[Splenic lymphoma, diagnosis and treatment]. / Lymphomes spléniques : diagnostic et prise en charge.

Some common clinical situations, such as splenomegaly or lymphocytosis, or less common, such as autoimmune hemolytic anemia, cold agglutinin disease, or cryoglobulinemia can lead to the diagnosis of splenic lymphoma. Splenic lymphoma is rare, mainly of non-hodgkinian origin, encompassing very different hematological entities in their clinical and biological presentation from an aggressive form such as hepato-splenic lymphoma to indolent B-cell lymphoma not requiring treatment such as marginal zone lymphoma, the most frequent form of splenic lymphoma. These entities can be challenging to diagnose and differentiate. This review presents different clinical and biological manifestations suspicious of splenic lymphoma and proposes a diagnosis work-up. We extended the strict definition of splenic lymphoma (lymphoma exclusively involving the spleen) to lymphoma thant can be revealed by a splenomegaly and we discuss the differential diagnosis of splenomegaly.

Long-term follow-up analysis of 100 patients with splenic marginal zone lymphoma treated with splenectomy as first-line treatment.

Splenectomy is considered as one of the first-line treatments for symptomatic patients with splenic marginal zone lymphoma (SMZL). Between 1997 and 2012, 100 hepatitis C virus-negative patients with SMZL were treated by splenectomy as first-line treatment. At 6 months, all patients but three recovered from all cytopenias. The median lymphocyte count at 6 months and 1 year was 11.51 × 10(9)/L and 6.9 × 10(9)/L, respectively. Median progression-free survival (PFS) was 8.25 years. The 5-year and 10-year overall survival (OS) rates were 84% and 67%, respectively. Histological transformation occurred in 11% of patients, and was the only parameter significantly associated with a shorter time to progression (p = 0.0001). Significant prognostic factors for OS were age (p = 0.0356) and histological transformation (p = 0.0312). In this large retrospective cohort, we confirmed that splenectomy as first-line treatment in patients with SMZL corrected cytopenias and lymphocytosis within the first year and was associated with a good PFS.

Acute renal failure and hypercalcemia as onset in splenic lymphoma.

Hypercalcemia is a rare metabolic disorder in course of B cell lymphoma. The mechanism of hypercalcemia in patients with malignancy may include the increased extrarenal production of vitamin D from tumoral cells or neighboring macrophages, i-PTH or PTHrP from tumoral cells. In this case we reported a 34 years old caucasian woman with acute renal failure and hypercalcemia as onset of splenic lymphoma in absence of abnormal levels of serum vitamin D and PTHrP. Because of dramatic recovery of renal function and hypercalcemia after splenectomy, we can speculate that main mechanism of hypercalcemia is related to vitamin D production from neighboring lymphoma macrophages.

Non-gastric marginal zone B-cell lymphoma in Korea: clinical features, treatment, and prognostic factors.

Marginal zone B-cell lymphoma (MZL) is the second most common subtype of non-Hodgkin's lymphoma in Korea (17.3%). Mucosa-associated lymphoid tissue (MALT) can develop in almost any organ as a result of exposure to a persistent stimulus, such as chronic infection or certain autoimmune processes. Under conditions of prolonged lymphoid proliferation, a malignant clone may emerge, which is followed by the development of a MALT lymphoma. Whereas MALT lymphoma of the stomach is the most common and the most extensively studied, we focus on non-gastric MZL studies conducted in Korea that highlight the most recent advances with respect to MZL definition, etiology, clinical characteristics, natural history, treatment approaches, outcomes, and prognostic factors. Moreover, we discuss current organ-specific considerations and controversies, and identify areas for future research.

Pure hepatoid adenocarcinoma of the stomach with spleen and lymph-node metastases.

The authors report a rare case of hepatoid adenocarcinoma of the stomach, presenting initially with spleen and lymph node metastases.

Splenic infarct as a diagnostic pitfall in radiology.

Follow-up of colorectal carcinoma after therapy is based on symptoms, tumor markers, and imaging studies. Clinicians sometimes face diagnostic dilemmas because of unusual presentations on the imaging modalities coupled with rising serum markers. We report a case of colorectal carcinoma that presented with gastrointestinal symptoms 14 months after completion of treatment. Investigations showed rise in carcinoembryonic antigen (CEA). Suspecting disease recurrence, complete radioimaging workup was performed; the only abnormality detected was a smooth, hypodense area in the posterior third of the spleen on contrast-enhanced computed tomography abdomen. In view of the previous diagnosis of carcinoma colon, the symptoms reported by the patient, the elevated CEA, and the atypical CECT appearance, a diagnosis of splenic metastasis was made. The patient was subjected to splenectomy as a curative treatment. However, the histopathological report revealed it to be a splenic infarct. The present case reemphasizes the limitations of radiological studies in the follow-up of carcinoma colon.

Splenic marginal zone lymphoma proposals for a revision of diagnostic, staging and therapeutic criteria.

