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Sarcomatoid Carcinoma/ Carcinosarcoma of Gall Bladder

 
 

                  

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Syn: spindle cell carcinoma

Sarcoma and carcinosarcoma constitute 1.5% of malignant tumours of the gall bladder.

There is female preponderance and a high association with gall stones.

They have a poor prognosis and spread to the liver and hilar lymph nodes.

Squamous cells may be seen. Neoplastic glands may contain mucin. The sarcomatous part is usually spindle celled. Pleomorphic cells may be present together with areas of heterologous osteosarcoma, chondrosarcoma or rhabdomyosarcoma.   Image Link

Pleomorphic spindle cell carcinoma or poorly differentiated adenocarcinoma must be excluded by immunohistochemistry.

Leiomyosarcoma, angiosarcoma, liposarcoma, neurofibrosarcoma, fibrosarcoma and rhabdomyosarcoma have all been reported but are uncommon.

                     

Tumours of the gallbladder and extrahepatic bile ducts. In: Hamilton SR, Aaltonen LA, eds. World Health Organization classification of tumors: pathology and genetics of tumours of the digestive system. Lyon, France: IARC,2000 : 204-217.

Primary liposarcoma of gallbladder diagnosed by preoperative imagings: a case report and review of literature.World J Gastroenterol. 2006 Mar 7;12(9):1472-5.

A 49-year-old Japanese woman was referred to our department because of high fever and a huge abdominal mass. Computed tomography (CT) and magnetic resonance (MR) imagings revealed a tumor, about 30 cm in diameter,occupied the right hepatic lobe and the peritoneal cavity. Abdominal angiography showed that the tumor was fed mainly by the cystic artery. We preoperatively diagnosed angiosarcoma of the gallbladder and performed tumor resection with cholecystectomy because the tumor was almost capsulated,however the posterior wall of the gallbladder attached to the tumor firmly. Histologically,the tumor was composed of spindle cells including lipoblasts with cellular pleomorphism, which were also detected in the muscular layer of the gallbladder. We finally diagnosed pleomorphic liposarcoma of the gallbladder.At 10 mo and 29 mo after the first operation, she underwent two more operations because of recurrence.Now she has a good quality of life 3 yr and 6 mo after the first operation.

Leiomyosarcoma of the gallbladder: a case report.Am Surg. 2001 Sep;67(9):873-4.

Primary sarcoma of the gallbladder is a rare disease. The tumor occurs more frequently in women. Usually gallstones are present. Symptoms resemble those of cholelithiasis or cholecystitis. The diagnosis is rarely made preoperatively. The patient was a 51-year-old woman with a 2-month history of right upper quadrant pain, nausea, vomiting, and a 10-pound weight loss. Ultrasound showed cholelithiasis and cholecystitis. Laparoscopic cholecystectomy was converted to open as a result of dense tissue in the middle to distal gallbladder. Exploration by a right subcostal incision revealed multiple implants on the surface of the liver and the peritoneum of the upper abdomen. The wall of the gallbladder was very thick and inflamed. Cholecystectomy with liver biopsy was performed. Pathology revealed poorly differentiated epithelioid leiomyosarcoma of the gallbladder with extension to the liver. The disease followed a very aggressive course and the patient died 3 weeks after the procedure. Recommended treatment is extensive surgical resection that can be followed by radiotherapy or chemotherapy. The tumor follows a very aggressive course, which often lasts a few weeks. Prognosis is poor with rare reported 5-year survivals.

Epithelioid angiosarcoma of the gallbladder: case report.J Gastrointest Surg. 2005 Jul-Aug;9(6):822-5.

A patient with epithelioid angiosarcoma of the gallbladder is described. This is only the second case of an extremely rare but highly aggressive tumor reported in the international literature. Pathophysiological, clinical, and therapeutic aspects are discussed in relation to the available data on angiosarcomas of the gallbladder.

Gallbladder carcinosarcoma: a case report and literature review.J Gastrointest Surg. 2005 Jul-Aug;9(6):818-21.

