[Primary hepatic sarcomatoid carcinoma, an unusual case]. / Carcinoma sarcomatoide hepático primario metastásico, una neoplasia infrecuente.

Primary hepatic sarcomatoid carcinoma is a very aggressive tumor, representing 0.4-0.7% of all primary hepatic neoplasms. The disease is associated with liver disease due to hepatotropic viruses and is more prevalent in Asians. Histology shows sarcomatous and carcinoma components. It does not have pathognomonic clinical or imaging characteristics and its diagnosis is based on the pathological and immunohistochemistry findings. Surgery could prolong survival in localized stages. We report the case of a 72-year-old Korean patient with a history of chronic liver disease due to B virus, who was diagnosed with primary hepatic sarcomatoid carcinoma with bone and lymph node metastases.

El carcinoma sarcomatoide primario hepático es un tumor agresivo que representa el 0.4-0.7% de todas las neoplasias primarias hepáticas. Se asocia a hepatopatía por virus hepatotropos, es más prevalente en la población asiática y en su histología se evidencian componentes de carcinoma y sarcoma. No posee características clínicas ni imagenológicas patognomónicas y su diagnóstico se realiza en base a los hallazgos de la anatomía patológica e inmunohistoquímica. La cirugía en estadio localizado representa la única modalidad terapéutica con impacto en la sobrevida. Reportamos el caso de una paciente de 72 años, coreana, con antecedentes de hepatopatía crónica por virus B, a quien se le diagnosticó un carcinoma sarcomatoide hepático primario con metástasis ósea y ganglionares.

A comparative analysis of anatomopathological features and COX-2 expression of mammary neoplasms with malignant mesenchymal components in female dogs / Análise comparativa das características anatomopatológicas e expressão de COX2 em neoplasias mamárias com componentes mesenquimais malignos em cadelas

Canine mammary neoplasms with malignant mesenchymal components, such as carcinosarcomas and sarcomas, belong to an uncommon and histologically heterogeneous group. Little is known about the biological behavior of these histogenic variants. This study aimed to compare the clinicopathological characteristics and the COX-2 immunohistochemical expression of different histologic subtypes of carcinosarcomas and sarcomas. Samples of 23 carcinosarcomas and 15 sarcomas from the mammary glands of female dogs were studied. Medical records were reviewed to obtain clinical data. Subsequently, histology microscope slides were analyzed to assess for mesenchymal subtypes, necrosis, vascular invasion, histologic grades, and lymph node metastasis. Immunohistochemistry was used to assess the COX-2 expression. The malignant mesenchymal proliferation was categorized into osteosarcomas (23/40), fibrosarcomas (5/40), liposarcomas (6/40) and chondrosarcomas (4/40). The osteosarcomatous differentiation was the most predominant type among the sarcomas and carcinosarcomas and was associated with vascular invasion (P=0.010) and lymph node metastases (P=0.014). High COX-2 expression was detected in 14.3% of the carcinosarcomas (carcinoma and/or sarcoma cells) and 27.3% of the sarcomas. The carcinosarcomas and sarcomas had similar clinical and pathological characteristics and developed as large tumors, with intratumoral necrosis and a predominance of high histologic grades, although the frequency of vascular invasion and lymph node metastasis was low. Osteosarcoma subtypes presented more aggressive characteristics than non-osteosarcoma subtypes.

Neoplasias mamárias caninas com componentes mesenquimais malignos, como carcinossarcomas e sarcomas, são um grupo de neoplasias pouco frequentes e histologicamente heterogêneas e pouco se sabe sobre o comportamento biológico das variantes histogênicas. O objetivo desse estudo é comparar as características anatomopatológicas e a expressão imunoistoquímica de COX-2 de diferentes subtipos histológicos de carcinossarcomas e sarcomas. Foram estudados 23 carcinosarcomas e 17 sarcomas da glândula mamária de cadelas. Os prontuários médicos foram revisados para obtenção de dados clínicos. Posteriormente, as lâminas histológicas foram avaliadas para acessar os subtipos mesenquimais, necrose, invasão vascular, grau histológico, metástase linfonodal. A imunoistoquímica foi realizada para avaliar a expressão de COX-2. Os tipos encontrados de proliferação mesenquimal maligna foram osteossarcoma (23/40), fibrossarcoma (7/40), lipossarcoma (6/40) e condrossarcoma (4/40). A diferenciação osteossarcomatosa foi predominante entre os sarcomas e carcinossarcomas e foi associado com invasão vascular (P=0,006) e metástase linfonodal (P=0,014). Uma expressão alta de COX-2 foi detectada em 14,3% dos carcinossarcomas (células carcinomatosas e/ou sarcomatosas) e 27,3% dos sarcomas. Os carcinossarcomas e sarcomas apresentaram características clínicas e patológicas semelhantes e se desenvolveram como tumores grandes, com necrose intratumoral e predomínio de alto grau histológico, mas com baixa frequência de invasão vascular e metástase distante. Os subtipos osteossarcomatosos apresentaram características mais agressivas quando comparados com subtipos não osteossarcomatosos.

