of nuclear INI-1, while the MEST component retained nuclear expression of this antigen. With these features taken together, our case represents a malignant rhabdoid tumor arising in a background of MEST. To our knowledge, this case represents the first case of a MEST showing malignant transformation in the form of malignant rhabdoid tumor in a male patient in the English language literature.     

Glomus tumor of renal pelvis: a case report and review of the literature

Glomus tumors are uncommon benign perivascular neoplasms that have rarely been described outside of their usual peripheral soft tissue sites. We report a unique case of glomus tumor of the renal pelvis in a 53-year-old woman who presented with microscopic hematuria associated with obstruction of the ureteropelvic junction and marked hydronephrosis. At initial gross examination, the tumor mimicked a urothelial carcinoma.     

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 juxtaovarian yolk sac tumor concurrent with an ipsilateral ovarian mature teratoma in an adult woman: a rare association

Objectives: Yolk sac tumor (YST) is a rare neoplasm that primarily occurs in the ovary in children and young women. Previously, it has been reported that the teratomatous components can be recognized in part of YSTs or appear in the contralateral ovary. Here, we report a rare case of an adult woman with a juxtaovarian YST concurrent with an ipsilateral ovarian mature teratoma. Methods: A 47-year-old woman found a pelvic mass for nine days and subsequently underwent debulking operation. The specimens were evaluated for detailed characterizations through gross examination, microscopy and immunohistochemistry. A literature review was performed and the pathogenesis was briefly discussed about the generation of an extraovarian YST concurrent with a teratoma. Results: The right juxtaovarian tumor showed typical histological patterns of YST. Immunostaining demonstrated the YST nature of Gly-3 and AFP positive tumor cells. The ipsilateral ovarian tumor was a common mature cystic teratoma with chronic fibrotic changes. According to the differences of the origin and the differentiation of the two germ cell tumors, we suspect that the occurrence of the teratoma is earlier than the YST. Conclusions: To our knowledge, this is the first report of an adult woman with a juxtaovarian YST concurrent with an ipsilateral ovarian mature teratoma.     

A young man with primary prostatic extra-gastrointestinal stromal tumor: a rare case report and review of the literature

Mounting evidence demonstrates the presence of extragastrointestinal stromal tumor (EGIST) which originates from tissues outside the gastrointestinal (GI) tract and shares overlapping immunohistological features with gastrointestinal stromal tumor (GIST). GIST emanating from prostate is extremely rare. To our knowledge, there are only 3 definitely reported cases of primary prostatic EGIST. Herein, we report a case of prostatic EGIST in 31-year-old man with low urinary tract symptoms who was initially misdiagnosed as sarcoma of prostate. Imaging studies assist in determining the origin and location of EGIST. Immunohistochemical assessment (DOG-1, CD117, and CD34) helps in differentiating such lesion from other stromal tumors and in addressing an appropriate and optimal therapeutic strategy.