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Cutaneous carcinosarcoma: adnexal vs. epidermal types define high- and low-risk tumors. Results of a meta-analysis.

OBJECTIVE: We report four cases of cutaneous carcinosarcoma (CS) and perform a meta-analysis of the cutaneous CS literature. RESULTS: CS occurred in elderly patients (mean of 80 years) on sun-damaged skin, and were keratotic papules of short duration. They did not recur after excision. CS exhibited basal cell carcinoma mixed with atypical fibroxanthoma cell populations. Immunophenotyping revealed vimentin+/keratin- spindle cells and vimentin-/keratin+ epithelial cells. Three cases exhibited p53 protein expression of both carcinomatous and sarcomatous components. Literature review identified 38 cases of cutaneous CS that could be broadly classified into two distinct groups. Epidermal-derived (basal or squamous cell carcinoma epithelial component) CS arose on the sun-damaged skin of the head and neck of elderly males (mean age 72 years) and had a 70% 5-year disease-free survival. In contrast, adnexal CS (spiradenocarcinoma, porocarcinoma, proliferating tricholemmal cystic carcinoma, or matrical carcinoma) occurred in younger patients (mean age 58 years), showed recent growth in a long-standing nodule and had a 25% 5-year disease-free survival. Age less than 65 years, recent growth, long-standing skin tumor, and tumor size greater than 2 cm significantly correlated with poor outcome. CONCLUSIONS: Cutaneous CS is an aggressive skin cancer with high risk for advanced disease. Significant risk factors exist whose identification will allow for better management of CS patients.

Aged↗

Biphasic sarcomatoid basal cell carcinoma (carcinosarcoma): four cases with immunohistochemistry and review of the literature.

BACKGROUND: Biphasic sarcomatoid carcinoma (BSC), or carcinosarcoma, is an uncommon biphasic neoplasm that has been reported in diverse anatomical sites. The tumor is composed of a malignant epithelial component intimately associated with a malignant mesenchymal component, which may be homologous or heterologous. Twenty-three cases of primary cutaneous BSC have been reported in the English literature. In only eight of these cases was basal cell carcinoma the epithelial component. METHODS: We report a further four cases of primary cutaneous biphasic basal cell carcinoma, and include the clinical, histological and immunohistochemical features. RESULTS: The four cases showed basal cell carcinoma associated with a pleomorphic sarcomatous stroma. In addition, myofibroblastic differentiation and foci of osteoid were present in one case, and leiomyosarcomatous areas in another. The epithelial components were positive for several epithelial markers. The mesenchymal components were positive for vimentin and CD99, and negative for epithelial markers. p53 was positive with equal intensity in both epithelial and mesenchymal components. A significantly worse outcome was observed in patients with tumors measuring 40 mm or more at excision. CONCLUSIONS: The sarcomatous component of the tumor is best regarded as a metaplastic transformation of the carcinomatous component. These tumors are potentially aggressive if incompletely excised, and complete resection is recommended.

12E7 Antigen↗

Bladder carcinosarcomas: three cases with diverse histogenesis.

Carcinosarcomas of the bladder are rare. We describe three such tumours, including an apparently unique case in which the components comprised liposarcoma and poorly-differentiated transitional cell elements. A second example included chondrosarcomatous elements. These two tumours showed architectural and immunocytochemical features which suggested that they had originated as carcinomas but had subsequently differentiated along both epithelial and mesenchymal pathways. The third tumour contained both carcinoma and osteosarcoma and may represent a collision tumour.

Aged↗

Mixed pulmonary blastoma and carcinosarcoma.

Pulmonary blastomas and carcinosarcomas are uncommon tumours, accounting for less than 1% of all lung neoplasms. Three previously described mixed epithelial and mesenchymal tumours having both adult and embryonal elements were termed 'transitional'. We report a similar case and evaluate the application of the term 'transitional tumour'.

Aged↗

Carcinosarcoma of the prostate.

The present report describes a rare prostatic tumor occurring in a 78-year-old Japanese male. The histological features were those of carcinosarcoma involving an adenocarcinoma and a chondrosarcoma, with metastasis to the liver. The carcinomatous component stained positively for prostatic acid phosphatase (PAP) by an immunoperoxidase technique. The literature is reviewed.

Adenocarcinoma↗

Carcinosarcoma of the esophagus. An immunohistochemical and electron microscopic study.

