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Carcinosarcoma of the submandibular gland. An autopsy case.

We present a rare case of submandibular gland carcinosarcoma occurring in a 45-year-old male patient. His clinical history revealed that the carcinosarcoma had developed from a carcinoma ex mixed tumor in three years. In spite of repeated resection, intensive chemotherapy and irradiation, the tumor recurred and grew rapidly, and the patient died of hemothorax caused by rupture of a pulmonary metastatic tumor. The fourth recurrent tumor and autopsy specimens showed features of carcinosarcoma consisting of three tumor components, i.e., undifferentiated carcinoma, and chondrosarcomatous and osteosarcomatous growth. The metastatic nodules in both lungs and pulmonary hilar lymph nodes showed the same pattern. Immunohistochemically, the chondrosarcomatous cells were positive for vimentin and S-100 protein, and for epithelial markers such as epithelial membrane antigen (EMA) and cytokeratin (MA-902). Undifferentiated carcinoma cells, on the other hand, were partially positive for muscle actin other than cytokeratin (KL 1). Ultrastructurally, desmosome-like structures were seen in the chondrosarcomatous cells. These findings suggest that the sarcomatous lesions might have originated from epithelial cells.

Actins↗

Gastric stump carcinosarcoma with rhabdomyosarcomatous differentiation.

Gastric carcinosarcoma is an unusual tumor and its occurrence in the gastric stump is extremely rare. A report is presented here of a unique case of gastric stump carcinosarcoma with rhabdomyosarcomatous differentiation in a 74-year-old man. The patient had undergone partial gastrectomy with gastrojejunostomy (Billroth II method) 30 years previously. The tumor had both adenocarcinoma and sarcoma components, and an immunohistochemical study suggested a focal transition between these components. The main sarcomatous components showed fibrosarcomatous features with a scattered distribution of rounded tumor cells, whose rhabdomyosarcomatous differentiation was immunohistochemically determined. Ultrastructural examination supported the rhabdomyosarcomatous natures. Experience with the present tumor indicates that carcinosarcoma with rhabdomyosarcomatous differentiation can occur in the gastric stump and that this disease is capable of aggressive behavior.

Aged↗

Esophageal carcinosarcoma with basaloid squamous carcinoma and rhabdomyosarcoma components with TP53 mutation.

Carcinosarcoma of the esophagus is a rare tumor with a distinct pathological entity having squamous cell carcinoma as the most described carcinomatous component. This paper reports the first case of carcinosarcoma of the esophagus that showed predominant basaloid squamous carcinoma component in addition to squamous cell carcinoma and poorly differentiated carcinoma and sarcoma component. A 64-year-old male patient consulted for dysphasia and chest pain was examined and found to have gastrointestinal fiber-endoscope and a polypoid growth in the lower third of the esophagus. Partial esophagectomy was performed and the excised tumor showed histological features of carcinosarcoma with heterogeneous carcinomatous components with dominance of basaloid squamous carcinoma and minority of squamous cell carcinoma, poorly differentiated carcinoma, and sarcomatous component, immunohistochemically proven to be rhabdomyosarcoma. Immunohistochemical study and TP53 mutation analysis was carried out to explain the histogenesis of this rare tumor. The distinct immunohistochemical profiles of the carcinomatous and sarcomatous components suggested the possibility of transition from a carcinomatous to a sarcomatous component. The similar TP53 mutation in the carcinomatous and sarcomatous component suggested each of these components had the same origin, that is, the tumor was monoclonal in origin.

Biomarkers, Tumor↗

Carcinosarcoma of the liver producing granulocyte-colony stimulating factor.

An autopsy case of carcinosarcoma of the liver producing granulocyte-colony stimulating factor (G-CSF) is reported. The patient, a 74-year-old Japanese man, presented with multiple liver masses. His serum G-CSF was elevated to 286 pg/mL and a marked leukocytosis of 19 100/microL was observed. The patient had a rapidly aggravated clinical course and died 57 days after admission. Autopsy revealed a liver carcinosarcoma composed both of hepatocellular carcinoma (HCC) and sarcomatous elements immunoreactive with alpha-smooth muscle actin and desmin. Immunohistochemistry revealed positive staining of G-CSF in the cytoplasm of HCC, whereas none of the spindle cells was positively stained. Production of G-CSF was also confirmed by enzyme-linked immunosorbent assay, using the frozen tumor tissue taken at the autopsy. Similar to the majority of G-CSF-producing tumors in the literature, only the epithelial elements of the present case were immunopositive for G-CSF. Although a monoclonal origin of carcinosarcomas has generally been proposed, heterologous differentiation from a single clone might lead to the production of G-CSF only in the epithelial element in the present case. It is suggested that G-CSF was associated with the high-grade transformation of the epithelial elements, as well as the reported phenomenon of conventional HCC producing G-CSF.

