Review

Authors: Barbara Pro, Pamela Allen, Amir Behdad...

Hepatosplenic T-cell lymphoma (HSTCL) is a rare T-cell neoplasm that most commonly arises from a small subset of γ/δ T-cell receptor-expressing lymphocytes. HSTCL is more common in adolescent and young adults and has a rapidly progressive clinical course and poor outcome due to its refractoriness to conventional chemotherapy regimens. Approximately 20% of the cases arise in the background of chronic immunosuppression or immune dysregulation. Patients commonly present with constitutional symptoms, hepatic and liver enlargement, and cytopenias; hematophagocytic syndrome can also occur. The most frequent chromosomal aberrations associated with HSTCL are isochromosome 7q and trisomy 8, and most cases harbor mutations in genes involved in chromatin modification or the JAK/STAT pathway. The rarity of this disease, along with lack of nodal involvement and presenting symptoms that mimic different entities including infectious etiologies, makes this lymphoma a significant diagnostic challenge. In this review, we highlight the clinical and pathologic features of HSTCL. Moreover, we summarize the results of recent molecular studies suggesting potential targets for novel therapeutics strategies.

Topics: Animals; Humans; Liver Neoplasms; Lymphoma, T-Cell; Splenic Neoplasms

PubMed: 32756940
DOI: 10.1182/blood.2019004118

Review

Authors: Ioannis Ioannidis, Andrea G Kahn

Splenic lymphangioma is a rare malformation of the splenic lymphatic channels, mostly seen in children. It is characterized by the presence of cysts, resulting from increases in the size and number of thin-walled lymphatic vessels that are abnormally interconnected and dilated. The condition may be restricted to the spleen, but in most cases it involves multiple organs (systemic lymphangiomatosis). The clinical picture is variable; small lesions are often incidentally detected through imaging studies, while larger lesions can result in compression of organs, causing pain or rupture even after minor trauma. Therefore, splenic lymphangiomas should be considered in the differential diagnosis of splenomegaly or left upper quadrant pain even among adults and should be immediately treated with splenectomy; delay in the therapeutic intervention can lead to life-threatening complications.

Topics: Adult; Child; Cysts; Diagnosis, Differential; Humans; Lymphangioma; Prognosis; Spleen; Splenectomy; Splenic Neoplasms; Tomography, X-Ray Computed

PubMed: 25611113
DOI: 10.5858/arpa.2013-0656-RS

Authors: Christian D Vyrdal, Luit Penninga

Littoral cell angioma is a benign vascular tumour of the spleen, and malign transformation is seldom. The angioma is associated with a high risk of simultaneous occurrence of other primary cancers, and it is of utmost importance to perform extensive diagnostic investigations to detect other cancers. Definitive treatment of littoral cell angioma is surgical resection of the spleen. This is a unique case report about a 73-year-old woman who had a simultaneous adenocarcinoma of the colon and a gastrointestinal stromal tumour. She underwent simultaneous splenectomy with colonic and gastric resection.

Topics: Female; Humans; Aged; Splenic Neoplasms; Hemangioma; Splenectomy

PubMed: 37264861
DOI: No ID Found

Authors: Matteo Rossanese, Heather Williams, Benito de la Puerta...

OBJECTIVE

To determine the prevalence of splenic malignancy in cats undergoing splenectomy and to investigate possible factors associated with post-operative outcome.

ANIMALS

62 client-owned cats that underwent splenectomy.

METHODS

Medical records of 4 UK-based referral hospitals were searched and data reviewed retrospectively over 17 years. Factors associated with outcomes post-splenectomy were analyzed.

RESULTS

50 out of 62 cats (81%) were diagnosed with splenic neoplasia. Mast cell tumor ([MCT], 42%), hemangiosarcoma ([HSA], 40%), lymphoma and histiocytic sarcoma (6% each) were the most common tumor types. Fifteen cats (24%) presented with spontaneous hemoabdomen and were all diagnosed with splenic neoplasia. The diagnostic accuracy of cytology to detect splenic malignant lesions was 73% (100% for MCTs and 54% for mesenchymal tumors). Median survival time for cats with nonneoplastic splenic lesions was 715 days (IQR, 18 to 1,368) and 136 days for cats with splenic neoplasia (IQR, 35 to 348); median survival time was longer for cats with splenic MCT when compared to cats with HSA (348 vs 94 days; P < .001). Presence of metastatic disease and anemia (PCV < 24%) at diagnosis were associated with a poorer survival when considering all cats. Presence of anemia, a splenic mass on imaging or spontaneous hemoabdomen were associated with a diagnosis of HSA (P < .001).

CLINICAL RELEVANCE

Benign splenic lesions were uncommon in this cohort of cats. Spontaneous hemoabdomen should prompt the clinician to suspect neoplasia in cats with splenic disease. Anemia and evidence of metastasis at diagnosis were poor prognostic factors regardless of the final diagnosis.

