OBJECTIVE: To study the pathologic characteristics of bone marrow for CD5 positive small B cell lymphoma (SBL).
 METHODS: The pathologic profiles of 92 patients with CD5 positive SBL were retrospectively analyzed. The morphologic and immunophenotypic features were analyzed by flow cytometry and immunohistochemistry. IgH/CCND1 was examined by fluorescence in situ hybridization (FISH).
 RESULTS: A total of 92 patients with CD5 positive SBL were enrolled in this study, including 56 (60.9%) chronic lymphocytic leukemia /small lymphocytic lymphoma (CLL/SLL), 23 (25.0%) mantle cell lymphoma (MCL) and 13 other SBL (14.1%). Among the 13 other cases, 5, 4 and 4 cases were follicular lymphoma (FL), lymphoplasmacytic lymphoma (LPL) and splenic marginal zone lymphoma (SMZL), respectively. The frequency of patterns for bone marrow infiltration was as follow: diffuse pattern (19/92), mixed pattern (15/92), nodular pattern (9/92), interstitial pattern (8/92), and intrasinusodial pattern (2/92). All patients expressed CD19, CD20 and CD5. According to the immunophenotypic score system, all the CLL patients had 4-5 scores, while SMCL and other SBL patients had less than 3 scores. For the other SBL patients, 5 FL expressed CD10, while 3 FL, 1 LPL and 3 SMZL expressed CD23. There was a significant difference in the expression of CD23, sIgM, FMC7, CD11C and CD22 between the CLL and MCL groups (P<0.01). All 23 MCL patients expressed cyclin D1 and showed IgH/CCND1 gene translocation by FISH detection.
 CONCLUSION CD5 positive SBL includes a variety of types of lymphoma. Patterns of bone marrow for CD5 positive SBL are diversity. Immunophenotypic analysis by flow cytometry is essential in the diagnosis and differential diagnosis of CD5 positive SBL, especially for CLL.
Bone Marrow ; pathology ; CD5 Antigens ; metabolism ; Diagnosis, Differential ; Flow Cytometry ; Humans ; Immunohistochemistry ; In Situ Hybridization, Fluorescence ; Leukemia, Lymphocytic, Chronic, B-Cell ; diagnosis ; Lymphoma, B-Cell ; diagnosis ; Lymphoma, Follicular ; diagnosis ; Lymphoma, Mantle-Cell ; diagnosis ; Oncogene Proteins, Fusion ; metabolism ; Retrospective Studies ; Splenic Neoplasms ; diagnosis

OBJECTIVETo further elucidate the clinical and pathologic features of retiform hemangioendothelioma (RH) and its clinical spectrum.

METHODSEight cases of RH were reviewed. The clinicopathologic profiles, immunophenotypes and outcome data were investigated.

RESULTSAll 8 cases occurred in females with a mean age at presentation of 40 years (range, 13-69 years). Five tumors arose in the skin of the head and neck region and lower extremities, two in the long bones and one in the spleen. Clinically, the patients presented with a slowly growing cutaneous plaque or subcutaneous nodule, pain of the upper arm, and dull pain and discomfort in the left hypochondrium respectively. Grossly, the tumor appeared as a non-encapsulated gray-yellowish to tan-brown mass with a mean diameter of 2.6 cm (range, 0.8-5.0 cm). On histology, it was composed of delicate branches of elongated vessels lined by a layer of hobnail or matchstick endothelium, exhibiting a retiform pattern with close resemblance of the normal rete testis. Cords or solid nests of epithelioid cells were noted adjacent to the well-formed vessels. In three cases, dilated vascular spaces with formation of intravascular papillary tufts were observed, features overlapping with Dabska tumor. There was usually marked lymphocytic infiltration in the stroma which was also hyalinized in some cases. One case had regional lymph node metastasis. By immunohistochemistry, all cases consistently expressed endothelial markers, including CD31 (8/8), human coagulation factor VIII (5/8), CD34 (5/7) and D2-40 (1/2). Two of six cases with follow-up information (18-67 months) developed local recurrences, but distant metastasis was not identified.