Actins↗

Carcinosarcoma of the ureter: report of a case with unusual histology and review of the literature.

Carcinosarcomas of the ureter are rare biphasic neoplasms, with distinct malignant epithelial and mesenchymal components. To the authors' knowledge there have been only 12 cases reported in the literature. Primary cartilaginous tumors of the ureter are also rare tumors. Thus far there has been a single case report of an extraskeletal mesenchymal chondrosarcoma of the ureter in the German literature. Herein a unique case is reported of ureteral carcinosarcoma in an 81-year-old woman presenting with gross hematuria in which the sarcomatous component had extraskeletal mesenchymal chondrosarcomatous differentiation. This is thought to be the first reported case of a carcinosarcoma with such a differentiation in the sarcomatous component.

Aged, 80 and over↗

Serous ovarian carcinoma recurring as a heterologous carcinosarcoma.

Only two cases of recurrence of heterologous carcinosarcoma from adenocarcinoma of the ovary have been documented in the published literature. We report a case of serous ovarian carcinoma recurring as a heterologous carcinosarcoma. The immunohistochemical analysis of several biological parameters in primary ovarian adenocarcinoma and recurrent carcinosarcoma has been also performed.

Carcinosarcoma↗

Carcinosarcoma arising from atypical endometriosis in a cesarean section scar.

Malignant change of endometriosis in a cesarean scar (CS) is rare. We report a case of carcinosarcoma arising from atypical endometriosis in a CS scar, which was successfully treated with complete excision of the lesion and repair of the abdominal wall defect with autologous skin-muscle flap graft. A 41-year-old woman presented with a recurrent endometriosis in a CS scar. Within 16 years it changed from benign to atypical endometriosis and finally to carcinosarcoma after three operations. Complete excision of the tumor was performed, with a big defect of abdominal wall successfully repaired by autologous pedicle skin-muscle graft. The diagnosis of carcinosarcoma arising from atypical endometriosis was confirmed histologically. The lesion recurred 6 months after the fourth operation. She died of disease 15 months after the fourth operation. This case demonstrated that long-standing recurrent scar endometriosis could undergo malignant changes and should be made aware. The primary treatment is complete surgical excision.

Abdominal Wall↗

Carcinosarcoma of the ampulla of Vater: a case report with immunohistochemical and ultrastructural studies.

Carcinosarcoma of the duodenum has not been reported previously, although this type of tumor has been detected in other organs. We present here a case of carcinosarcoma of the duodenum, including immunohistochemical and electron microscopical findings. An ulcerating tumor, located in the duodenal ampullary region, contained two divergent components: ordinary differentiated tubular adenocarcinoma, and sarcomatoid tissue composed of spindle tumor cells. Immunohistochemically, the adenocarcinoma cells were stained with antibodies against epithelial markers including keratin and CA19-9. In contrast, the sarcomatoid tissue was strongly positive for vimentin and was focally positive for myoglobin, keratin, and CA19-9. We speculate that the sarcomatoid element of the carcinosarcoma arose from part of the ordinary adenocarcinoma tissue.

Ampulla of Vater↗

Carcinosarcoma in a bladder diverticulum. A case report and literature review.

Carcinosarcoma of the bladder is a rare neoplasm composed of a mixture of malignant epithelial and mesenchymal components. The presentation and clinical course of an infiltrating carcinosarcoma located in a bladder diverticulum coexisting with prostatic adenocarcinoma in a 69-year-old man are described. We believe this to be the fifth report of true carcinosarcoma appearing in a bladder diverticulum.

Adenocarcinoma↗

Primary heterologous carcinosarcoma of the ureter with necrotic malignant polyps. Report of a case and review of the literature.

