Korean J Hematol.  2011 Mar;46(1):52-56. 10.5045/kjh.2011.46.1.52.

Promyelocytic sarcoma of the sternum: a case report and review of the literature

• Thomas X
• Chelghoum

• KMID: 2252021
• DOI: http://doi.org/10.5045/kjh.2011.46.1.52

Abstract

This study reports a case of promyelocytic sarcoma that developed as a solitary sternal mass without any clinical evidence of acute promyelocytic leukemia. The case presented the diagnostic difficulties common to all aleukemic granulocytic sarcomas, and diagnosis was made possible by local identification of the PML/RARalpha fusion gene by fluorescent in situ hybridization. The patient was treated by surgery followed by chemotherapy plus all-trans retinoic acid therapy and local radiotherapy.

Keyword

Promyelocytic sarcoma; PML/RARalpha fusion; Acute promyelocytic leukemia

MeSH Terms

Humans
In Situ Hybridization, Fluorescence
Leukemia, Promyelocytic, Acute
Sarcoma
Sarcoma, Myeloid
Tretinoin
Tretinoin

Figure

• 

  Fig. 1 Magnetic resonance imaging of the sternum. Sagittal-weighted images (A to H) show injury to almost the entire sternum. The sternum shows an obvious abnormality at the distal end with a centro-osseous necrotic mass measuring 24 mm in transverse section, 7 mm in thickness, and 20 mm in height. The soft tissue swelling in the pre- and retro-sternal areas is significant with intense enhancement spanning nearly 50 mm.

• 

  Fig. 2 The reciprocal and balanced t(15;21), involving the PML gene on chromosome 15q22 and the RARα gene on chromosome 17q12.1, is a characteristic molecular feature of APL. In APL cells containing t(15;17), 1 orange (PML), 1 green (RARα), and 1 fusion (PML/RARα) signal pattern is observed (A). Two gene fusion products result from this translocation and encode the functional chimeric proteins: PML/RARα and RARα/PML. The LSI PML/RARα probe is a mixture of 2 FISH DNA probes. LSI PML is labeled in spectrum orange and LSI RARα is labeled in spectrum green (B).

Reference

1. Neiman RS, Barcos M, Berard C, et al. Granulocytic sarcoma: a clinicopathologic study of 61 biopsied cases. Cancer. 1981; 48:1426–1437. PMID: 7023656.

2. Jaffe ES, Harris NL, Stein H, Vardimann JW, editors. World Health Organization Classification of Tumours of Haematopoietic and Lymphoid Tissues. 2001. Lyon, France: IARC Press.

3. Liso V, Specchia G, Pogliani EM, et al. Extramedullary involvement in patients with acute promyelocytic leukemia: a report of seven cases. Cancer. 1998; 83:1522–1528. PMID: 9781945.

4. Sanz MA, Grimwade D, Tallman MS, et al. Management of acute promyelocytic leukemia: recommendations from an expert panel on behalf of the European LeukemiaNet. Blood. 2009; 113:1875–1891. PMID: 18812465.

5. Fukushima S, Terasaki M, Tajima Y, Shigemori M. Granulocytic sarcoma: an unusual complication of acute promyelocytic leukemia causing cerebellar hemorrhage. Case report. J Neurosurg. 2006; 105:912–915. PMID: 17405265.

6. Ajarim DS, Santhosh-Kumar CR, Higgy KE, el Saghir NS, Almomen AK, Shipkey FD. Granulocytic sarcoma of the thymus in acute promyelocytic leukaemia. Clin Lab Haematol. 1990; 12:97–99. PMID: 2344722.

7. Brown DM, Kimura AE, Ossoinig KC, Weiner GJ. Acute promyelocytic infiltration of the optic nerve treated by oral trans-retinoic acid. Ophthalmology. 1992; 99:1463–1467. PMID: 1407980.

8. Specchia G, Lo Coco F, Vignetti M, et al. Extramedullary involvement at relapse in acute promyelocytic leukemia patients treated or not with all-trans retinoic acid: a report by the Gruppo Italiano Malattie Ematologiche dell'Adulto. J Clin Oncol. 2001; 19:4023–4028. PMID: 11600603.

9. Ko BS, Tang JL, Chen YC, et al. Extramedullary relapse after all-trans retinoic acid treatment in acute promyelocytic leukemia - the occurrence of retinoic acid syndrome is a risk factor. Leukemia. 1999; 13:1406–1408. PMID: 10482992.

10. Savranlar A, Ustündag Y, Ozer T, et al. A thoracic-epidural granulocytic sarcoma case that was diagnosed preceding the onset of and that recurred co-incidental to acute promyelocytic leukemia, which developed after surgical treatment. Acta Med Okayama. 2004; 58:251–254. PMID: 15666994.

11. Kubonishi I, Ohtsuki Y, Machida K, et al. Granulocytic sarcoma presenting as a mediastinal tumor. Report of a case and cytological and cytochemical studies of tumor cells in vivo and in vitro. Am J Clin Pathol. 1984; 82:730–734. PMID: 6594928.

12. Tosi A, De Paoli A, Fava S, et al. Undifferentiated granulocytic sarcoma: a case with epidural onset preceding acute promyelocytic leukemia. Haematologica. 1995; 80:44–46. PMID: 7758990.

13. Worch J, Ritter J, Frühwald MC. Presentation of acute promyelocytic leukemia as granulocytic sarcoma. Pediatr Blood Cancer. 2008; 50:657–660. PMID: 17437290.

14. Gopal S, Marcussen S, Dobin SM, Koss W, Donner LR. Primary myeloid sarcoma of the testicle with t(15;17). Cancer Genet Cytogenet. 2005; 157:148–150. PMID: 15721636.

15. Tsimberidou AM, Kantarjian HM, Estey E, et al. Outcome in patients with nonleukemic granulocytic sarcoma treated with chemotherapy with or without radiotherapy. Leukemia. 2003; 17:1100–1103. PMID: 12764375.