Clinical presentation of gastric Burkitt lymphoma presenting with paraplegia and acute pancreatitis: A case report

BACKGROUNDThe incidence of gastric Burkitt lymphoma (BL), presenting as paraplegia and acute pancreatitis, is extremely low. BL is a great masquerader that presents in varied forms and in atypical locations, and it is prone to misdiagnosis and missed diagnosis. The prognosis of BL remains poor because of the difficulty in early diagnosis and the limited advances in chemotherapy.CASE SUMMARYA 53-year-old man was referred to our hospital from the local county hospital due to abdominal pain for two weeks and weakness in the lower extremities for one day. Magnetic resonance imaging of the abdomen and lumbar spine showed a swollen pancreas and gallbladder, with peripancreatic exudation and liquid collection, indicating acute pancreatitis and acute cholecystitis. Additionally, we observed abnormally thickened lesions of the gastric wall, multiple enlarged retroperitoneal lymph nodes and a well-demarcated, posterolateral extradural mass lesion between T9 and T12, with extension through the spinal foramen and definite bony destruction, suggesting metastasis in gastric malignancy. Subsequent whole-body positron emission tomography/computed tomography examination showed multifocal malignant lesions in the stomach, pancreas, gallbladder, bone, bilateral supraclavicular fossa, anterior mediastinum, bilateral axillary and retroperitoneal lymph nodes. Gastroduodenal endoscopy revealed primary BL with massive involvement of the gastric body and duodenum. The patient refused chemotherapeutic treatment and died one week later due to upper gastrointestinal hemorrhage. Afterward, we reviewed the characteristics of 11 patients with BL involving the stomach, pancreas or spinal cord.CONCLUSIONClinicians should be aware that BL can be the potential cause of acute pancreatitis or a rapidly progressive spinal tumor with accompanying paraplegia. For gastric BL, gastroscopy biopsies and pathology are necessary for a definite diagnosis.     

Primary pancreatic lymphoma： Report of six cases

AIM: To heighten recognition of primary pancreatic lym phoma (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 76.3μg/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 lympho-ma 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 immunohis-tochemical assays to obtain a final diagnosis on a small amount of tissue. Surgery and adjuvant chemotherapy or radiotherapy can produce fairly good outcomes.     

Gastric infiltration of diffuse large B-cell lymphoma： Endoscopic diagnosis and improvement of lesions after chemotherapy

Diffuse large B-cell lymphoma （DLBCL） is the most common histologic subtype of the non-Hodgkin’s lymphoma （NHL） accounting for about 40% of all NHLs. This is a case report about the endoscopic appearance of a DLBCL with infiltration to the stomach in a 39-year-old female. She had a 6-mo history of lumbar and left upper quadrant pain with intermittent episodes of melena. A computer tomograghy （CT） scan showed mural thickening of the gastric antrum. Endoscopic examination revealed multiple gastric ulcers. Definite diagnosis could be made by endoscopic biopsies and the patient had a good response to chemotherapy. This response correlated well with a further endoscopic follow-up. A follow-up endoscopic examination could be considered to evaluate a good response to chemotherapy in DLBCL patients with secondary gastric dissemination.     

Acute pancreatitis caused by an ectopic mediastinal parathyroid adenoma

A 22-year-old man was admitted to our hospital because of epigastric pain. Blood tests showed leukocytosis (8940?cells/mm³) and increased serum amylase levels (787?IU/L); an abdominal computed tomography (CT) scan revealed an enlarged pancreas and peripancreatic fluid collection. On the basis of these findings, he was diagnosed with acute pancreatitis. Hypercalcemia (13.5?mg/dL) and increased levels of parathormone (>3200?pg/dL) were also detected using a high-sensitivity assay; we therefore considered hypercalcemia and primary hyperparathyroidism to be the possible causes of the acute pancreatitis. A ^99mTc-sestamibi scan showed accumulation of parathyroid tissue in the left mediastinum, and a tumor was noted on the left side of the aortic arch on a thoracic CT scan. Our final diagnosis was acute pancreatitis due to hypercalcemia induced by an ectopic mediastinal parathyroid adenoma. Ectopic parathyroid tumors can thus cause acute pancreatitis, and ^99mTc-sestamibi and CT scans are useful for their diagnosis and localization.     