CONCLUSIONSRH is a distinctive vascular tumor of adulthood characterized by retiform growth of vessels with striking hobnail endothelium. Although the tumor occurs predominantly in the skin, the long bones and the spleen can be occasionally affected. The presence of Dabska tumor-like areas in RH may suggest a morphologic continuum between these two entities, comprising the family of hobnail hemangioendothelioma. Familiarity with the characteristic features of this vascular tumor of intermediate malignancy will help in the differential diagnosis of vascular neoplasms with hobnail endothelium.

Adolescent ; Adult ; Aged ; Biomarkers, Tumor ; Bone Neoplasms ; pathology ; Diagnosis, Differential ; Epithelioid Cells ; pathology ; Female ; Hemangioendothelioma ; pathology ; Humans ; Immunohistochemistry ; Immunophenotyping ; Middle Aged ; Neoplasm Recurrence, Local ; Skin Neoplasms ; pathology ; Splenic Neoplasms ; pathology ; Young Adult

We report a rare case of a patient who presented with pathological splenic rupture as the initial manifestation of chronic myeloid leukemia (CML) and was treated successfully by transcatheter arterial embolization. A 36-year-old man presented to the emergency department with a 1-day history of abdominal pain. Computed tomography showed gross hemoperitoneum with marked splenomegaly, with suspected focal rupture at the lower portion of the spleen and the extravasation of contrast material indicating active bleeding. Given the patient's hemodynamic stability, he was treated with partial splenic embolization by an interventional radiologist, and transfused with red blood cells. Examination of a bone marrow aspiration and biopsy led to a diagnosis of chronic phase CML. He was discharged from the hospital on day 13 post-embolization. Transcatheter arterial embolization should be considered as the initial treatment of spontaneous splenic rupture, especially in patients with hematological malignancies.
Abdominal Pain ; Adult ; Biopsy ; Bone Marrow ; Diagnosis ; Embolization, Therapeutic ; Emergency Service, Hospital ; Erythrocytes ; Hematologic Neoplasms ; Hemodynamics ; Hemoperitoneum ; Hemorrhage ; Humans ; Leukemia, Myelogenous, Chronic, BCR-ABL Positive* ; Rupture ; Spleen ; Splenic Rupture* ; Splenomegaly

Sclerosing angiomatoid nodular transformation (SANT) is a rare, benign vascular neoplasm. Most patients have no clinical symptoms, and the tumors are usually discovered incidentally on abdominal computed tomography or ultrasonography. Some studies have reported the clinical features and imaging findings of SANT, but the diagnosis is based on histopathologic examination of a tissue specimen obtained at splenectomy. We report herein an incidentally discovered case of SANT and review the related literature.
Diagnosis ; Humans ; Spleen* ; Splenectomy ; Splenic Neoplasms ; Ultrasonography ; Vascular Neoplasms

