Sarcomatous Component Research Articles

Angiosarcoma After Treatment of Primary Mediastinal Yolk Sac Tumor

SESSION TITLE: Student/Resident Case Report Poster - Disorders of the Mediastinum SESSION TYPE: Student/Resident Case Report Poster PRESENTED ON: Tuesday, October 25, 2016 at 01:30 PM - 02:30 PM INTRODUCTION: Germ cell tumors are often composed of several different cell types. Rarely, these tumors can include sarcomatous components, such as rhabdomyosarcoma or angiosarcoma, which may be realized after failure of chemotherapy to reduce tumor size. Currently, given the poor response rate of systemic therapy for angiosarcoma, surgical resection is considered the most effective therapy. Monitoring response to therapy with tumor markers and imaging is important, as it may identify chemo-refractory malignancies. CASE PRESENTATION: A 54-year-old male without prior medical history presented with dyspnea and 10-pound weight loss. His initial imaging identified a 5x13x8 cm anterior mediastinal mass; labs were notable for an elevated alpha fetoprotein (AFP). Biopsy of the mass revealed a yolk sac tumor. He was received four cycles of combination chemotherapy for non-seminomatous germ cell tumor with etoposide, ifosfamide, and cisplatin. Despite normalization of the AFP level, a repeat CT scan revealed that the mediastinal mass had increased in size. A repeat biopsy of the anterior mediastinum showed angiosarcoma; biopsy of a sacral lesion detected on SPECT scan also showed angiosarcoma, consistent with metastatic disease. The mediastinal tumor was partially resected, and the patient was subsequently initiated on paclitaxel. DISCUSSION: This case demonstrates the importance of monitoring for progression of germ cell tumors with serial imaging, in addition to tumor markers. Similar to other case reports, this patient experienced normalization of his tumor markers, but demonstrated increasing tumor size on imaging. Detecting the expanding tumor prompted a concern for the presence tumor cells that were unaffected by treatment, which, in this case, were revealed to be those of angiosarcoma. Because angiosarcomas are very unlikely to respond to chemotherapy, the prognosis is poor. Novel therapies are under investigation. CONCLUSIONS: Imaging is necessary in addition to tumor markers to identify unexpected outcomes in the course of treatment of germ cell tumors. Reference #1: Mediastinal Germ Cell Tumors with an Angiosarcomatous Component: A Report of 12 Cases. Alejandro Luina Contreras, MD et al. Hum Pathol. 2010 June; 41 (6): 832-837 Reference #2: Poor prognosis of mediastinal germ cell cancers containing sarcomatous components. Gonzalez-Vela et al. Cancer. 1990 Sep 15;66(6): 1114-6. Reference #3: Germ cell tumors with sarcomatous components: a clinicopathologic and immunohistochemical study of 46 cases. Malagon HD, Valdez AM, Moran CA, Suster S. Am J Surg Pathol. 2007 Sep;31(9):1356-62 DISCLOSURE: The following authors have nothing to disclose: Huy Do, Janine Vintch, Yvonne Carter, James Yeh, Justin Darrah No Product/Research Disclosure Information

Carcinosarcoma of the Pancreas: Case Report of a Rare Type of Pancreatic Neoplasia

