OBJECTIVETo investigate the clinical features and treatment in patients of marginal zone lymphoma (MZL)with monoclonal immunoglobulin (McIg).

METHODSThe clinical data of MZL patients with McIg, including 3 cases diagnosed and treated in Beijing Anzhen Hospital from Jan 2007 to Dec 2014 were retrospectively studied, meanwhile 36 patients searched from literatures were reviewed.

RESULTSOf a total of 39 patients, the ratio of male and female was 1.05∶1 with an average age of 65.1± 12.3 years old. 28 cases (71.8%)were with mucosa associated lymphoid tissue lymphomas (MALTL), 9 cases (23.1% )with nodal marginal zone lymphoma, and 2 cases (5.1%)with splenic marginal zone lymphoma. Nine cases (23.1% )were in the early stage, 30 cases (76.9%)in the advanced stage. The common initial symptom was non-mass lesions (65.5%), such as skin purpura, peripheral neuropathy; 13 patients (33.3% )were accompanied by autoimmune phenomenon, and most were with Sjogren's syndrome. Among MALTL patients, the common primary lesion was in non- gastrointestinal tract (17 cases, 60.7%). Most of patients with McIg were one with McIgM (82.0%); the others with McIgA, Mcκ-light chain, McIgG and double McIg. The level of plasma McIgM was (25.55±21.31)g/L, which was higher in advanced stage patients than in early stage ones [(29.85±20.60)g/Lvs (3.23±2.95)g/L,P= 0.008]. The complete remission (CR)rate was 56.0% and the overall response rate (ORR)92.0%, respectively in 30 patients treated by chemotherapy. At a median follow- up of 10 months, the 3- year progression free survival and the 3-year overall survival were 44.7% and 76.5%, respectively. The rates of ORR and CR in the patients received rituximab- included regimen were seemly better than those without rituximab one (100.0%vs 78.6%, 63.6%vs 50.0%;P>0.05), but no statistic differences were found. The CR rate in patients with McIgM was significantly higher than that with non- McIgM (P=0.026). The plasma McIgM level decreased after chemotherapy (P=0.002).

CONCLUSIONThe MZL with McIg, perhaps a kind of unique subtype, usually occurred in 60 years or older patients. It was often diagnosed in patients of advanced stage and susceptible to autoimmune phenomenon. MALTL in non- gastrointestinal tract was more prone to find McIg. In MZL patients with McIg, McIgM was more common and other McIg rare. Rituximab-included regimen produced a better therapeutic response.

Adult ; Beijing ; Disease-Free Survival ; Female ; Humans ; Lymphoma, B-Cell, Marginal Zone ; pathology ; Male ; Paraproteins ; metabolism ; Remission Induction ; Retrospective Studies ; Rituximab ; therapeutic use ; Sjogren's Syndrome ; complications ; Splenic Neoplasms ; pathology

OBJECTIVE: Distal pancreatectomy with splenectomy may be required for optimal cytoreductive surgery in patients with epithelial ovarian cancer (EOC) metastasized to splenic hilum. This study evaluates the morbidity and treatment outcomes of the uncommon procedure in the management of advanced or recurrent EOC. METHODS: This study recruited 18 patients who underwent distal pancreatectomy with splenectomy during cytoreductive surgery of EOC. Their clinicopathological characteristics and follow-up data were retrospectively analyzed. RESULTS: All tumors were confirmed as high-grade serous carcinomas. The median diameter of metastatic tumors located in splenic hilum was 3.5 cm (range, 1 to 10 cm). Optimal cytoreduction was achieved in all patients. Eight patients (44.4%) suffered from postoperative complications. The morbidity associated with distal pancreatectomy and splenectomy included pancreatic leakage (22.2%), encapsulated effusion in the left upper quadrant (11.1%), intra-abdominal infection (11.1%), pleural effusion with or without pulmonary atelectasis (11.1%), intestinal obstruction (5.6%), pneumonia (5.6%), postoperative hemorrhage (5.6%), and pancreatic pseudocyst (5.6%). There was no perioperative mortality. The majority of complications were treated successfully with conservative management. During the median follow-up duration of 25 months, nine patients experienced recurrence, and three patients died of the disease. The 2-year progression-free survival and overall survival were 40.2% and 84.8%, respectively. CONCLUSION: The inclusion of distal pancreatectomy with splenectomy as part of cytoreduction for the management of ovarian cancer was associated with high morbidity; however, the majority of complications could be managed with conservative therapy.
Adult ; Aged ; *Cytoreduction Surgical Procedures ; Disease-Free Survival ; Female ; Humans ; Middle Aged ; Neoplasms, Glandular and Epithelial/mortality/pathology/*surgery ; Ovarian Neoplasms/mortality/pathology/*surgery ; *Pancreatectomy/adverse effects ; Postoperative Complications/epidemiology/therapy ; *Splenectomy/adverse effects ; Splenic Neoplasms/pathology/*secondary/*surgery