Sinonasal teratocarcinosarcoma: a therapeutic dilemma.

Sinonasal teratocarcinosarcomas (SNTCSs) are rare and aggressive malignant tumours with histological features of the three embryonic layers. They have an elevated local recurrence rate, risk of metastasis and mortality. Moreover, the therapeutic options are limited, and optimal management is not yet clear. As fewer than 150 cases have been reported, therapeutic strategies remain a clinical challenge. Here, we discuss a case of a large SNTCS successfully treated with surgical resection followed by concurrent chemotherapy and radiation. Despite the significant size of the tumour and the inferred high recurrence risk, the patient has had no recurrence over the past 45 months. Although the optimal treatment of SNTCS is not clearly outlined, the very limited data suggests that a multidisciplinary approach with surgery, radiation and chemotherapy is the best option for patients.

Odontogenic Carcinosarcoma: Clinicopathologic Features of 2 Cases.

This study reports 2 odontogenic carcinosarcomas, including the clinicopathologic and immunoprofile characteristics of these rare tumors. The first case occurred in a 22-year-old male presenting a bilobular mass involving the gingiva and bone of the premolar region of the left mandible, with paresthesia of the lower lip. Microscopic examination revealed a tumor similar to ameloblastic fibrosarcoma, with atypical mesenchymal cells; however, the odontogenic epithelium also showed atypia. In the second case, a 16-year-old female had a painless, asymptomatic, large intraosseous mandibular lesion. The patient received radiotherapy to treat a rhabdomyosarcoma of the parotid 13 years before. The tumor was composed of atypical spindle cells, positive for vimentin and smooth muscle actin, intermingled with malignant odontogenic epithelium. Both epithelial and mesenchymal components of the tumors showed high index of p53- and Ki67-positive cells. The first case was diagnosed as odontogenic carcinosarcoma possibly originated from an ameloblastic fibrosarcoma, and the second as de novo odontogenic carcinosarcoma possibly caused by previous radiotherapy.

Clinicopathologic Diagnostic and Prognostic Factors of Spindle Cell Carcinoma of Upper Airway.

Spindle cell carcinoma (SpCC) is a rare tumor, which occurs in upper respiratory tract, mainly in larynx. This study aimed to review the clinical and pathological characteristics for diagnosis and prognosis. Retrospective cohort study. All patients with SpCC in upper respiratory tract treated for curative intent was included. All patients were reviewed in search of epithelial component and immunohistochemistry when not found. It was evaluated rate of recurrence and disease-free survival with univariate and multivariate analysis with Kaplan Meier and Cox Regression model adjusted to propensity score indexes (PSI) according to age, gender, site of tumor, stage, surgical treatment, status of margins of surgical resection, lymphatic invasion. There were 16 cases of SpCC.31% were diagnosed with light microscopy and others with immunohistochemistry for epithelial marker. Disease-free survival was higher in early stage disease in univariate and multivariate analysis, as the main prognostic factor. Surgical treatment increases in 2.54 the rate of survival. The SpCC is a rare tumor considered a highly malignant variant of squamous cell carcinoma. It has male predominance and tobacco use as risk factors. Its treatment should follow the same recommendations for squamous cell carcinoma, with surgery as the maintain treatment. Immunohistochemistry is an adjuvant important tool for diagnosis of SpCC.

Carcinosarcoma of the ampulla of Vater.

Carcinosarcomas, are very rare tumors in gastrointestinal tract, and at the ampulla of Vater location, are extremely uncommon. They are also called spindle cell carcinomas or sarcomatoid carcinomas. These tumors have an aggressive clinical course with frequent metastasis. We report the case of a male patient of 64 y.o with anemia and jaundice, and a diagnosis of carcinosarcoma of the ampulla of Vater.

Odontogenic carcinosarcoma: morphologic and immunohistochemical description of a case.