Carcinosarcoma of the pancreas: case report of a rare type of pancreatic neoplasia Introduction Carcinosarcoma of the pancreas is a rare type of pancreatic neoplasms and only few cases of carcinosarcoma have been reported in the literature. Carcinosarcoma of the pancreas have cells recognizable as adenocarcinoma as well as sarcomatous components. We present a rare case of pancreatic carcinosarcoma. Case Presentation: An 85 year-old male was transferred to our hospital for further management of pancreatic mass. He presented to outside hospital with complains of abdominal pain, jaundice and weight loss. Imaging showed a mass in the head of the pancreas. Endoscopic ultrasound (EUS) showed a 7.5 cm X 7.0 cm hypoechoic mass in the head of pancreas (figure 1). EUS guided fine needle biopsy suggested an atypical spindle and myxoid type sacrcoma. No evidence of distant metastasis was identified. An extended pancreaticoduodenectomy was performed. We will plan for adjuvant chemotherapy after adequate postoperative recovery. Grossly, the tumor consisted of a 12.0 x12.0 x 8.0 cm soft mass localized in head of the pancreas with extension into duodenum and peripancreatic soft tissue (Figure 2). Histologically, the tumor showed two components. Sections of the tumor showed a high-grade sarcomatoid component with myxoid features, some floret like cells, many mitotic figures and geographic areas of necrosis. There was focal cartilaginous differentiation. In addition there was a glandular component consistent with invasive ductal adenocarcinoma (Figure 3). Immunostains were also used in evaluation and the overall findings were consistent with carcinosarcoma (pT3pNo).Figure 1Figure 2Figure 3Discussion: Carcinosarcomas are rare tumors characterized by a carcinomatous and a sarcomatous component. Carcinosarcomas are predominantly located in the uterus and only few cases of carcinosarcoma of pancreas have been reported in literature. These tumors have cellular elements derived from two different histological origins proliferating in one tumor. Although controversial but several studies have suggested a monoclonal origin for carcinosarcomas using diverse immunohistochemical and molecular analyses. EUS guided core needle biopsy of pancreatic mass was not specific and only showed the sarcomatous component in our patient. Based on the very limited number of reported cases, the prognosis of carcinosarcoma of the pancreas appears dismal.

Carcinoma Fallopian Tube with Sarcomatous Component in the Metastatic Omental Deposit: A Rare Event

Background Carcinosarcoma (malignant mixed mullerian tumour) is a rare malignancy of the female genital tract and rarer to arise from the fallopian tube. Approximately 60 cases are reported in world literature so far. Carcinosarcoma with the sarcoma component only in the metastatic deposit is highly uncommon.

Sarcomatoid renal cell carcinoma: genomic insights from sequencing of matched sarcomatous and carcinomatous components.

The estimated new kidney cancer cases diagnosed each year in the United Sates and in the world are ~63,000 and ~300,000, respectively (1,2). Renal cell carcinoma (RCC) represents over 90% of kidney cancer and consists of a group of malignancies arising from the renal epithelium and exhibiting distinct histopathological features (3-6). The 2004 WHO classification listed 12 different subtypes of RCC (4). With a better understanding of the molecular pathogenesis of RCC, the 2013 International Society of Urological Pathology (ISUP) consensus conference added several new entities (6).

Abstract 131: Allelic imbalance analysis of uterine carcinosarcoma: An inquiry into the dual nature of the neoplasm

Abstract Uterine carcinosarcoma (UCS), also known as Malignant Mixed Mullerian Tumor, is a neoplasm of the female genital tract that shows histological features of both carcinoma and sarcoma. Some studies have indicated that UCS arises from sarcomatous differentiation of high-grade carcinoma while others have confirmed a bi-clonal nature of the tumor. Given these differences, further investigation in the origin of this tumor with newer approaches and technologies is warranted. In this study we determined the allelic imbalance (AI) profiles of the two components of UCS and compared the AI events that were shared by, or differed between, them. Samples were obtained from 10 patients diagnosed with UCS and were preserved in formalin fixed paraffin embedded (FFPE) blocks. The two components (carcinoma & sarcoma) were identified and micro-dissected, and whole-genome DNA micro-arrays were applied to the isolated DNA. We then applied a sensitive computational method that combines haplotype information with SNP array data to identify somatic segmental copy number variations and copy-neutral loss of heterozygosity (cnLOH). After we obtained the AI events we filtered small events (<2 MB) to rule out germline duplications. We then obtained events that are common to both components by using a criterion of 80% reciprocal overlap. All such events were classified as a gain, loss or cnLOH. We found that, on an average, 80% of the events found in the carcinoma component are common with the sarcoma component. Conversely, only 64% of events seen in the sarcoma component are found in the carcinoma. This may be in part due to the fact that the sarcoma component usually showed greater number of events and most of these events were larger than the corresponding carcinoma component. Also the sarcoma component showed an increased degree of allelic imbalance than the counterpart. We then looked into particular regions of known tumor suppressor and oncogenes and identified loss in the TP53 gene region (60% of tumors), gains in PIK3CA and PTEN gene regions (40% of tumors), among other events. Our analysis showed that the sarcomatous component shared major portions of the AI profile with the carcinomatous component, but also showed additional events and a greater degree of AI. One explanation for this observation can be that the sarcomatous component retains the AI profile of the carcinomatous component and also gathers additional imbalance. This suggests directionality to the development of UCS: that it arises by sarcomatous differentiation of already existing carcinoma of the uterus. We are pursuing additional assessments of point mutations and expression profiles to complement our existing analyses. Citation Format: Aditya S. Deshpande, Zachary Weber, Raed Sulaiman, Natasha Flier, Cheryl Ageton, Mary Fagerness, Joseph Sulaiman, Gareth E. Davies, David Starks, Luis Rojas-Espaillat, Paul A. Scheet, Erik Ehli. Allelic imbalance analysis of uterine carcinosarcoma: An inquiry into the dual nature of the neoplasm. [abstract]. In: Proceedings of the 107th Annual Meeting of the American Association for Cancer Research; 2016 Apr 16-20; New Orleans, LA. Philadelphia (PA): AACR; Cancer Res 2016;76(14 Suppl):Abstract nr 131.

