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| LETTER TO THE EDITOR |
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| Year : 2015 | Volume
: 52
| Issue : 1 | Page : 21 |
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Superior vena cava syndrome: Initial presentation of acute myeloid leukemia in a child
A Gogia1, A Sharma1, V Raina1, A Chopra2
1 Department of Medical Oncology, Dr. B. R.A. Institute Rotary Cancer Hospital, All India Institute of Medical Sciences, New Delhi, India
2 Lab Oncology, Dr. B. R.A. Institute Rotary Cancer Hospital, All India Institute of Medical Sciences, New Delhi, India
| Date of Web Publication | 3-Feb-2016 |
Correspondence Address:
A Sharma
Department of Medical Oncology, Dr. B. R.A. Institute Rotary Cancer Hospital, All India Institute of Medical Sciences, New Delhi
India
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/0019-509X.175592
How to cite this article:
Gogia A, Sharma A, Raina V, Chopra A. Superior vena cava syndrome: Initial presentation of acute myeloid leukemia in a child. Indian J Cancer 2015;52:21 |
Sir,
Granulocytic sarcoma (GS) is extra medullary manifestation of acute myeloid leukaemia (AML). It has been frequently reported in the skin, orbits, gingiva, maxilla and lymph nodes.GS in the mediastinum is rare and often mistaken as lymphoblastic lymphoma in paediatric age group. Superior venacaval obstruction (SVCO) due to mediastinal GS as initial presentation of AML is rare.
A 17 year old female presented with acute onset of breathlessness, facial edema and distended superficial veins over neck and chest, all features of SVCO. Chest radiograph revealed anterior mediastinal mass causing marked widening of the mediastinum and right side moderate pleural effusion. Hemogram showed haemoglobin 10.1 g/dL, white blood cell count 8.2 × 10 9/L and platelet count 125 × 109/L. Diagnosis of lymphoblastic lymphoma was considered in different hospital. CT scan revealed 12 × 10 cm anterior mediastinal mass with right sided pleural effusion [Figure 1]a. The peripheral blood smear [Figure 1]b, and bone marrow examination revealed 80% monoblasts which on immunophenotyping expressed positivity for HLADR, CD117, CD13, CD33, CD14 and CD64, confirming the diagnosis of acute monoblastic leukaemia[Figure 1]c,[Figure 1]d, [Figure 1]e. Bone marrow Cytogenetics was normal. She was started on standard induction using daunomycin and cytosar (3 and 7) following which bone marrow examination was in morphological remission; however, there was persisting mediastinal mass. She was re-induced with high dose cytarabine (HiDAC). On day 15 she developed neutropenic fever, hypotension, and died on same day. | Figure 1: CT scan chest showing 12 × 8 cm anterior mediastinal mass and pleural effusion (a) Peripheral blood smear shows monoblast (b), positive for CD13 and CD33 (c,d,e)
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GS develops in only 2% to 7% of patients with acute myeloid leukaemia (AML).[1] It is an extramedullary solid collection of myeloid blasts and any site of the body can be involved including, skin, soft tissue, bone, periosteum and gastrointestinal tract. The mediastinum is the unusual sites of presentation.[2] The most common AML subtype presenting with GS are FAB M2 with t(8;21) and monocytic leukaemia (M4/M5) with MLL gene abnormality.[2] Mediastinal GS presenting as SVCO, in pediatric age group is rare.[3],[4] Literature review of mediastinal GS presents as SVCO revealed, majority of patients had complex cytogenetic abnormalities, refractory to treatment and a poor outcome. We are reporting this case to emphasize mediasinal GS can be present as SVCO in young patients, and should be kept in mind as differential diagnosis other than Lymphoma. Since it is difficult to achieve complete remission in these cases, allogenic stem cell transplant should be considered when available.
| » References | |  |
| 1. | Ramasamy K, Lim Z, Pagliuca A, Devereux S, Ho AY, Mufti GJ. Acute myeloid leukaemia presenting with mediastinal myeloid sarcoma: Report of three cases and review of literature. Leuk Lymphoma 2007;48:290-4.  |
| 2. | PaydasS, Zorludemir S, Ergin M. Granulocytic sarcoma: 32 cases and review of literature. Leuk Lymphoma 2006;47:2527-41. |
| 3. | Ali R, Ozkalemkas F, Yerci O, Ozcelik T, Ozkocaman V. Superior vena cava syndrome: Initial presentation of acute myeloid leukaemia (AML-M0) with near-tetraploidy+/TdT+/CD7+/CD34+/HLA-DR+. Leuk Lymphoma 2006;47:937-40. |
| 4. | Tsai MH, Yang CP, Chung HT, Shih LY. Acute myeloid leukaemia in a young girl presenting with mediastinal granulocytic sarcoma invading pericardium and causing superior vena cava syndrome. J Pediatr Hematol Oncol 2009;31:980-2. |
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