Immunohistochemical and electron microscopic examinations were made of a carcinosarcoma of the esophagus in an 80-year-old man. An immunohistochemical examination showed that sarcomatous spindle cells were vimentin-positive, whereas squamous carcinoma cells were keratin-positive. No coexistence of vimentin and keratin in a single tumor cell was found. Electron microscopically, the sarcomatous spindle cells were characterized by well-developed rough endoplasmic reticulum, abundant intermediate filaments, and the occasional presence of peripheral aggregates of microfilaments. No definite desmosomes were identified among these cells. These results appear to indicate that most of the spindle-shaped tumor cells assume fibroblastic cellular features and synthesize the intermediate filament protein usually expressed in mesenchymal cells, even though such tumor cells could be epithelial in origin.

Aged↗

Carcinosarcoma of the gallbladder. Report of a case and review of the literature.

An extremely rare case of carcinosarcoma of the gallbladder is reported. The tumor presenting as a large, soft polypoid growth was microscopically characterized by an admixture of adenocarcinomatous and sarcomatous elements, the latter containing specific heterologous mesenchymal elements such as osteoid, cartilage, and rhabdomyoblasts. The cytoplasm of neoplastic rhabdomyoblasts offered unequivocal cross striations and was strongly positive for myoglobin, determined by the immunoperoxidase procedure. Metastatic tumors in the liver also exhibited a dual composition of adenocarcinoma and rhabdomyosarcoma. This appears to be the second documented case of this type of neoplasm with the rhabdomyosarcomatous component. The clinical settings and morbid prognosis were similar to events in cases of carcinoma of the gallbladder.

Carcinosarcoma↗

Carcinosarcoma of the prostate.

A unique case of carcinosarcoma of the prostate occurring in a 32 year old man is presented. This is the youngest case reported to date among nine well-documented examples. The patient underwent a total prostatectomy under the diagnosis of prostatic sarcoma. Despite adjuvant chemotherapy and full-dose radiotherapy being undertaken, the patient died from multiple lung metastases about 8 months after the operation. The surgically resected primary tumor was composed of two histologically distinct elements, these being an undifferentiated carcinoma and a sarcoma with various mesodermal components. These elements were intimately intermingled and transitional forms were often noticed. The sarcomatous portion contained myxoid areas, spindle cell sarcomas with nuclear palisading, areas of osteoid formation and small islands of chondromatous differentiation. The pathogenesis of this complex neoplasm is discussed, and it is suggested that vestigial embryologic Müllerian mesodermal tissue may be capable of diverse differentiation.

Adult↗

Carcinosarcoma of the urinary bladder: expression of epithelial markers and different expression of heat shock proteins between epithelial and sarcomatous elements.

A case of carcinosarcoma composed of both adenocarcinoma and sarcomatous elements in the non-trigone region of the urinary bladder is presented. The epithelial element was a well to poorly differentiated adenocarcinoma with focal squamous metaplasia. The sarcomatous elements disclosed spindle cell sarcoma with focal epithelioid pattern and myxoid change in the stroma, together with chondrosarcomatous and rhabdomyosarcomatous elements. By immunohistochemical examination, not only the carcinoma element but also the sarcomatous elements showed a positive immunoreaction for cytokeratin (CK), epithelial membrane antigen (EMA) and carcinoembryonic antigen. Some population of sarcomatous elements expressed smooth muscle actin and muscle specific actin (MSA) and a limited portion of epithelioid area showed a positive immunoreaction for desmin, MSA and myoglobin, indicating leiomyosarcomatous and rhabdomyosarcomatous differentiation, respectively. Unexpectedly, tumor cells in the chondrosarcomatous element revealed a simultaneous positivity of CK and EMA as well as S-100 protein. Both epithelial and sarcomatous elements showed an intensive positive immunoreaction for p53 and heat shock protein (HSP) 70. However, HSP27 and HSP60 were detected in most epithelial elements and only in a small number of tumor cells in the sarcomatous area. These findings indicate that sarcomatous elements, including heterologous elements, may derive from epithelial elements with partial or complete loss of epithelial features, and different factors other than p53 and HSP70 may associate with the morphological alteration of carcinoma.

Biomarkers↗

Carcinosarcoma in the region of the female urethra.

A 61-year-old woman with acute urinary retention was found to have a carcinosarcoma in the region of the urethra. Evaluation of the computed tomogram suggested a urethral tumor, which was resected by a transperineal approach. She received local radiotherapy after surgery, and is alive at 1-year follow-up with a tumor metastasis to the pelvis.

Carcinosarcoma↗

Case of uterine cervical carcinosarcoma.