Topics: Humans; Cats; Animals; Dogs; Splenectomy; Retrospective Studies; Prevalence; Splenic Neoplasms; Anemia; Hemoperitoneum; Dog Diseases; Hemangiosarcoma; Cat Diseases

PubMed: 37582488
DOI: 10.2460/javma.23.05.0258

Authors: Marta Grau, Cristina López, Alba Navarro...

The genetic mechanisms associated with splenic marginal zone lymphoma (SMZL) transformation are not well defined. We studied 41 patients with SMZL that eventually underwent large B-cell lymphoma transformation. Tumor material was obtained either only at diagnosis (9 patients), at diagnosis and transformation (18 patients), and only at transformation (14 patients). Samples were categorized in 2 groups: (1) at diagnosis (SMZL, n = 27 samples), and (2) at transformation (SMZL-T, n = 32 samples). Using copy number arrays and a next-generation sequencing custom panel, we identified that the main genomic alterations in SMZL-T involved TNFAIP3, KMT2D, TP53, ARID1A, KLF2, 1q gains, and losses of 9p21.3 (CDKN2A/B) and 7q31-q32. Compared with SMZL, SMZL-T had higher genomic complexity, and higher incidence of TNFAIP3 and TP53 alterations, 9p21.3 (CDKN2A/B) losses, and 6p gains. SMZL and SMZL-T clones arose by divergent evolution from a common altered precursor cell that acquired different genetic alterations in virtually all evaluable cases (92%, 12 of 13 cases). Using whole-genome sequencing of diagnostic and transformation samples in 1 patient, we observed that the SMZL-T sample carried more genomic aberrations than the diagnostic sample, identified a translocation t(14;19)(q32;q13) present in both samples, and detected a focal B2M deletion due to chromothripsis acquired at transformation. Survival analysis showed that KLF2 mutations, complex karyotype, and International Prognostic Index score at transformation were predictive of a shorter survival from transformation (P = .001; P = .042; and P = .007; respectively). In summary, SMZL-T are characterized by higher genomic complexity than SMZL, and characteristic genomic alterations that could represent key players in the transformation event.

Topics: Humans; Splenic Neoplasms; Mutation; Translocation, Genetic; Lymphoma, Large B-Cell, Diffuse; Leukemia, Lymphocytic, Chronic, B-Cell

PubMed: 36995085
DOI: 10.1182/bloodadvances.2022009415

Authors: Giulio Illuminati, Giulia Pizzardi, Francesco Calio...

INTRODUCTION

Hemangiopericytoma of the spleen is a very rare tumor, with 14 isolated reports. It was our aim to review our experience and compare it with all the reported cases in an attempt to standardize surgical treatment, adjuvant treatment and follow-up protocol of this infrequent condition.

METHODS

A consecutive case series study, with a mean follow-up of 44 months. Five patients (mean age, 49 years) underwent simple splenectomy for hemangiopericytoma limited to the spleen followed by adriamycin-based chemotherapy in one patient.

RESULTS

All the patients are alive and free from disease.

CONCLUSIONS

For tumors confined to the spleen, simple splenectomy can be considered curative, without any need for further adjuvant treatment. On review of the medical literature, cure can still be achieved with complete resection of recurrences, when feasible, with adjuvant chemotherapy being also indicated. The slow-growing pattern of the tumor suggests a 10-year follow-up.

Topics: Adult; Antibiotics, Antineoplastic; Chemotherapy, Adjuvant; Cohort Studies; Doxorubicin; Female; Hemangiopericytoma; Humans; Male; Middle Aged; Splenectomy; Splenic Neoplasms; Treatment Outcome

PubMed: 25638732
DOI: 10.1016/j.ijsu.2015.01.018

Authors: Kamran S Hamid, Joaquin A Rodriguez, Terry C Lairmore...

Primary splenic angiosarcoma is a rare, aggressive malignant neoplasm arising from splenic vascular endothelium. A 70-year-old woman presented with shortness of breath and chest discomfort secondary to a left-sided pleural effusion. A thoracentesis revealed a reactive effusion suspicious for malignancy. Splenic enlargement with heterogeneous enhancement was identified on CT of the abdomen. Laboratory findings at initial presentation revealed mild anemia (10.5 g/dL) with normal platelets (300 × 10⁹/L). Laparoscopic splenectomy was performed, and a primary splenic angiosarcoma was discovered. After 2 rounds of chemotherapy, a CT scan showed progressive disease with metastasis to the liver and lung. The patient's antineoplastic regimen was switched to Ifosfamide and Doxorubicin. She is currently alive with evidence of disease at 9 months but without further progression. Primary splenic angiosarcoma is almost universally fatal despite treatment. The best chance for survival is early diagnosis and prompt splenectomy prior to splenic rupture.