Carcinosarcoma of the gallbladder is a rare malignancy characterized by both malignant epithelial and mesenchymal components. The clinical behavior of this tumor is extremely aggressive. Only 26 cases have been reported in the world literature to date. We report the case of a 64-year-old woman who had fever associated with a right upper quadrant mass. An exploratory laparotomy through a right upper quadrant incision was performed at another institution, and the patient was thought to have severe acute cholecystitis that would require additional antibiotic therapy before attempted resection. She was referred to our center, where abdominal CT showed a 6.4 x 8.2 cm pericholecystic mass involving the hepatic flexure of the colon. The patient underwent cholecystectomy and hepatic wedge resection, pancreaticoduodenectomy, and right hemicolectomy. Pathologic examination of the surgical specimen revealed two histologic components consisting of squamous cell carcinoma and spindle cell sarcoma of gallbladder origin, consistent with carcinosarcoma. All seven lymph nodes in the pancreaticoduodenectomy specimen were negative for tumor. We present this case and a review of the literature and current treatment recommendations.

Loss of heterozygosity in clonal evolution with genetic progression and divergence in spindle cell carcinoma of the gallbladder.Hum Pathol. 2004 Apr;35(4):418-23.

Spindle cell carcinoma (SpCC) of the gallbladder is a rare neoplasm that shows carcinoma with a variable component of sarcomatoid spindle cells. The clinical and pathological features of this neoplasm have been well documented, but the histogenesis has long been a matter of speculation. In an attempt to clarify the clonality and genetic relationships involved in the evolution of this neoplasm, we microdissected a total of 18 carcinomatous and sarcomatous foci from 2 gallbladder SpCCs and analyzed the allelic status with 42 microsatellite markers on chromosomal arms 1p, 1q, 3p, 4q, 5q, 6q, 8p, 9p, 10q, 11p, 11q, 13q, 16q, 17p, 17q, 18q, and 22q. The 2 cases examined had a polypoid tumor in the gallbladder, in which both adenocarcinomatous and sarcomatoid spindle cell components were identified histologically. In both SpCCs, homogenous allelic losses were identified in both the carcinomatous and sarcomatous components; 17p, 18q, and 5q in case 1 and 17p and 11q in case 2. These indicated that both SpCCs had a single clonal origin. In case 1, additional loss of heterozygosity (LOH; 6q) consisting of genetic progression occurred in both the carcinomatous and sarcomatoid components. In case 2, there was additional LOH (9p) in the carcinomatous components and additional microsatellite instability at D5S644 in both the carcinomatous and sarcomatoid components, indicating a monoclonal neoplasm with genetic progression and divergence. In the 2 cases, the genetic changes indicated that an original clone of a pure adenocarcinoma apparently acquired sarcomatoid spindle cell phenotype by successive genetic changes. On the other hand, we saw no evidence of tumors in which a sarcomatoid spindle cell appeared to give rise to a carcinomatous subclone in the examined cases. In conclusion, the current study includes the first LOH analyses of SpCC of the gallbladder. Our data support the concept that gallbladder SpCC is derived from a single clone originating from a carcinoma. Furthermore, we showed genetic heterogeneity accompanying the phenotypic divergence, with patterns of genetic alterations that are consistent with both the progression and divergence within the individual tumors.

Sarcomatoid carcinoma with components of small cell carcinoma and undifferentiated carcinoma of the gallbladder.Pathol Int. 2004 Nov;54(11):866-71.

We report a case of sarcomatoid carcinoma with components of small cell carcinoma and undifferentiated carcinoma of the gallbladder. An 84-year-old woman was admitted to our university hospital with right upper abdominal pain and back pain. Clinical diagnosis of a gallbladder tumor was made based on the findings of abdominal ultrasonography, computed tomography and endoscopic retrograde cholangiopancreatography, and a cholecystectomy was carried out. On gross examination a pedunculated polypoid tumor protruded into the lumen of the gallbladder. Histologically the tumor was composed of carcinomatous and sarcomatous components; the carcinomatous component consisted mainly of small cell carcinoma and undifferentiated carcinoma. In general, the carcinomatous component of sarcomatoid carcinoma of the gallbladder consists of adenocarcinoma, and there have only been two previously reported cases in which the carcinomatous component consisted of small cell carcinoma or undifferentiated carcinoma. Because the patient's prognosis may be influenced by the peculiar carcinomatous component in such cases, it is important to accumulate case reports that clarify their clinicopathological features.

