Sclerosing Extramedullary Hematopoietic Tumor Mimicking Intra-abdominal Sarcoma

Karaarslan, Serap; Neşe, Nalan; Öncel, Güray; Özsan, Nazan; Kaplan, Hasan; Büyükkeçeci, Filiz; Hekımgıl, Mıne

doi:10.4132/jptm.2015.04.22

Cited by 3 publications

(8 citation statements)

References 10 publications

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“…Extramedullary hematopoiesis, defined as the presence and growth of immature hematopoietic cells in other locations than the bone marrow in some benign or malignant diseases, is a consequence of a low production of hematopoietic cells in the bone marrow from various causes [ 3 , 11 ]. Extramedullary hematopoiesis is common in patients with CMPD, especially PMF [ 4 ], and more commonly affects the liver, spleen and lymph nodes, with hepatosplenomegaly, sometimes significant, and lymphadenopathy; however, it has been reported in other rare locations: heart, thymus, lung, pleura, dura mater, paraspinal region, gastrointestinal tract, peritoneum, mesentery, retroperitoneum, kidneys, adrenal glands, pelvis, uterus, breast, skin and soft tissue [ 2 , 4 , 5 , 7 , 8 , 9 , 12 , 13 , 14 , 15 , 16 ].…”

Section: Discussionmentioning

confidence: 99%

“…Unlike EMH, from which it should be distinguished, the fibrous hematopoietic tumor described under this name by Beckman and Oehrle in 1982 [ 1 ] and later called SEHT [ 8 ] forms a solid tissue mass by reactive fibroblast proliferation, it is less cellular and has the peculiarity of exhibiting the presence of atypical megakaryocytes [ 4 , 13 ].…”

Section: Discussionmentioning

confidence: 99%

“…Atypical megakaryocytes are IHC positive for factor VIII, CD31, CD61 and CD41 [ 5 ]. Granulocytes are highlighted by the positive reaction for myeloperoxidase, and erythrocyte precursors by the positivity for glycophorin A [ 4 ] and hemoglobin [ 8 ].…”

Section: Discussionmentioning

confidence: 99%

“…Sclerosing Extramedullary Hematopoietic Tumor (SEHT), an extremely rare and difficult-to-diagnose lesion, was first described in 1982 [ 1 ] and was later reported in case reports [ 1 , 2 , 3 , 4 , 5 , 6 , 7 ] or small series of cases [ 1 , 8 ], usually in elderly patients with CMPD, especially primary myelofibrosis (PMF) [ 5 ].…”

Section: Introductionmentioning

confidence: 99%

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Sclerosing Extramedullary Hematopoietic Tumor (SEHT) Mimicking a Malignant Bile Duct Tumor-Case Report and Literature Review

Dema¹,

Lazăr

Barna

et al. 2021

Medicina

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Introduction: Sclerosing Extramedullary Hematopoietic Tumor (SEHT) is a very rare lesion associated with chronic myeloproliferative disorders (CMPD). SEHT can mimic morphologically, both macroscopically and microscopically, a wide variety of tumors/lesions. Case presentation: We present the case of a female patient diagnosed with gallstones for which surgery was decided. Intraoperatively, a malignant tumor of extrahepatic bile ducts was suspected. A frozen section examination raised the suspicion of a mesenchymal tumor or an inflammatory pseudotumor. The histological evaluation of the permanent sections, supplemented with an immunohistochemical investigation (IHC), was the one that established the diagnosis of SEHT, based on the presence of areas of sclerosis, atypical CD31+ megakaryocytes, myeloid and erythroid elements. Conclusions: The authors present the difficulties of a morphological diagnosis on the frozen section and on permanent sections in the absence of relevant clinical information and make a review of the literature data dedicated to the subject.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 2 more Smart Citations

Sclerosing Extramedullary Hematopoietic Tumor (SEHT) Mimicking a Malignant Bile Duct Tumor-Case Report and Literature Review