Abstract 5190: Correspondence between clinical patient outcome and in vivo carboplatine response of high grade ovarian cancer PDX

Abstract Survival rate for high grade ovarian cancer (HGOC) remains low and has not significantly evolved for several years. This poor outcome is due to several mechanisms of resistance (initial or acquired) in different context (Homologous recombination deficience, CCNE1 amplification). We developed a collection of High-grade ovarian cancer Patient Derived Xenografts (PDX) with extensive characterization (CGH, transcriptome and exome sequencing, Colombo et al, Oncotarget. 2015 Sep 29;6(29):28327-40). Our objective is to create a panel of HGOC PDX which encompasses main types of platinium sensitivity/resistance in HGOC. We selected 10 PDX models (9 serous histiotypes and one carcinosarcomas) among our collection of 39 HGOC PDXs from two patient extreme platinium response categories (resistant with a progression-free inferior to 6 months and sensible with a progression-free higher than 18 months, some of the sensitives with BRCA1 mutations). These PDXs included PDXs were grafted sub-cutaneously on nude mice and submitted to carboplatin treatment (50mg/kg twice a week, for 4 weeks) and impact on tumor growth was evaluated as well as histological characterization. For the carcinosarcomas, no selection of the epithelial or sarcomatous component upon treatment was observed. Outgrowth of the tumors after end of treatment is currently under assessment. Remarkably, tumor PDXs response was in accordance with clinical status with complete and partial response for the sensitive PDXs; slowed growth under treatment or stabilization for resistant PDXs. Outgrowth of the tumor after the end of treatment is currently under assessment. For each PDXs model, the large majority of the 7-9 treated tumor reacted in identical fashion if classified according to RECIST criteria with occasionally divergent responses reflecting tumor heterogeneity. Genetic and histological characterization of these outliers is ongoing. This collection is a valuable resource to model carboplatine resistance in a well-defined context and will be the cornerstone of our future studies to conduct preclinical drugs tests, in particular for combination selected in vitro and to evaluate clonal evolution under treatment. Citation Format: Stan P. du Manoir, Helene Delpech, Beatrice Orsetti, Jeremy Tessier, Pierre-Emmanuel Colombo, Charles G. Theillet. Correspondence between clinical patient outcome and in vivo carboplatine response of high grade ovarian cancer PDX. [abstract]. In: Proceedings of the 107th Annual Meeting of the American Association for Cancer Research; 2016 Apr 16-20; New Orleans, LA. Philadelphia (PA): AACR; Cancer Res 2016;76(14 Suppl):Abstract nr 5190.

Significance of histologic pattern of carcinoma and sarcoma components on survival outcomes of uterine carcinosarcoma

