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LETTER TO EDITOR
Year : 2012  |  Volume : 60  |  Issue : 5  |  Page : 522-524

Unusual central nervous system presentation of ALK-positive anaplastic large cell lymphoma in a child


1 Department of Pathology, All India Institute of Medical Sciences, New Delhi, India
2 Department of Neurosurgery, All India Institute of Medical Sciences, New Delhi, India
3 Department of Medical Oncology, All India Institute of Medical Sciences, New Delhi, India
4 Department of Neuroradiology, All India Institute of Medical Sciences, New Delhi, India

Date of Web Publication3-Nov-2012

Correspondence Address:
Vaishali Suri
Department of Pathology, All India Institute of Medical Sciences, New Delhi
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0028-3886.103206

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How to cite this article:
Ahuja A, Suri V, Suri A, Sharma MC, Prakash G, Bakhshi S, Garg A, Sarkar C. Unusual central nervous system presentation of ALK-positive anaplastic large cell lymphoma in a child. Neurol India 2012;60:522-4

How to cite this URL:
Ahuja A, Suri V, Suri A, Sharma MC, Prakash G, Bakhshi S, Garg A, Sarkar C. Unusual central nervous system presentation of ALK-positive anaplastic large cell lymphoma in a child. Neurol India [serial online] 2012 [cited 2019 Aug 18];60:522-4. Available from: http://www.neurologyindia.com/text.asp?2012/60/5/522/103206


Sir,

Primary or secondary involvement of central nervous system (CNS) by anaplastic large cell lymphoma (ALCL) in childhood is extremely rare. [1],[2] Secondary spread of primary nodal or systemic ALCL to the CNS is also unusual. Activin receptor-like kinase-1 (ALK-1) protein expression can be demonstrated in 80% to 90% of all systemic ALCLs in children.

An 11-year-old boy landed in emergency in a semiconscious state with a history of headache, generalised tonic-clonic seizures and vomiting of 6 months duration. On examination there was no lymphadenopathy or organomegaly. Contrast-enhanced computed tomography (CECT) scan revealed multiple dural based homogenously enhancing lesions along the left side of cavernous sinus, left tentorium, right tentorial edge, and left posterior temporal convexity dura with significant oedema in left tempro-occipital brain [Figure 1]a-c. On magnetic resonance imaging (MRI), the lesions were isointense on T1-weighted, hypointense on T2-weighted and show homogenous enhancement [Figure 1]d-f. In addition, on MRI the left posterior temporal brain showed cystic changes [Figure 1]d and e. He underwent left temporoparietal craniotomy and subtotal decompression of a tentorial based ill defined, vascular, fleshy, pinkish grey tumour in an emergency. Diagnosis of primary CNS ALK- 1 positive anaplastic large cell lymphoma of T-cell immunophenotype (common type) was established on histopathology after doing a panel of immunostains. Tumour cells were immunopositive for LCA, CD3, CD30, EMA and ALK-1 and immunonegative for CD20, MPO, synaptophysin, chromogranin, GFAP and S100 protein. Patient was referred to paediatric oncology clinic for further management. Post-operative CECT of head, chest, abdomen and pelvis revealed enhancing lesion at the site of surgery associated with inflammation of residual mass along with enlarged retroperitoneal lymph nodes. PET-CT whole body showed avid FDG uptake in the bilateral cervical, supraclavicular, left upper mediastinal, left internal mammary, retroperitoneal and pelvic group of lymph nodes. Bone marrow examination and cerebrospinal fluid (CSF) cytological examination for staging did not show lymphoma infiltration. With the presence of generalised lymphadenopathy, the diagnosis was subsequently revised as secondary ALK-positive ALCL with CNS involvement.
Figure 1: (a-c) Axial CECT shows multiple dural based homogenously enhancing lesions along the left side of cavernous sinus (a) left tentorium (b-c) right tentorial edge (thick arrow; b) and, left posterior temporal convexity dura (c) with significant oedema in left tempro-occipital brain. (d-f) On MRI, the lesions are isointense on T1-weighted (thin arrow; d), hypointense on T2-weighted (thin arrow; e) and show homogenous enhancement. Note that left posterior temporal brain is showing cystic changes (thick arrow; d, e)