Splenic lymphoma of Ki-1 positive B-cell type treated with CHOP]

A 75-year old man was admitted to our hospital by complaining of his left supraclavicular lymphadenopathy. Physical examination also revealed splenomegaly and cervical lymphadenopathy. An abdominal CT scan showed paraaortic lymphadenopathy and splenomegaly with multiple low density area. A pathological diagnosis of Ki-1 lymphoma was made by the biopsied supraclavicular lymph node because the node consists of large cells with clear cytoplasms and conspicuous nucleoli and large cells were positive for IgM, lambda, CD20 and CD30 (Ki-1). The primary lesion of lymphoma was suspected to be spleen because of the radiological finding of the spleen. The patient responded to systemic chemotherapy (CHOP) with complete remission. Pathological and clinical characteristics of this B-cell Ki-1 lymphoma were discussed.     

Leiomyosarcoma of the pancreas

Summary A 45-yr-old female patient was admitted with signs and symptoms of acute pancreatitis. She had no history of gallstones, alcohol, or trauma. Her CT scan was consistent with a cystic lesion of the pancreas. Because of her presenting symptoms and signs, she was initially treated as an acute pancreatitis. Followup CT scan showed absolutely no change in the cyst. Surgical consultation was requested. A diagnosis of a neoplastic cyst was made, and the patient was taken to surgery where a distal pancreatectomy and splenectomy was performed. Pathology revealed a leiomyosarcoma of the pancreas. Only 15 other cases of leiomyosarcoma of the pancreas have been reported thus far in literature.     

Primary malignant lymphoma of uterine corpus: case report and review of the literature

We describe a patient presenting with postmenopausal vaginal bleeding and a uterine mass subjected to endometrial biopsy that showed a high-grade non-Hodgkin's lymphoma, consistent with a diffuse large B-cell lymphoma. Staging computed tomography (CT) scans of the chest, abdomen, and pelvis revealed three lung nodules in addition to the uterine mass. Fine needle aspirate of one lung lesion showed lymphomatous involvement. She was treated with intensive chemotherapy alone and has remained in complete remission 21 months after diagnosis. The literature on primary lymphoma of the uterine corpus is reviewed.     

Effective combined modality therapy for a patient with primary adrenal lymphoma

Primary adrenal lymphoma is a rare lymphoma with clinical features consisting of a high incidence of bilateral adrenal involvement, diffuse large B-cell histology and secondary adrenal insufficiency. We report a successful treatment of a patient with primary adrenal lymphoma using a combined modality therapy (CMT). A 62-year-old man was hospitalized with pain of the flank, and a computed tomography (CT) scan of the abdomen revealed very large, bilateral adrenal masses. A needle biopsy of the left adrenal mass revealed diffuse large B-cell lymphoma. After irradiation of both adrenal lymphomas and CHOP therapy accompanied by intrathecal treatment and rituximab, the patient underwent a left adrenalectomy and high-dose chemotherapy with autologous peripheral blood stem cell transplantation. The patient has been disease-free for 2 years after the diagnosis of primary adrenal lymphoma. In contrast to the previous reports of poor response to conventional-dose chemotherapy alone and short-term survival of patients with primary adrenal lymphoma, our patient has demonstrated that radiation therapy combined with chemotherapy and rituximab may be an effective modality as a first-line therapeutic regimen for localized primary adrenal lymphoma.     

Mucinous cystic tumour of the pancreas presenting with acute pancreatitis

BackgroundThe optimal therapy for mucinous neoplasms of the pancreas is surgical resection because these tumours are either premalignant (cystadenoma) or malignant.Case outlineA 44-year-old previously fit woman presented with sudden onset of epigastric pain. Clinical and laboratory findings were consistent with acute pancreatitis. Abdominal ultrasound scan demonstrated a mature 6-cm cyst in the tail of pancreas and no findings suggestive of cholelithiasis. These findings were confirmed by a CT scan, which also demonstrated splenic infarction and evidence of recent haemorrhage into the cyst. The patient''s abdominal pain persisted after amylase levels returned to normal.ResultsSplenic infarction, a mature cyst in the tail of the pancreas, and peripancreatic inflammation consistent with recent pancreatitis were found at laparotomy. Enbloc distal pancreatectomy and splenectomy were performed. Histological examination of the cyst wall demonstrated a focus of mucinous cystadenoma.DiscussionThis case demonstrates that acute pancreatitis may be the first presentation of a cystic neoplasm.     

