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Sclerosing Extramedullary Hematopoietic Tumor Mimicking Intra-abdominal Sarcoma.

Karaarslan S, Nese N, Oncel G, Ozsan N, Akalin T, Kaplan H, Buyukkececi F, Hekimgil M - J Pathol Transl Med (2015)

View Article: PubMed Central - PubMed

Affiliation: Department of Pathology, Sifa University Faculty of Medicine, Izmir, Turkey.

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Sclerosing extramedullary hematopoietic tumor (SEMHT) is a rare tumor that occurs in patients with chronic myeloproliferative disorders (CMPDs)... EMH is seen in many disorders such as sickle cell anemia, hemoglobinopathies, thalassemia, hereditary spherocytosis, and MF... EMH is most commonly observed in the liver and spleen and is rarely found in the peritoneum, lymph nodes, kidneys, thymus, central nervous system, retroperitoneum, myocardium, uterus, pleura, paraspinal region, or intestines... Lymphoid follicles with prominent germinal centers were observed mostly in the periphery of the mass, and some contained the large cells described above (Fig. 2A, B)... No mitosis or necrosis was found... Glycophorin staining revealed some precursor cells belonging to the erythroid series (Fig. 3B)... We concluded that all these characteristics were related to SEMHT... Detailed investigation of the patient history revealed that she had been diagnosed with MF after a bone marrow biopsy 14 years prior... A liver biopsy had been performed at that time, and EMH was reported... The previous bone marrow biopsy evaluation found hypercellular bone marrow (90%) with a prominent increase in occasional clustering of megakaryocytes... SEMHT is a rare tumor more commonly seen with chronic idiopathic MF rather than CMPDs It is usually located in the abdomen, retroperitoneum, and mesenteric region... It can develop as a result of bone marrow failure due to various causes in a wide range of disorders including MF... A detailed immunohistochemical (IHC) panel is used together with morphological indicators of the degree of differentiation, number of mitoses, and the presence/absence of necrosis in the pathologic evaluation of soft tissue tumors... In this case, there was no necrosis in the background and no mitosis in the large cells, which did not invade the surrounding tissues, vessels, or nerves... All were negative (large cells were negative for CD34, DKA, S-100, CD31, desmin, vimentin, CD117, CD10, ALK), and the diagnosis of SEMHT was made with the help of a detailed medical history and the positivity for CD61 and glycophorin.

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(A) Large cells are CD61-positive on immunohistochemical stain, indicating that they are megakaryocytes. (B) Glycophorin positivity in precursor cells (green arrows) belonging to the erythroid lineage and surrounding erythrocytes (red arrow).
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f3-jptm-49-4-335: (A) Large cells are CD61-positive on immunohistochemical stain, indicating that they are megakaryocytes. (B) Glycophorin positivity in precursor cells (green arrows) belonging to the erythroid lineage and surrounding erythrocytes (red arrow).

Mentions: The patient was referred for general surgery, and the right lower quadrant mass was removed with a preliminary diagnosis of intra-abdominal malignant tumor. Macroscopically, the soft tissue mass was solid, off-white in color, and 9.5×7×3 cm in size. Microscopically, there were thick fibrotic bands, areas with more prominent collagen bands, mixed inflammatory cell infiltration including eosinophils, and occasional foci with individual or grouped enlarged cells with large cytoplasm and pleomorphic nucleoli in the background. Lymphoid follicles with prominent germinal centers were observed mostly in the periphery of the mass, and some contained the large cells described above (Fig. 2A, B). No mitosis or necrosis was found. Increased collagen was seen in the background on Masson’s trichrome stain (Fig. 2C). Immunohistochemistry revealed suspicious large cells that were negative for CD34, DKA, S-100, CD31, desmin, vimentin, CD117, CD10, CD68, CD30, mast cell tryptase, CD1a, CD45, CD30, CD15, CD3, CD20, CD21, CD23, anaplastic lymphoma kinase (ALK), pancytokeratin, and epithelial membrane antigen. The Ki-67 proliferation index was very low at about 2%. The background lymphoid follicle structures became more evident with CD3 and CD20 staining. Additional stains were then applied, and the large cells were positive for CD61 (Fig. 3A), indicating dysplastic megakaryocytes. Some myeloperoxidase-positive cells with mononuclear or polynuclear morphology were seen, indicating granulocytic series. Glycophorin staining revealed some precursor cells belonging to the erythroid series (Fig. 3B). We concluded that all these characteristics were related to SEMHT.


Sclerosing Extramedullary Hematopoietic Tumor Mimicking Intra-abdominal Sarcoma.