Since the initial description of splenic marginal zone lymphoma (SMZL) in 1992, an increasing number of publications have dealt with multiple aspects of SMZL diagnosis, molecular pathogenesis and treatment. This process has identified multiple inconsistencies in the diagnostic criteria and lack of clear guidelines for the staging and treatment. The authors of this review have held several meetings and exchanged series of cases with the objective of agreeing on the main diagnostic, staging and therapeutic guidelines for patients with this condition. Specific working groups were created for diagnostic criteria, immunophenotype, staging and treatment. As results of this work, guidelines are proposed for diagnosis, differential diagnosis, staging, prognostic factors, treatment and response criteria. The guidelines proposed here are intended to contribute to the standardization of the diagnosis and treatment of these patients, and should facilitate the future development of clinical trials that could define more precisely predictive markers for histological progression or lack of response, and evaluate new drugs or treatments.

Hemolymphangiomatosis of the spleen: imaging features.

Hemolymphangiomatosis is an extremely rare entity that has never been described in the diffuse form. Its main pathologic feature is proliferation of lymphatic and vascular cells inducing interstitial and hematic lakes and then splenomegaly. We report a complete imaging study, including magnetic resonance (MR) imaging, after administration of a superparamagnetic contrast agent. Ultrasonography (US), basal MR imaging, and late computed tomography (CT) contrastographic features are quite similar to those of other diffuse benign vascular neoplasms, without real hemangioma-like enhancement during the vascular phase. Its late contrastographic pattern could be considered specific if CT and iron oxide-enhanced MR imaging are applied as complementary diagnostic tools, however.

[Rupture of the spleen in angiosarcoma: a case report and review of the literature]. / Rottura di milza in angiosarcoma: considerazioni su di un caso clinico e revisione della letteratura.

Splenic angiosarcoma is a rare neoplasm originating from endothelial cells of the blood vessels. Its incidence is about 0.14-0.25 per million. We report the case of a patient admitted in a state of hypovolaemic shock with haemoperitoneum due to rupture of the spleen. Splenectomy was performed with evacuation of the haemorrhagic effusion. The blood was aspirated and in part instilled during the operation through intraoperative blood salvage due to the large haemoperitoneum. Histological examination revealed a splenic angiosarcoma. Splenic angiosarcoma should be suspected in cases of splenomegaly with unknown anaemia and no lymphoma, leukaemia or myelofibrosis, because of its neoplastic aggressiveness and its invariably fatal outcome. It is important to perform a splenectomy before splenic rupture owing to its negative impact on long-term survival.

Splenic metastasis of a fallopian tube adenocarcinoma.

Clinical, diagnostic and therapeutic characteristics of a case of a fallopian tube adenocarcinoma splenic metastasis are reported. This case illustrates the diagnostic difficulties of this situation. These difficulties are both related to the poorly specific symptomatology and the low specificity of the current imagery. Percutaneous biopsy aspiration guided by ultrasonography or CT-scan could be helpful. The treatment is surgery, eventually combined with adjuvant radiotherapy: it can allow a long survival time. Splenic metastasis should be systematically checked up in every tumoral context.

Magnetic resonance imaging of the spleen and pancreas.

MRI of the spleen and pancreas requires specialized sequences which diminish artifacts in the upper abdomen. High temporal resolution sequences (e.g., spoiled gradient echo) acquired immediately after intravenous Gd-DTPA administration are necessary for imaging both the spleen and pancreas. In evaluating the spleen, early post Gd-DTPA images are essential as many focal disease processes (e.g., lymphomatous deposits or metastases) equilibrate rapidly (< 2 min) with splenic parenchyma. Complete pancreatic examination also requires the use of T1-weighted fat suppressed spin echo. T2-weighted images provide complementary information in a number of settings, in particular in the evaluation of islet cell tumors. Pancreatic ductal carcinoma is low signal on T1-weighted images and enhances in a diminished fashion on immediate post Gd-DTPA images. These tumors are well differentiated from focal chronic pancreatitis and islet celltumors based on their appearances on combined T1, T2-weighted and immediate post Gd-DTPA enhanced images.

Splenic hamartoma: report of a case and review of the literature.

A 39-year-old Japanese man with a splenic hamartoma who underwent surgery, is reported herein. The patient had a volvulus of the transverse colon and acute Type A hepatitis. His symptoms included fever, abdominal pain and icterus and the serum chemistry showed liver dysfunction and an elevation of hepatitis A antibody levels. Barium enema revealed a volvulus of the transverse colon. Ultrasonogram showed a hypoechoic mass and angiogram showed evidence of stretching of the intrasplenic vessels in the arterial phase and a hypovascular mass lesion in the venous phase. Macroscopically, the tumor was a well-defined, whitish-yellow, hard nodule. Microscopically, the tumor was composed of fibrous scar tissue containing scattered red pulp tissues and the histologic diagnosis was a splenic hamartoma of the fibrous type. The patient's postoperative recovery was uneventful. The clinical features of splenic hamartoma are reviewed in this report and the differential diagnosis is also discussed.