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Laryngeal osteosarcoma: a clinicopathologic analysis of four cases and comparison with a carcinosarcoma.

We report the clinicopathologic findings of four cases of laryngeal osteosarcoma and one of carcinosarcoma with osteosarcoma components. The tumors occurred in men ranging in age from 50 to 69 years within a median age of 63.3 years. The most common symptoms were hoarseness, dyspnea, and obstruction, or a combination of these. Biopsy material and intraoperative frozen sections usually showed a high-grade sarcoma, but the diagnosis of osteosarcoma was quite apparent on microscopic examination of the surgical specimen. The primary differential diagnosis is spindle cell carcinoma with osteoid formation. Pulmonary metastases and local recurrences were common. Surgery is the treatment of choice, followed by adjuvant chemotherapy. Whether to use radiotherapy or not should be decided on an individual basis.

Aged↗

Carcinosarcoma of the mandibular mucosa: the first report.

A 67-year-old man was referred with a squamous cell carcinoma of the left mandibular alveolar ridge verified elsewhere by an exploratory excision. After extensive staging and preoperative irradiation, a radical surgical attempt was performed. Histopathological examination and immunohistochemical analysis revealed the first case of a carcinosarcoma of the mandibular alveolar ridge. Clinical and histopathological features are presented, and treatment and prognosis are discussed.

Aged↗

Carcinosarcoma of the lung, a tumour which has a poor prognosis and is extremely rarely diagnosed preoperatively.

Out of about 2,000 patients operated on for lung cancer in the period from 1975 to 1990, four were found with the very rare condition carcinosarcoma. The diagnosis was verified by immunohistochemical investigation. The problems concerning this rare tumour are discussed with reference to four case descriptions and with consideration of the pertinent literature. On the one hand, they comprise the difficulty of making a correct histological diagnosis preoperatively, and on the other hand the poor prognosis, even in the NO stage.

Adult↗

A position 12-activated H-ras oncogene in all HS578T mammary carcinosarcoma cells but not normal mammary cells of the same patient.

Among 21 human mammary tumors analyzed for transforming genes by transfection of NIH/3T3 cells, only DNA of a carcinosarcoma cell line, HS578T, registered as positive. A Harvey (H)-ras oncogene identified in this line was cloned in biologically active form and the activating lesion was identified as a single nucleotide substitution of adenine for guanine in the 12th codon. This results in substitution of aspartic acid for glycine at this position of the p21 coding sequence. Knowledge that this alteration creates a restriction site polymorphism for Msp I/Hpa II in the H-ras protooncogene made it possible to survey for the presence of the activated H-ras allele in normal cells as well as in clonally derived tumor cell lines of the same patient. We demonstrated the presence of unaltered H-ras alleles in normal HS578 cells. In contrast, every clonally derived HS578T tumor cell line analyzed contained the H-ras oncogene possessing the genetic alteration at position 12. These findings establish that activation of this oncogene was the result of a somatic event selected within all HS578T tumor cells.

Amino Acid Sequence↗

Characterization of platelet aggregation induced by the human carcinosarcoma Colo 526: role of platelet activation, tumor cell cytoskeleton and tumor cell plasma membrane.

Tumor cell-platelet interactions have been shown to be involved in the process of metastasis. This study characterizes the aggregation of washed platelets induced by the human uterine carcinosarcoma Colo 526. Ultrastructural studies revealed a two-stage process in which the earliest events were the adhesion and degranulation of individual platelets in contact with the tumor cell membrane. The second stage consisted of a wave of aggregation involving all residual platelets. We found that the first stage was initiated by a factor integral to the tumor cell plasma membrane which acted independently of the tumor cell cytoskeleton or metabolic processes. This factor was found to be a glycoprotein or glycolipid with functionally important sialic acid and N-linked carbohydrate residues. The initial stage was not dependent on platelet activation as neither aspirin nor prostacyclin prevented adhesion or degranulation. The second stage was found to be dependent on platelet activation. These results suggest that platelet aggregation induced by Colo 526 involves a distinctive primary stage which is initiated by a factor on the tumor cell plasma membrane resulting in the degranulation and lysis of individual platelets. This process can occur independently of platelet activation or aggregation and thus may have some relevance to the clinical use of platelet antagonists as antimetastatic agents.