We report a case of pathological splenic rupture as a manifestation of malignant metastatic spindle cell tumour. To the best of our knowledge, this is the first case report of an atraumatic-pathological rupture of the spleen secondary to metastatic malignant spindle cell tumour. A 63-year-old man with a previous history of right upper limb amputation for an axillary malignant spindle cell tumour was admitted with an acute abdomen. Computed tomography showed a ruptured spleen. The patient subsequently underwent splenectomy. Histopathology confirmed the presence of malignant metastatic spindle cell tumour. Pathological splenic rupture is a rare manifestation of metastatic malignant spindle cell tumour. Background oncological history and thorough examination of the musculoskeletal system may provide important clues to make a prompt diagnosis.
Humans ; Male ; Middle Aged ; Rupture, Spontaneous ; diagnostic imaging ; etiology ; Sarcoma ; complications ; diagnostic imaging ; pathology ; Spleen ; diagnostic imaging ; pathology ; Splenectomy ; Splenic Neoplasms ; complications ; diagnostic imaging ; pathology ; Splenic Rupture ; diagnostic imaging ; etiology ; Tomography, X-Ray Computed

Gastrointestinal stromal tumor (GIST) represents the most common intramural mesenchymal tumor of the gastrointestinal tract, but the synchronous occurrence of GIST in the stomach and gynecological cancer is rare. We present a unique case of a 56-year-old female patient who was diagnosed with the synchronous development of GIST and an isolated parenchymal splenic metastasis of ovarian cancer. She underwent a wide local excision of gastric lesions with splenectomy. A morphological (histological and immunohistochemical) study established a spindle-cell type of gastrointestinal tumor that expressed CD117, and a parenchymal recurrence of ovarian papillary serous adenocarcinoma. The patient has remained alive and disease-free for 30 months since the last operation. A small GIST concomitant with an isolated parenchymal splenic metastasis of ovarian cancer is rarely encountered. The coexistence of GIST with other malignancies constitutes an intriguing oncologic model. Surgeons are advised to be alert against possible primary GIST accompanying other neoplasms.
Female ; Gastrointestinal Stromal Tumors ; diagnostic imaging ; secondary ; Humans ; Middle Aged ; Ovarian Neoplasms ; complications ; Radiography ; Splenic Neoplasms ; secondary

Adult ; Antineoplastic Combined Chemotherapy Protocols ; therapeutic use ; CD56 Antigen ; metabolism ; Cyclophosphamide ; therapeutic use ; Doxorubicin ; therapeutic use ; Fever of Unknown Origin ; etiology ; Humans ; Lymphoma, Extranodal NK-T-Cell ; complications ; metabolism ; pathology ; therapy ; Male ; Prednisone ; therapeutic use ; Splenectomy ; Splenic Neoplasms ; complications ; metabolism ; pathology ; therapy ; Vincristine ; therapeutic use ; Young Adult

OBJECTIVETo study the clinicopathologic features and immunophenotype of splenic littoral cell angioma.

METHODSThe clinical features, radiologic findings and histopathology of 17 cases of splenic littoral cell angioma were retrospectively reviewed. Immunohistochemical study was carried out on paraffin-embedded tissues, using normal spleen, cases of congestive splenomegaly and cavernous hemangioma as controls.

