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PubMedDate not supplied

Cure of metastatic uterine carcinosarcoma to lungs: a case report.

Most patients with advanced or recurrent uterine sarcoma experience disease progression and ultimately die. We present a case of uterine sarcoma with lung metastasis treated with systemic chemotherapy and with no evidence of disease for more than 5 years. A 77-year-old woman underwent total abdominal hysterectomy with bilateral salpingo-oophorectomy for carcinosarcoma of the uterus followed by external pelvic radiotherapy. Ten months later, the tumor recurred in the apex of the vagina and was treated with brachytherapy. After 6 months of remission, she presented with pulmonary metastasis. After four cycles of systemic chemotherapy with cisplatin and ifosfamide, the pulmonary nodules completely disappeared. Currently she is still in complete remission after more than 5 years, but unfortunately she has developed myelodysplastic syndrome. This is the first reported case in the literature of cured metastatic uterine carcinosarcoma to lungs, with long-term survival of 5 years.

Aged↗
PubMed2000

Clinicopathological studies of esophageal carcinosarcoma: analyses of its morphological characteristics using endoscopic, histological, and immunohistochemical procedures.

BACKGROUND AND STUDY AIMS: Carcinosarcoma of the esophagus is a rare malignant neoplasm consisting of both carcinomatous and sarcomatous components, which characteristically forms polypoid tumors. PATIENTS AND METHODS: Seven carcinosarcomas were analyzed using endoscopic, histological, and immunohistochemical procedures. Endoscopically, six of the seven lesions were found to be of the protruding type, while the other one was an ulcerating tumor. RESULTS: In all seven cases, the carcinomatous component consisted of differentiated squamous cell carcinoma, and the sarcomatous component was spindle cell carcinoma. Histological analyses demonstrated that the majority of the protruding tumors consisted of the sarcomatous component, while the ulcerating tumor mainly consisted of squamous cell carcinoma. The Ki-67 (MIB-1) labeling index (LI) of the carcinomatous component (28.2%) did not differ significantly from that of the sarcomatous component (25.5%). The sarcomatous component showed abundant expression of type IV collagen and laminin. CONCLUSIONS: It is conceivable that the carcinomatous and sarcomatous components grow separately from the early stage of the tumors, and that the sarcomatous component forms a protruding tumor mass because it has abundant stroma positive for type IV collagen and laminin.

Aged↗
PubMed1993

Carcinosarcomas of the lung. Three case reports and literature review.

Therapy and course of pulmonary manifestations of carcinosarcomas in three patients are described. For this rare mixed tumor there is a prevalence among men between the ages of forty and seventy. Operative therapy should be employed whenever possible, because of the poor results achieved thus far using radiotherapy or chemotherapy. Carcinosarcomas were thought to have a worse prognosis than other non-small-cell bronchial carcinomas but an increasing number of reports indicate a similar clinical course and prognosis. Pneumonectomy was required for two of our patients and lobectomy was performed in the third. The period of observation extended over two years. One patient, tumor stage T4N0M0 died of his disease after seven months. The two other patients, tumor stages T3N0M0 and T2N1M0, were operated on thirty-three and thirty-five months ago respectively and are clinically disease-free and in good health.

Aged↗
PubMed2003

Carcinosarcoma and squamous cell carcinoma of the renal pelvis associated with nephrolithiasis: a case report of each tumor type.

Both squamous cell carcinoma and carcinosarcoma of the renal pelvis are uncommon. We report on two cases, one carcinosarcoma and one squamous cell carcinoma of the renal pelvis. In the patient with squamous cell carcinoma of the left kidney, the tumor was neither detectable on preoperative radiological evaluation nor grossly visible in the surgical specimen. This patient, a 56-year-old man, presented with left lumbar pain, hematuria, fever, 4-5 kg weight loss, and untreated nephrolithiasis of the left kidney that had been diagnosed 20 years earlier. The second patient, an 87-year-old woman, also had a long history of left kidney nephrolithiasis and presented with left lumbar pain and hematuria. Both patients underwent nephrectomy for removal of the non-functioning hydronephrotic left kidney. In both cases, microscopic examination of the surgical specimen revealed squamous metaplasia and dysplasia in the pelvicalyceal mucosa, and islands of atypical squamous cells in the renal parenchyma. In the second case, the kidney also showed sarcomatous changes in the pelvis.

