HISTOPATHOLOGY INDIA.COM Atypical Fibroxanthoma

 

               

Syn:Anaplastic; Pleomorphic Large Cell; Pleomorphic Giant Cell; Sarcomatoid Carcinoma.

Visit: Pancreatic Pathology Online ; Exocrine Pancreatic Tumours.

It constitute about 2-7% of exocrine pancreatic cancers.

Age and sex:  The tumours have similar age and sex distribution like

ductal adenocarcinoma  but are clinically more aggressive.

Gross features:  Grossly, they are large and soft with prominent

hemorrhage, necrosis and cystic changes.

Microscopic features:  Microscopically, the tumour consists of large

mononuclear pleomorphic cells and bizarre multinucleated giant cells,

with scanty fibrous stroma.  Mitoses are frequent.

Visit: Undifferentiated Carcinoma with Osteoclast-like Giant Cell

Spread: There is extensive perineural, lymphatic and vascular invasion.

                  

Anaplastic carcinoma of the pancreas with squamous features: report of a case and immunohistochemical study.Med Sci Monit. 2005 Nov;11(11):CS65-8.

BACKGROUND: Anaplastic carcinomas of the pancreas are rare aggressive tumors with survival measurable in weeks. Many terms have been applied used to describe these tumors, and anaplastic foci are identified in ductal adenocarcinomas and in ectopic pancreata, but are not the dominant pattern of growth. We herein present our experience with a case of anaplastic carcinoma of the pancreas with squamous features in order that allowed us to delineate the clinicopathologic and immunohistochemical features of this rare entity. CASE REPORT: According to imaging findings, the 77-year-old Japanese man was diagnosed as the malignant pancreatic tumor, and underwent a surgical resection. Histopathologically, anaplastic tumor cells showed focal ductal and squamous features infiltrated into pancreatic parenchyma, extrapancreatic fatty tissue, and stomach. The tumor cells showed strong reactivity for cytokeratin, alpha-SMA, vimentin, NSE, and S-100 protein. Although immunoreactivity against p53 was negative, strong positive immunostaining for proliferating cell nuclear antigen and interleukin-1 receptor type I (IL-1RI) was observed in a the majority of tumor cells, while the alpha6 integrin subunit was predominantly strong expressed in the adenocarcinomatous lesion. The patient's postoperative course was uneventful and he was treated with a chemotherapy consisting of gemcitabine. After discharging from the hospital, he had subsequently been observed as an outpatient and same chemotherapy was followed by weekly. However, the patient suffered from peritonitis carcinomatosa and re-increases of multiple liver metastases, and he died in the fourteenth month after surgery. CONCLUSIONS: Our immunohistochemical studies suggested that the prognosis of the case with anaplastic carcinoma presented here would be poor, due to the strong expression of integrins and IL-1RI.

Undifferentiated pancreatic carcinomas. Leap into chaos.Pathologe. 2005 Feb;26(1):18-21.

Undifferentiated pancreatic carcinomas are rare anaplastic variants of ductal adenocarcinoma of the pancreas. They have to be distinguished immunohistochemically from metastases of malignant melanoma or an infiltrating sarcoma. An uncommon variant is undifferentiated pancreatic carcinoma with osteoclast-like giant cells. This variant can be associated with mucinous cystic neoplasms and is characterized by abundant non-neoplastic osteoclast-like giant cells.

Anaplastic pancreatic carcinoma. A case report and review of literature . JOP. 2004 Nov 10;5(6):512-5.

CONTEXT: Anaplastic pancreatic carcinoma is an aggressive neoplasm with survival measurable in weeks. It presents as a large cystic mass with loco-regional and distant spread. Three histological types have been described: pleomorphic, spindle cell and sarcomatoid. CASE REPORT: We describe the case of a 74-year-old woman with pleomorphic anaplastic carcinoma of the pancreas diagnosed after laparoscopic biopsy. The patient had a rapid downhill course with progression of the disease and demise within 4 weeks after diagnostic laparoscopy. CONCLUSION: Due to the rapid spread of the disease, no effective cure exists for these tumors. A brief review of the histological and radiological findings and the possible mechanisms of the pathogenesis of anaplastic tumors is included in the discussion.

A clinicopathologic and immunohistochemical study of 35 anaplastic carcinomas of the pancreas with a review of the literature. Ann Diagn Pathol. 2001 Jun;5(3):129-40.

Anaplastic pancreatic carcinomas are rare tumors, frequently displaying a variety of growth patterns. The literature lacks a comprehensive study of this tumor. Thirty-five cases of anaplastic carcinoma of the pancreas diagnosed between 1955 and 1997 were retrieved from the Endocrine Registry at the Armed Forces Institute of Pathology. Histology, immunophenotype, molecular analysis, and patient follow-up were analyzed. The tumors of 10 women and 25 men, aged 34 to 85 years (mean age at presentation, 62.5 years), were studied. Patients had vague symptoms (weight loss, pain, and fatigue, nausea, or vomiting), lasting an average of 13.2 weeks. The tumors, of an average size of 9.2 cm, were usually in the head or tail of the pancreas. The tumors were widely infiltrative, histomorphologically separated into predominantly large, pleomorphic cell, or spindle cell groups. Tumor phagocytosis and necrosis were noted. Immunohistochemical studies confirmed an epithelial origin with at least one epithelial marker in 78% of the tumors. K-ras mutations by sequence analysis were found in eight of 12 cases tested. Surgical biopsy/excision was used in all patients. Twenty-nine of 35 patients died of disease (average, 5.2 months), three died with no evidence of disease (average, 56.9 months), and three patients were alive at last follow-up (average, 94.0 months), one with residual disease. There was no statistically significant difference in survival between patients with and without a K-ras mutation. Anaplastic carcinoma of the pancreas usually occurs in the head of the pancreas in older men. The epithelial nature of the pleomorphic cells (giant or spindled) can usually be documented. Patients with K-ras mutations have a shorter survival time, even though the overall prognosis for all anaplastic carcinomas is fatal (93% fatality; average survival, 448 days).