RESULTSAll the 17 cases had similar clinical manifestations and radiologic findings. There was mild to moderate splenomegaly, with solitary or multifocal space-occupying lesions. Hepatic cysts were observed in 5 of the 17 cases. One case was also accompanied by serous cystadenoma of ovary. Gross examination revealed enlarged spleen containing single or multiple tan-colored nodules which ranged from 0.2 cm to 6.0 cm in diameter. Histologically, the lesions consisted of anastomosing vascular channels, sometimes with papillary or cavernous appearance. Two types of component cells were identified. A population of smaller cells lined the vascular channels, while another population of larger cells often floated in the vascular lumen. Both cell populations showed little cytologic atypia. Immunohistochemical study demonstrated that the smaller cells of all cases were positive for CD31 and polyclonal factor VIII-related antigen. They were negative for CD34, histiocytic markers and S-100 protein. CD8 and CD21 were expressed in 1 and 1 of the 17 cases respectively. On the other hand, the larger cells expressed histiocytic markers, including CD68 (KP1 and PG-M1), CD163 and lysozyme. There was also focal positivity for CD31. The staining for CD34, monocolonal factor VIII-related antigen and S-100 protein was negative. The immunophenotype of splenic littoral cell angioma was different from that of the controls.

CONCLUSIONSLittoral cell angioma is a benign condition, likely secondary to hemodynamic disturbance in spleen. The littoral cells become hyperplastic and anastomose, resulting in a hemangioma-like growth associated with histiocytic reaction. Attention to the characteristic histopathologic findings and immunophenotype are crucial for diagnosis.

Adult ; Aged ; Antigens, CD ; metabolism ; Antigens, Differentiation, Myelomonocytic ; metabolism ; Diagnosis, Differential ; Female ; Follow-Up Studies ; Hemangioma ; immunology ; pathology ; Hemangioma, Cavernous ; immunology ; pathology ; Humans ; Immunohistochemistry ; Male ; Middle Aged ; Platelet Endothelial Cell Adhesion Molecule-1 ; metabolism ; Receptors, Cell Surface ; metabolism ; Retrospective Studies ; Spleen ; pathology ; Splenic Neoplasms ; complications ; immunology ; pathology ; Splenomegaly ; metabolism ; pathology ; von Willebrand Factor ; metabolism

OBJECTIVETo investigate the clinical manifestations, pathologic features and laboratory findings in two Proteus syndrome patients with giant hemangiomas in the spleen and chronic DIC.

METHODSUltrasound imaging and magnetic resonance imaging (MRI) were used for analysing the characteristics of the giant hemangiomas in the spleen. The spleen specimen was examined pathologically for the feature of the hemangioma. Homostatic tests were performed by routine laboratory methods.

RESULTSTwo Proteus syndrome patients with giant hemangiomas in the spleen causing chronic DIC (Kasabach-Merritt syndrome) were first reported. They were recovered after splenectomy.

CONCLUSIONProteus syndrome when accompanied giant hemangioma could cause chronic DIC. Significantly decreased plasma fibrinogen level in this case might be helpful for the differential diagnosis from DIC caused by other diseases.

Adolescent ; Disseminated Intravascular Coagulation ; etiology ; Female ; Hemangioma, Cavernous ; complications ; diagnostic imaging ; surgery ; Humans ; Proteus Syndrome ; complications ; Splenectomy ; Splenic Neoplasms ; complications ; diagnostic imaging ; surgery ; Ultrasonography

