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Primary neoplasms of the pancreas are most often adenocarcinoma.

Non-Hodgkin's lymphoma (NHL) involving the pancreas is less common but well documented.

Primary pancreatic Non-Hodgkin lymphoma predominantly involving the pancreas is a rare tumour and accounts for less than 0.7% of all pancreatic malignancies  and 1% of extranodal lymphomas.

The expression "pancreatic lymphoma" has been used to describe both primary lymphoid neoplasms originating in the pancreatic parenchyma and tumours invading from a peri-pancreatic lymphadenopathy.

The majority of patients with pancreatic cancer, whether it is adenocarcinoma or lymphoma, present with a mass in the head of the gland.

Pancreatic lymphoma is often described as a large homogeneous mass with extra-pancreatic extension, with or without associated lymphadenopathy.

Less common presentations are masses in the body or tail, or more rarely diffuse involvement of the pancreas.

Percutaneous fine-needle aspiration (FNA) of the pancreas requires experienced cytopathologists as well as advanced immunohistochemical assays to obtain a final diagnosis on a small amount of tissue.

Histopathological examination is usually mandatory to obtain a definitive diagnosis since symptoms and radiological features are quite similar to those of other pancreatic masses.

Follicular lymphoma is a rare subtype of primary pancreatic lymphoma.

In case of primary pancreatic lymphoma, surgery and adjuvant chemotherapy or radiotherapy can produce fairly good outcomes.

                   

Follicular lymphoma of the pancreas: a case report and proposed new strategies for diagnosis and surgery of benign or low-grade malignant lesions of the head of the pancreas.: JOP. 2007 Jan 9;8(1):44-9.

CONTEXT: Primary pancreatic lymphoma is a rare form of extranodal lymphoma originating in the pancreas. The present report describes a case of follicular lymphoma of the pancreas with unique CT and MRI findings. CASE REPORT: A 58-year-old male complained of sudden abdominal pain, and routine ultrasonography detected an 8 cm hypoechoic tumor in the head of the pancreas. The 3D image generated using multi-cholangiography and virtual duodenography provided the information necessary for a laparotomy. The tumor was enucleated for diagnosis. Follicular lymphoma is quite rare in the pancreas and gastrointestinal tract. A considerable number of pancreatic lymphoma subtypes have been reported. The expression "pancreatic lymphoma" has been used to describe both primary lymphoid neoplasms originating in the pancreatic parenchyma and tumors invading from a peri-pancreatic lymphadenopathy. The present case belongs to the latter, which might explain the unique imaging findings and histological type. These subtypes display different imaging findings and different clinical characteristics. In the future, primary pancreatic lymphoma should be discussed separately depending on the subtype. CONCLUSION: We propose a new subtype of primary pancreatic lymphoma. Multi-cholangiography and virtual duodenography provided the information necessary for a laparotomy in the present case. Enucleation is indicated for benign and low-grade malignant tumors of the pancreas, even if the tumor is located in the head of the pancreas.

Problems in diagnosing lymphoma of the pancreas with computed tomography. A case report. J Gastrointestin Liver Dis. 2007 Mar;16(1):101-3.

Primary lymphoma of the pancreas is a rare form of extranodal lymphoma accounting for less than 0.5% of pancreatic tumors. Percutaneous fine-needle aspiration of the pancreas with histopathological examination and immunohistochemical assay confirm the diagnosis. A 73 year old male presented with recurrent pancreatic type abdominal pain with significant weight loss over 1 year. He was pale with ill defined epigastric mass. Contrast enhanced CT showed an ill defined poorly marginated non enhancing hypodense mass lesion involving the body of the pancreas. CA 19-9 was normal. CT guided aspiration cytology was suggestive of hemolymphoid malignancy. Immunohistochemistry was positive for Leukocyte Common Antigen and CD 34. It was negative for CD3 and CD 20 indicating an undifferentiated lymphoma. Patient received two sessions of chemotherapy and was followed-up.

Primary pancreatic lymphoma: diagnostic and therapeutic dilemma. Pancreas. 2006 Aug;33(2):192-4.