Aged↗
PubMedDate not supplied

Differential diagnosis between uterine carcinosarcoma versus carcinoma with sarcomatous metaplasia: an immunohistochemical and ultrastructural case study.

Uterine carcinosarcomas are biphasic neoplasms with carcinomatous and sarcomatous elements. However, several elements suggest that carcinosarcomas may be more closely related to carcinoma of the endometrium and that they arise from an unique stem cell. Recently, the authors observed an uterine tumor that at histologic examination showed an apparently double population of cells: malignant epithelial element admixed with mesenchymal spindle-shaped cells. The immunohistochemical stainings instead showed cytokeratin positivity and negativity for stromal markers. Electron microscopy showed the neoplastic tissue to be made of a single population of poorly differentiated epithelial cells, thus confirming the immunohistochemical findings and leading to the diagnosis of uterine metaplastic carcinoma.

Adenocarcinoma↗
PubMed2003

Laparoscopic small bowel resection of metastatic pulmonary carcinosarcoma.

Carcinosarcoma of the lung is a rare malignancy consisting of both epithelial and mesenchymal components. Although metastatic disease is common, metastatic disease to the small intestine is rare and has been reported only once in the literature. An unusual case of pulmonary carcinosarcoma with metastasis to the small bowel is presented. At the time of laparoscopic exploration, a 4 x 4 cm mass was seen in the jejunum involving the adjacent mesentery. The affected small bowel and mesentery were resected laparoscopically and bowel continuity was restored through an entirely intracorporeal technique. Laparoscopic small bowel resection can be performed safely in the setting of malignant disease and imparts many of the benefits of minimally invasive surgery.

Carcinosarcoma↗
PubMed1994

Biphasic and monophasic sarcomatoid carcinomas of the lung. A reappraisal of 'carcinosarcomas' and 'spindle-cell carcinomas'.

To address the premise that pulmonary "carcinosarcomas" and spindle-cell carcinomas are part of a single clinicopathologic continuum, the authors studied 21 examples of such lesions as defined by World Health Organization criteria. Two biphasic tumors demonstrated an admixture of overt carcinoma with other foci showing partial rhabdomyogenic differentiation; 15 others were histologically similar but lacked "heterologous" sarcoma-like elements; and four lesions were monophasic spindle-cell sarcomatoid carcinomas. One of the latter also contained rhabdomyosarcoma-like areas by light microscopy. Sarcomatoid components were reactive for keratin and/or epithelial membrane antigen (EMA) in 18/21 cases. In addition, desmin and muscle-specific actin were co-detected in the same spindle cells that were keratin-positive in 4 tumors, 3 of which were those with partially myogenic histologic features. Vimentin was present in keratin- or EMA-reactive sarcomatoid cells in 12 neoplasms, and all cases were labeled with an antibody to collagen type IV. Survival was poor in this group of patients; only 1 was alive at last contact. These data support the contention that "carcinosarcoma" of the lung is part of a spectrum with "spindle-cell carcinoma." It is proposed that the terms "biphasic sarcomatoid carcinoma" and "monophasic sarcomatoid carcinoma" are more apt descriptors for such tumors.

Adult↗
PubMed1995

Carcinosarcoma (malignant mixed müllerian tumor) of the uterus with a rhabdoid tumor component. An immunohistochemical, ultrastructural, and immunoelectron microscopic case study.

A case of uterine carcinosarcoma (malignant mixed müllerian tumor) of the uterus containing a rhabdoid tumor element is described. In addition to a malignant glandular component and anaplastic sarcomatous areas, this tumor had multiple foci with histologic and ultrastructural features of malignant rhabdoid tumor. Vimentin positivity by immunohistochemistry was confirmed by immunoelectron microscopy. Although three malignant rhabdoid tumors of the uterus have been previously described, in addition to rhabdoid differentiation in an endometrial stromal sarcoma, to our knowledge this is the first report of rhabdoid tumor differentiation occurring within a uterine carcinosarcoma.

Aged↗
PubMed1990

Carcinosarcoma arising in eccrine spiradenoma. A clinicopathologic and immunohistochemical study of two cases.

Malignant change in a benign eccrine spiradenoma is rare. Only 13 such cases have been previously reported. Two further cases are described herein, both of which were carcinosarcomas and arose in middle-aged women. In each case continuity was demonstrated between the benign and malignant epithelial components and the sarcomatous element. In case 1 the sarcomatous component showed osteocartilaginous and rhabdomyoblastic differentiation, whereas in case 2 only osteosarcoma was evident. To our knowledge, carcinosarcoma has not been previously described arising in eccrine spiradenoma. The literature regarding malignant eccrine spiradenoma is reviewed.