PURPOSE: A pancreas-preserving total gastrectomy (PPTG) was introduced to decrease the postoperative complications due to pancreatic resection. However, some complications, such as leakage of pancreatic juice, are still reported. Thus, the purpose of this study was to propose a supplementary procedure based on the results of treatment for gastric cancer at our hospital. MATERIALS AND METHODS: From Jan. 1997 to Dec. 2004, the cases of 141 patients who underwent a PPTG for gastric cancer were reviewed retrospectively. The patients were divided into Group A (38 cases), patients who were treated using a conventional PPTG, and Group B (103 cases), patients who were treated using a new and improved PPTG. Their postoperative complications were compared. RESULTS: No statistically significant differences in clinicopathologic data were noted between the two groups. The comparison of complications showed for groups A and B, respectively, 4 and 0 cases of pancreatic fistula, 1 and 0 cases of intraabdominal abscess, 2 and 0 cases of intraoperative pancreatic necrosis, and 2 and 2 cases of minor leakage. The difference in the prevalence of complications between the two groups was statistically significant (P=0.0001). CONCLUSION: In order to reduce the risk of PPTG-related complications, we used vascular clamps to observe the necrosis of the pancreatic tail before dividing the splenic artery, and this method resulted in a significant decrease in postoperative complications. Thus, we conclude that our use of vascular clamps in a PPTG is a simple and useful method for preventing postoperative complications.
Abscess ; Gastrectomy* ; Humans ; Necrosis ; Pancreatic Fistula ; Pancreatic Juice ; Postoperative Complications ; Prevalence ; Retrospective Studies ; Splenic Artery ; Stomach Neoplasms

We report here on a case of primary extraskeletal mesenchymal chondrosarcoma that arose from the pancreas. A 41-year-old man was evaluated by CT to find the cause of his abdominal pain. The CT scans showed a heterogeneously enhancing necrotic mass with numerous areas of coarse calcification, and this was located in the left side of the retroperitoneal space and involved the body and tail of the pancreas. Portal venography via the celiac axis also showed invasion of the splenic vein. Following excision of the mass, it was pathologically confirmed to be primary extraskeletal mesenchymal chondrosarcoma that arose from the pancreas.
Abdominal Pain/etiology ; Adult ; Chondrosarcoma, Mesenchymal/complications/*diagnosis/surgery ; Contrast Media/administration & dosage ; Diagnosis, Differential ; Humans ; Iohexol/analogs & derivatives/diagnostic use ; Male ; Necrosis ; Pancreas/pathology/radiography ; Pancreatic Neoplasms/complications/*diagnosis/surgery ; Portal Vein/radiography ; Radiographic Image Enhancement/methods ; Rare Diseases ; Retroperitoneal Space/radiography ; Splenic Vein/radiography ; Tomography, X-Ray Computed/methods

To explore the clinical and pathological characteristics of hepatosplenic gammadelta T-cell lymphoma and its relationship with Epstein-Barr virus infection, the clinical features of a 9-year-old girl with hepatosplenic gammadelta T-cell lymphoma were investigated, the smears of bone marrow was stained with Wright' s stain, biopsies of bone marrow and liver specimen were embedded in plastic and sliced about 4 microm in thickness and routinely stained with HE staining, the immunohistochemical staining was used to mark the tumor cells, and EBER probes were used to detect Epstein-Barr virus RNA. The results showed that the girl presented with prolonged fever, anemia, thrombocytopenia, hepatosplenomegaly, chronic active Epstein-Barr virus infection, and elevated levels of serum ferritin and lactate dehydrogenase. Bone marrow aspirate revealed the infiltration of atypical lymphocytes in the bone marrow stroma. The liver biopsy specimen revealed the infiltration of lymphocytes in the sinusoids, which was positive for the T-cell associated marker CD3 and activated cytotoxicity-associated marker granzyme B. In-situ hybridization analysis with EBER probes revealed that the above-mentioned characteristics were negative in neoplastic cells. It is concluded that hepatosplenic gammadelta T-cell lymphoma is a disease with distinctive clinical, histopathologic, and phenotypic characteristics. Hepatic and/or splenic and/or bone marrow biopsy with combined phenotype is beneficial to diagnosis. Epstein-Barr virus infection is late event involving an already transformed gammadelta T-cell clone.
Child ; Epstein-Barr Virus Infections ; complications ; Female ; Humans ; Liver Neoplasms ; diagnosis ; pathology ; virology ; Lymphoma, T-Cell, Peripheral ; diagnosis ; pathology ; virology ; Receptors, Antigen, T-Cell, gamma-delta ; analysis ; Splenic Neoplasms ; diagnosis ; pathology ; virology