Dema¹,

Lazăr

Barna

et al. 2021

Medicina

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“…SEMHT usually develops after the diagnosis of chronic myeloproliferative disorders whereas EMH can be observed in other diseases including sickle cell anemia, thalassemia, hemoglobinopathies, and hereditary spherocytosis. 4 SEMHT may be misdiagnosed not only as EMH but also other neoplasms including Hodgkin's disease (where the atypical cells can be mistaken for Reed Sternberg cells), carcinoma, or sarcoma.…”

Section: Discussionmentioning

confidence: 99%

Sclerosing Extramedullary Hematopoietic Tumor: A Case Report

Wang

Castro

Rao

et al. 2020

Journal of Investigative Medicine High Impact Case Reports

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Sclerosing extramedullary hematopoietic tumor (SEMHT) is a rare disease that was originally named fibrous hematopoietic tumor or myelosclerosis. The process typically occurs in patients with a history of chronic myeloproliferative disorders and may afflict the skin, lung, breast, gastrointestinal tract, breast, kidney, lymph node, and thyroid gland. In this article, we report the case of a 37-year-old female with more than 5 years history of acute B-cell lymphoblastic leukemia who presented with right upper quadrant pain and tenderness. Computed tomography scan showed multiple new ring-enhancing low-density lesions within the right and left hepatic lobes. A computed tomography–guided liver core biopsy was performed on one of the new liver lesions and showed extramedullary hematopoiesis with atypical megakaryocytes and sinusoidal capillarization with associated fibroblastic proliferation. Numerous atypical megakaryocytes were scattered in the background of haphazard collagen deposition. No significant blasts or a leukemic process were identified. Bone marrow aspiration and biopsy showed extensive reticulin fibrosis (MF-3), trilineage dysplasia, increased blasts (10% to 19%), and hypercellularity (close to 100%), which was consistent with MDS-EB-2. Cytogenetics was reported as follows: 44~46,XX,-3,add(3)(p13),-5,-6,-7,17,del(17)(p12),+1~5mar[cp9]/46,XX. Molecular analysis was negative for both JAK2 V617F and CALR exon 9 activating mutations. In summary, we contributed a new case of SEMHT diagnosis in a synchronous presentation with poor clinical evolution associated chromosome 7 deletion and intact JAK2 and CALR exon 9. Care should be taken when diagnosing intraabdominal and retroperitoneal soft tissue masses with the history of hematological disorders.

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Sclerosing extramedullary hematopoietic tumor of the colon: A case report and literature review

Zuo

Zhang

et al. 2023

Exp Ther Med

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Sclerosing extramedullary hematopoietic tumor (SEMHT) is a rare tumor that can occur in association with some chronic myeloproliferative neoplasms, particularly myelofibrosis. The morphology of SEMHT can mimic that of a wide variety of other lesions, both macroscopically and microscopically. SEMHT originating from the colon is extremely rare. The present study reports a case of SEMHT in the colon with involvement of the peri-intestinal lymph nodes. On the basis of the clinical symptoms and endoscopic results, a malignant tumor of colon was suspected. Pathological examination revealed the deposition of collagen and hematopoietic components in the fibrous mucus background. Immunohistochemical staining for CD61 confirmed the presence of atypical megakaryocytes, while immunohistochemical staining for myeloperoxidase and glycophorin A highlighted the existence of granulocyte and erythrocyte precursors, respectively. These findings combined with a clinical history of myelofibrosis led to the final diagnosis of SEMHT. The presence of atypical megakaryocytes with immature hematopoietic cell morphology and a good understanding of the clinical history of the patient are essential to prevent misdiagnosis. The present case emphasizes the necessity of reviewing previous hematological history and considering clinical findings together with the associated pathological results.

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Sclerosing Extramedullary Hematopoietic Tumor Mimicking Intra-abdominal Sarcoma

Cited by 3 publications

References 10 publications

Sclerosing Extramedullary Hematopoietic Tumor (SEHT) Mimicking a Malignant Bile Duct Tumor-Case Report and Literature Review

Sclerosing Extramedullary Hematopoietic Tumor (SEHT) Mimicking a Malignant Bile Duct Tumor-Case Report and Literature Review

Sclerosing Extramedullary Hematopoietic Tumor: A Case Report

Sclerosing extramedullary hematopoietic tumor of the colon: A case report and literature review

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