Sarcomatoid carcinoma of the gallbladder with a rhabdoid tumor component.Arch Pathol Lab Med. 2003 Oct;127(10):e406-8.

We report the case of a sarcomatoid carcinoma with a rhabdoid tumor component originating in the gallbladder, along with immunohistochemical and electron microscopic findings. A 61-year-old woman presented with a 5-month history of right upper quadrant pain. Ultrasonography and a computed tomographic scan indicated gallbladder cancer. She underwent a cholecystectomy and a common bile duct resection. A firm mass (4.5 cm in greatest dimension) was present in the neck portion of the gallbladder. The mass was firm, solid, yellowish gray, and granular with areas of necrosis. Microscopically, the tumor was a biphasic sarcomatoid carcinoma and consisted of diffusely arranged pleomorphic cells, focally showing rhabdoid features and neoplastic glands with focal mucin production. Heterologous components such as osteoid, chondroid, and rhabdomyoblastic elements were not identified. By immunohistochemical staining, we demonstrated that the rhabdoid cells coexpressed cytokeratin and vimentin. On electron microscopic examination, the rhabdoid tumor cells showed cytoplasmic whorls of intermediate filaments in the cytoplasm and eccentric nuclei. Two months postoperatively, the follow-up computed tomographic scan showed multiple intrahepatic metastases and omental seedings.

So-called carcinosarcoma of the gallbladder; spindle cell carcinoma of the gallbladder: report of a case.Surg Today. 2002;32(5):462-7.

We report a so-called carcinosarcoma of the gallbladder in a 53-year-old man. The findings of ultrasonography, computed tomography, endoscopic retrograde cholangiopancreatography, and angiography revealed a large mass of the gallbladder with a cholesterol stone. He underwent three operations, and died from liver failure with multiple liver metastasis immediately after the third operation. A pathological examination revealed neoplastic tissue composed of sarcomatous and glandular components. Hematoxylin-eosin stain proved the presence of carcinosarcoma in the gallbladder, but an immunohistochemical study proved that the sarcomatous component was stained by antivimentin and also anticytokeratin antibodies, which thus proved it to be so-called carcinosarcoma of the gallbladder. As a result, an immunohistochemical study is considered to provide valuable information regarding the identification of sarcomatous elements in such cases.

Carcinosarcoma of the gallbladder with chondroid differentiation.J Gastroenterol. 2002;37(11):966-71.

Carcinosarcoma of the gallbladder is an uncommon neoplasm. We herein report the case of a patient with carcinosarcoma of the gallbladder with chondroid differentiation, treated by cholecystectomy with liver segmentectomy and lymph node dissection for a tumor which occupied the entire gallbladder and spread to the liver. Histologically, the tumor contained two distinct components: a mixture of both well and poorly differentiated tubular adenocarcinoma and sarcomatoid tissue with chondroid differentiation. From a review of the literature, it was seen that carcinosarcomas of the gallbladder could be divided into two groups: one group with apparent sarcomatous differentiation, such as chondroid, osteoid, and rhabdomyosarcomatous differentiation, and the other group, of carcinosarcomas with a sarcomatous portion composed of anaplastic spindle cells. Each group had a poor prognosis in spite of surgical resection of tumors. Our patient died of peritoneal dissemination 7 months after surgery.

Sarcomatoid carcinoma (carcinosarcoma) of the gallbladder.Gen Diagn Pathol. 1998 Apr;143(5-6):321-5.