The splenic marginal zone lymphoma (SMZL) is a relatively rare chronic B lymphoproliferative disease, which primarily manifest increase of peripheral blood lymphocyte count and/or scale, and splenomegaly, while the peripheral superficial lymph nodes are often not swollen. Therefore, the splenectomy are usually needed to confirm the diagnosis, but the majority of patients could not accept such management, resulting in early difficult diagnosis. This study was purposed to explore the more prior way for diagnosis based flow cytometry (FCM). Six patients with suspected diagnosis of SMZL were used as research objects, 10 healthy bone marrow donors and 10 cases of chronic lymphocytic leukemia (CLL), 3 cases of hairy cell leukemia (HCL), 3 cases of lymphatic plasma cell lymphoma/Waldenströ's macroglobulinemia (LPL/WM) were selected as control. The immunophenotype of bone marrow cells were analyzed and compared by FCM using a panel of antibodies including CD45, CD5, CD10, CD19, CD20, CD22, CD23, CD25, CD103, CD11c, CD123, κ,λ, Cyclin D1, and combined with bone marrow cell morphology. The results indicated that 6 cases of suspected SMZL showed a large increase of lymphocytes and splenomegaly. Because absence of peripheral lymphadenopathy, 6 patients did not suffer from lymph node biopsy, only 1 patient underwent diagnostic splenectomy. The immunophenotypes of bone marrow in patients and controls were analyzed by FCM, as a result, except for the healthy donors, varying degrees of abnormal mature B cell clones were found in bone marrow of all patients, and the further differentiation from other B-cell tumors was performed through CD5, CD10 expression and combination with other B-cell phenotype. All 6 cases of SMZL patients expressed CD19(+) and CD20(+), but CD10 expression was negative, 4 patients expressed CD5(-), 2 patients expressed CD5(+). The expressions of CD23, CD38, ZAP-70, CD11c, CD103, CD123, Cyclin D1 were negative. The morphological examination of bone marrow cells showed velutinous abnormal lymphocytes. Combined with clinical characteristics, 6 patients were diagnosed as SMZL, 1 patient suffered from splenectomy because of concurrent hypersplenism, and this postoperative pathologic examination confirmed the patient with SMZL. Ten cases of CLL mainly expressed CD5, CD23; 3 cases of HCL had more typical morphology of "hair like" in addition to CD11c, CD103 and CD123 positive; 3 cases of LPL/WM had significantly increased light chain restriction expression, IgM, plasmacytoid lymphocytes. It is concluded that the FCM immunophenotype analysis can be used as a powerful tools for clinical diagnosis of SMZL.
Adult ; Aged ; Aged, 80 and over ; B-Lymphocytes ; Female ; Flow Cytometry ; Humans ; Immunophenotyping ; Lymphoma, B-Cell, Marginal Zone ; diagnosis ; Lymphoproliferative Disorders ; diagnosis ; Male ; Middle Aged ; Splenic Neoplasms ; diagnosis

OBJECTIVETo study the clinicopathologic features and differential diagnosis of splenic B-cell marginal zone lymphoma (SMZL) involving bone marrow.

METHODSThe clinical and pathologic features of 22 patients with SMZL were retrospectively studied. Immunophenotypic analysis was carried out by flow cytometry and immunohistochemistry. Immunoglobulin heavy chain rearrangement study was performed using polymerase chain reaction-based method.

RESULTSVillous lymphocytes were found in peripheral blood smears of 11/18 of the patients. In bone marrow aspirates, lymphocytosis (> 20%) was demonstrated in 15 cases (15/18) and villous lymphocytes in 6 cases (6/18). Flow cytometry showed CD19(+) CD20(+) FMC7(+) CD22(+) CD10(-) CD2(-) CD3(-) CD7(-) in 18 cases. Bone marrow biopsies of all the 22 patients revealed various degrees and patterns of neoplastic infiltration, as follows: mild (4 cases, 18.2%), moderate (11 cases, 50.0%) or severe (7 cases, 31.8%); intrasinusoidal (16 cases, 72.7%), interstitial (14 cases, 63.6%), nodular (11 cases, 50.0%) or diffuse (1 case, 4.5%). Reactive germinal center formation (CD23(+) bcl-2(-)) was found in 2 cases (91.0%). Immunohistochemical study showed the following results: CD20(+) PAX5(+) CD3(-) CD5(-) CD10(-) cyclin D1(-) CD23(-) CD43(-) Annexin A1(-) CD11C(-) CD25(-) in all the 22 cases, CD38(+) in 2 cases (9.1%) and CD138(+) in 2 cases (9.1%).

CONCLUSIONSDifferent and overlapping patterns of bone marrow involvement are observed in SMZL. As the histologic and immunophenotypic features are not specific to SMZL, distinction from other types of mature B-cell lymphomas is necessary.

