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Small cell carcinoma (SCC) of the pancreas is a rare malignancy with an extremely poor prognosis.   Visit: Small Cell Carcinoma of the Lung

This type of carcinoma accounts for about 1% of all pancreatic cancers and is commoner in elderly males who have a history of cigarette smoking.

There is usually a rapid progression of disease and widespread dissemination, especially if untreated.  The tumour is successfully treated by chemotherapy.

Differential diagnosis: Islet Cell Tumours

Primary small cell carcinoma of the pancreas with an unusual sonographic appearance. J Clin Ultrasound. 2007 Feb;35(2):82-4.

We present a case of primary pancreatic small cell carcinoma with an unusual sonographic feature. A 75-year-old woman presented with poor appetite and weight loss. Abdominal sonographic examination revealed a diffusely enlarged pancreas with relative increased echogenicity and smooth contour. CT also confirmed the diffuse infiltrative pattern of the tumor. The diagnosis was confirmed via sonographically guided biopsy. The tumor was composed of small cells with hyperchromatic nuclei and scanty cytoplasm infiltrating the pancreatic tissue, consistent with small cell carcinoma of the pancreas. Primary pancreatic small cell carcinoma rarely presents as the diffuse infiltrating type. These unusual sonographic features must be differentiated from other pancreatic tumors presenting as diffuse pancreatic enlargement.

Changes to levels of serum neuron-specific enolase in a patient with small cell carcinoma of the pancreas. J Hepatobiliary Pancreat Surg. 2005; 12(1): 93-8.

Small cell carcinoma (SCC) of the pancreas is a rare disease, with an extremely poor prognosis; only 24 cases have been reported in the literature. However, as some patients have been successfully treated with combination chemotherapy, it is important to obtain both a definite diagnosis and a precise evaluation of the effect of the treatment. A 69-year-old woman presented with an abdominal tumor and pain. She had been observed for sensory neuropathy and swelling of the pancreatic head by the referring doctor over the previous 9 months. The patient was diagnosed with SCC of the pancreas after surgery and had two courses of combination chemotherapy (cisplatin and etoposide). Initially, the tumor disappeared completely on computed tomography (CT) scans, but she died of disease recurrence 3 months after completing the chemotherapy. Changes in serum neuron-specific enolase (NSE) levels were monitored constantly during the progress of the disease. NSE levels had already increased above the upper limit of normal 8 months before the patient's admission to our hospital, and levels changed concurrently not only with tumor growth but also subsequently with remission and then relapse of the disease after treatment. These results indicate that NSE is a good marker, both as a diagnostic indicator for SCC of the pancreas and as a means of evaluating response to treatment.

Poorly differentiated small cell carcinoma of the pancreas. A case report and review of the literature.Pancreatology. 2004;4(6):521-6.

Small cell carcinoma (SCC) of the pancreas is a rare malignancy with an extremely poor prognosis. We present the case of a 74-year-old man with a 2-month history of upper abdominal discomfort who was diagnosed with SCC of the pancreas tail, involvement of peripancreatic and mesenteric lymph nodes and multiple liver metastases (extended disease). A CT scan and a positive somatostatin receptor scintigraphy showed no evidence of a primary lung tumour. The diagnosis of a SCC was confirmed by biopsy. Local tumour control could be achieved by gemcitabine once a week and a long-acting somatostatin analogue once a month, but liver metastasis showed progress. Thus, 5-fluorouracil on a weekly basis was started. The patient died 8 months after diagnosis and had not been hospitalised in the meantime.

                   

Curative resection of a small cell carcinoma of the pancreas: report of a case of long survival without chemotherapy. J Gastroenterol Hepatol. 2004 Sep;19(9):1087-91.

