Primary squamous cell carcinoma of the ovary. Review of the literature.

PURPOSE: Primary squamous cell carcinoma (SCC) of the ovary is rare. Most cases arise from a cystic teratoma or less frequently from Brenner tumor or endometriosis. We reviewed 36 cases of primary ovarian SCC reported in the literature including a case diagnosed and treated in our institution. METHODS: Data was collected by using the key-words "primary squamous cell carcinoma" and "ovary" on Google Scholar and PubMed in April 2018. All reviewed cases were analyzed according to diagnosis, surgical approach, adjuvant therapy and outcome. RESULTS: To date 23 articles presenting 36 cases of primary ovarian SCC are reported. Nine patients had stage I, 8 stage II, 11 stage III and 5 stage IV disease, whereas 3 patients had in situ carcinoma. All patients underwent surgery (mainly hysterectomy with bilateral salpingo-oophorectomy). Adjuvant therapy was reported in 24 patients, 15 of which received chemotherapy, 6 radiotherapy and 3 a combination of both. Chemotherapy regimens were similar to the ones used in ovarian carcinoma (more often platinum plus paclitaxel). Follow-up period was in general short and survival varied between 9 days and 14 years, depending on the stage at diagnosis. CONCLUSIONS: Primary ovarian SCC is a rare entity with poor prognosis, compared to serous carcinoma. Treatment is usually extrapolated from classical ovarian carcinoma algorithms, including surgical management combined with adjuvant chemotherapy with or without radiotherapy. Further investigations are needed to define optimal treatment, such as chemotherapy regimens and the role of radiotherapy and lymph node dissection.

Synchronous presentation of GISTs and other primary neoplasms: a single center experience.

BACKGROUND: Gastrointestinal stromal tumors (GISTs) are common mesenchymal neoplasms of the digestive tract and may occasionally arise within the abdomen without gastrointestinal tract connection. GISTs have recently attracted widespread interest because of the development of effective targeted molecular agents against it. While synchronous occurrence of a GIST with a tumor of different histogenesis was thought to be very rare, it is now apparent that they are more common than previously believed. PATIENTS AND METHODS: We report our experience with GISTs and also six cases of GIST coexisting with other primary neoplasms. Using immunohistochemistry and mutational analysis, a possible correlation was investigated. A review of the literature was also conducted. RESULTS: There were no significant differences in the immumohistochemical and molecular profile between single GISTs and GISTs coexisting with other tumors, nor was there any mutational correlation between GISTs and the coexistent tumors of different histogenesis regarding KIT and PDGFRA genes. CONCLUSION: Further molecular biology studies are required in order to investigate thoroughly the simultaneous development of tumors with different histotypes.

Expression of E-cadherin, beta-catenin and topoisomerase IIalpha in leiomyosarcomas.

INTRODUCTION: The expression of E-cadherin, beta-catenin and topoisomerase II has been associated with clinical outcome of several cancers including sarcomas. We aimed to evaluate the expression of these markers in leiomyosarcomas (LMS). MATERIALS AND METHODS: Paraffin blocks of 19 primary, nonmetastatic LMS were analysed immunohistochemically for the expression of the above-mentioned markers with a cutoff level for positivity of 20% of cell staining. RESULTS: Expression of E-cadherin was negative in all LMS. Nuclear expression of beta-catenin was also negative in all cases, while positive cytoplasmic beta-catenin expression was observed in approximately half of the patients. The majority of LMS had expression of topoisomerase IIalpha, although only in 10 patients was this expression in more than 20% of tumour cells. From the analysed factors, tumour size was statistically significantly correlated with relapse-free survival. CONCLUSIONS: Further evidence with larger series is required in order to determine the implication of these markers in LMS.

Successful en bloc resection of primary hepatocellular carcinoma directly invading the stomach and pancreas.

