Acute promyelocytic leukemia presenting as isolated femoral granulocytic sarcoma
Background. Granulocytic sarcoma (GS), or myeloid sarcoma or chloroma, is a tumoral mass containing myeloblasts and immature granulocytes in an anatomic site other than the bone marrow. GS is very rare in children with acute promyelocytic leukemia (APL). This case report presents a rare case of GS...
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Hacettepe University Institute of Child Health
2025-02-01
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| Series: | The Turkish Journal of Pediatrics |
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| Online Access: | https://turkjpediatr.org/article/view/4583 |
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| author | Ayşe Şimşek Hüseyin Tokgöz Ümran Çalışkan |
| author_facet | Ayşe Şimşek Hüseyin Tokgöz Ümran Çalışkan |
| author_sort | Ayşe Şimşek |
| collection | DOAJ |
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Background. Granulocytic sarcoma (GS), or myeloid sarcoma or chloroma, is a tumoral mass containing myeloblasts and immature granulocytes in an anatomic site other than the bone marrow. GS is very rare in children with acute promyelocytic leukemia (APL). This case report presents a rare case of GS manifesting as a solitary bone mass.
Case. A 15-year-old female presented with left knee pain. Complete blood count and biochemistry were normal. No blasts or early granulocytic elements were observed in the peripheral blood smear. Magnetic resonance imaging (MRI) revealed a 4x4-cm solid lesion extending to the physis line in the distal metaphyseal section of the left femur. A Tru-cut biopsy of the mass confirmed GS with immature promyelocytic cell infiltration containing Auer rods and immature myeloid cells. The t(15;17) mutation was highly positive in the tissue suspension. Bone marrow aspiration performed afterward showed no abnormalities, and acute myeloid leukemia and acute lymphoblastic leukemia mutation panels were negative. The patient was diagnosed as having APL presenting as GS of isolated femoral origin. Treatment with standard-risk chemotherapy, including all-trans retinoic acid (ATRA) according to the BFM 2013 protocol, was initiated. After 2 months, a repeat biopsy showed no pathologic promyelocytic infiltration and a negative t(15;17) mutation. However, the patient died of severe neutropenia, sepsis, and typhoid fever.
Conclusion. This case contributes to the literature as a rare presentation of APL as isolated femoral GS. It is the first reported case of an isolated femoral mass in this context to the best of our knowledge.
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| format | Article |
| id | doaj-art-86a2eceb2282473ba932cff40cc711d2 |
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| issn | 0041-4301 2791-6421 |
| language | English |
| publishDate | 2025-02-01 |
| publisher | Hacettepe University Institute of Child Health |
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| series | The Turkish Journal of Pediatrics |
| spelling | doaj-art-86a2eceb2282473ba932cff40cc711d22025-08-20T02:01:50ZengHacettepe University Institute of Child HealthThe Turkish Journal of Pediatrics0041-43012791-64212025-02-0167110.24953/turkjpediatr.2025.4583Acute promyelocytic leukemia presenting as isolated femoral granulocytic sarcomaAyşe Şimşek0https://orcid.org/0000-0001-8777-3046Hüseyin Tokgöz1https://orcid.org/0000-0002-3064-4646Ümran Çalışkan2https://orcid.org/0000-0003-4695-7046Department of Pediatric Hematology and Oncology, University of Health Sciences, Konya City Hospital, Konya, TürkiyeDepartment of Pediatric Hematology and Oncology, Faculty of Medicine, Necmettin Erbakan University, Konya, TürkiyeDepartment of Pediatrics, Faculty of Medicine, KTO University, Konya, Türkiye Background. Granulocytic sarcoma (GS), or myeloid sarcoma or chloroma, is a tumoral mass containing myeloblasts and immature granulocytes in an anatomic site other than the bone marrow. GS is very rare in children with acute promyelocytic leukemia (APL). This case report presents a rare case of GS manifesting as a solitary bone mass. Case. A 15-year-old female presented with left knee pain. Complete blood count and biochemistry were normal. No blasts or early granulocytic elements were observed in the peripheral blood smear. Magnetic resonance imaging (MRI) revealed a 4x4-cm solid lesion extending to the physis line in the distal metaphyseal section of the left femur. A Tru-cut biopsy of the mass confirmed GS with immature promyelocytic cell infiltration containing Auer rods and immature myeloid cells. The t(15;17) mutation was highly positive in the tissue suspension. Bone marrow aspiration performed afterward showed no abnormalities, and acute myeloid leukemia and acute lymphoblastic leukemia mutation panels were negative. The patient was diagnosed as having APL presenting as GS of isolated femoral origin. Treatment with standard-risk chemotherapy, including all-trans retinoic acid (ATRA) according to the BFM 2013 protocol, was initiated. After 2 months, a repeat biopsy showed no pathologic promyelocytic infiltration and a negative t(15;17) mutation. However, the patient died of severe neutropenia, sepsis, and typhoid fever. Conclusion. This case contributes to the literature as a rare presentation of APL as isolated femoral GS. It is the first reported case of an isolated femoral mass in this context to the best of our knowledge. https://turkjpediatr.org/article/view/4583granulocytic sarcomamyeloid sarcomachloromaacute promyelocytic leukemianeutropenia |
| spellingShingle | Ayşe Şimşek Hüseyin Tokgöz Ümran Çalışkan Acute promyelocytic leukemia presenting as isolated femoral granulocytic sarcoma The Turkish Journal of Pediatrics granulocytic sarcoma myeloid sarcoma chloroma acute promyelocytic leukemia neutropenia |
| title | Acute promyelocytic leukemia presenting as isolated femoral granulocytic sarcoma |
| title_full | Acute promyelocytic leukemia presenting as isolated femoral granulocytic sarcoma |
| title_fullStr | Acute promyelocytic leukemia presenting as isolated femoral granulocytic sarcoma |
| title_full_unstemmed | Acute promyelocytic leukemia presenting as isolated femoral granulocytic sarcoma |
| title_short | Acute promyelocytic leukemia presenting as isolated femoral granulocytic sarcoma |
| title_sort | acute promyelocytic leukemia presenting as isolated femoral granulocytic sarcoma |
| topic | granulocytic sarcoma myeloid sarcoma chloroma acute promyelocytic leukemia neutropenia |
| url | https://turkjpediatr.org/article/view/4583 |
| work_keys_str_mv | AT aysesimsek acutepromyelocyticleukemiapresentingasisolatedfemoralgranulocyticsarcoma AT huseyintokgoz acutepromyelocyticleukemiapresentingasisolatedfemoralgranulocyticsarcoma AT umrancalıskan acutepromyelocyticleukemiapresentingasisolatedfemoralgranulocyticsarcoma |