Mural nodules in ovarian mucinous cystic tumors are uncommon, with only 80 cases reported over the last 30 yr. The literature describes only 5 cases of carcinosarcomatous mural nodules with mucinous ovarian neoplasm. We compared and summarized the literature related to mural nodules in mucinous ovarian tumors to elaborate on the clinical and histomorphologic features. A 21-yr-old woman presented with 2 mo history of abdominal distension. Physical examination showed a palpable pelvic mass. Radiologic investigation showed a 31×18.6×25 cm large right ovarian cyst. Few nodular solid masses were also seen, the largest mass measured 3.5×3.1 cm. On histomorphology and immunohistochemistry, it was a mucinous ovarian carcinoma with carcinosarcomatous mural nodules. Carcinosarcomatous mural nodules with ovarian mucinous neoplasm affects younger females. It presents at an early stage and does not carry an adverse prognosis.

Anaplastic carcinoma arising in a mucinous tumour of the ovary and rarely in the retroperitoneum is an uncommon neoplasm with three morphological patterns; rhabdoid, sarcomatoid and pleomorphic. We investigated expression of switch/sucrose non-fermentable (SWI/SNF) chromatin remodelling complex components and claudin-4 expression.

Twenty-two ovarian and three retroperitoneal mucinous tumours were investigated using antibodies against SMARCB1, SMARCA4, SMARCA2, ARID1A and claudin-4. Loss of nuclear staining for any SWI/SNF protein was observed in the anaplastic component of nine of 25 (36%), with retained expression within the mucinous component of all tumours. Five (56%) showed loss of more than one protein, with dual loss of SMARCA4 and SMARCA2 in two, loss of SMARCA2 and ARID1A in two and loss of SMARCB1 and SMARCA2 in one. Retained expression of claudin-4 was seen in 39% of the anaplastic carcinomas and within the mucinous component of all tumours. Rhabdoid morphology was associated with poor prognosis [stages III or IV disease (six of six, 100% versus four of 14, 29%; P = 0.0108] and death from disease (three of four, 75% versus one of 13, 8%; P = 0.0223). Although loss of a SWI/SNF protein was not significantly associated with death from disease (three of five, 60% versus one of 12, 8%; P = 0.0525), it showed a trend in correlation with poor prognosis and was often noted in tumours with rhabdoid morphology within this small cohort.

Our report adds to the growing list of female genital tract malignancies with loss of SWI/SNF proteins, underlining their broad differential diagnosis and the importance of careful, context-dependent interpretation of SWI/SNF protein loss.

Epithelial ovarian cancer development before menarche is extremely rare.

We report a prepubertal girl who developed ovarian mucinous carcinoma with mural carcinosarcomatous components.

Magnetic resonance imaging showed a polycystic mass with solid components. The left adnexa was removed. Histological analysis revealed a mucinous tumor with mural carcinosarcomatous components. Three weeks later, ascites and peritoneal metastasis were detected. The patient received a combination therapy of paclitaxel with carboplatin. After 4 chemotherapy cycles the right adnexa, uterus, partial omentum, and pelvic peritoneum were removed. Four additional paclitaxel/carboplatin therapy cycles were administered. She remains free from recurrence after 29 months. To our knowledge, this is the first report of ovarian mucinous carcinoma with mural carcinosarcomatous components in a prepubertal girl.

Carcinosarcoma mural nodules arising form a mucinous ovarian neoplasm is very rare and only two published cases have been reported. We report a case of a 29-year-old female patient who suffered from severe lower abdominal pain unrelated to menstruation for 1 year. She came to our (Shin-Kong Hospital) gynecology outpatient department in February 2010. The CT scan revealed a large cystic tumor, measuring approximately 36 cm in greatest dimension and at least 2 solid foci were noted. The patient underwent left salpingo-oophorectomy. A carcinosarcoma mural nodule arising within a mucinous ovarian neoplasm was diagnosed. The patient was treated by further chemotherapy and was free of the disease at time of publication. We reviewed the published studies, and in particular looked at the histology and immunohistochemistry of tumors, in which sarcomatoid carcinoma and carcinosarcoma like nodules were diagnosed. We also discussed the differential diagnosis of the mural nodule in a mucinous cystic neoplasm.