Multivisceral surgical resection for cure was successfully performed in a 70-year-old man suffering from a primary hepatocellular carcinoma (HCC) associated with direct invasion to the stomach and pancreas. The patient presented with gastric outlet obstruction, upper abdominal pain and a history of chronic liver disease due to hepatitis B virus (HBV) infection. Upper gastrointestinal (GI) endoscopy revealed an infiltrating tumor protruding through the gastric wall and obliterating the lumen. Computer tomograghy (CT) and magnetic resonance imaging (MRI) scan demonstrated a 15-cm tumor in the left lateral segment of the liver with invasion to the stomach and pancreas. Alpha-foetoprotein (AFP) levels and liver function tests were normal. The patient underwent an en bloc left hepatectomy, total gastrectomy, distal pancreatectomy with splenectomy and radical lymphadenectomy. Pathology revealed a poorly differentiated, giant cell HCC involving the stomach and pancreas. Disease-free margins of resection were achieved. The patient's postoperative course was uneventful. Sixteen months after surgery, he has no recurrence or distal metastasis. Direct invasion of HCC into the GI tract is rarely encountered. Complete surgical resection should be considered in selected patients with an appropriate hepatic functional reserve.

Concomitant gastric adenocarcinoma and stromal tumor in a woman with polymyalgia rheumatica.

Gastrointestinal stromal tumors (GISTs) are rare neoplasms (1%) of the gastrointestinal tract and to our knowledge only rare cases of synchronous presentation of gastric carcinomas and GISTs are reported in the literature. A 72-year-old female with a simultaneous presentation of gastric adenocarcinoma and GIST is presented. Moreover, due to polymyalgia rheumatica the patient received corticosteroids as treatment for the last 3 years. The concomitant occurrence of these neoplasms may involve common carcinogenic factors and there could be an association with polymyalgia rheumatica either as a paraneoplastic presentation or due to its treatment with corticosteroids.

Primary and metastatic ovarian cancer in patients with prior breast carcinoma. Pre-operative markers and treatment results.

BACKGROUND: The pre-operative diagnosis of primary and metastatic malignant ovarian tumors in patients treated for breast cancer is difficult. The objective of this study was to analyze and compare the characteristics and outcome of women with a history of breast cancer in order to identify pre-operative markers useful in differential diagnosis and the role of surgery in their management. MATERIALS AND METHODS: The medical records of 36 patients with a history of breast cancer, who had been operated on either for primary or metastatic cancer between 1987 and 2003, were reviewed retrospectively. RESULTS: Twenty-seven patients had been diagnosed with primary epithelial ovarian cancer (POC) and nine had metastatic disease (MOC), resulting in a 3:1 ratio. The median age of breast and ovarian cancer diagnosis was 45 and 56 years, respectively, and the median interval was 8 years. The serum CA 125 level was elevated in the majority of cases, in 70% of the POC group and 56% of the MOC, but the median level was higher, though not statistically significant, in the former. Serum CA 15-3 levels were elevated >100 U/ml in 89% of patients with MOC (p = 0.0002). BrCA mutation risk, as calculated with the BRCAPRO software program, was 41.8% and 9% in primary and metastatic tumors, respectively (p=0.0477). Ovarian spread was not the only site of metastatic breast cancer in 55.5% of the MOC group, compared to 11% of the POC patients Disease was disseminated in the abdominal cavity at the time of diagnosis in both groups, however, 78% of patients had unilateral tumors in the POC group and bilateral disease in the MOC (p=0.0133). Cytoreduction to less than 2 cm tumor diameter was feasible in 67% of primary and 44% of metastatic neoplasms. In the follow-up period (12-204 months), the median survival was 10 months for patients with metastatic disease, compared to 33 months for those with primary tumors (p<0.05). CONCLUSION: Small bilateral ovarian enlargements and minor serum elevation of CA 125 titers in patients with initial Stage IV breast cancer, suffering from multiple metastatic disease, are likely to illustrate MOC. Unilateral ovarian mass and high serum levels of CA 125 in apparently disease-free patients with a positive family history and high prevelance of BRCA mutations are suggestive of primary tumors. Optimal cytoreduction was feasible in both groups, but survival was longer in patients with primary tumors (p<0.05).

Solitary parenchymal splenic recurrence of ovarian adenocarcinoma: a case report and review of the literature.