Non-Hodgkin lymphoma as a cause of obstructive jaundice with simultaneous extrahepatic portal vein obstruction： A case report

Non-Hodgkin lymphoma is a rare cause of biliary obstruction. To the best of our knowledge, non-Hodgkin lymphoma in the peripancreatic region causing obstructive jaundice with simultaneous portal vein （PV） invasion has not yet been reported. We present a 50-year-old patient with obstructive jaundice whose extrahepatic portal vein was obstructed by the invasion of a peripancreatic non-Hodgkin lymphoma. The patient denied any other symptoms such as recurrent fever, night sweat and loss of body weight. Computed tomography （CT） revealed a 10 cm mass in the retroperitoneal space behind the head of the pancreas causing obstruction of the distal bile duct and the PV. A pylorus-preserving pancreaticoduodenectomy combined with a PV resection was performed. The PV was reconstructed using an au- tologous right internal jugular vein graft. The resected specimen showed endoluminal invasion of both the bile duct and the PV. Histological examination showed the mass consisting of diffuse sheets of large malignant lymphoid cells. These cells were positive for CD20 and CD79a, partially positive for CD10, and negative for CD3, CD4, CD5, CD8 and CD30. The pathologic diagnosis was diffuse large B-cell type non-Hodgkin lymphoma and the patient was transferred to the Department of Hematology and Oncology for chemotherapy. He received four cycles of combined chemotherapy including cyclophosphamide, doxorubicin, vincristine and prednisone plus rituximab, and three cycles of intrathecal chemoprophylaxis including methotorexate, cytosine arbinoside and prednisone. The patient is alive with no evidence of the disease for 7 mo after operation and will receive additional courses of chemotherapy.     

Aggressive non-Hodgkin's lymphoma after successful eradication of Helicobacter pylori and regression of gastric lymphoma of mucosa-associated lymphoid tissue

A 57-year-old woman presented to our clinic with low-grade gastric lymphoma of mucosa-associated lymphoid tissue (stage IE) and Helicobacter pylori infection. She received a 2-week course of omeprazole and clarithromycin, resulting in eradication of H. pylori and histological disappearance of the lymphoma. However, 9 months later (May 1996), multiple mass lesions were found around the pancreas and hepato-duodenal ligament on abdominal computed tomography. Inguinal lymph node biopsy revealed aggressive nodal type B-cell non-Hodgkin's lymphoma, diffuse large cell type. She received chemotherapy with cyclophosphamide, adriamycin, vincristine, and prednisolone, but failed to achieve remission and died in December 1996. There was no evidence of recurrent gastric lymphoma. This case emphasizes the importance of performing follow-up examinations to detect other neoplasms in patients with gastric lymphoma of mucosa-associated lymphoid tissue. Received Aug. 21, 1997; accepted Mar. 27, 1998     

Intravascular T-cell lymphoma with bowel involvement: case report and literature review

Intravascular lymphoma (IVL) is a rare form of non-Hodgkin lymphoma characterized by massive proliferation of large, neoplastic cells in small- and medium-sized blood vessels. Most cases of IVL are of B-cell immunophenotype; fewer than 15 cases of T-cell IVL have been reported. A 23-year-old male presented with acute abdominal pain, fever, and tender lower abdomen. Pathology at laparotomy revealed infiltration of colonic vessels with large lymphoid cells compatible with IVL. We reviewed all cases of IVL diagnosed at the Queen Elizabeth II Health Sciences Centre in Halifax, Nova Scotia, from August 1992 to August 2002. A literature review was also performed. Five additional cases of IVL were identified at this institution during a 10-year period. Three patients presented with neurological symptoms, and two with abdominal pain. In 4 of 5 cases, patients died of lymphoma within 3 months of presentation; one patient experienced a 10-month remission. While visceral involvement with IVL is common at autopsy, IVL presenting as an acute abdomen in an immunocompetent patient has not previously been described. Among the 15 cases of T-cell IVL reported in the literature, only two occurred in people under age 30. Given the rarity of T-cell IVL, it is remarkable that three cases of T-cell IVL have been diagnosed at our institution during a 10-year period.     

Acute pancreatitis induced by traditional acupuncture therapy

Although rare, it is possible for acute pancreatitis to develop after blunt or penetrating abdominal trauma. We report here a very rare case of acute pancreatitis induced by traditional acupuncture therapy. A 42-year-old woman with a low body mass index had suffered from functional dyspepsia for one year. She visited an acupuncture clinic and underwent long needle (13 cm) and gold thread needle (3 mm) acupuncture therapy. Five hours later, she presented at our emergency room complaining of severe periumbilical pain. Levels of serum amylase (1162 U/l, normal <220 U/l), and lipase (5195 IU/l, normal <60 IU/l) were high, and an abdominal computed tomography scan showed a diffusely swollen pancreas and ill-defined infiltration of the peripancreatic fat, indications of possible acute pancreatitis. Multiple small acupunctured gold thread needles were also found on the anterior abdominal wall and back muscles. After excluding other possible causes of pancreatitis, traumatic pancreatitis was diagnosed as an adverse effect of the long acupuncture needle therapy.     