We report a case of carcinoma of the gallbladder in a 67-year-old woman. The description comprises the histological, immunohistochemical, ultrastructural and cytogenetical picture of the tumor. The ultrastructural features as well as chromosomal changes may denote the epithelial derivation of the tumor studied.

Carcinosarcoma of the gallbladder.Ann Ital Chir. 1997 May-Jun;68(3):375-8; discussion 379.

The authors analyse and comment on a case of carcinosarcoma of the gallbladder which they have recently observed. Given the rarity of this form of cancer, it is interesting to note that this case presents some peculiar aspects which have not been recorded in the few cases previously published on the subject. The peculiarities are such that they require a revision of what have until now been considered as intrinsic characteristics in the clinical observation of this kind of cancer. The authors maintain that the peculiarities of this case concern: a) the clinic onset of the illness and the relative aspecific symptomatology: b) that fact that laboratory tests did not signal any biochemical alteration; c) that the instrumental investigation's results pointed toward a non neoplastic pathology; d) that even the intra-operatory examination did not reveal any suspicious element; and last but by no means least the good survival of the patient which is definitely superior to other cases already reported in literature. The neoplasia, which manifested itself through a hemorrhage in the gallbladder, represented in fact the very initial stage of the illness, a condition rarely observed.

 
January 2008
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Anatomy of Gall Bladder

Arterial and Lymphatic Supply of Gall Bladder

Physiology of Gall Bladder

Macroscopic examination and dissection of Gall Bladder

Acute Cholecystitis

Acalculous Cholecystitis

Emphysematous Cholecystitis

Eosinophilic Cholescystitis

Xanthogranulomatous Cholecystitis

Cholelithiasis  

Cholesterol stone

Pigment stones 

"Mixed" stones

Melanosis of Gall Bladder

Porcelain Gall Bladder

Adenomyomatous Hyperplasia of Gall Bladder

Adenoma of Gall Bladder

Lymphoma of Gall Bladder

Myxoid Tumours of Soft Tissue

Classification of Soft Tissue Tumour

Gross examination of soft tissue specimen          

A practical approach to histopathological reporting of soft tissue tumours

Grading of soft tissue tumours

Lipomatous tumours

Neural tumours

Vascular tumours

Myogenic tumours

Fibroblastic/Myofibroblastic tumours

Myofibroblastic tumours

Fibrohistiocytic tumours

ChondroOsseous tumours

Soft TissueTumours of Uncertain Differentiation               

Notochordal Tumour - Chordoma

Extra-adrenal Paraganglioma

Gastrointestinal Stromal Tumour

Carcinosarcoma of the gallbladder.Gastroenterol Hepatol. 1995;18(2):81-3.

The authors present a case of carcinosarcoma of the gallbladder, discussing its possible histological origin, clinical picture, and recommended therapeutic approaches. Only 30 cases have so far been reported. The case described was seen because of a painless large volume mass in the right hypochondrium. She was surgically treated with a complete resection of the gross tumor including a portion of choledochus, followed by chemotherapy. The survival was of 20 months.

Carcinosarcoma of the gallbladder. A case report with immunohistochemical and ultrastructural studies.Cancer. 1990 Sep 1;66(5):992-7.

The authors present the histologic features, immunohistochemical findings, and ultrastructure of a carcinosarcoma of the gallbladder containing rhabdomyosarcoma as a mesenchymal element. A pedunculated polypoid tumor protruded into the lumen from the fundus of the gallbladder. The neoplasm contained two divergent components. One was malignant mesenchymal tissue with rhabdomyoblastic differentiation; the other was ordinary adenocarcinoma which was observed predominantly at the base of the polyp. Immunohistochemically, the cytoplasm of the rhabdomyoblasts stained with anti-myoglobin, myosin, and muscle actin antibodies. Ultrastructurally, there were a large number of malignant mesenchymal tissues in which various stages of differentiated rhabdomyoblasts were noted. Ultrastructural study was particularly valuable for the identification of sarcomatous elements.

Carcinosarcoma of the gallbladder. Report of a case and review of the literature.Acta Pathol Jpn. 1986 Jun;36(6):913-20.

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.