Topics: Aged; Diagnosis, Differential; Female; Hemangiosarcoma; Humans; Laparoscopy; Splenectomy; Splenic Neoplasms; Tomography, X-Ray Computed

PubMed: 21333203
DOI: 10.4293/108680810X12924466006521

Evaluation of the validity of the double two-thirds rule for diagnosing hemangiosarcoma in dogs with nontraumatic hemoperitoneum due to a ruptured splenic mass: a systematic review.

Authors: Ashley R Schick, Janet A Grimes

OBJECTIVE

To evaluate the validity of the double two-thirds rule for a diagnosis of splenic hemangiosarcoma in dogs with nontraumatic hemoperitoneum due to a ruptured splenic mass.

SAMPLE

Systematic literature review.

PROCEDURES

3 databases (PubMed, CAB abstracts, and World of Science) were searched in November 2020. Articles were included if data on dogs with nontraumatic hemoperitoneum due to a splenic mass were included and subsequent pathologic diagnosis could be determined.

RESULTS

In total, 2,390 unique articles were identified, with 66 articles meeting the criteria for full-text review and 14 articles included for analysis. A total of 1,150 dogs were evaluated, with 73.0% (840/1,150) of dogs being diagnosed with a malignant splenic lesion and 27.0% (310/1,150) being diagnosed with a benign splenic lesion. Of the malignancies, 87.3% (733/840) were hemangiosarcoma. Levels of evidence were low, and bias was high as most included studies were retrospective case series.

CLINICAL RELEVANCE

The double two-thirds rule should be refined when evaluating dogs with nontraumatic hemoperitoneum from a ruptured splenic mass, with more dogs being diagnosed with a malignancy and hemangiosarcoma specifically than the double two-thirds rule indicates. These findings may be useful in an emergency setting to guide owners on potential diagnoses for dogs with nontraumatic hemoperitoneum due to a ruptured splenic mass. However, there remains a portion of these dogs with benign conditions and nonhemangiosarcoma malignancies that may have a good long-term prognosis compared to dogs with hemangiosarcoma. Studies with higher levels of evidence, lower risks of bias, and large case numbers are needed in the literature.

Topics: Dogs; Animals; Hemangiosarcoma; Hemoperitoneum; Retrospective Studies; Dog Diseases; Splenic Diseases; Splenic Neoplasms

PubMed: 36322487
DOI: 10.2460/javma.22.08.0389

Review

Authors: Piers Blombery, Daphne de Jong, Judith A Ferry...

The mature splenic B-cell lymphomas are an enigmatic group of lymphoid neoplasms that have long caused significant difficulty for the practicing pathologist due to overlapping diagnostic features among entities and the decreasing availability of splenic tissue for assessment. While some entities have highly characteristic and specific clinicopathological features (e.g. hairy cell leukaemia), others are substantially more difficult to recognise (e.g. splenic diffuse red pulp lymphoma). At the same time, classification systems have been evolving, resulting in multiple changes to the boundaries among these entities and even the existence of some entities in their own right. Moreover, unbiased multi-omic interrogation (whole genome/transcriptome sequencing, methylome) of the splenic B-cell lymphomas over the past decade has given us significant insights into the underling biology of these neoplasms. We present a clinicopathological perspective on the historical, current and future state of the diagnosis and classification of splenic B-cell lymphomas integrating multi-omic data and highlighting areas of focus for the field in order to continue to strive to improve patient outcomes through accurate diagnosis.

Topics: Humans; Splenic Neoplasms; Lymphoma, B-Cell; Spleen

PubMed: 39403047
DOI: 10.1111/his.15323

Authors: Brigita Ziogaite, Elena T Contreras, Jason E Horgan...

The objectives of this study were to evaluate the risk and predictive factors of splenic malignancy and hemangiosarcoma in dogs undergoing splenectomy at a surgical specialty clinic. Medical records, hematologic results, surgical reports, and histopathologic results from 182 dogs that underwent splenectomy for the treatment of splenic masses or nodules were reviewed retrospectively. The majority of dogs (57.7%) had benign splenic diagnoses with no malignancy. Hemangiosarcoma was diagnosed in 32.4% of the dogs. A final multivariable model indicated that thrombocytopenia, anemia, and a smaller diameter of the largest splenic nodule were risk factors for hemangiosarcoma (P<0.001), and hemoperitoneum (P = 0.01) was an additional risk factor when nodule diameter was not evaluated. There were 91 dogs that had hemoperitoneum, and 60.4% of those dogs had malignant splenic lesions. Of the 33 dogs that underwent a splenectomy for incidentally identified splenic lesions, 93.9% had benign splenic lesions. Breed size was not a significant predictor of splenic malignancy risk; however, all 6 of the German shepherds included in the study had a hemangiosarcoma diagnosis. Overall prevalence of splenic malignancy including HSA may be overestimated in some canine populations.

Topics: Dogs; Animals; Splenectomy; Hemangiosarcoma; Splenic Neoplasms; Dog Diseases; Male; Female; Retrospective Studies; Incidence; Risk Factors; Spleen

PubMed: 39625875
DOI: 10.1371/journal.pone.0314737