OBJECTIVES: Non-Hodgkin lymphoma predominantly involving the pancreas is a rare tumor and accounts for less than 0.7% of all pancreatic malignancies and 1% of extranodal lymphomas. Diagnosis of primary pancreatic lymphoma can be difficult because it may mimic carcinoma. The principal aims of this review were to highlight the difficulties encountered in making a diagnosis and to identify the role of surgery. METHODS: A PubMed search was conducted using the following terms: primary pancreatic lymphoma and non-Hodgkin lymphoma of the pancreas. Additional references were sourced from key articles. RESULTS: A total of 89 reported cases of pancreatic lymphoma between 1951 and 2005 were reviewed. An accurate preoperative diagnosis of primary pancreatic lymphoma is not always possible. A complete response rate of 100% and a long-term survival rate of 94% have been reported with surgery and adjuvant chemotherapy when compared with a 5-year survival rate of less than 50% and an overall 3-year disease-free survival rate of 44% with current chemotherapy, radiotherapy, or combined methods. CONCLUSION: Pancreaticoduodenectomy may have a therapeutic role in association with chemotherapy.

Primary pancreatic lymphoma: report of six cases.World J Gastroenterol. 2006 Aug 21;12(31):5064-7.

AIM: To heighten recognition of primary pancreatic lymphoma (PPL) in clinical practice. METHODS: A retrospective review of the clinical presentation, imaging characteristics and pathological features of PPL patients were presented, as well as their diagnosis and treatment, in combination with literature review. RESULTS: Histological diagnosis was made in four patients by surgery and in two patients by EUS-FNA. The six PPL patients (5 males and 1 female; age range, 16-65 years; mean age, 46 years) had the duration of symptoms for two weeks to three months. The primary presenting symptoms, though not characteristic, were abdominal pain, abdominal masses, weight loss, jaundice, nausea and vomiting. One of the patients developed acute pancreatitis. In one patient, the level of serum CA19-9 was 76.3 microg/L. Abdominal CT scan showed that three of the six tumors were located in the head of pancreas, two in the body and tail, and one throughout the pancreas. Diameter of the tumors in the pancreas in four cases was more than 6 cm, with homogeneous density and unclear borders. Enhanced CT scan showed that only the tumor edges were slightly enhanced. The pancreatic duct was irregularly narrowed in two cases whose tumors were located in the pancreatic head and body, in which endoscopic retrograde cholangiopancreatography (ERCP) showed that the proximal segment was slightly dilated. Two patients underwent Whipple operation, one patient underwent pancreatectomy, and another patient underwent operative biliary decompression. PPL was in stage I E in 2 patients and in stage II E in 4 patients according to the Ann Arbor classification system. The diagnosis of B-cell non-Hodgkin's lymphoma was made in all patients histopathologically. All six patients underwent systemic chemotherapy, one of whom was also treated with gamma radiometry. One patient died two weeks after diagnosis, two patients lost follow-up, two patients who received chemotherapy survived 49 and 37 mo, and the remaining patient is still alive 21 mo, after diagnosis and treatment. CONCLUSION: PPL is a rare form of extranodal lymphoma originating from the pancreatic parenchyma. Clinical and imaging findings are otherwise not specific in the differentiation of pancreatic lymphoma and pancreatic cancer, which deserves attention. EUS-guided fine-needle aspiration (EUS-FNA) of the pancreas requires experienced cytopathologists as well as advanced immunohistochemical assays to obtain a final diagnosis on a small amount of tissue. Surgery and adjuvant chemotherapy or radiotherapy can produce fairly good outcomes.

A case of primary pancreatic non-Hodgkin's lymphoma. Korean J Intern Med. 2006 Jun;21(2):123-6.