Adenoma, Sweat Gland↗
PubMed1997

Primary metaplastic carcinoma (carcinosarcoma) of the skin. A clinicopathologic study of four cases and review of the literature.

Metaplastic carcinoma (carcinosarcoma, sarcomatoid carcinoma, malignant mixed tumor) is a biphasic tumor comprising malignant epithelial and heterologous mesenchymal elements. Primary cutaneous cases are rare, with only seven cases documented in the English literature to date. We present four further cases, including three that developed in association with squamous cell carcinoma and one in an eccrine porocarcinoma. Heterologous malignant mesenchymal elements included malignant osteosarcoma, chondrosarcoma, leiomyosarcoma, and rhabdomyosarcomas. In contrast to metaplastic carcinomas arising in visceral sites, those primarily arising in the skin do not appear to behave in a very aggressive manner (Recurrence rate 22%, metastasis rate 22%, overall mortality 11%). However, the numbers involved are small and the follow-up period is short. In view of recent developments and progress in our understanding of the possible histogenesis of such tumors, we suggest that metaplastic carcinoma rather than carcinosarcoma is the most appropriate term with which to describe these very rare cutaneous neoplasms.

Acrospiroma↗
PubMed2004

Low-grade trichoblastic carcinosarcoma of the skin.

We present an unusual case of cutaneous carcinosarcoma with the epithelial component closely resembling nodular basal cell carcinoma, and the mesenchymal component composed of cells constituting extended follicular papillae. A solitary tumor was excised in an 80-year-old man. Histologic sections revealed an ulcerated, asymmetric, poorly circumscribed neoplasm composed of epithelial cells arranged in lobules with peripheral palisading or in a cribriform pattern. The epithelial cells were darkly basophilic with scant cytoplasm and round or oval nuclei with an indistinct chromatin pattern and nucleoli. Nuclei crowding and mitotic figures were observed. Some lobules contained melanin. There were no shadow cells, sebaceous or apocrine glandular differentiation. Each epithelial nodule was surrounded by multiple rows of cells with pale vesicular nuclei and scant cytoplasm. Smaller epithelial aggregations were encircled by these cells concentrically; in larger ones these cells were aligned across a broad front resembling so-called "continuous papillae". Additionally, numerous small follicular germ-like structures associated with papillae were seen. The cells composing "continuous papillae" showed nuclear pleomorphism, numerous mitotic figures including atypical ones, and nuclear crowding. At foci, the transition from the multilayered arrangement of these cells into their diffuse proliferation in the stroma was seen. There were no transitions between the epithelial and stromal component; both were intermingled as though being mutually dependent, with no areas revealing a high-grade tumor or dedifferentiation. Immunohistochemically, the epithelial cell component stained with cytokeratins. The cells of the mesenchymal component tested positive for vimentin and negative for desmin and cytokeratins. The proliferation index (Ki-67) was high in both components. There were also a high number of p53-positive cells in both compartments. We propose the term "low-grade trichoblastic carcinosarcoma" for this neoplasm. We are not aware of a similar tumor published in the English literature.

Aged↗
PubMed1992

Adrenal carcinosarcoma presenting in a woman with clinical signs of virilization. A case report with immunohistochemical and ultrastructural findings.

An adrenal carcinosarcoma is reported in a 29-year-old female presenting with clinical signs of virilization. This is the first reported case of a functioning adrenal carcinosarcoma in the English language literature. The tumor measured 12.5 cm in greatest dimension, weighed 610 g, and consisted of large areas of typical adrenal cortical carcinoma that was, however, interspersed with multiple foci of sarcoma. Rhabdomyosarcomatous elements were identified and confirmed both immunohistochemically and ultrastructurally. After radical resection, the patient received adjuvant mitotane therapy but developed rapid local and metastatic recurrence. Systemic chemotherapy was unsuccessful, and the patient died 8 months after surgery.

Adrenal Cortex Neoplasms↗
PubMed1993

Carcinosarcoma of the adrenal cortex presenting with mineralocorticoid excess.