Inflammatory pseudotumor of the pancreas

Summary We describe a rare example of inflammatory pseudotumor of the pancreas in a 42-yr-old woman, which developed following chemotherapy for lymphoma of the uterine cervix. The patient had developed fatigue, weight loss abdominal pain, and anemia; abdominal CT scan showed a large mass in the pancreas. Examination of the resected specimen revealed a fleshy, well-circumscribed, 7-cm mass., Histologically, there was a hypocellular to moderately hypercellular, bland spindle-cell proliferation admixed with a prominent infiltrate of lymphocytes, histiocytes, and plasma cells. The spindle cells were vimentin positive but negative for muscle markers; electron microscopy revealed only fibroblastic cells. DNA analysis revealed a diploid population with low S-phase fraction. The patient was well at 6-mo follow-up. It is important for the pathologist to be aware of the existence of this entity in unusual locations such as the pancreas so as to avoid a mistaken diagnosis of malignancy.     

Primary diffuse large B-cell lymphoma of the fallopian tube treated with a combination of surgery and chemotherapy: Case report

Rationale:Primary female genital tract lymphomas are sporadic neoplasms, accounting for 0.2% to 1.1% of all cases of extranodal lymphoma. The most common genital localizations are the cervix, the uterine corpus and the ovary, while primary lymphomas of the fallopian tube are quite unusual. According to literature searching in PubMed, this is the first reported case of primary diffuse large B-cell lymphoma of the fallopian tube.Patient concerns:A 52-year-old woman presented with a more than 2 months history of intermittent lower abdominal pain. The gynecological examination showed that the uterus, as big as 3 months of pregnancy, had weak activity and no tenderness. The uterine rectum lacuna was like a hard nodule of about 3 × 2 cm, and an irregular solid mass was fixed and inactive in the right adnexa.Diagnoses:In accordance with Ann Arbor staging system, a stage IE primary diffuse large B-cell lymphoma of fallopian tube was diagnosed for this patient, based on the tumor pathology, the results of bone marrow biopsy and computed tomography (CT) scan.Interventions:After gynecological/urinary ultrasound, blood test, pelvic contrast enhanced CT scan and CT angiography of iliac artery, exploratory laparoscopy and following hysterectomy with bilateral salpingo-oophorectomy were performed. After the surgery, the patient was treated with combined Rituximab and chemotherapy and got complete response (CR).Outcomes:After the operation and R-CHPOP, following up for more than 1 year so far, the patient has no tumor recurrence and is still in good condition.Lessons:It is very difficult to diagnose the primary diffuse large B-cell lymphoma of fallopian tube, not only because of its rarity, but also because of its non-specific clinical manifestations. It easily be treated as late ovarian cancer by gynecologist. So the pathology diagnosis and surgeons’ decision is very important. Because lymphoma is pretty sensitive to chemotherapy and easy to get complete response, so we no need to do an operation like ovarian cancer and should put chemotherapy as a primary method for lymphomas of the female genital tract.     

骨源性淋巴瘤伴继发性甲状旁腺机能亢进症1例报告并文献复习

目的：探讨骨源性非霍奇金淋巴瘤(NHL)(弥漫型大B细胞性)伴继发性甲状旁腺机能亢进的诊断及机制。方法：对1例全身骨痛伴昏迷患者进行了骨髓图、骨髓病理及免疫组织化学、血液生化和CT检查，并复习文献就其诊治问题进行讨论。结果：CT显示多部位骨质破坏，血钙及甲状旁腺激素水平显著升高，骨髓病理及免疫组织化学检查证实为骨源性NHL(弥漫型大B细胞性)，最终诊断为骨源性NHL(弥漫型大B细胞性)伴继发性甲状旁腺机能亢进。结论：骨源性NHL的诊断有赖于病理组织学、免疫学和细胞遗传学检查以及CT及核素扫描。淋巴瘤细胞分泌甲状旁腺激素相关蛋白或相关多肽(PTHrP)，细胞因子与PTHrP的释放及溶骨损害是引起高钙血症的原因。     

A case of primary pancreatic lymphoblastic lymphoma diagnosed by endoscopic ultrasound-guided fine-needle aspiration

Pancreatic involvement is an extremely rare manifestation of lymphoblastic lymphoma (LBL), and only a few cases have been reported. We report a case of LBL arising from the pancreas that was diagnosed using endoscopic ultrasound-guided fine-needle aspiration (EUS-FNA). The patient was a 57-year-old female who had suffered from abdominal pain for 2 weeks. A physical examination revealed an upper abdominal mass, but did not detect peripheral lymphadenopathy. Imaging studies including computed tomography (CT) and ¹⁸F-fluorodeoxy glucose (FDG)-positron emission tomography-CT revealed an enlarged pancreatic body, which was positive for FDG uptake. EUS-FNA detected medium-sized proliferating atypical lymphocytes, and immunohistochemical staining demonstrated that these cells were positive for CD20, CD10, PAX5, and terminal deoxynucleotidyl transferase. A bone marrow examination was negative for lymphoma infiltration, and a diagnosis of LBL arising from the pancreas was made. The patient was successfully treated with a combination of chemotherapy and pancreatic irradiation.     