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Patient was treated with chemotherapy Berlin-Frankfurt-Mόnster Group Trial (BFM) 90 NHL protocol. It includes high dose methotrexate along with combination chemotherapy like ifosfamide, cytosine arabinoside, vincristine, dexamethasone, adriamycin, etoposide and intrathecal administration of methotrexate, hydrocortisone and cytosine arabinoside. Patient was given six cycles of chemotherapy followed by a CNS irradiation and remission was achieved. However, patient relapsed after 6 months. Thereafter, he was given second line chemotherapy (ifosfamide, carboplatin and etoposide) followed by autologus peripheral stem cell transplant with conditioning regimen (cyclophosphamide, carmustine and etoposide). The patient is in complete remission at 7 months of regular follow up.

ALCL accounts for approximately 3% of adult and 10- 15% of childhood non-Hodgkin lymphoma. It frequently involves both nodal and extranodal sites. Extranodal sites which are most commonly involved are skin, bone, soft tissue and liver. [3] Primary CNS lymphomas are rare, mostly represented by diffuse large B cell lymphoma. Till date in literature only 15 cases of primary ALCL of CNS have been reported. [1],[2],[4],[5] CNS is a rare site to be involved by systemic ALCL. Somewhat similar case has been reported by Kaplinsky et al.[4] in a 2-year-old boy, however, there was generalized lymphadenopathy, skin involvement, hepatosplenomegaly and pulmonary infiltrates along with CNS involvement. A case reported by Karikari et al. [5] as a primary ALK-1 positive ALCL developed cervical lymphadenopathy 3 weeks after the diagnosis of primary CNS lymphoma. In their report, they have discussed the possibility of a primary nodal lymphoma with metastasis to the brain. This exceptionally unusual case widens the differential diagnosis of intracranial space occupying lesions in childhood and emphasizes that thorough workup and clinical examination are the most important for early diagnosis and treatment.[Figure 2]
Figure 2: (a) Photomicrograph shows singly lying tumour cells with moderate to marked pleomorphism, prominent nucleoli and scant to moderate amount of cytoplasm with focal giant cell formation (H&E, x400); (b-d) Tumour cells show immunopositive for CD20(b; × 400), CD30 (c; × 400), and ALK-1 (d; × 200)

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  References Top

1.George DH, Scheithauer BW, Aker FV, Kurtin PJ, Burger PC, Cameselle-Teijeiro J, et al. Primary anaplastic large cell lymphoma of the central nervous system: Prognostic effect of ALK-1 expression. Am J Surg Pathol 2003;27:487-493.  Back to cited text no. 1
    
2.Merlin E, Chabrier S, Verkarre V, Cramer E, Delabesse E, Stéphan JL. Primary leptomeningeal ALK+ lymphoma in a 13-year-old child. J Pediatr Hematol Oncol 2008;30:963-7.  Back to cited text no. 2
    
3.Brugières L, Deley MC, Pacquement H, Robert A Meguerian-Bedoyan Z, Terrier-Lacombe MJ, et al. CD30+ anaplastic large-cell lymphoma in children: Analysis of 82 patients enrolled in two consecutive studies of the French Society of Pediatric Oncology. Blood 1998;92:3591-8.  Back to cited text no. 3
    
4.Kaplinsky C, Toren A, Neumann Y, Mandel M, Kenet G, Sharon N, et al. Central nervous system involvement at diagnosis in a case of pediatric CD30 + anaplastic large cell lymphoma. Med Pediatr Oncol 1997;28:132-5.  Back to cited text no. 4
    
5.Karikari IO, Thomas KK, Lagoo A, Cummings TJ, George TM. Primary cerebral ALK-1-positive anaplastic large cell lymphoma in a child. Case report and literature review. Pediatr Neurosurg 2007;43:516-21.  Back to cited text no. 5
    


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