An adrenal carcinosarcoma is reported in a 79-year-old woman presenting with clinical signs of hyperaldosteronism. The tumor weighed 199 g and consisted of areas typical of adrenal carcinoma and areas of sarcoma. The sarcomatous component of the tumor showed osteogenic and chondroid differentiation. Vimentin stained both the carcinomatous and sarcomatous regions. Four months after resection, the patient developed metastases. This is the third reported case of adrenal carcinosarcoma and the only case in which hyperaldosteronism or bony differentiation was observed.

Adrenal Cortex Neoplasms↗
PubMed1995

Adenoid cystic carcinoma of the uterine cervix with malignant stroma. An unusual variant of carcinosarcoma?

An 80-year-old woman developed vaginal bleeding caused by a large tumor of the cervix. Histologically, the tumor proved to be composed predominantly of adenoid cystic carcinoma focally associated with squamous cell carcinoma. Of special interest was the stroma, which displayed pleomorphism, multinucleated tumor giant cells, and numerous mitoses, justifying the designation of carcinosarcoma. A single similar reported case also regarded as a carcinosarcoma described a primary cervical neoplasm in a postmenopausal woman containing an admixture of heterologous sarcoma and conventional cervical adenocarcinoma, which focally demonstrated areas of adenoid cystic carcinoma.

Aged↗
PubMed1996

Monoclonal origins of malignant mixed tumors (carcinosarcomas). Evidence for a divergent histogenesis.

Malignant mixed tumors (carcinosarcomas) are examples of unusual neoplasms whose occurrences have been observed in increasingly diverse sites but whose pathogenesis remains a complete mystery. Two antithetical hypotheses that have been advanced to explain the histogenesis of these tumors include the convergence hypothesis, which proposes an origin from two or more stem cells (multiclonal hypothesis), and the divergence hypothesis, which proposes an origin from a single totipotential stem cell that differentiates into separate epithelial and mesenchymal directions (monoclonal hypothesis). To test these hypotheses, a novel strategy for the determination of clonality from as few as 100 tumor cells obtained by enzymatic digestion of either fresh or formalin-fixed, paraffin-embedded tissues and cell sorting was used that exhibited the polymerase chain reaction (PCR) in amplifying a 511-bp region located within the first intron of the human hypoxanthine phosphoribosyl transferase gene, a site that contains inactive X chromosomal obligately methylated HpaII/MspI sites and single-base allelic polymorphisms in 5% females. Carcinoma cells gated on the basis of fluorescein isothiocyanate (FITC)-anti-cytokeratin and sarcoma cells gated on the basis of FITC-antivimentin or FITC-anti-desmin were sorted to homogeneity on FACSTAR and then subjected to genomic DNA extraction and Hpa II digestion before PCR amplification and subsequent analysis of the product on denaturing gradient gel electrophoresis. The comigrations of the single homoduplexes generated from both the carcinoma cells and sarcoma cells in six different malignant mixed tumors obtained from four different organs indicated clonal identity and monoclonality in all cases. These findings of monoclonality were confirmed independently by two other methods of clonality determination. The findings of a monoclonal origin of carcinosarcomas support the single totipotential stem-cell-divergence hypothesis.

Base Sequence↗
PubMed1997

Thymoma with pseudosarcomatous stroma: report of an unusual histologic variant of thymic epithelial neoplasm that may simulate carcinosarcoma.

Six cases are described of an unusual type of primary thymic epithelial neoplasm characterized by a biphasic epithelial/spindle cell morphology that closely resembled a carcinosarcoma. The patients were two women and four men 28-70 years of age. The tumors presented clinically as asymptomatic anterior mediastinal masses found incidentally on routine chest radiographs. All patients were treated by complete surgical excision. Grossly, the tumors consisted of well-circumscribed, encapsulated masses that measured 6-14 cm in greatest diameter and showed a gray-white, homogeneous, rubbery cut surface. Histologically, the lesions were composed of anastomosing islands and cords of oval to polygonal epithelial cells displaying large nuclei with occasional prominent nucleoli and rare mitotic figures, separated by areas containing a highly cellular spindle cell proliferation without nuclear atypia. Thymic remnants could be identified in the periphery of the lesions in four cases. Immunohistochemical stains showed diffuse strong positivity for keratin and focally for epithelial membrane antigen (EMA) in the epithelial cell component, and strong positivity for vimentin and focally for actin in the spindle cell stromal component. Stains for keratin, EMA, desmin, S-100 protein, and CD34 were negative in the spindle stromal cells in all cases except one, in which EMA positivity was present; CD5 stains were negative in the epithelial cells in all cases examined. Electron microscopic examination in one case showed well-formed desmosomes and tonofilaments in the epithelial elements, as well as features indicative of fibroblastic differentiation in the spindle stromal cells. Because of the unusually florid spindle cell stromal component and the focally atypical features of the epithelial cells, some of these tumors initially were misinterpreted as examples of carcinosarcoma. Clinical follow-up in five cases showed that the patients were alive and without evidence of disease over a period of 5-20 years (mean follow-up 10 years), suggesting a benign or very low grade malignant biologic behavior. The present cases appear to represent an unusual, previously undescribed morphologic variant of thymoma characterized by a prominent pseudosarcomatous stromal component. Because of the distinctive histologic appearance and indolent clinical behavior, these lesions should be distinguished from other more aggressive anterior mediastinal neoplasms displaying a biphasic morphology.