Karaarslan S, Nese N, Oncel G, Ozsan N, Akalin T, Kaplan H, Buyukkececi F, Hekimgil M - J Pathol Transl Med (2015)

(A) Large cells are CD61-positive on immunohistochemical stain, indicating that they are megakaryocytes. (B) Glycophorin positivity in precursor cells (green arrows) belonging to the erythroid lineage and surrounding erythrocytes (red arrow).
© Copyright Policy
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4508572&req=5

f3-jptm-49-4-335: (A) Large cells are CD61-positive on immunohistochemical stain, indicating that they are megakaryocytes. (B) Glycophorin positivity in precursor cells (green arrows) belonging to the erythroid lineage and surrounding erythrocytes (red arrow).
Mentions: The patient was referred for general surgery, and the right lower quadrant mass was removed with a preliminary diagnosis of intra-abdominal malignant tumor. Macroscopically, the soft tissue mass was solid, off-white in color, and 9.5×7×3 cm in size. Microscopically, there were thick fibrotic bands, areas with more prominent collagen bands, mixed inflammatory cell infiltration including eosinophils, and occasional foci with individual or grouped enlarged cells with large cytoplasm and pleomorphic nucleoli in the background. Lymphoid follicles with prominent germinal centers were observed mostly in the periphery of the mass, and some contained the large cells described above (Fig. 2A, B). No mitosis or necrosis was found. Increased collagen was seen in the background on Masson’s trichrome stain (Fig. 2C). Immunohistochemistry revealed suspicious large cells that were negative for CD34, DKA, S-100, CD31, desmin, vimentin, CD117, CD10, CD68, CD30, mast cell tryptase, CD1a, CD45, CD30, CD15, CD3, CD20, CD21, CD23, anaplastic lymphoma kinase (ALK), pancytokeratin, and epithelial membrane antigen. The Ki-67 proliferation index was very low at about 2%. The background lymphoid follicle structures became more evident with CD3 and CD20 staining. Additional stains were then applied, and the large cells were positive for CD61 (Fig. 3A), indicating dysplastic megakaryocytes. Some myeloperoxidase-positive cells with mononuclear or polynuclear morphology were seen, indicating granulocytic series. Glycophorin staining revealed some precursor cells belonging to the erythroid series (Fig. 3B). We concluded that all these characteristics were related to SEMHT.

View Article: PubMed Central - PubMed

Affiliation: Department of Pathology, Sifa University Faculty of Medicine, Izmir, Turkey.

AUTOMATICALLY GENERATED EXCERPT
Please rate it.

Sclerosing extramedullary hematopoietic tumor (SEMHT) is a rare tumor that occurs in patients with chronic myeloproliferative disorders (CMPDs)... EMH is seen in many disorders such as sickle cell anemia, hemoglobinopathies, thalassemia, hereditary spherocytosis, and MF... EMH is most commonly observed in the liver and spleen and is rarely found in the peritoneum, lymph nodes, kidneys, thymus, central nervous system, retroperitoneum, myocardium, uterus, pleura, paraspinal region, or intestines... Lymphoid follicles with prominent germinal centers were observed mostly in the periphery of the mass, and some contained the large cells described above (Fig. 2A, B)... No mitosis or necrosis was found... Glycophorin staining revealed some precursor cells belonging to the erythroid series (Fig. 3B)... We concluded that all these characteristics were related to SEMHT... Detailed investigation of the patient history revealed that she had been diagnosed with MF after a bone marrow biopsy 14 years prior... A liver biopsy had been performed at that time, and EMH was reported... The previous bone marrow biopsy evaluation found hypercellular bone marrow (90%) with a prominent increase in occasional clustering of megakaryocytes... SEMHT is a rare tumor more commonly seen with chronic idiopathic MF rather than CMPDs It is usually located in the abdomen, retroperitoneum, and mesenteric region... It can develop as a result of bone marrow failure due to various causes in a wide range of disorders including MF... A detailed immunohistochemical (IHC) panel is used together with morphological indicators of the degree of differentiation, number of mitoses, and the presence/absence of necrosis in the pathologic evaluation of soft tissue tumors... In this case, there was no necrosis in the background and no mitosis in the large cells, which did not invade the surrounding tissues, vessels, or nerves... All were negative (large cells were negative for CD34, DKA, S-100, CD31, desmin, vimentin, CD117, CD10, ALK), and the diagnosis of SEMHT was made with the help of a detailed medical history and the positivity for CD61 and glycophorin.

No MeSH data available.