Carcinosarcoma (CS) is a rare neoplasm that is called a mixed epithelial and mesenchymal malignancy. CS of the uterine cervix is much less common than its counterparts in the uterine corpus. A 61-year-old, gravida 2, para 2 woman, who had undergone menopause 16 years prior to the presentation, was diagnosed with CS of the uterine cervix. A semiradical hysterectomy was carried out on the diagnosis of stage Ib1 cervical cancer. The patient underwent whole pelvic 45 Gy radiation as a postoperative additional treatment, but she died from multiple organ failure by metastasis 17 months after the operation. The tumor protruded from the cervix to the vagina and measured 4.5 x 3.0 cm. Histologically, the tumor was characterized as a squamous cell carcinoma and mesenchymal malignancy, represented by osteosarcomatous components. The stroma was largely composed of atypical spindle-shaped cells, which were immunohistochemically demonstrated to be of epithelial origin. Uterine cervical CS is one of the aggressive malignancies, and squamous cell carcinomas are common epithelial counterparts of cervical CS as well as adenocarcinomas.

Carcinosarcoma↗

Carcinosarcoma of the parotid gland: an unusual case with large-cell neuroendocrine carcinoma and rhabdomyosarcoma.

We report a case of carcinosarcoma of the parotid gland in a 72-year-old Japanese man. The patient noticed a rapidly enlarging hard mass in the right parotid gland. He underwent radical parotidectomy with cervical lymph node dissection. The resected tumor measured 3.5 x 4.5 cm and histopathologically showed carcinomatous and sarcomatous components. The carcinomatous component consisted of large-cell neuroendocrine carcinoma (LCNEC), squamous cell carcinoma and adenocarcinoma not otherwise specified, while the sarcomatous component included spindle cell sarcoma not otherwise specified, so-called myxosarcoma and rhabdomyosarcoma. The LCNEC component was predominant within the whole tumor. The diagnoses of LCNEC and rhabdomyosarcoma were also confirmed immunohistochemically. With regard to histopathogenesis, based on the lack of histopathological evidence and antecedent history of pleomorphic adenoma, we considered the present case to be de novo, not expleomorphic adenoma.

Aged↗

Carcinosarcoma of the skin.

A 91-year-old patient with an extraskeletal osteosarcoma (characterized by atypical mesenchymal cells with ossification) of the forehead associated with a basal cell carcinoma is reported. Both osteoid and basal cell carcinoma nests were found embedded in a stroma containing markedly atypical cells. The 2 previously reported carcinosarcomas of the skin are reviewed.

Aged↗

Establishment and characterization of JHUCS-1 cell line derived from carcinosarcoma of the human uterus.

The cell line designed JHUCS-1 was established from a carcinosarcoma (malignant mixed mesodermal tumor) of the uterus that was surgically removed from a 57-year-old Japanese woman. We carefully examined the histopathology of the original tumor after the cell line was established and noted differentiation into a neuroendocrine carcinoma within the tumor's epithelial components. Immunohistochemical staining of the tumorous tissue that had been heterotransplanted was positive for Leu7. Additionally, secretary granules were observed in the grafted cells as determined by electron microscopy. These results support the existence of neuroendocrine cells within the JHUCS-1 cell line.

Animals↗

Carcinosarcoma with a large cell neuroendocrine epithelial component: first report of an unusual biphasic tumour of the urinary bladder.

This article describes the first reported case of carcinosarcoma of the urinary bladder with a large cell neuroendocrine epithelial component. A 61 year old man presented with gross haematuria and underwent resection of a biphasic bladder tumour. The malignant epithelial component showed large cell neuroendocrine differentiation with immunohistochemical reactivity for neurone specific enolase, synaptophysin, and chromogranin. The malignant mesenchymal component did not show specific differentiation by histological or immunohistochemical examination. The differential diagnosis of biphasic tumours in the urinary bladder is discussed, along with a review of the literature.

Carcinosarcoma↗

Carcinosarcoma arising in a dermoid cyst of the ovary.

A case of carcinosarcoma arising within an otherwise benign cystic teratoma is reported. The patient, a 78 year old nulliparous woman, presented with right sided abdominal pain of short duration and subsequently underwent a bilateral salphingo-oophorectomy. Slicing of the left ovary revealed a unilocular cyst containing hair admixed with soft yellow material with a thin wall apart from a solid area at one pole. Extensive areas of necrosis and cystic degeneration were present within this mass. Histologically, the large cyst was a typical mature cystic teratoma, containing carcinomatous and sarcomatous elements. Mature cystic teratomas have been reported in association with a variety of malignant ovarian tumours such as mucinous cystadenocarcinoma and malignant germ cell neoplasms. Secondary malignant transformation within a dermoid cyst is a much rarer occurrence, estimated as less than 2% of all such lesions. Adenocarcinomas are the second most common malignancies arising within dermoid cysts. Sarcomas alone or in combination with squamous carcinoma have been described arising in a mature cystic teratoma. To the best of our knowledge, no case of sarcoma arising in association with adenocarcinoma has been described before.

Actins↗