Cystic tumors of ovary, whether benign, borderline, or malignant may be associated with mural nodule of various types, including sarcomas, sarcoma-like mural nodules (SLMN), and foci of anaplastic carcinoma. Cases of serous borderline ovarian tumor with mural nodules of mixed type are very rare.

A 54-year-old woman referred with abdominal swelling. Imaging studies revealed a huge mass localized in pelvis and lower abdomen and grade 1-2 left renal hydronephrosis. Preoperative Ca-125 was 798 U/ml. In exploratory laparotomy there was a 16 cm mass adherent to lateral abdominal wall and intestines. Adhesiolysis and de-bulking surgery were performed including bilateral pelvic, para-aortic lymphadenectomy, appendectomy and omentectomy. Left ureter was found to be dilated because of the infiltration of distal part by the tumor, so distal ureteral resection and neoureterocystostomy were performed. Final pathology revealed borderline serous ovarian tumor with mural nodules which were consisted of SLMNs, multiple and sharply demarcated from the adjacent tumor, and sarcomatous nodules showing infiltrative appearance in metastatic regions. Mural nodules showed a positive reaction for vimentin and SMA but were negative for cytokeratin and also necrosis, hemorrhage, and 10-15 mitoses in 10 high power fields were noted. She had postoperative chemotherapy and follow-up is going on without metastases in her first year.

The existence of sarcomatous nodules combined with the SLMN necessitates a careful histologic analysis for treatment and the determination of prognosis. However, too few cases of mixed type mural nodules have been published to warrant a conclusion regarding their prognosis.

We first established a human ovarian sarcomatoid carcinoma cell line designated BUPH:OVSC from primary culture. The specimen was derived from the mural nodule in an ovarian mucinous tumor and cultured in vitro. To date, the cell line has been maintained for over 100 passages. Its biologic characteristics were studied by light and electron microscopy, which revealed spindle-shaped or polygonal cells with a doubling time of 39.5 h. The agglutination test of BUPH:OVSC was positive, and cell colonies were formed in soft agar. Chromosome analysis revealed its karyotype to be a pseudodiploidy. One X chromosome deletion and chromosome 20 addition were detected, and aberrant chromosomes t (1q;12q) and 14p(+) were its chromosome markers. BUPH:OVSC was tumorigenic in nude mice. Hematoxylin and eosin staining of transplanted tumors showed that the cells were morphologically sarcomatoid. However, the transmission electron microscopic observation exposed its epithelial origin. The cell line coexpresses cytokeratin and vimentin. It dose not appear to express estrogen and progesterone receptors or the CA125 tumor marker. Alcian blue/periodic acid-Schiff staining indicates that the cells could secrete acid mucopolysaccharide. In conclusion, BUPH:OVSC displays unique cellular properties, which make it a useful model for the study of human ovarian sarcomatoid carcinomas.

Epithelial ovarian tumors of borderline malignancy are tumors with histologic features and biologic behavior between benign and frankly malignant epithelial ovarian neoplasms. To date, we cannot accurately predict the patients who are prone to an aggressive course of disease. Here, we present a 35-year-old patient with carcinosarcoma-like mural nodule in intestinal-type mucinous ovarian tumor of borderline malignancy. Foci of intraepithelial carcinoma (about 10%) without stromal invasion are also noted. Total hysterectomy, bilateral salpingo-oophorectomy, appendectomy, and omentectomy were performed, and the frozen pathology during operation showed mucinous tumor of borderline malignancy of left ovary on April 18, 2002. The patient was followed at our outpatient department for 19 months after operation and was free of the disease without any adjuvant chemotherapy. It is difficult to determine whether intestinal-type borderline mucinous tumors with intraepithelial carcinoma are associated with a worse prognosis compared with those with epithelial atypia alone due to disparate results in the published literature. In contrast, most patients with mural nodules of anaplastic carcinoma have had a malignant, often rapid, course. However, too few cases of carcinosarcoma-like mural nodule in mucinous tumor have been published to warrant a conclusion regarding their prognosis.