OBJECTIVES: The goal of this study was to describe an extremely rare case of odontogenic carcinosarcoma and compare the findings with those of a literature review. STUDY DESIGN: The clinical and pathologic data of an odontogenic carcinosarcoma affecting the posterior maxilla of a 42-year-old male patient was described. The lesion was immunostained for cell-cycle, cytokeratin, and mesenchymal markers. A review of literature from 1960 to 2017 was conducted in a search for similar well-documented case reports. Descriptive statistics were calculated to compare clinical and pathologic variables. RESULTS: In the reported case, the percentage of Ki-67-positive epithelial and mesenchymal cells was estimated as 40% and 25%, respectively. Epithelial cells were focally positive for cytokeratin 7, -8, -14, and -18, and diffusely positive for cytokeratin 19, p53, and p16. Mesenchymal cells were strongly positive for desmin, HHF-35, and vimentin. Our review showed that odontogenic carcinosarcoma is diagnosed mostly in the advanced stage. All patients with relapsed tumors had died as a result of the disease. CONCLUSIONS: Very few cases have been reported in the literature supporting that most odontogenic carcinosarcoma develop in the posterior mandible in a wide age range, without gender and racial predilections. Only one case of odontogenic carcinosarcoma in the maxilla other than the one described here has been reported. Until today, the best treatment remains unknown.

Odontogenic carcinosarcoma: A systematic review.

The aim of this study was to integrate the available data published on odontogenic carcinosarcoma into a comprehensive analysis of their features, treatment and recurrence. An electronic search with no publication date or language restriction was undertaken in March 2018 in the following databases: Medline Ovid, PubMed, Web of Science, Scopus and LILACS. Eligibility criteria included publications having enough clinical, imaginological and histopathological information to confirm a definite diagnosis of the neoplasm. Data were evaluated descriptively and statistically using the MedCalc software. The Kaplan-Meier method was used for survival analysis. The systematic review detected nine articles from eight countries. Six cases with no age predilection occurred in male individuals complaining of painful swelling in the posterior mandible. Radiographically, the lesions were large, with expansive radiolucency and with ill-defined borders and seven cases were associated with preexisting odontogenic lesions. Radical surgery was the treatment of choice in the majority of cases. Recurrences (n = 6), metastasis (n = 4) and death (n = 4) were frequently observed in many cases. Odontogenic carcinosarcoma is a very aggressive neoplasm with a poor prognosis. This study provides knowledge that could help surgeons, oncologists, otorhinolaryngologists and oral maxillofacial pathologists with the diagnosis and management of these lesions.

The demographic features, clinical outcomes, prognosis and treatment options for patients with sarcomatoid carcinoma of the urinary bladder: a single centre experience

ABSTRACT Introduction Carcinosarcoma of the bladder is a very rare neoplasm. The pathogenesis of carcinosarcomas is not clearly understood and remains a subject of debate. Whilst there is some research conceptualizing the histopathological findings of bladder carcinosarcomas, the demographic features, clinical outcomes, prognosis and treatment options remain unclear. Materials and Methods We analyzed 12 consecutive cases of patients with sarcoma-toid bladder cancer who were treated surgically at a single Urology Department be-tween 1999 and 2015. Radiology, pathology and surgical reports were reviewed to determine the pathological staging at the time of cystectomy. These were directly compared with 230 patients having cystectomies for urothelial cell carcinoma. The sarcomatoid patients, were compared to patients with urothelial cell cancers. The other histological sub types, squamous cell (17), neuroendocrine (9), metastatic (7), mixed (4), adenocarcinoma (3), were not included. Results and conclusion Carcinosarcoma of the urinary bladder is often described in the literature as a highly malignant neoplasm that is rapidly lethal. We found that the sarcoma does not offer a worse prognosis than conventional high-grade urothelial car-cinoma. There is no significant difference in grade, stage, positive surgical margin rate, nodal involvement, associated prostate cancer or incidence rates of progression, all cause or disease specific mortality. There was a barely significant difference in carcinoma in-situ. However, carcinosarcomas are three times the volume of urothelial cell tumors which may contribute to its reputation as an aggressive tumour (44cc v 14cc). Sarcomatous elements do not appear, from our small study, to bestow a worse prognosis.

Cutaneous Metaplastic Carcinoma: Report of a Case With Sebaceous Differentiation.