Primary pancreatic lymphoma is rare, comprising 0.2-4.9% of all pancreatic malignancies and less than 1% of cases of non-Hodgkin's lymphoma. Many patients are diagnosed with lymphoma after radical resection. We report a rare presentation of diffuse large B cell lymphoma, appearing as a primary tumor of the pancreas. A 61-year old female was admitted to the hospital with the complaint of right upper abdominal pain. Computed tomography of the abdomen showed a well defined mass located at the head of the pancreas. A frozen section of pancreas, during laparotomy, revealed lymphoma. The patient received 6 cycles of chemotherapy and is currently in complete remission. This case underscores the importance of differentiating primary lymphoma from the more common adenocarcinoma of the pancreas as treatment and prognosis differ significantly. Primary pancreatic lymphoma should be considered in the differential diagnosis of pancreatic tumors and an attempt to obtain a tissue diagnosis is always necessary before proceeding to radical surgery, especially on young patients.

Pancreatic primary lymphoma: a case report and review of the literature. Hepatobiliary Pancreat Dis Int. 2005 Nov;4(4):622-6.

BACKGROUND: Primary pancreatic lymphoma is a rare but treatable malignancy (less than 1% of pancreatic tumors) that may be clinically confused with pancreatic adenocarcinoma. METHODS: In a patient with upper abdominal pain, ultrasonography and CT detected a mass in pancreatic head, which compressed the common bile duct. The patient received a Whipple's operation and intraoperative frozen sections of the mass showed an anaplastic carcinoma. Immunohistochemical staining was used to indicate the origin and prognosis of tumor. RESULTS: Grossly the tumor involved the pancreatic head, soft in consistence and invaded part of the gastric wall. Histologically, the tumor was composed mainly of large and moderate neoplastic cells, which were diffusely positive for CD20 and Bcl-6 antigens, indicating the features of diffusely large B cell lymphoma. CONCLUSIONS: The proper diagnosis of the tumor should be made with CD20 and Bcl-6 immunohistochemical study. Radical surgery is indicated for resectable carcinoma but not for a chemosensitive lymphoma.

Anaplastic large cell lymphoma of the pancreas: a pediatric case and literature review.J Pediatr Hematol Oncol. 2004 Dec;26(12):840-2.

A 13-year-old boy presented with acute abdominal pain and later became jaundiced. Medical imaging subsequently demonstrated a 7 x 8 cm pancreatic mass. Examination of a biopsy specimen obtained by open laparotomy revealed malignant lymphoma. Histology, immunohistochemistry, and cytogenetics confirmed the diagnosis of ALK1-positive anaplastic large cell lymphoma. He was treated with multiagent chemotherapy and remains in complete remission 12 months off treatment. This is the first case of primary pancreatic ALK1-positive anaplastic large cell lymphoma described in a child and shows that aggressive surgical resection of pancreatic tumors is not always necessary to achieve a cure.

Pitfalls in diagnosis of non-Hodgkin-lymphoma of the pancreas.Ultraschall Med. 2002 Dec;23(6):407-10.

A 74 year-old woman was admitted because of upper abdominal pain radiating to the back. The patient was in good health prior to onset of symptoms. Both ultrasound and endoscopic ultrasound revealed a lesion of low echogenicity in the pancreatic head without indication of hepatic lesions, enlarged lymph nodes or vascular infiltration. An computed tomography (CT) did not show the lesion in the pancreas. Explorative laparotomy was performed. The biopsy specimen revealed a diffuse large cell Non-Hodgkin-Lymphoma. This case report illustrates, how simple ultrasound studies can detect lesions not seen in more expensive, complex, and time consuming imaging techniques like CT. The patient underwent a postoperative chemotherapy and is currently well.

Primary pancreatic lymphoma. Surg Endosc. 2002 Jul;16(7):1107-8. 