To examine the effect of the histology of carcinoma and sarcoma components on survival outcome of uterine carcinosarcoma. A multicenter retrospective study was conducted to examine uterine carcinosarcoma cases that underwent primary surgical staging. Archived slides were examined and histologic patterns were grouped based on carcinoma (low-grade versus high-grade) and sarcoma (homologous versus heterologous) components, correlating to clinico-pathological demographics and outcomes. Among 1192 cases identified, 906 cases were evaluated for histologic patterns (carcinoma/sarcoma) with high-grade/homologous (40.8%) being the most common type followed by high-grade/heterologous (30.9%), low-grade/homologous (18.0%), and low-grade/heterologous (10.3%). On multivariate analysis, high-grade/heterologous (5-year rate, 34.0%, P = 0.024) and high-grade/homologous (45.8%, P = 0.017) but not low-grade/heterologous (50.6%, P = 0.089) were independently associated with decreased progression-free survival (PFS) compared with low-grade/homologous (60.3%). In addition, older age, residual disease at surgery, large tumor, sarcoma dominance, deep myometrial invasion, lymphovascular space invasion, and advanced-stage disease were independently associated with decreased PFS (all, P < 0.01). Both postoperative chemotherapy (5-year rates, 48.6% versus 39.0%, P < 0.001) and radiotherapy (50.1% versus 44.1%, P = 0.007) were significantly associated with improved PFS in univariate analysis. However, on multivariate analysis, only postoperative chemotherapy remained an independent predictor for improved PFS [hazard ratio (HR) 0.34, 95% confidence interval (CI) 0.27-0.43, P < 0.001]. On univariate analysis, significant treatment benefits for PFS were seen with ifosfamide for low-grade carcinoma (82.0% versus 49.8%, P = 0.001), platinum for high-grade carcinoma (46.9% versus 32.4%, P = 0.034) and homologous sarcoma (53.1% versus 38.2%, P = 0.017), and anthracycline for heterologous sarcoma (66.2% versus 39.3%, P = 0.005). Conversely, platinum, taxane, and anthracycline for low-grade carcinoma, and anthracycline for homologous sarcoma had no effect on PFS compared with non-chemotherapy group (all, P > 0.05). On multivariate analysis, ifosfamide for low-grade/homologous (HR 0.21, 95% CI 0.07-0.63, P = 0.005), platinum for high-grade/homologous (HR 0.36, 95% CI 0.22-0.60, P < 0.001), and anthracycline for high-grade/heterologous (HR 0.30, 95% CI 0.14-0.62, P = 0.001) remained independent predictors for improved PFS. Analyses of 1096 metastatic sites showed that carcinoma components tended to spread lymphatically, while sarcoma components tended to spread loco-regionally (P < 0.001). Characterization of histologic pattern provides valuable information in the management of uterine carcinosarcoma.

Pancreatic carcinosarcoma with the same KRAS gene mutation in both carcinomatous and sarcomatous components: molecular evidence for monoclonal origin of the tumour.

To better understand the histogenesis, prognosis and feasible treatment of pancreatic carcinosarcoma, a rare type of neoplasia. We investigated eight additional cases of pancreatic carcinosarcoma at a single institution, including the clinicopathological, immunohistochemical, and KRAS mutation characteristics. We have also reviewed the current literature on this rare type of neoplasia, and summarized the clinicopathological features and feasible treatments. As a result, concordant strong nuclear immunoreactivity for P53 protein and the same type of KRAS gene mutation, c.35G>A (p.G12D) or c.35G>T (p.G12V), were showed in both carcinomatous and sarcomatous components in five of eight cases. Furthermore, we found that the patients treated with surgery plus postoperative chemotherapy had longer survival than those treated with surgery only (P = 0.034 and P = 0.131 for overall survival and disease-free survival, respectively), although Cox regression multivariate analysis indicated that it was not an independent predictor. In addition, we found KRAS mutant allele-specific imbalance in four of our five cases of pancreatic carcinosarcoma, which was associated with advanced disease and a worse prognosis. This is the largest panel of cases of pancreatic carcinosarcoma studied so far, including clinicopathological, immunohistochemical and molecular cytogenetic features. Our findings indicate that the tumour could have been of monoclonal origin, and that the sarcomatous components might have arisen from metaplastic transformation of the carcinomatous components. Our results also suggest that surgery plus POC including gemcitabine may be a good choice for patients with pancreatic carcinosarcoma.

Characterization of actionable somatic alterations in microdissected gynecologic carcinosarcomas.

5594Background: Gynecologic carcinosarcomas (CS) are rare tumors most often arising in the uterus or ovary that histologically contain both carcinomatous and sarcomatous components. CS is thought t...

GHRH Receptor Expression in Malignant Mixed Müllerian Tumors: A Potentially Targetable Biopredictor.