In this study, a case of a 56-year-old woman with cutaneous metaplastic carcinoma in the neck is reported. It harbored basal cell carcinoma, squamous cell carcinoma, and sebaceous carcinoma, as well as osteosarcoma (with fibroblastic, osteoblastic, aneurysmal bone cyst-like, and chondroblastic patterns). To our knowledge, the literature does not mention sebaceous carcinoma in cutaneous metaplastic carcinoma. Epidemiology and intact mismatch repair proteins of this case support sporadic pathogenesis for this neoplasm. The patient has been followed up for 3 years after surgery with free margins, and no local recurrence or distant metastases have been observed.

The demographic features, clinical outcomes, prognosis and treatment options for patients with sarcomatoid carcinoma of the urinary bladder: a single centre experience.

INTRODUCTION: Carcinosarcoma of the bladder is a very rare neoplasm. The pathogenesis of carcinosarcomas is not clearly understood and remains a subject of debate. Whilst there is some research conceptualizing the histopathological findings of bladder carcinosarcomas, the demographic features, clinical outcomes, prognosis and treatment options remain unclear. MATERIALS AND METHODS: We analyzed 12 consecutive cases of patients with sarcoma-toid bladder cancer who were treated surgically at a single Urology Department be-tween 1999 and 2015. Radiology, pathology and surgical reports were reviewed to determine the pathological staging at the time of cystectomy. These were directly compared with 230 patients having cystectomies for urothelial cell carcinoma. The sarcomatoid patients, were compared to patients with urothelial cell cancers. The other histological sub types, squamous cell (17), neuroendocrine (9), metastatic (7), mixed (4), adenocarcinoma (3), were not included. RESULTS AND CONCLUSION: Carcinosarcoma of the urinary bladder is often described in the literature as a highly malignant neoplasm that is rapidly lethal. We found that the sarcoma does not offer a worse prognosis than conventional high-grade urothelial car-cinoma. There is no significant difference in grade, stage, positive surgical margin rate, nodal involvement, associated prostate cancer or incidence rates of progression, all cause or disease specific mortality. There was a barely significant difference in carcinoma in-situ. However, carcinosarcomas are three times the volume of urothelial cell tumors which may contribute to its reputation as an aggressive tumour (44cc v 14cc). Sarcomatous elements do not appear, from our small study, to bestow a worse prognosis.

Carcinosarcoma de laringe: reporte de dos casos / Carcinosarcoma of the larynx: report of two cases

El carcinosarcoma de laringe es un tumor bifásico raro que representa menos del 1% de todos los tumores malignos de laringe. Debido a su doble naturaleza epitelial y mesenquimal esta neoplasia ha sido denominada de distintas maneras en la literatura, siendo indispensable el estudio mediante inmunohistoquímica para establecer un diagnóstico correcto. Se presentan 2 casos de carcinosarcoma de laringe, confirmados mediante estudio con inmunohistoquímica, ambos tratados mediante laringectomía total. Se elabora una discusión de los principales aspectos clínicos, histopatológicos y terapéuticos de esta infrecuente neoplasia.

The larynx carcinosarcoma is a rare biphasic tumor that represents less than 1% of all malignant tumors of the larynx. Because of its biphasic epithelial and mesenchymal nature this neoplasm has been called in different ways in the literature being indispensable the study by immunohistochemistry to establish a proper diagnosis. We present 2 cases of larynx carcinosarcoma confirmed by immunohistochemical study, both treated with total laryngectomy. A discussion of the main clinical, histopathological and therapeutic aspects of this rare neoplasm is made.

Cytogenetic aspects of a canine breast carcinosarcoma - a case report.