A case of age-related EBV-associated B-cell lymphoproliferative disorder metachronously showing two distinct morphologic appearances, one of a polymorphic disease resembling classical Hodgkin lymphoma, and the other of a large-cell lymphoma

We report a case of age-related EBV-associated B-cell lymphoproliferative disorder (age-related EBV+ B-cell LPD) metachronously showing two distinct morphologic appearances: one of a polymorphic disease resembling classical Hodgkin lymphoma (CHL), and the other of a large-cell lymphoma. A 71-year-old man was admitted to the St. Marianna University Hospital because of fever and generalized lymphadenopathy. Right axillary lymph node biopsy revealed mixed cellularity classical Hodgkin lymphoma (MCHL). The patient was referred to the Tokyo Medical Center, where he was treated with chemotherapy and obtained CR. One year later, the patient again developed fever and generalized lymphadenopathy. Biopsy of the right cervical mass revealed a diagnosis of diffuse large B-cell lymphoma. The patient was treated with salvage chemotherapies and obtained the second CR. Two years later, the patient developed acute myeloid leukemia (AML). Although CR was achieved with chemotherapy, AML relapsed 5 months later and proved to be refractory. Two and a half years later, the patient developed right cervical lymph node enlargement. The biopsy again revealed diagnosis of MCHL. The patient died 2 months later. On reviewing all of the biopsy specimens, including the findings of immunohistochemistry and in situ hybridization, possibility of CHL was ruled out, because neoplastic giant cells resembling Hodgkin and Reed-Sternberg (HRS) cells were positive for both Oct2 and BOB.1, which has not been reported in CHL. Both HRS-like cells at the time of diagnosis of Hodgkin lymphoma and lymphoma cells at the time of diagnosis of non-Hodgkin lymphoma were positive for CD20 and EBV-encoded small RNAs. This case was finally diagnosed as having age-related EBV+ B-cell LPD. We report the case here as it underscores the difficulty in diagnosing age-related EBV+ B-cell LPDs and also suggests an important role of EBV in the pathogenesis of lymphoid neoplasms.     

Acute liver failure as the sole manifestation of relapsing non-Hodgkin's lymphoma.

Acute liver failure due to hepatic involvement by haematological malignancies is very rare, and usually has a rapidly fatal prognosis. We describe the case of a man who was treated for diffuse large B-cell lymphoma, and achieved a complete remission after eight courses of chemotherapy. He then presented with acute liver failure. Transjugular liver biopsy revealed massive infiltration by lymphomatous cells, with extensive necrosis. A CT scan did not show any evidence of extrahepatic lymphoma. The patient recovered from the liver failure following combined immuno-chemotherapy. Recurrence of the disease should be included in the differential diagnosis of patients with haematological malignancies and acute liver failure, even without evidence of extrahepatic involvement.     

Lymphoma Metastasizing to the Pituitary: An Unusual Presentation of a Treatable Disease

Lymphoma involving the pituitary gland is particularly rare. We present two cases of patients with pituitary lymphoma, both of whom were symptomatic from pituitary dysfunction. The first patient demonstrated pituitary involvement on imaging, with mild biochemical diabetes insipidus but clear hypoadrenalism. Both adrenals were grossly enlarged on CT scanning and biopsy of one of the adrenal masses confirmed the diagnosis of diffuse large B cell lymphoma. The second patient presented with clinical diabetes insipidus but with no obvious abnormalities on pituitary imaging. CT scanning of abdomen and pelvis, however, revealed widespread lymphadenopathy. Lymph node biopsy revealed a T cell-rich B cell lymphoma. Review of the English-language literature of all published cases of pituitary lymphoma in the presence of generalised disease in immunocompetent patients revealed 13 cases. Most patients had large B cell non-Hodgkin's lymphoma. Involvement of the anterior lobe of the pituitary was more frequently seen than in patients developing pituitary metastases from solid tumour primaries. Patients with advanced lymphoma including the pituitary also appear to have a better prognosis than patients presenting with pituitary metastases. This is an important diagnosis to make as rapidly as possible to allow the early institution of effective therapy.