Adenosine Diphosphate↗

Carcinosarcoma of the lung. Report of two cases and review of the literature.

Two cases of carcinosarcoma of the lung are presented. Until now, 76 cases have been recorded in the literature. The lesion contains an epithelial and a mesenchymal component, both with cytologic features of malignancy. A central endobronchial and a peripheral invasive type have been described. Fiberoptic biopsies usually show no tumour at all or contain only one of the two components. Treatment consists of lobectomy or pneumonectomy. Prognosis is not better for this tumour than for non-small cell bronchogenic carcinoma, and may even be worse.

Aged↗

Carcinosarcoma of the skin. Case report and literature review.

A case of carcinosarcoma arising in the skin of the left arm of a 69-year-old woman is reported with a review of the literature. The tumor was composed of low-differentiated squamous cell carcinoma, which was intermingled with a pleomorphic sarcoma. The carcinomatous component had keratin and lacked vimentin, whereas the phenotype of the sarcomatous portion was the reverse. The former presented additionally focal expression of S-100 protein, which was lacking in other portions of the carcinoma. The phenotypic data, supplemented by p53 immunostaining, which was present in both components, suggest their common origin in this tumor.

Aged↗

Carcinosarcoma of the prostate.

Carcinosarcoma of the prostate is a biphasic tumor containing adenocarcinoma (ACA) and recognizable sarcomatous components. It is a rare neoplasm with only 12 previous reported cases. We describe three additional cases arising between 4 and 6 years after initial diagnosis of prostatic ACA. Two patients were initially treated by prostatectomy, pelvic external beam radiotherapy, and hormonal manipulation. The third patient was treated by pelvic lymphadenectomy and 125I implants. After the development of the sarcomatous component, the first two are still alive with distant metastases and residual pelvic disease at 9 and 17 months. The third patient died with disease 7 months after diagnosis. Histologically, prostatic ACA was recognized in all three cases, as well as a neoplastic mesenchymal component that appeared later. Foci of osteosarcomatous, chondrosarcomatous, and myosarcomatous differentiation were recognized in two of the three cases. Based on the chronologic and the histologic evolution of the neoplasm, we favor sarcomatoid transformation of the ACA as the most likely histogenesis. It appears that radiotherapy and hormonal therapy may be important in the development of at least some of these tumors.

Aged↗

Carcinosarcoma of the spleen. An extragenital malignant mixed müllerian tumor?

A 55-year-old woman with painful splenomegaly underwent surgical exploration and resection of the spleen. Pathologic examination revealed a neoplasm of the spleen exhibiting malignant stromal and epithelial components with müllerian features. In the absence of a recognized neoplasm elsewhere, the carcinosarcoma most likely arose as a primary splenic tumor and may represent an extrauterine malignant mixed müllerian tumor arising in the spleen.

Carcinosarcoma↗

Carcinosarcoma of the uterine cervix: a report of eight cases with immunohistochemical analysis and evaluation of human papillomavirus status.