Adult ; Aged ; Aged, 80 and over ; Antigens, CD20 ; metabolism ; Bone Marrow ; pathology ; Diagnosis, Differential ; Female ; Gene Rearrangement, B-Lymphocyte, Heavy Chain ; Humans ; Leukemia, Lymphocytic, Chronic, B-Cell ; metabolism ; pathology ; Lymphoma, B-Cell, Marginal Zone ; genetics ; metabolism ; pathology ; Lymphoma, Follicular ; metabolism ; pathology ; Lymphoma, Mantle-Cell ; metabolism ; pathology ; Male ; Middle Aged ; Neoplasm Invasiveness ; Retrospective Studies ; Splenic Neoplasms ; genetics ; metabolism ; pathology ; Waldenstrom Macroglobulinemia ; metabolism ; pathology

Aged ; CDX2 Transcription Factor ; Cysts ; metabolism ; pathology ; surgery ; Diagnosis, Differential ; Female ; Homeodomain Proteins ; metabolism ; Humans ; Keratin-20 ; metabolism ; Mucous Membrane ; pathology ; Peritoneal Neoplasms ; metabolism ; pathology ; surgery ; Pseudomyxoma Peritonei ; metabolism ; pathology ; surgery ; Rupture ; Splenic Neoplasms ; metabolism ; pathology ; surgery

Sclerosing angiomatoid nodular transformation (SANT) of the spleen, a newly defined primary lesion of the spleen, is very rare. Immunohistochemistry is the only way to confirm the diagnosis of SANT. We present the clinical characteristics and postoperative outcomes of five SANT cases that underwent splenectomy from January 2007 to October 2010. Although SANT had specific imaging findings, differential diagnosis from other splenic tuomrs or malignant lesions preoperatively was difficult. The hand-assisted laparoscopic splenectomy was a useful and effective technique for the management and postoperative diagnosis of SANT. All SANT patients had good prognosis without recurrence after splenectomy.
Adult ; Angiomatosis ; diagnosis ; pathology ; surgery ; Female ; Humans ; Immunohistochemistry ; Male ; Middle Aged ; Spleen ; pathology ; surgery ; Splenic Neoplasms ; diagnosis ; pathology ; surgery

Accessory spleen can be mistaken as a gastric subepithelial mass, and may not be differentiated in CT or endoscopic ultrasonography (EUS). A gastric subepithelial mass was detected on routine endoscopy in a 39-year old woman with history of splenectomy. In subsequent CT and EUS, the subepithelial mass was located on the fourth layer of the stomach. To make a definite diagnosis, EUS-guided fine needle aspiration (FNA) was performed, and a splenic tissue was demonstrated in histologic examination. EUS-guided FNA can be beneficial in the diagnosis of accessory spleen which mimics a gastric subepithelial mass.
Adult ; Antigens, CD34/metabolism ; Biopsy, Fine-Needle ; Endosonography ; Female ; Gastroscopy ; Humans ; Immunohistochemistry ; Splenic Diseases/pathology ; Stomach Neoplasms/*diagnosis/pathology ; Tomography, X-Ray Computed

Portal annular pancreas is one of the pancreatic fusion anomalies in which the uncinate process of the pancreas extends to fuse with the dorsal pancreas by encircling the portal vein or superior mesenteric vein. We report two consecutive patients with portal annular pancreas. The first case is a 71-year-old male patient who underwent a pancreaticoduodenectomy for intraductal papillary mucinous neoplasm in the head of pancreas. His preoperative computed tomography scan showed the suprasplenic type portal annular pancreas. The second case is a 74-year-old female patient who underwent a laparoscopic anterior radical antegrade modular pancreatosplenectomy (RAMPS) for pancreatic body cancer. In operative finding, portal confluence (superior mesenteric vein-splenic vein-portal vein) was encased with the uncinate process of pancreas in both cases. Therefore, they required pancreatic division at the pancreatic neck portion twice. During the postoperative period, grade B and A, respectively, postoperative pancreatic fistulas occurred and were controlled by conservative management. Surgeons need to know about this rare pancreatic condition prior to surgical intervention to avoid complications, and to provide patients with well-designed, case-specific pancreatic surgery.
Adenocarcinoma, Mucinous/diagnosis/surgery ; Aged ; Female ; Humans ; Male ; Mesenteric Veins/radiography ; Pancreas/abnormalities ; Pancreatic Diseases/*diagnosis/therapy ; Pancreatic Fistula/etiology ; Pancreatic Neoplasms/diagnosis/surgery ; Pancreaticoduodenectomy/adverse effects ; Portal Vein/radiography ; Splenic Vein/radiography ; Tomography, X-Ray Computed