Malignant mixed Müllerian tumors (MMMTs) are aggressive malignant neoplasms with a high recurrence rate and poor prognosis. Despite advances in adjuvant therapies in recent years, the prognosis of these tumors has not improved. Growth hormone-releasing hormone (GHRH) is produced by a variety of malignant tumors and acts as a growth factor in an autocrine/paracrine manner. Its function requires the presence of its receptors to exert its effects on neoplastic cells. In this study, we evaluated the expression of GHRH receptors (GHRH-R) in a group of MMMTs. Thirty-one examples of MMMTs from endometrium, ovary, uterine tube, and pelvic peritoneum were retrieved from the files of Department of Pathology at the University of Miami, Jackson Memorial Hospital. Immunohistochemistry for GHRH-R was performed on paraffin sections and the staining results were evaluated separately in both epithelial and mesenchymal components of each tumor. The presence of pituitary type growth hormone-releasing hormone receptor mRNA and that of its biologically active splice variant were also evaluated by RT-PCR in 6 of the tumors. Positive immunohistochemical reaction for GHRH-R was detected in 30 tumors (96%). The epithelial and sarcomatous components were positive in 30 (96%), whereas one endometrial tumor was negative in both components. The mRNA for GHRH-R and its splice variant was found in all 6 tested tumors. This study shows that GHRH-R is expressed by the majority of MMMTs in both epithelial and mesenchymal components. This finding could potentially serve as a basis for therapeutic approaches using synthetic peptide antagonists of GHRH-R that have shown significant efficacy with minimal side effects in experimental models.

An Unusual Site of Metastasis of Malignant Mixed Müllerian Tumor of the Uterus

Malignant mixed Mullerian tumors (carcinosarcomas) are biphasic tumors of the uterus and uterine cervix with carcinomatous and sarcomatous components. Malignant mixed Mullerian tumors (MMMT) are rare tumors, accounting for <5% of uterine cancers. They most commonly metastasize to the vagina, pelvic wall, lungs, and ovaries. We report a unique case of MMMT, which developed in a 69-year-old woman after she presented with hip and knee pain. Ultimately, her pain was found to be caused by metastatic tumor deposits in her psoas muscle. The primary tumor was found to be a MMMT of the uterine corpus. We recommend practitioners be aware of this entity and include it and other metastatic lesions in their differential diagnosis in difficult cases of non-resolving pain.

Cooperation of Sox4 with β-catenin/p300 complex in transcriptional regulation of the Slug gene during divergent sarcomatous differentiation in uterine carcinosarcoma.

BackgroundUterine carcinosarcoma (UCS) represents a true example of cancer associated with epithelial-mesenchymal transition (EMT), which exhibits cancer stem cell (CSC)-like traits. Both Sox and β-catenin signal transductions play key roles in the regulation of EMT/CSC properties, but little is known about their involvement in UCS tumorigenesis. Herein, we focused on the functional roles of the Sox/β-catenin pathway in UCSs.MethodsEMT/CSC tests and transfection experiments were carried out using three endometrial carcinoma (Em Ca) cell lines. Immunohistochemical investigation was also applied for a total of 32 UCSs.ResultsEm Ca cells cultured in STK2, a serum-free medium for mesenchymal stem cells, underwent changes in morphology toward an EMT appearance through downregulation of E-cadherin, along with upregulation of Slug, known as a target gene of β-catenin. The cells also showed CSC properties with an increase in the aldehyde dehydrogenase (ALDH) 1high activity population and spheroid formation, as well as upregulation of Sox4, Sox7, and Sox9. Of these Sox factors, overexpression of Sox4 dramatically led to transactivation of the Slug promoter, and the effects were further enhanced by cotransfection of Sox7 or Sox9. Sox4 was also able to promote β-catenin-mediated transcription of the Slug gene through formation of transcriptional complexes with β-catenin and p300, independent of TCF4 status. In clinical samples, both nuclear β-catenin and Slug scores were significantly higher in the sarcomatous elements as compared to carcinomatous components in UCSs, and were positively correlated with Sox4, Sox7, and Sox9 scores.ConclusionsThese findings suggested that Sox4, as well as Sox7 and Sox9, may contribute to regulation of EMT/CSC properties to promote development of sarcomatous components in UCSs through transcriptional regulation of the Slug gene by cooperating with the β-catenin/p300 signal pathway.Electronic supplementary materialThe online version of this article (doi:10.1186/s12885-016-2090-y) contains supplementary material, which is available to authorized users.