A 41-year-old asymptomatic man was, by chance, diagnosed as having a mass in the left upper quadrant of the abdomen by ultrasound. Computed tomography and magnetic resonance imaging showed a slightly enhanced heterogeneous mass, measuring about 5 cm in diameter, adjacent to the pancreas tail and spleen. On abdominal angiography the tumor was found to be fed by the splenic artery, and no encasement was observed. At operation the tumor was connected to the pancreas tail and attached to the spleen, and no metastasis was evident. As a result of these observations, a curative resection was performed. On histological examination the tumor cells had no ductal or architectural organization and were continuous to the normal pancreatic tissues. The cells were negative for Grimelius argylphile and the periodic acid schiff stain. Immunohistochemistry indicated that the tumor cells were positive for keratin and epithelial membrane antigen, but negative for chromogranin A, vimentin, alpha1-antitrypsin and alpha1-antichymotrypsin. The tumor was diagnosed as a small cell carcinoma of the pancreas, which is a rare disease. In previously reported cases curative resection of the tumor had not been possible because of its rapid progression and metastasis, and the prognosis is generally very poor. In our case, however, curative resection of the tumor was done, and the patient is healthy with no signs of recurrence for 56 months after the operation and with no additional therapy.

Diagnostic features on images in primary small cell carcinoma of the pancreas. Am J Gastroenterol. 1997 Feb;92(2):319-22.

Small cell carcinoma of the pancreas is a rare tumor with very poor prognosis, but the tumor is successfully treated by chemotherapy. Therefore, small cell carcinoma must be differentiated from other pancreatic tumors on images. A 71-year-old man presented with digestive symptoms. Multiple hypoechoic masses were detected on transabdominal ultrasonography and lateral shadow and posterior echo enhancement on endoscopic ultrasonography. The dynamic computed tomography scan revealed that the tumor walls were hypervascular at an early phase, and that the central portions were gradually stained at a late phase with differences among the tumors; tumor stains were also found on angiography. The distal site of the main pancreatic duct was completely interrupted, as seen on endoscopic retrograde pancreatography. The resected specimens were composed of circumferential hard walls and internally necrotic, hemorrhagic contents of different degrees and were microscopically diagnosed as small cell carcinoma. The tumor has to be differentiated from hypervascular pancreatic tumors, especially islet cell tumors.

Primary small cell carcinoma of the pancreas.Pathology.1993;25(3):240-2.

A 59 yr old male presented with abdominal pain and jaundice. CT scan showed a tumor in the pancreas with secondaries in the liver, peri-pancreatic lymph nodes, vertebral bone marrow and subcutaneous tissue of the neck together with a cervical lymph node. The patient died after 2 wks and autopsy revealed complete replacement of the pancreas by tumor. Careful dissection of both lungs failed to show any tumor. A diagnosis of primary small cell carcinoma of the pancreas was made. Review of the literature indicates that this type of carcinoma accounts for about 1% of all pancreatic cancers and is commoner in elderly males who have a history of cigarette smoking. There is usually a rapid progression of disease and widespread dissemination, especially if untreated. Some success has been obtained with chemotherapy.

Small cell undifferentiated carcinoma of the pancreas. Report of a patient with tumor marker studies.Cancer. 1992;70(6):1514-9.

BACKGROUND. Small cell undifferentiated carcinoma of the pancreas is a rare type of pancreatic neoplasm. METHODS. The authors report the clinical and pathologic aspects of a patient with this malignant lesion and an extensive serologic and immunohistochemical survey of potential ectopically produced hormones or tumor markers. RESULTS. Neuron-specific enolase (NSE) emerged as a tumor marker. CONCLUSIONS. NSE could be valuable in the diagnosis and management of other patients with this rare disease.


September 2007

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Anatomy of Normal Pancreas

Normal Islets of Langerhans

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

Non-Neoplastic Tumour-Like Lesions of the Pancreas

Neoplasms of the Endocrine Tumours

Islet Cell Tumours

Glucagonomas

Insulinomas

Somatostatinoma

Enterochromaffin Cell (Carcinoid) Tumours

Pancreatic Gastrinoma

Multiple Endocrine Neoplasia (MEN) Syndrome

Carcinoma of the Pancreas