Carcinosarcomas (malignant Müllerian mixed tumors [MMMTs]) of the uterine cervix are rare neoplasms. This report describes the morphology, immunohistochemical profiles, and human papillomavirus (HPV) status of eight cervical MMMTs. Patients' ages ranged from 32 to 93 years (mean, 61 years). Seven cases showed in situ squamous cell carcinoma (SCC). The invasive epithelial component (EC) was composed of combined adenoid basal carcinoma, basaloid SCC, and adenoid cystic carcinoma (ACC) in two cases. Keratinizing SCC, large cell nonkeratinizing SCC, undifferentiated carcinoma, and basaloid SCC predominated in the remaining tumors, one of which had admixed ACC. The sarcomatous component (SC) was homologous and spindled with admixed myxoid areas in three lesions. The ECs and SCs in six MMMTs showed dual immunostaining with epithelial membrane antigen and the pan-keratin marker, MNF116. The SC was vimentin-positive in seven cases. Five tumors had a vimentin-positive EC. The SC was positive for muscle specific actin and/or smooth muscle actin in seven lesions, of which four were desmin-positive. Polymerase chain reaction (PCR) using GP5+/GP6+ L1 consensus primers detected HPV DNA in all eight cases. Nonisotopic in situ hybridization with digoxigenin-labeled probes to HPV types 6, 11, 16, 18, 31 and 33 demonstrated integrated HPV 16 in three cases, not only in the EC, but also in nuclei of the SC. This is the first study to implicate HPV in the evolution of cervical MMMTs. The above observations lend support to a metaplastic theory of histogenesis.

Adult↗

Carcinosarcoma (malignant mixed mesodermal tumor) of the uterus. A Gynecologic Oncology Group pathologic study of 203 cases.

We report on the pathologic findings in primary tumors and metastases in 203 cases of stage I and II endometrial carcinosarcoma (malignant mixed mesodermal tumor) subjected to hysterectomy and staging laparotomy. Metastases were studied in 40 of these cases, including 34 with positive findings in the pelvic and/or para-aortic lymph nodes. Features of the stromal component of the primary tumors, including grade, mitotic index, and the presence and types of heterologous elements, showed no relation to the presence of metastases at operation. High-grade, serous, and clear cell carcinomatous components, on the other hand, were associated with a higher frequency of metastases, as were deep myometrial invasion, lymphatic or vascular space invasion, and involvement of the isthmus or cervix. The current concepts of histogenesis and differentiation of these tumors are discussed, and the suggestion is made that they might represent metaplastic carcinomas.

Carcinosarcoma↗

Primary mullerian carcinosarcoma of the retroperitoneum: report of a case.

A müllerian carcinosarcoma or malignant mesodermal mixed tumor (MMMT) originated from the retroperitoneum of a 51-year-old woman; only two previous similar cases have been reported. The 1,040 gm tumor was found in the left retroperitoneal space; the center of the tumor was extensively liquified. The uterus, fallopian tubes, and ovaries were unremarkable. The histopathological features of the tumor were typical of MMMT, homologous type. The adenocarcinomatous component of the tumor was immunohistochemically positive for cytokeratin 7 and negative for cytokeratin 20. MMMTs arising in extragenital sites are rare, and most of them arise from the peritoneum. The histogenesis of extragenital MMMTs remains speculative, but the origin from the "secondary mullerian system" is most likely.

Carcinosarcoma↗

Carcinosarcoma of the gallbladder. A case report and review of the literature.

We report a carcinosarcoma (CS) of the gallbladder in an 83-year-old woman. Ultrasonography found an enlarged gallbladder with thickened walls, a 3-cm gallstone, and a polypoid mass in the fundus. Pathological examination revealed neoplastic tissue composed of sarcomatous and glandular components. Twelve months later, the patient is alive. We review 24 other cases in the literature to outline the characteristics of this tumor.

Aged↗

Malignant mixed mesodermal tumors and carcinosarcoma of the ovary: report of eight cases and review of literature.

Eight cases of malignant mixed mesodermal tumors and carcinosarcoma of the ovary from our files and 193 cases from the literature were reviewed. An attempt is made to analyze these cases in regard to clinical feature, treatment results, and their outcome. A detailed review of the histogenesis is presented. These tumors grow very rapidly and are usually in advanced stages when diagnosed. Most patients are postmenopausal. Surgery, radiation therapy, and chemotherapy, either alone or in combination, have been used to treat this neoplasm, but 77.6 per cent of the patients are dead of their disease within 1 year. Limited data suggests that patients treated with surgery and combination chemotherapy survive longer. However, further investigation in finding new combination therapy is desired.

Aged↗

Carcinosarcoma arising in a patient with multiple cylindromas.