Clinical outcome of gliosarcoma compared with glioblastoma multiforme: a clinical study in Chinese patients

Gliosarcoma (GSM) is a rare biphasic neoplasms of the central nervous system composed of a glioblastoma multiforme (GBM) admixed with a sarcomatous component. In clinical practice GSM is generally managed similarly to GBM. However, there are conflicting reports regarding their clinical aggressiveness, cell line of origin and possible prognosis compared with those of GBM. The objective of this study was to compare clinic-pathological features in GSM patients with the GBM patients during the same study period. 518 patients with GBM were treated at our hospital between 2008 and 2013, among them 51 were GSM. In this series the GSMs represented 9.8% of all GBMs and included 58.8% male with a median age of 44.7 years. The locations, all supratentorial, included temporal in 41.2%, frontal in 25.5%, parietal in 19.6%, and occipital in 13.7%. All patients underwent tumor resection followed by post-operative radiation and adjuvant chemotherapy. The O6-methylguanine-DNA methyltransferase promoter methylation studies were significantly more frequent in the GBMs than GSMs (80.1% vs. 44.7%, P < 0.001). The median progression free survival and overall survival for the patients with GSM were 8.0 and 13.0 months, respectively, as compared with 9.0 and 14.0 months in the GBM group (log rank test P = 0.001 and 0.004, respectively). The Cox proportional hazards regression model indicated that the extent of tumor resection (HR = 1.518, P = 0.009) and pathological types (HR = 0.608, P = 0.002) were the significant prognostic factors in our own series. With regard to clinical features and outcomes, GSM and GBM cannot be distinguished clinically. GSM in China may be managed similarly to GBM, with maximal safe surgical resection followed by chemo-radiotherapy. Our study adds further evidence to support GSM as a unique clinical entity with a likely worse prognosis than GBM.

Key Features in the Management of Pulmonary Carcinosarcoma.

Pulmonary carcinosarcoma represents a category of extremely rare tumours accounting for 0.1% of all lung malignancies. It is defined as a poorly differentiated non-small-cell carcinoma that contains a component of sarcoma or sarcoma-like elements. These biphasic tumours typically have a poor prognosis due to late diagnosis and early metastases. Preoperative tissue diagnosis is usually difficult due to the heterogeneity of the tumour, with biopsies often just reflecting one element of the tumour. By means of a case illustration and review of the literature, we discuss the optimal management of patients with pulmonary carcinosarcoma.

Carcinomes sarcomatoïdes pulmonaires

Pulmonary sarcomatoid carcinomas are a rare group of tumors accounting for about one percent of non-small cell lung carcinoma (NSCLC). In 2015, the World Health Organization classification united under this name all the carcinomas with sarcomatous-like component with spindle cell or giant cell appearance, or associated with a sarcomatous component sometimes heterologous. There are five subtypes: pleomorphic carcinoma, spindle cell carcinoma, giant cell carcinoma, carcinosarcoma and pulmonary blastoma. Clinical characteristics are not specific from the other subtypes of NSCLC. Epithelial to mesenchymal transition pathway may play a key role. Patients, usually tobacco smokers, are frequently symptomatic. Tumors are voluminous more often peripherical than central, with strong fixation on FDG TEP CT. Distant metastases are frequent with atypical visceral locations. These tumors have poorer prognosis than the other NSCLC subtypes because of great aggressivity, and frequent chemoresistance. Here we present pathological description and a review of literature with molecular features in order to better describe these tumors and perhaps introduce new therapeutics.

Cytogenetic Findings of an Undifferentiated Testicular Sarcoma

Primary testicular sarcomas are extremely rare. In contrast, germ cell tumors (GCTs) with sarcomatous components (SCs) that can have similar pathologic features are more frequently reported. We report a primary undifferentiated testicular sarcoma with cytogenetic analysis for amplification of 12p. A 36-year-old male initially presented with 9.4 cm right testicular tumor and no metastatic disease. He was lost to follow-up but re-presented 2 years later with tumor growth to 21 cm and multiple pulmonary and bone metastases. Histologically, the testicular tumor revealed an undifferentiated sarcoma. Fluorescence in situ hybridization (FISH) of paraffin-embedded tissue showed no amplification of 12p. Gain of isochromosome 12p (i(12p)) is a specific chromosomal anomaly present in most GCTs. The cytogenetic analysis indicated the possibility that the tumor was not of GCT origin. This is the first report of primary testicular undifferentiated sarcoma with cytogenetic analysis for i(12p). J Med Cases. 2016;7(1):7-12 doi: http://dx.doi.org/10.14740/jmc2356w