This study searched a rare and aggressive type of cancer in dogs and humans, the breast carcinosarcoma. Both clinical and pathological traits of mammary carcinosarcomas in dogs are similar to humans, such as infrequent occurrence, fast tumor growth, and unfavorable prognosis when compared to carcinomas. Other possible alterations include chromosomal abnormalities that can be useful for the identification of tumoral cells and diagnosis. The aim of this study was to compare the chromosomal features of peripheral lymphocytes and tumor cells in a mammary carcinosarcoma of a 14-year-old female Poodle. Chromosomes were analyzed from 210 metaphases by conventional Giemsa staining, C-banding, and base-specific fluorochrome staining with chromomycin A3 (CMA3+) and DAPI. Of the 105 blood cells, 56.3% followed the standard karyotype of dogs (2n = 78). In contrast, the carcinosarcoma cells showed high chromosomal numbers (104 to 153), divided into 80% hypertriploid (118 to 136 chromosomes), 10.5% hypotetraploid (137 to 153 chromosomes), 5.7% hypotriploid (104 to 116 chromosomes), and 3.8% triploid cells (117 chromosomes). Among the aneuploid cells identified, we highlighted the trisomy of pair 1 and X chromosome once these elements were easily recognized in karyotype because of their size (pair 1) or differential morphology. Heterochromatin in normal cells was restricted to the pericentromeric region of all chromosomes while few C-bands were observed in tumor cells. This apparent loss of heterochromatin in neoplastic cells was supposed to favor centric fusion among formerly acrocentric chromosomes. Fluorochrome staining reinforced this hypothesis once GC-rich segments (CMA3+) were identified on 10 chromosomes from normal cells (2n = 78) whereas carcinosarcoma metaphases had up to 11 chromosomes bearing CMA3 signals in spite of their remarkable high chromosomal numbers. We concluded that, like in humans, the carcinosarcoma in dogs caused genome instability that eventually led to structural and numerical chromosomal aberrations. Besides, this study reinforced the importance of cytogenetic studies in dogs as a reference material for human cancer studies, especially in rare cases, since it is possible to increase knowledge about the characteristics of breast neoplasms in which there is a little availability of similar cases for comparative studies.

Carcinosarcoma of the bladder following local schistosomiasis infection.

A young patient from central Africa presented acute renal insufficiency due to extrinsic compression of the distal ureters by a pelvic mass. After initial medical management, a biopsy revealed poorly differentiated bladder cancer and Schistosoma haematobium eggs embedded in the bladder wall. The initial workup showed evidence of locoregional disease. Radical cystectomy with an incontinent urinary diversion was performed with no complications. Carcinosarcoma of the bladder was diagnosed by pathological analysis of the surgical specimen. After a short follow-up, the patient was readmitted presenting with lung and bone metastases. At 60 days after diagnosis, he died of respiratory insufficiency caused by pulmonary metastatic disease.

Cutaneous invasion from sarcomatoid urothelial carcinoma: clinical and dermatopathologic features.

In Brazil, without considering the non-melanoma skin tumors, bladder cancer in men is the eighth most common, and the urothelial carcinoma or transitional cell carcinoma is the most common among these. Cutaneous metastases from urothelial neoplasms appear as single or multiple erythematous, infiltrated nodules or plaques, and like other cases of distant disease, it is indicative of poor prognosis. The invasive urothelial carcinoma is recognized for its ability to present divergent differentiation and morphological variants. The sarcomatoid urothelial carcinoma is a rare cancer that consists of two different components: one composed of epithelial tissue and the other with sarcomatoid features of mesenchymal origin. The authors describe a case of cutaneous metastasis of sarcomatoid urothelial carcinoma in a 63-year-old male patient.

An uncommon response to metronomic therapy in a heavily pretreated patient with metastatic carcinosarcoma: a case report.

BACKGROUND: Uterine carcinosarcoma is well known for its aggressive behavior. There is little evidence regarding the gold standard combination chemotherapy in metastatic or locally advanced carcinosarcoma, due to poor survival outcomes obtained with conventional scheduled chemotherapy. This case report represents the first-ever reported objective response to a metronomic chemotherapy regimen and adds to the current literature. CASE PRESENTATION: We describe a case of a Caucasian woman diagnosed with metastatic carcinosarcoma that had already been treated with multiple lines of conventional chemotherapy, with progressive disease. This patient had a surprising clinical and imaging response when treated with oral metronomic cyclophosphamide. CONCLUSIONS: We reviewed the mechanism of action implicated in metronomic chemotherapy, and correlated it with the biology of disease in carcinosarcoma. This information may add to the current literature, providing important insights to future clinical trials in this patient population.

Cutaneous invasion from sarcomatoid urothelial carcinoma: clinical and dermatopathologic features

Abstract In Brazil, without considering the non-melanoma skin tumors, bladder cancer in men is the eighth most common, and the urothelial carcinoma or transitional cell carcinoma is the most common among these. Cutaneous metastases from urothelial neoplasms appear as single or multiple erythematous, infiltrated nodules or plaques, and like other cases of distant disease, it is indicative of poor prognosis. The invasive urothelial carcinoma is recognized for its ability to present divergent differentiation and morphological variants. The sarcomatoid urothelial carcinoma is a rare cancer that consists of two different components: one composed of epithelial tissue and the other with sarcomatoid features of mesenchymal origin. The authors describe a case of cutaneous metastasis of sarcomatoid urothelial carcinoma in a 63-year-old male patient.