Phenotypical characteristics of undifferentiated carcinoma of the pancreas: a comparison with pancreatic ductal adenocarcinoma and relevance of E-cadherin, alpha catenin and beta catenin expression.Oncol Rep. 2001 Jul-Aug;8(4):745-52.

The characteristics of undifferentiated (anaplastic) carcinoma (UC) of the pancreas were analysed and compared with those of poorly differentiated adenocarcinoma (PA). Eight cases of UC of the pancreas were evaluated by clinicopathological and immunohistochemical methods and compared with 20 cases of PA. Large size of the tumor, local invasion and lymph node metastasis were frequently seen in UC, leading to a significantly worse prognosis compared to that of PA (p=0.022). Immunohistochemically, E-cadherin expression was completely lost in 7/8 UC cases, whereas half the PA cases revealed a strong reactivity for E-cadherin. alpha- and beta-catenin expressions tended to be impaired in UC compared with PA. The results suggest that the altered expression of adhesion molecules is correlated with dedifferentiated change and is contributory to its aggressive biological behaviour.


Anatomy of Normal Pancreas ;Normal Islets of Langerhans ; The Apud Concept ; An approach to reporting of pancreatic specimen ; Reporting of pancreatic biopsies for the diagnosis of neoplastic lesions ; Reporting of ampullary and periampullary biopsies for the diagnosis of neoplastic lesions ;Reporting of Pancreatico duodenectomy (Whipple's operation) specimen ; Reporting of Distal Pancreatectomy Specimen ; Developmental Defects of Pancreas ; Nesidioblastosis ; Pancreas Divisum ; Aberrant(Ectopic)Pancreas ; Annular Pancreas ; Pancreatic Agenesis ; Non-Neoplastic Pancreatic Cysts  ; Pancreatitis ; Acute Pancreatitis ; Chronic Pancreatitis ; Autoimmune Pancreatitis ; Herpes Simplex Pancreatitis ; Diabetes Mellitus ; Neoplasms of the Endocrine Tumours ; Islet Cell Tumours ; Glucagonomas  ; Insulinomas  ; Somatostatinoma ; VIPomas ; Pancreatic Polypeptide-Secreting Tumours ; Enterochromaffin Cell (Carcinoid) Tumours ; Pancreatic Gastrinoma ; Corticotropinoma ; Multiple Endocrine Neoplasia (MEN) Syndrome ; Carcinoma of the Pancreas ;Exocrine Pancreatic Tumours and Tumour -Like Lesions ; Paediatric Pancreatic Tumours ; Acinar cell carcinoma ; Pancreatoblastoma ; Adenosquamous Carcinoma of the Pancreas ;
September 2007

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Undifferentiated carcinoma of the pancreas: analysis of intermediate filament profile and Ki-ras mutations provides evidence of a ductal origin.
J Pathol. 1998 May;185(1):53-60. 

Undifferentiated carcinomas and osteoclast-like giant cell tumours of the pancreas commonly contain foci of neoplastic ductal glands. To test the hypothesis that undifferentiated carcinomas and osteoclast-like giant cell tumours have a ductal origin, the immunocytochemical cytokeratin pattern and the frequency and type of Ki-ras mutations at colon 12 were studied in a series of 17 undifferentiated carcinomas and two osteoclast-like giant cell tumours. The cytokeratin features of undifferentiated carcinomas and osteoclast-like giant cell tumours were compared with those found in 10 ductal adenocarcinomas, 20 acinar cell carcinomas, 25 neuroendocrine tumours, and 15 solid-pseudopapillary tumours. All undifferentiated carcinomas and osteoclast-like giant cell tumours stained with at least one cytokeratin antibody, and 13/19 of them with antibodies against cytokeratins 7, 8, 18, and 19. The latter cytokeratins were expressed in all ductal adenocarcinomas, but only in 15/20 acinar cell carcinomas, 2/25 neuroendocrine tumours, and 1/15 solid-pseudopapillary tumours. In addition to cytokeratin, 15/19 undifferentiated carcinomas/osteoclast-like giant cell tumours were positive for vimentin. Ki-ras mutations at codon 12 were found in 10 undifferentiated carcinomas and one osteoclast-like giant cell tumour from which DNA could be successfully amplified. The Ki-ras mutation patterns were analysed in six tumours and corresponded to those typical of ductal adenocarcinomas. In tumours with ductal and anaplastic components, both components revealed identical mutation patterns. From these findings, it is concluded that both undifferentiated carcinomas and osteoclast-like giant cell tumours belong to the pancreatic tumours that show a ductal phenotype. Since undifferentiated carcinomas and osteoclast-like giant cell tumours share the same cytokeratin and Ki-ras features, they are probably derived from the same cell lineage.