A pediatric case of primary cutaneous anaplastic large cell lymphoma: An unusual presentation

Gunjan Verma; Mukesh Manjhi; HK Kar

doi:10.4103/2319-7250.193029

CASE REPORT

Year : 2017 | Volume : 18 | Issue : 4 | Page : 324-326

A pediatric case of primary cutaneous anaplastic large cell lymphoma: An unusual presentation

Gunjan Verma, Mukesh Manjhi, HK Kar
Department of Dermatology, Post Graduate Institute of Medical Education and Research, Dr. Ram Manohar Lohia Hospital, New Delhi, India

Date of Web Publication

29-Sep-2017

Correspondence Address:
Gunjan Verma
Post Graduate Institute of Medical Education and Research, Dr Ram Manohar Lohia Hospital, New Delhi - 110 001
India

Source of Support: None, Conflict of Interest: None

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DOI: 10.4103/2319-7250.193029

Abstract

Primary cutaneous anaplastic large cell lymphoma (PCALCL) is a rare form of ALCL, usually seen in elderly patients which is clinically characterized by single or multifocal nodules that ulcerate, autoregressive, and recurrent. Extracutaneous dissemination may occur, especially to regional lymph nodes. In this report, we present an unusual case of an 8-year-old boy with a noduloulcerativelesion present on the upper back with axillary painful lymphadenopathy, initially mimicking as scrofuloderma which was later histopathologically and immunohistochemically diagnosed as CD30+ PCALCL.

Keywords: Lymphoma, neoplasm, primary cutaneous lymphoma

How to cite this article:
Verma G, Manjhi M, Kar H K. A pediatric case of primary cutaneous anaplastic large cell lymphoma: An unusual presentation. Indian J Paediatr Dermatol 2017;18:324-6

How to cite this URL:
Verma G, Manjhi M, Kar H K. A pediatric case of primary cutaneous anaplastic large cell lymphoma: An unusual presentation. Indian J Paediatr Dermatol [serial online] 2017 [cited 2019 Dec 14];18:324-6. Available from: http://www.ijpd.in/text.asp?2017/18/4/324/193029

Introduction

Lymphomas constitute 11% of all childhood malignancies.^[1] Anaplastic large cell lymphoma (ALCL) is recognized as a subtype of large cell lymphoma and is mostly of T-cell origin.^[2],[3] Three forms of ALCL have been identified: systemic, anaplastic lymphoma kinase (ALK) positive; systemic, ALK−; and primary cutaneous ALCL (PCALCL).^[1],[2] In the pediatric age group, non-Hodgkin's lymphomas (NHL) may present in extranodal locations.^[4] The incidence of PCALCL among other types of peripheral T-cell NHL is 1.7%. Most patients present with solitary or localized nodules, papules, or plaques. However, up to 20% of patients may have multiple lesions. Ulceration may be present or not. The lesions usually occur on the trunk, face, extremities, and buttocks and are usually asymptomatic.^[5] It is an indolent neoplasm with good prognosis and 5-year survival rate between 76% and 96%.^[6]

We here report a case of CD30+ ALCL who presented with ulcerated plaques, which is very rare in childhood.

Case Report

An 8-year-old boy presented with asymptomatic raised lesion over the right upper back and right axilla for last 1 year. Over the period of 4 months, patients developed ulceration over the nodule. He had no systemic complaints. On examination, a well-defined noduloulcerative plaque present on the right side of upper back, of size 4 cm × 5 cm with necrotic black crust in center, on removal of which ulcer is seen with undermined margin and the surrounding skin is erythematous. Few lesions present in axilla consist of erythematous nodules covered with scales. Right axillary lymphnode were matted, enlarged, and tender [Figure 1]a and [Figure 1]b.

Figure 1: (a) Noduloulcerative plaques present on back extending till axilla with lymphadenopathy. (b) Noduloulcerative plaques present on back extending till axilla with lymphadenopathy

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Biopsy from the ulcerated lesion revealed orthokeratosis, hyperkeratosis, mild irregular acanthosis, lymphocytic exocytosis, and spongiosis. Superficial dermis shows mild perivascular and interstitial mononuclear infiltrate. Deep dermis and subcutaneous shows collection of atypical lymphoid cells, showing mild to moderate nuclear, moderate cytoplasm with few mitosis [Figure 2]a and [Figure 2]b. Immunohistochemistry showed that the tumor cells are CD30+, EMA focal+, LCA−, CD3−, Cd1a−, pan-CK− which is suggestive of CD30+ ALCL [Figure 3]a,[Figure 3]b,[Figure 3]c.

Figure 2: (a) H and E stain showing atypical lymphoid cell in deep dermis and subcutis. (b) Large power views showing large cell

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Figure 3: (a) Immunohistochemistry showing CD 30 positivity. (b) Immunohistochemistry showing CD 3 negativity. (c) Immunohistochemistry showing leucocyte common antige negativity

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Discussion

ALCL was first recognized in 1985. Primary cutaneous CD30+ ALCL is characterized with good prognosis. Unlike in the systemic forms, a 4-year survival rate of 92% is reported. CD30+ ALCL can be misdiagnosed as Hodgkin's lymphoma.^[7],[8] Immunohistochemical studies are important in discriminating ALCL from Hodgkin's lymphoma.^[9]

Pediatric large cell lymphomas usually present with abdominal and mediastinal involvement. Lymph nodes, soft tissues, bone marrow, and internal organs can be involved in disseminated ALCL.^[10] Cutaneous manifestations of ALCL in the skin are characterized by papules, nodules, or plaques, often with ulceration. This uncommon manifestation in children is characterized by infiltration of the dermis by large neoplastic cells which mostly show T-cell markers. Mora et al. studied 52 pediatric patients with large cell lymphoma, only nine of whom showed skin involvement.^[1] There is a case report from the recent literature of generalized papulonodular eruption which comes out to be nodal ALCL (CD30+ and ALK+) in a 7-year-old boy who was on chemotherapy.^[11] Furthermore, Mahajan et al. reported a case of ALCL, which presented as ulcerated nodule on the left breast-sparing nipple areola complex which shows atypical cereberiform cells in HPE and CD30 positivity.^[12]

We report this case as it is important to realize that this uncommon presentation of ALCL can be confused with other diseases such as cutaneous tuberculosis clinically and histologically. Alhough having a much benign course, the patients should be closely monitored because of the potential risk of dissemination or extracutaneous spread, besides recurrence of the disease or even development of other malignancies, such as mycosis fungoides, Hodgkin, or NHL.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

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