Duodenal Metastasis of Pulmonary Pleomorphic Carcinoma: A Case Report

Pleomorphic carcinomas of the lung form a subgroup of general carcinomas exhibiting pleomorphic, sarcomatoid, or sarcomatous elements. The World Health Organization defines pleomorphic carcinoma of the lung as non-small cell lung cancer (with a carcinomatous or epithelial component) combined with neoplastic spindle or giant cells (with a sarcomatous or mesenchymal component) or as a carcinoma consisting only of spindle and giant cells (1). Pulmonary pleomorphic carcinoma associated with intraperitoneal metastasis is extremely rare. We report an instance of the duodenal metastasis of a pulmonary pleomorphic carcinoma, in addition to providing a review of the literature.

A Rare Case of Prostatic Extra Gonadal Germ Cell Tumor in a Young Adult: Review of the Literature

We report the case of a 29 year-old male who presented with hemospermia. A biopsy revealed an embryonal rhabdomyosarcoma of the prostate and the patient was treated with pre-operative chemotherapy according to the pediatric protocol IVAD (Ifosfamide - Actinomycin - Doxorubicin – Vincristine) followed by prostatectomy. Pathologic examination of the prostate showed the persistence of an immature teratoma without residual rhabdomyosarcoma. The choice of consolidation chemotherapy with a platinum-based regimen was based on the diagnosis of an extra-gonadal non-seminomatous germ cell tumor with an excellent clinical outcome. Extragonadal germ cell tumors of the prostate in adults are very rare and aggressive. The presence of a sarcomatous component represents a poor prognostic factor and it may pose diagnostic pitfalls just like in the case we report. Early diagnosis and multimodality treatment are necessary to improve overall survival.

Dedifferentiated chondrosarcoma of right proximal femur

Dedifferentiated chondrosarcoma (DDCS) comprises approximately 10% of all chondrosarcomas. The tumor generally occurs after the age of 50 years, with equal distribution among males and females. It is most often located in the bones of the pelvis, the proximal femur or humerus, the distal femur, and the ribs. Regardless of treatment, the prognosis is ominous with 90% of patients dying with distant metastases within 2 years. We report a case of DDCS of right proximal femur in a 73-year-old woman. The most important factors affecting survival are early recognition of the radiographic features, adequate histological sampling, and wide-margin resection of the lesion. So for the correct histopathological diagnosis the biopsy sample should be adequate to visualize both cartilaginous and noncartilaginous sarcomatous component which helps to increase the survival of patients before the distant metastasis occurs.

Epithelial Malignancies Arising in Ovarian Mature Cystic Teratoma: A Series, of 26 Cases

Objective: To report a series of epithelial malignancies arising within ovarian teratomas along with clinical followup. Some previously unreported combinations of malignant component with MCT are also being reported. Material and Methods: Clinico-pathological details and clinical follow up of twenty six cases reported during January 2000 to March 2014 in the Section of Histopathology, of a tertiary care hospital of Pakistan are included. Results: Total 1418 cases of MCTs were reported, 26 (1.8%) of them showed epithelial malignancies. The mean age at presentation was 42.5 years (range; 15-70 years). Commonest clinical presentation was with abdominal pain and mass. All the patients underwent surgery as first line treatment. Malignant transformation in form of Squamous cell carcinoma was seen in 20 cases. In one case, in addition to squamous cell carcinoma, atypical proliferative serous tumor and high grade sarcomatous component was also present. Adenosquamous carcinoma, Poorly differentiated carcinoma, Endometrioid adenocarcinoma and Adenocarcinoma with mucinous differentiation represented malignant components in additional 4 cases respectively. Cases demonstrating endometrioid features and atypical proliferative serous tumor represent collision tumors. Two of the cases had papillary carcinoma (follicular variant) of thyroid arising within monodermal teratoma (struma ovarii). Clinical follow up was available in 13 cases and it is noted that patients with squamous cell carcinoma had a worse outcome. Conclusions: Malignancy occurring in a MCT is an extremely rare occurrence. It is very challenging to diagnose this malignant transformation clinically.