Familial cylindromatosis (Brooke-Spiegler syndrome) is a rare autosomal dominant inherited disease characterized by the development of adnexal tumors, mostly cylindromas, but also trichoepitheliomas and spiradenomas. Malignant tumors may occur, usually with the features of a cylindrocarcinoma. The authors describe the case of a 75-year-old woman with the Brooke-Spiegler syndrome who presented with multiple nodules of the scalp, face, and trunk. In 1997 she underwent surgical excision of the entire forehead and scalp with skin grafting. Histologic examination revealed multiple cylindromas, some with areas of spiradenoma and one with an extensive adenomatous component; some trichoepitheliomas were also evident. In 2002, a nodule of the trunk suddenly increased in size and became painful. The lesion was excised and histologic and immunohistochemical evaluation revealed a malignant cutaneous biphasic tumor extending into the subcutis, consisting of a major portion with the features of an adnexal carcinoma and of a minor one of atypical spindle cells. Biphasic malignant skin tumors are rare and only a limited number have been described, none in association with the Brooke-Spiegler syndrome. The authors discuss the morphogenesis of the folliculosebaceous-apocrine unit from which the tumors in this syndrome derive, and the pivotal role of mesenchymal cells in determining the process. Since the Brooke-Spiegler syndrome is characterized by a germline mutation in the CYLD oncosuppressor gene, a biphasic tumor in this setting may represent a true carcinosarcoma.

Aged↗

Endometrioid carcinomas of the uterine corpus with sex cord-like formations, hyalinization, and other unusual morphologic features: a report of 31 cases of a neoplasm that may be confused with carcinosarcoma and other uterine neoplasms.

We describe a series of unusual endometrioid carcinomas (ECs) of the uterine corpus characterized in significant part by cords of epithelioid cells, spindle cells, and a hyalinized stroma that sometimes formed osteoid. These features, particularly when prominent, produced an appearance strikingly different from that of conventional EC, sometimes resulting in problems in differential diagnosis, especially with a malignant mullerian mixed tumor (carcinosarcoma). The 31 patients ranged in age from 25 to 83 years (mean, 52 years). The proportion within each stage were as follows: stage Ia, 9.7%, stage Ib, 45.2%, stage Ic, 9.7%, stage IIb, 16.1%, stage IIIc 3.2%, and stage IV, 3.2%. In 4 patients (12.9%), staging information was not available. On microscopic examination, typical EC, which accounted for 10% to 90% of the tumor, was admixed in 90% of cases with cords of epithelioid or spindle cells within a hyalinized stroma. In 3 cases, the tumor contained cords of cells without a hyalinized stroma. Areas with a diffuse growth of fusiform cells suggesting endometrial stromal cells were also occasionally seen in minor amounts. Seventy percent of the tumors exhibited squamous differentiation, and in 50% of the tumors there was a background of endometrial hyperplasia. Two thirds of the tumors were grade 2 and the remainder were grade 1. Vascular space invasion was identified in seven tumors. On immunohistochemical analysis, the typical EC component was strongly positive for keratin, whereas the keratin staining was more focal and variable in the epithelial cells in the cords. Muscle markers (desmin, actin), CD10, and inhibin were negative in the latter. Overexpression of p53 was found in only 1 case. Eighty-three percent of the patients were alive with no evidence of disease on follow-up (range, 2-115 months; mean, 34.4 months). The clinical features, including a typically low stage and generally good prognosis, and histologic findings are different from those of malignant mullerian mixed tumors that are characterized by both high-grade carcinomatous and sarcomatous components and an aggressive clinical course. Confusion with other neoplasms, particularly those with sex cord-like growth, such as uterine tumors resembling ovarian sex cord tumors and epithelioid smooth muscle tumors, may also arise. We refer to tumors with the features described herein as "corded and hyalinized endometrioid carcinomas," a designation that reflects their two most striking and consistent features. Corded and hyalinized endometrioid carcinomas are yet another example of the protean phenotype of endometrioid adenocarcinomas of the female genital tract that has been appreciated only in the last two decades.

Adult↗