We report a rare case of solitary parenchymal splenic recurrence of epithelial ovarian cancer which developed 27 months after the initial treatment. The patient, a 53-year-old woman, with a history of breast cancer, underwent total abdominal hysterectomy bilateral salpingo-ophorectomy (TAH & BSO), omentectomy and pelvic lymph node sampling for a serous carcinoma of the ovaries (stage IIIB). She subsequently received 6 cycles of cisplatinum chemotherapy. During follow-up, rising CA 125 serum levels heralded the 6 x 6 cm parenchymal splenic lesion which was documented by CT scan. She underwent splenectomy after pneumococcal vaccination, sandostatin and chemoprophylaxis. Histopathological evaluation revealed metastatic parenchymal disease consistent with recurrent ovarian cancer. She remains alive and disease-free for 20 months since the last operation. Isolated parenchymal splenic lesions are very rare and may occur as a late recurrence in epithelial ovarian cancer. Splenectomy can be performed with acceptable morbidity and confers a substantial survival benefit to patients.

Unusual metastatic spread of follicular thyroid carcinoma: report of a case.

Concurrent skull and liver metastases from follicular thyroid carcinoma is a very rare event. We herein present the case of a 72-year-old woman who initially presented with a swelling in the right supraorbital region that proved to be metastasis from a well-differentiated follicular thyroid carcinoma of clear-cell type. The metastatic workup disclosed a huge liver metastasis and an additional metastasis in the left iliac fossa. The treatment of this patient included a total thyroidectomy, an excision of the skull lesion, and the administration of radioiodine therapy, as well as thyroid-stimulating hormone (TSH) suppression therapy. However, the course of her disease was relentless. Although well-differentiated thyroid carcinoma tends to show an excellent course, the presence of metastatic disease leads to a very dismal prognosis.

Metaphase and interphase cytogenetics in fibroadenomas of the breast.

Short-term cultures of fifty-two samples of fibroadenomas were cytogenetically analyzed. Thirty-three of the successfully karyotyped fibroadenomas were further investigated for the presence of amplifications in the CCND1, c-MYC and HER/2-neu genes by means of FISH analysis. Compared to carcinomas, fibroadenomas seem to have less complex cytogenetic rearrangements and limited alterations on HER-2/neu, CCND1 and c-MYC loci. A cytogenetic subgroup of fibroadenomas with hyperdiploid karyotypes and only numerical changes was observed. Amplification of CCND1 seems to play a more substantial role in benign tumor progression. These findings confirm that fibroadenomas do have genetic alterations and support the hypothesis that a fibroadenoma subset displays changes also found in carcinomas, thus indicating that patients belonging to this group might have an increased risk for subsequent breast cancer.

Cytogenetic profile of unknown primary tumors: clues for their pathogenesis and clinical management.

Unknown primary tumors (UPTs) represent an entity of great clinical and biological interest, whose origin cannot be determined even after medical workup. To better understand their pathogenesis by outlining their genetic composition, 20 UPTs were investigated by G-banding, supplemented with Fluorescence In Situ Hybridization and Comparative Genomic Hybridization analyses. The data obtained were sufficient to reach a diagnosis in five cases-four lymphomas and one Ewing sarcoma-demonstrating that in a subset of UPTs, cytogenetics can be an adjunct for differential diagnosis. In the remaining 15 UPTs, an aggressive cytogenetic pattern was revealed. The most frequently rearranged chromosome regions were 1q21, 3p13, 6q15-23, 7q22, 11p12-5, and 11q14-24, pinpointing gene loci probably associated with the peculiar pathogenesis of UPTs. The preferential involvement of 4q31, 6q15, 10q25, and 13q22 in adenocarcinomas (whereas 11q22 is involved in the rest of the carcinomas)-in addition to the marked divergence in the mean average of chromosomal changes, 16 and 3, respectively-demonstrates genotypic differences between the two histologic subgroups. Furthermore, the significantly shorter survival in cases displaying massive chromosome changes compared with those having a few changes indicates that the cytogenetic pattern might be used as a tool to assess prognosis in UPTs, even without the detection of their primary site.