|Year : 2019 | Volume
| Issue : 2 | Page : 95-97
A rare case of paediatric non-Hodgkin's lymphoma
D Bhargavi, T Kannan, KL Priyadarshini, V Santhosh, KM Ramprahlad, B Sreekanth
Department of Medical Oncology, Sri Venkateswara Institute of Medical Sciences, Tirupati, Andhra Pradesh, India
|Date of Web Publication||11-Nov-2019|
Assistant Professor, Department of Medical Oncology, Sri Venkateswara Institute of Medical Sciences, Tirupati, Andhra Pradesh
Source of Support: None, Conflict of Interest: None
Anaplastic large cell lymphoma (ALCL) is a rare disease accounting for 10%–15% of all non-Hodgkin's lymphomas in children. It is distinguished from other lymphomas by their anaplastic cytology and constant membrane expression of the CD30 antigen. We report a 9-year-old boy presented with a left calf swelling. Biopsy from swelling revealed as small cell variant of anaplastic lymphoma kinase-positive ALCL. Staging with positron emission tomography–computed tomography showed lymph nodal and multiple extra-nodal involvement. The extra-nodal sites were lung, pancreas, bone, sphenoid sinus and muscles. He was started on ALCL-99 protocol.
Keywords: Anaplastic large cell lymphoma, anaplastic large cell lymphoma-99, anaplastic lymphoma kinase positive, extra-nodal, small cell variant
|How to cite this article:|
Bhargavi D, Kannan T, Priyadarshini K L, Santhosh V, Ramprahlad K M, Sreekanth B. A rare case of paediatric non-Hodgkin's lymphoma. J Clin Sci Res 2019;8:95-7
|How to cite this URL:|
Bhargavi D, Kannan T, Priyadarshini K L, Santhosh V, Ramprahlad K M, Sreekanth B. A rare case of paediatric non-Hodgkin's lymphoma. J Clin Sci Res [serial online] 2019 [cited 2020 Jun 2];8:95-7. Available from: http://www.jcsr.co.in/text.asp?2019/8/2/95/270745
| Introduction|| |
Anaplastic large cell lymphoma (ALCL) is a rare disease in paediatric population, accounting for 10%–15% of all childhood non-Hodgkin's lymphomas. It was first described by Stein et al. in 1985. Depending on the expression of anaplastic lymphoma kinase (ALK), it is divided into ALK-positive and ALK-negative subtypes. ALK-positive variant was most commonly observed during the first three decades of life and is associated with male predominance. Majority of patients found to have advanced stage at initial presentation. Both nodal (50%–70%) and extra-nodal involvement (60%) are common. Among extra-nodal involvement, the most common sites are skin, bone, soft tissue, liver and spleen., Morphologically, it is characterised by large pleomorphic blasts with horseshoe-shaped nuclei and multiple nucleoli (hallmark cells). Small cell type accounts for 5%–10% of cases and is associated with poor prognosis. As the hallmark cells are very scanty in small cell subtype, it is very difficult to diagnose this entity.
| Case Report|| |
A 9-year-old boy born of non-consanguineous marriage presented with swelling of the left calf for 2 months duration. The swellings increased in size progressively. The child was unable to walk due to pain in the left calf. High-grade, intermittent fever was present, and there was no history of weight loss and night sweats. On physical examination, a single, tender, ulcerated swelling was noted in the left calf region measuring 10 cm × 7 cm × 5 cm. A solitary hard painless swelling measuring 2 cm × 2 cm was present over the right parietal region of the scalp. Per-abdominal examination showed a subcutaneous nodule measuring 2 cm × 2 cm palpable in the right hypochondriac region. There is no involvement of peripheral lymph nodes. Testicular examination was normal. Complete haemogram, renal function tests and liver function tests were normal. Serum lactate dehydrogenase was 197 units/L. Core needle biopsy was done from the left calf swelling. Histological examination of the biopsy specimen showed small and large lymphoid cells arranged in sheets with few cells showed nucleoli [Figure 1]. Hallmark cells are seen but are scattered. Mitosis is brisk. Immunohistochemical examination revealed that these cells tested positive for CD45, CD30 and ALK-1 [Figure 2] and negative for CD3, CD20, CD34, MPO and CD99. Ki-67 is 80%. Positron emission tomography–computed tomography (PET-CT) revealed metabolically active lymph nodes in multiple stations on both sides of the diaphragm with non-contiguous involvement of non-lymphatic organs such as sphenoid sinus, anal canal, pancreas, lung and multiple soft-tissue deposits in the abdomen and para-spinal, right gluteus, right tensor fascia lata, left quadriceps, left gastrocnemius and left tibialis muscles with largest deposit noted in the left gastrocnemius muscle. Bone marrow aspiration and biopsy were negative for lymphomatous infiltration. The final diagnosis of small cell variant of ALK-positive ALCL was made. He was started on ALCL-99 protocol. He achieved partial response after the course 1.
|Figure 1: Photomicrograph showing diffuse proliferation of small and large cells with few hallmark cells (Haematoxylin and eosin, × 400)|
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|Figure 2: Immunohistochemistry showing anaplastic lymphoma kinase positivity (× 400)|
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| Discussion|| |
Most common non-Hodgkin's lymphomas in paediatric age group occur in the following order: Burkitt's lymphoma, lymphoblastic lymphoma and diffuse large B-cell lymphoma. ALCL is rare T-cell lymphoma that accounts for 10% of paediatric population. Majority of these cases present during the first three decades of life. Our patient presents in his first decade.
Extra-nodal presentations are common, the most common sites being skin, bone, lung and liver. In the present case, the patient presented with calf muscle and scalp swelling. The differential diagnosis with this clinical presentation would be Ewing's sarcoma, rhabdomyosarcoma and rarely haematolymphoid malignancies such as ALCL and myeloid sarcoma. Morphologically, the tumour is composed of small and medium cells with few scattered hallmark cells. The positivity for CD45, CD30 and ALK-1 and negativity for CD3, CD20, CD34, MPO and CD99 confirm that the final diagnosis of small cell variant of ALCL was made. In a study, they found that increased expression of CD99 (70%) in ALK-positive lymphomas. Hence, ALK must include in immunohistochemistry differentiating small cell variant of ALCL from Ewing's sarcoma.
Small cell variant of ALCL most commonly presents with disseminated disease and is associated with poor prognosis. The 2-year survival is 50%. Our patient presented with disseminated disease and advanced stage. PET/CT has facilitated the diagnosis of extra-nodal involvement in lymphoma. In the present case, the patient had involvement of sphenoid sinus, pancreas and wide spread skeletal muscle involvement on PET-CT.
In 1999, the European intergroup for childhood non-Hodgkin's lymphoma conducted the large randomised trial in ALCL patients who are <22 years of age. ALCL-99 has become the choice of chemotherapy regimen. Our patient was treated with ALCL-99 protocol.
We report a rare case of small cell variant of ALK-positive ALCL presenting as calf muscle swelling and ulceration. The correct pathological diagnosis of small cell variant is very challenging as the hallmark cells are very few in this type. Pathologists must be aware of this entity when considering the differential diagnosis of small round cell tumour, particularly in a young patient.
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[Figure 1], [Figure 2]