Carcinosarcoma is a rare and aggressive disease characterized by biphasic neoplasms with distinct mesenchymal and epithelial components. We report a case of ureteral carcinosarcoma with malignant necrotic polyps. The patient was a 58-year-old woman with painless hematuria, who was later diagnosed as having ureteral carcinosarcoma. Three long pendulous polypoid-shape tumors consisting of high-grade transitional cell carcinoma with chondrosarcomatous and osteosarcomatous elements were found. Two months after nephroureterectomy, the tumor relapsed in the bladder. Despite anterior exenteration, the patient died of local recurrence 6 months after her initial visit. To our knowledge, only 10 cases of this disease have been reported in the literature.

Carcinoma, Transitional Cell↗

Complete cytoreduction combined with early postoperative intraperitoneal chemotherapy for ovarian carcinosarcoma. Report of two cases.

BACKGROUND/AIMS: The purpose of the study is to report two long-term survivors with ovarian carcinosarcomas, which are rare intrapelvic malignancies with unfavorable prognosis. METHODS: Two middle-aged women with palpable abdominal tumors underwent complete cytoreductive surgery and no macroscopic tumor was left behind. Early postoperative intraperitoneal chemotherapy with doxorubicin was used as an adjuvant treatment. RESULTS: The patients are disease-free 2 and 3 years, respectively after the initial therapy. CONCLUSION: Complete cytoreduction combined with early postoperative intraperitoneal chemotherapy seems to be an effective treatment for ovarian carcinosarcoma with peritoneal spread. Further studies are required to define the value of intraperitoneal chemotherapy in ovarian carcinosarcoma.

Antibiotics, Antineoplastic↗

Carcinosarcoma of the prostate.

A very rare case of carcinosarcoma of the prostate is reported. The patient was a 77-year-old man in whom both primary and metastatic tumors presented the pathology of carcinosarcoma of the prostate. The carcinosarcoma was resistant to anti-androgen therapy, and the patient showed low level of serum prostatic acid phosphatase and was free from bony metastases despite multiple metastases to the lung, liver, pancreas, para-aortic lymph nodes, spleen and penis. The sarcomatous component consisted of chondrosarcoma and fibrosarcoma, both of which were positive for vimentin. The carcinomatous component was positive for both keratin and prostatic acid phosphatase.

Acid Phosphatase↗

Carcinosarcoma of the uterus: magnetic resonance imaging.

We report the magnetic resonance imaging (MRI) findings with a paramagnetic enhancement agent (gadopentetate dimeglumine (Gd-DTPA)) in a 70-year-old woman with carcinosarcoma of the uterus. In the present patient, the polypoid portion histologically most abundant in the sarcomatous component was strongly enhanced by Gd-DTPA and was clearly demonstrated with high signal intensities on contrast-enhanced T1-weighted images. We suggest that MRI with Gd-DTPA may be clinically useful for detection of carcinosarcoma, and that any patient having endometrial carcinoma with the polypoid portion, which is more strongly enhanced by Gd-DTPA within the tumor, may be suspected of carcinosarcoma.

Aged↗

Carcinosarcoma of monoclonal origin arising in a dermoid cyst of ovary: a case report.

BACKGROUND: Transformation of a cystic benign teratoma of the ovary into a "carcinosarcoma" has very rarely been reported and its histogenetic origin is still debated. CASE PRESENTATION: A case of carcinosarcoma arising from a dermoid cyst is reported. The tumor showed cystic areas delimited by normal squamous epithelium, with transitional areas through dysplastic epithelium to "in situ" and infiltrating squamous cell carcinoma (SCC). The sarcomatous component showed compact tissue composed of round cells concentrically arranged around small vessels, spindle, and pleomorphic cells with a high nuclear-cytoplasmic ratio. Positive staining for vimentin, alpha smooth muscle actin and CD10, as well as P53 and P63, was found in the sarcomatous component and in some atypical basal cells of the squamous epithelium, which also showed the usual epithelial markers. CONCLUSION: To the best of our knowledge, this is the first case of carcinosarcoma arising from a dermoid cyst in which a histogenetic origin from totipotent stem cells, located in the basal squamous layer, is supported by immunohistochemical findings.

Aged↗

Histomorphological investigation regarding to malignant transformation of pleomorphic adenoma (so-called malignant mixed tumor) of the salivary gland origin: special reference to carcinosarcoma.