Adult↗
PubMed2002

Molecular pathogenesis of pulmonary carcinosarcoma as determined by microdissection-based allelotyping.

Pulmonary carcinosarcoma is a rare, biphasic tumor composed of malignant epithelial and mesenchymal elements. Its histogenesis is controversial in light of the presence of divergent cell lineages and the clonal nature of malignancy. To address these issues, we performed an extensive comparative genotypic analysis using microdissection to secure representative mesenchymal and epithelial components from each of six cases of pulmonary carcinosarcoma. Loss of heterozygosity was analyzed with a panel of 12 polymorphic microsatellite markers designed to indicate allelic loss and situated in proximity to known tumor suppressor genes located on 1p, 3p, 5q, 9p, 10q, and 17p. In accordance with the relatively greater biologic aggressiveness of this tumor type, both the epithelial and mesenchymal components showed extensive allelic loss, most notably for 3p, 5q, and 17p. More importantly, we found overall equivalent patterns of acquired allelic loss between the two components on an individual case basis, strongly supporting the monoclonal origin of these neoplasms. Minor differences in the allelic fingerprint between the two cell lineages could be explained by progressive accumulation of allelic loss alterations that appear to occur more frequently in the mesenchymal component of the tumor. The data support the efficacy of microdissection-based allelic fingerprinting to delineate the relationship between different morphologic components of a single neoplasm.

Carcinosarcoma↗
PubMed2004

Aberrant nuclear/cytoplasmic localization and gene mutation of beta-catenin in classic pulmonary blastoma: beta-catenin immunostaining is useful for distinguishing between classic pulmonary blastoma and a blastomatoid variant of carcinosarcoma.

It is now known that gene mutation of beta-catenin with subsequent nuclear/cytoplasmic (N/C) overaccumulation of the protein plays an important role in tumorigenesis of various organs. We recently demonstrated that low-grade adenocarcinoma of the fetal lung type (L-FLAC)/well-differentiated fetal adenocarcinoma (WDFA), the epithelial prototype of classic pulmonary blastoma (CPB), shows N/C localization of beta-catenin with genetic mutation. This prompted us to further investigate the state of beta-catenin abnormality in CPB and related neoplasms. We studied 9 lung tumors previously diagnosed as biphasic pulmonary blastoma (PB). Histologically, 4 cases (median age 34 years) were CPB with l-FLAC/WDFA as the epithelial component, whereas 5 cases (median age 65 years) were a variant of carcinosarcoma with high-grade FLAC/clear cell adenocarcinoma with fetal lung features as the epithelial component, which we term the blastomatoid variant of carcinosarcoma (BCS). Immunohistochemically, all 4 CPBs showed aberrant N/C localization of beta-catenin both in the epithelial and mesenchymal components, with especially high staining intensity in the budding glands and morules. In contrast, all 5 BCSs showed preserved or diminished membranous expression and no significant N/C expression of beta-catenin in the epithelial component, and absent or focal N/C expression of beta-catenin in the mesenchymal component. Mutational analysis of exon 3 of the beta-catenin gene revealed that 3 CPBs harbored missense mutations (S29F, S37F, and S37F), whereas none of the 5 BCSs had this mutation. This study suggests that beta-catenin gene mutations may play a role in the tumorigenesis of CPB. Although CPB and BCS have often been grouped together as biphasic PB, they are different entities based on immunohistochemical and molecular analysis of beta-catenin. Immunostaining for beta-catenin is useful for the discrimination.

Adult↗