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Anaplastic Large Cell Lymphoma Presenting as a Soft Tissue Mass

Case report

Abstrak

Limfoma sel besar anaplastik (ALCL) adalah tumor yang jarang berlaku. Limfoma ini terdiri daripada kira-kira 3% daripada limfoma bukan-Hodgkin dewasa. Kerapkali ALCL sistemik primer melibat pada kedua-dua nodus limfa dan tapak ekstranodus. Seorang wanita berumur 44 tahun menampilkan dengan massa padat di bahagian fosa iliak kiri. Penemuan ultrasound menunjukkan massa tisu lembut inhomogenous yang agak berbatas jelas, berukuran 4x4x2.6cm di bahagian subkutanus dalam. Pemeriksaan histopatologi menunjukkan sel limfoid yang besar dengan nukleus yang atipikal, termasuk nukleus berbentuk ginjal. Sel neoplastik ini mengekspres ALK (pewarnaan pada kedua-dua sitoplasma dan nuklues), CD30 dan EMA tetapi penanda bukan pada sel-T (CD45RO dan CD3) dan sel-B (CD20 dan CD79α). Analisis hibridisasi in situ fluoresens (FISH) menunjukkan translokasi kromosom, t(2;5)(p23;q35). Berikutnya pesakit mengalami kesesakan nafas yang mendadak dan skan tomografi berkomputer torasik (CT) menunjukkan massa mengelilingi bronkus lobus atasan kanan. Pesakit juga mempunyai nodus limfa aksilari bilateral, berukuran 1 sm diameter (biopsi tidak dilakukan). Kawasan mediastinum dan endobronkus tidak menunjukkan sebarang keabnormalan. Pesakit menerima rawatan kemoterapi CHOP sebanyak 6 pusingan (cycle) dan pesakit ini sembuh daripada penyakit ini. ALCL jarang menampil sebagai tumor tisu lembut dan adalah disyorkan supaya penyakit ini dimasukkan dalam senarai sebagai diagnosis diferensial pada sebarang massa tisu lembut.

Abstract

Anaplastic large cell lymphoma (ALCL) is a rare tumour, accounting for approximately 3% of adult non-Hodgkin lymphomas.1 Primary systemic ALCL frequently involves both lymph nodes and extranodal sites. A 44-year-old woman presented with a firm, mobile mass in the left iliac fossa region. Ultrasound findings showed a well defined inhomogenous soft tissue mass, measuring 4x4x2.6cm in the deep subcutaneous region. Histopathological examination revealed that the mass was infiltrated by large lymphoid cells with marked nuclear atypia including kidney-shaped nuclei. These neoplastic cells expressed anaplastic lymphoma kinase (ALK) (both nuclear & cytoplasmic staining), CD30 and EMA but not for T-cell (CD45RO and CD3), and B-cell (CD20 & CD79α) markers. Fluorescence in situ hybridization (FISH) analysis showed a t(2;5)(p23;q35) chromosomal translocation. Subsequently the patient developed shortness of the breath and a thoracic computed tomography (CT) scan showed a mass encasing the right upper lobe bronchus. She also had bilateral axillary lymph nodes, measuring 1 cm in diameter (biopsy was not done). The mediastinum and endobronchial region did not show any abnormalities. She received 6 cycles of CHOP chemotherapy and remained disease free 2 years after diagnosis. ALCL, rarely present as a soft tissue tumour and this disease should be included as a differential diagnosis of any soft tissue mass.