Pleomorphic adenoma is the best-recognized tumor among those of salivary gland origin that also has the highest incidence. However, it is not uncommon for it to become malignant, and it has been noted that the frequency of malignancy increases as a result of the tumor persisting without being treated following the onset of adenoma. Carcinomatous components demonstrate various histological appearances, and in particular, there have been sporadic reports of carcinosarcoma in which malignant findings are not only observed in epithelial components, but in mesenchymal components as well. We conducted a histopathological study on cases of malignant-transformed pleomorphic adenoma, namely so-called malignant mixed tumor observed in our department. The subject cases consisted of a total of 19 cases registered in the diagnostic files of our department and included 12 males and 7 females. Age ranged from 46 to 87 (average 67.7) years old. The sites of occurrence consisted of the parotid gland in 17 cases, and palatal and buccal minor salivary glands in one case each. Histological types consisted of 7 cases of adenocarcinoma not otherwise specified, 3 cases of squamous cell carcinoma, 3 cases of salivary duct carcinoma, and one case each of polymorphous low-grade adenocarcinoma and epithelial-myoepithelial carcinoma, while the remaining were 2 cases of sarcomatoid carcinoma and 2 carcinosarcoma. We described herein their histological characteristics, discussed diagnostic problems in various instants in which each phenotype on this setting, and reviewed the literature, especially as to carcinosarcoma in contrast with those derived from other organs.

Adenoma↗

Carcinosarcoma of the liver.

An autopsy case of carcinosarcoma of the liver in an 84-year-old man is described. The 14 x 6-cm solid tumor was located in the hilus to the left lobe and was grayish-white with some translucent areas. Histologically, the tumor consisted of an intimate mixture of adenocarcinomatous and chondrosarcomatous elements with transitional areas in between. Immunohistochemically, cells of the adenocarcinomatous elements were positive for cytokeratin but negative for S100 protein, whereas cells of the chondrosarcomatous elements showed the reverse staining pattern. Cells of transitional areas were positive for both cytokeratin and S100 protein. Most previously reported cases of carcinosarcoma of the liver have involved elderly men and have had a poor prognosis. The findings of the present case support the view that carcinosarcomas represent carcinomas that develop a sarcomatous element via metaplasia of the epithelial element.

Adenocarcinoma↗

Carcinosarcoma of the urinary bladder following cyclophosphamide therapy: evidence for monoclonal origin and chromosome 9p allelic loss.

We report a case involving a 45-year-old man with a 12-year history of Wegener granulomatosis, who developed a carcinosarcoma of the urinary bladder after long-term cyclophosphamide therapy. Cyclophosphamide is well recognized as an etiologic agent for urothelial carcinoma of the urinary bladder. However, only 5 cases of carcinosarcoma of the urinary bladder following cyclophosphamide therapy have been reported. We used loss of heterozygosity studies and microsatellite markers to define the molecular basis of this rare neoplasm. These studies revealed evidence supporting a monoclonal origin for the 2 components of this tumor. We also demonstrated allelic loss of chromosome 9p. This loss associated with carcinosarcoma of the urinary bladder is in agreement with previous studies, suggesting a possible role for the tumor suppressor gene p16 in the pathogenesis of this tumor.

Carcinosarcoma↗

Carcinosarcoma of the liver: a case report and review of the literature.

No more than 11 cases of carcinosarcoma of the liver have been reported in the past 40 years that fulfill the definition of hepatocellular carcinoma combined with differentiated sarcomatous elements. Most cases consist of hepatocellular carcinoma with 1 to 2 heterologous elements. We report a case of a 51-year-old woman with liver carcinosarcoma consisting of 3 carcinomatous components and 4 sarcomatous components. Hepatocellular carcinoma, fibrolamellar type, was accompanied by neuroendocrine carcinoma (neuron-specific enolase and synaptophysin positive) and adenocarcinoma (cytokeratin 7 and 20 positive). The sarcomatous elements consisted of poorly differentiated spindle cell neoplasm (vimentin positive), leiomyosarcoma (smooth muscle actin positive), rhabdomyosarcoma (desmin positive), and osteosarcoma. To our knowledge, this is the first case of liver carcinosarcoma with this many differentiated heterologous features. There are differing views on the pathogenesis of this tumor. Findings in this case support the view that metaplasia of carcinomatous cells gives rise to the sarcomatous elements.

Antineoplastic Agents↗