Primary pancreatic lymphoma (PPL) is a rare form of extranodal lymphoma (less than 0.5% of pancreatic tumors) originating from the pancreatic parenchyma. Histopathological examination is usually mandatory to obtain a definitive diagnosis since symptoms and radiological features are quite similar to those of other pancreatic masses. Percutaneous fine-needle aspiration (FNA) of the pancreas requires experienced cytopathologists as well as advanced immunohistochemical assays to obtain a final diagnosis on a small amount of tissue. A 46-year-old man complaining of watery diarrhea and severe weight loss (more than 20 kg) for more than 1 year was admitted to our hospital due to severe diabetic crisis. Enlarged lymph nodes (2.5 x 1 cm) were found at the right axillary stations. Abdominal ultrasound revealed the presence of a large hyperechogenic mass, mainly located at the pancreatic head. Abdominal computed tomography scan confirmed a diffuse enlargement of the head and body of the pancreas associated with lymphadenopathy along the lesser gastric curvature. Percutaneous ultrasound-guided FNA of the pancreas as well as gross biopsy of the axillary lymph nodes were unable to identify the nature of the mass. Diagnostic laparoscopy was performed: several enlarged lymph nodes along the lesser gastric curvature were revealed. Multiple biopsies of the pancreatic head were taken and lymphadenectomy along the lesser curvature and the hepatic hilus was also performed. The definitive histopathological examination of the pancreatic specimen revealed a primary low-grade non-Hodgkin B cell pancreatic lymphoma. The postoperative course was unremarkable; the patient underwent systemic chemotherapy regime for low-grade B cell Hodgkin lymphoma and he was symptom free at 9-month follow-up.

Pancreatic involvement by non-Hodgkin's lymphoma.J Hepatobiliary Pancreat Surg. 2000;7(6):610-3.

A case of pancreatic involvement by non-Hodgkin's lymphoma is presented. The patient, a 63-year-old man had a large tumor in the head of the pancreas, without obstructive jaundice. Invasion of the tumor into the duodenum and transverse colon induced progressive anemia and ileus. Therefore, pancreatoduodenectomy and right hemicolectomy were performed, although a definitive preoperative diagnosis was not obtained. This tumor was identified, by histopathology and immunohistochemistry, as diffuse mixed type lymphoma with a B-cell phenotype. Postoperatively, the patient had severe congestive heart failure, and he died without receiving chemotherapy. It is important to establish a definitive diagnosis for this disease, to remove the tumor, and to treat the patient with appropriate chemotherapy.

Primary pancreatic lymphoma: a case report, literature review, and proposal for nomenclature. Med Oncol. 2000 Aug;17(3):237-47.

Primary neoplasms of the pancreas are most often adenocarcinoma. Non-Hodgkin's lymphoma (NHL) involving the pancreas is less common but well documented; the pancreas as the primary site of NHL is rare. The majority of patients with pancreatic cancer, whether it is adenocarcinoma or lymphoma, present with a mass in the head of the gland. Pancreatic lymphoma is often described as a large homogeneous mass with extra-pancreatic extension, with or without associated lymphadenopathy. Less common presentations are masses in the body or tail, or more rarely diffuse involvement of the pancreas. We present a case of diffuse pancreatic lymphoma with extra-pancreatic dissemination to the spleen and lymph nodes, and review the literature on pancreatic lymphoma. Because the definition of pancreatic lymphoma and primary pancreatic lymphoma varies, we also propose a nomenclature system to make future studies of pancreatic lymphoma more comparable.


September 2007

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Primary lymphoma of pancreas. Rev Gastroenterol Peru. 2006;26 (4):390-4.

Pancreas lymphoma is very rare, we report a 29 years old man, treated at Daniel A. Carrion Hospital. He presented jaudince and abdominal pain . Ecography and tomography showed a mass in pcreas head . the patient underwent pancreaticoduodenectomy. The final diagnosis was Large B cell diffuse lymphoma. CD 20 +, CD3 -, CD45 + without extrapancreatic disease, ACE -. We report the case and literature review.

Primary non-Hodgkin lymphoma of the pancreas.Chirurg.1998;69(3): 301-5.

A 71-year-old patient had been suffering from pain-free obstructive jaundice for 8 weeks. Ultrasonography and computed tomography revealed an inhomogeneous mass (diameter 7 x 6 cm) in the head of the pancreas. In combination with a CA 19-9 of 329 U/l, the findings were highly suggestive of a pancreatic carcinoma. Endoscopic implantation of a pigtail drain into the dilated choledochal duct was performed. A partial duodenopancreatectomy (Whipple's procedure) became necessary because of continuous bleeding with hemodynamic disorders after endoscopic papillotomy. In the histopathological examination a low-grade malignant non-Hodgkin lymphoma of the pancreas (follicular centroblastic-centrocytic) was diagnosed. The differential diagnosis of primary pancreatic lymphoma from pancreatic carcinoma is usually impossible. Neither clinical nor laboratory nor imaging methods indicate to the correct diagnosis. In cases of relatively large pancreatic tumor masses and impression of the pancreatic duct without infiltration, a primary pancreatic lymphoma should be considered and a histological diagnosis by biopsy should be performed.