Ten mucinous cystic ovarian tumors that contained sarcoma-like mural nodules are described. The nodules were studied by conventional and immunohistochemical methods. The sarcoma-like mural nodules occurred predominantly in middle-aged women, were multiple and sharply demarcated from the adjacent mucinous tumor, had small size, and exhibited a heterogeneous cell population. Distinction of these lesions from true sarcomatous nodules and foci of anaplastic carcinoma is important because of the worse prognosis of the two latter tumors compared with the favorable behavior of the sarcoma-like mural nodules. Six of the eight patients with follow-up information were alive and clinically free of recurrence at a mean follow-up interval of 12 years. Two patients died of other causes (thyroid and breast carcinomas). The nature of the nodules is not clear. Sarcoma-like mural nodules probably represent a reactive and self-limited phenomenon within a neoplasia. Their coexpression of vimentin and cytokeratins is consistent with an origin from submesothelial mesenchymal cells, which undergo partial transformation into epithelial cells.

A case of ovarian mucinous cystadenocarcinoma with malignant mural nodules is reported. The patient was a 28-year-old Japanese female (gravida 0, para 0) with a 2 year history of increasing abdominal fullness and edema of the lower extremity. Physical examination showed a large mass in the abdomen. An abdominal ultrasound and computed tomography demonstrated a multilocular cyst with a solid component. Lymph node and distant metastases were not found. These tests were followed by surgery. The resected right ovarian tumor measured 25 x 22 x 18 cm. On cut sectioning, it was multilocular with multiple mural nodules. Microscopically, the cyst wall was lined with papillary infoldings of atypical mucinous epithelium (intestinal type). Nuclear stratification, cribriform and back-to-back glandular patterns and stromal invasion were observed. In addition, the solid area of the mural nodules showed spindle or polygonal cells with increased mitotic activity including atypical mitoses. Nuclear pleomorphism was marked. Necrosis and hemorrhage were also present. Reticulin stain showed these pleomorphic cell clusters circumscribed by reticulin fibers and these cells were immunoreactive for vimentin and p53.

Mural nodules associated with mucinous and serous tumors of the ovary may represent a reactive process, a benign tumor, or a malignant neoplasm. Mural leiomyomatous nodule in mucinous cystadenoma is extremely rare. Two such cases had been described previously. In this case a 43-year-old white female presented with 24-h history of left quadrant pain and a left adenexal cystic mass on ultrasound examination. An exploratory laparotomy revealed a left ovarian mass with torsion on its pedicle. Frozen section of the cystic mass showed a mucinous cystadenoma with mural smooth muscle proliferation. A total abdominal hysterectomy and bilateral salpingo-oophorectomy were performed. Histologic examination of the mass revealed a mucinous cystadenoma with a mural leiomyomatous nodule and an enlarged ovary with massive stromal edema. This is the first case of a mural leiomyomatous nodule in association with a mucinous cystadenoma in an ovary with massive edema. This case broadens the histologic spectrum in which a mural leiomyomatous nodule may be encountered.

We present a rare case of a 31-year-old woman with a mucinous ovarian tumor with sarcoma-like mural nodules. The epithelial elements consisted of a mixture of benign, borderline-malignant, and mucinous adenocarcinoma; the sarcoma-like mural nodules consisted of pleomorphic and epulis-like cells. The lesion was resected for an ovarian tumor. In an immunohistochemical study, these cells stained for lysozyme and vimentin. The patient received 2 courses of cisplatin (100 mg/m2). There was no evidence of disease at a second-look laparotomy after 1 year, and none upon examination 6 years after the initial operation. The mural nodules might be of non-epithelial origin. The prognosis of patients with this rare lesion appears favorable, according to this and previous reports.