Primary pancreatic lymphoma.Surgery. 1998 ; 123(4):382-90.

BACKGROUND: Primary pancreatic lymphoma is a rare neoplasm that may be confused with pancreatic adenocarcinoma. We reviewed retrospectively our contemporary experience with this disease to define more clearly the clinical presentation of this disease and the proper role for percutaneous fine-needle aspiration biopsy and surgery. METHODS: From 1980 to 1995, 11 patients with primary pancreatic lymphoma were treated at The University of Texas M. D. Anderson Cancer Center. Patient demographics, radiographic studies, fine-needle aspiration biopsy findings, operative procedures, and other treatment data were reviewed. RESULTS: The median age of the 11 patients was 64 years (range, 37 to 74 years). Abdominal pain was the most common symptom at presentation. Five patients had an elevated lactate dehydrogenase level, and only two patients had hyperbilirubinemia. Computed tomography scan demonstrated encasement of the superior mesenteric artery or superior mesenteric-portal vein confluence in six patients. Seven patients underwent computed tomography-guided fine-needle aspiration; five had findings of lymphoma. Two patients underwent distal pancreatectomy and splenectomy, and one underwent pancreatico-duodenectomy. All patients were treated with combinationchemotherapy, and seven received radiotherapy. Only two patients have died of disease (12 and 16 months after diagnosis) at a median follow-up time of 67 months. CONCLUSIONS: In the majority of patients, pancreatic lymphoma can be distinguished from pancreaticadenocarcinoma on the basis of symptoms, laboratory and radiographic findings, and fine-needle aspiration biopsy results. Once the diagnosis is established, all patients should undergo systemic chemotherapy followed by involved-field radiotherapy if the tumor has not been resected.

Primary lymphoma of the pancreas. Aust N Z J Surg. 1994;64(6):444-6.

Lymphoma of the pancreas is uncommon. Secondary invasion from contiguous, retroperitoneal lymph node disease is the predominant mode of involvement. Lymphoma originating in and remaining localized to the pancreas is very rare. A case of primary lymphoma confined to the pancreas, in a 35 year old man, is described. Only two other similar cases could be found in the English literature.

Pancreatic lymphoma. Ann Chir Gynaecol.1993;82(3):207-9.

Primary lymphoma of the pancreas is an extremely rare condition. There are no previous reports from northern Europe. It is important to differentiate pancreatic carcin oma from lymphoma because there is significant difference between the prognosis and treatment. We describe here a case of primary pancreatic lymphoma in a 64-year-old woman who was admitted to hospital because of a sudden abdominal pain and vomiting. Ultrasound, computed tomography and endoscopic retrograde cholangiopancreatography findings are presented, and the features of pancreatic lymphoma in these imaging studies are discussed.

A case of the primary pancreatic lymphoma.Rinsho Ketsueki. 1991;32 (4):414-8.

Primary pancreatic lymphoma is extremely rare. A case of the primary pancreatic lymphoma was described. A 74-year-old man was admitted to our hospital on May 26, 1990, because of epigastralgia and 5-kg weight loss. On admission, physical examination revealed mild epigastric tenderness without hepatosplenomegaly and superficial lymphadenopathy. The serum amylase level and trypsin level were normal but serum CA19-9 was slightly elevated. The diagnosis of the pancreatic cancer (body and tail) was strongly suspected on subsequent examination by US, CT, ERP and angiography. Partial pancreatectomy and splenectomy were performed. Pathological examination of the tumor in the pancreas showed diffuse non-Hodgkin's lymphoma and B-cell origin. Peripancreatic fat was involved but peripancreatic lymph nodes and spleen were intact, which strongly suggested that the isolated lymphoma was pancreatic origin.