Context—The year 2010 commemorates the 25th year since the seminal publication by Karl Lennert and Harald Stein and others in Kiel, West Germany, describing an unusual large cell lymphoma now known as anaplastic large cell lymphoma (ALCL). Investigators at many universities and hospitals worldwide have contributed to our current in-depth understanding of this unique peripheral T-cell lymphoma, which in its systemic form, principally occurs in children and young adults. Objective—To summarize our current knowledge of the clinical and pathologic features of systemic and primary cutaneous ALCL. Particular emphasis is given to the biology and pathogenesis of ALCL. Data Sources—Search of the medical literature (Ovid MEDLINE In-Process & Other Non-Indexed Citations and Ovid MEDLINE: 1950 to Present [National Library of Medicine]) and more than 20 years of diagnostic experience were used as the source of data for review. Conclusions—Based on immunostaining for activation antigen CD30 and the presence of...

Anaplastic Large Cell Lymphoma: Twenty-Five Years of Discovery

Background Lymphomas with involvement of soft tissues as a primary event are very rare. The published studies have a small sample size, most of them being reported as case reports. Patients and Methods In this article we describe our experience with soft tissue non-Hodgkin lymphomas (NHL) diagnosed and treated in our institution over a 15-year period. Moreover, we systematically review the available data from the literature in the past 2 decades, considering all the published series and case reports available from 1990 to 2011 using a PubMed access. Results In the monocentric analysis, 16 consecutive patients treated at our Institution from 1996 to 2011 were considered. In the literature search, we selected 16 case reports (18 patients) and 5 case series (49 patients), including a total of 67 patients. Eighty-three patients were finally considered in the combined analysis. The most common histologic subtype was diffuse large B cell lymphoma (DLBCL) (>50% of cases in both groups). In both analyses we observed an inferior outcome for DLBCL compared with indolent B-cell NHL (5-year progression free survival: 34% vs. 64%, respectively, in the combined analysis; P = .01). Furthermore, the prognosis in the DLBCL group appears to be worse compared with the historical data of DLBCL patients treated with chemoimmunotherapy. Conclusions Though indolent soft tissue B-cell NHLs appear to have a good outcome, soft tissue DLBCLs represent an anatomic-clinical entity with aggressive features, and dismal prognosis. Strategies of first-line therapy intensification could be considered. Studies aiming to a better biologic characterization of this peculiar entity are warranted.

https://www.clinical-lymphoma-myeloma-leukemia.com/article/S2152-2650(12)00253-4/pdf

Non-Hodgkin Lymphomas Presenting as Soft Tissue Masses: A Single Center Experience and Meta-Analysis of the Published Series

Primary anaplastic large cell lymphoma (ALCL) of skeletal muscle is very rare. We report a case of ALCL arising from the left psoas muscle. A 14-year-old girl presented with a large left inguinal tumor. She complained of a 2-month history of left leg pain, which had been exacerbated upon leg extension, and she had become aware of a rapidly growing left inguinal tumor 3 weeks before admission. CT scan and MRI revealed a large tumor arising from the left major psoas muscle and protruding into the inguinal region. In view of the tumor's location and the patient's age, soft tissue tumors such as rhabdomyosarcoma and primitive neuroectodermal tumor were initially considered. However, histopathological examination yielded a diagnosis of anaplastic lymphoma kinase-positive ALCL. The serum level of soluble interleukin-2 receptor was markedly elevated at 50,414 U/ml, and this also strongly suggested ALCL. Although rarely reported, ALCL is an important entity to consider in the differential diagnosis of skeletal muscle tumors in children and young adults.

Primary anaplastic large cell lymphoma of the psoas muscle: a case report and literature review.

By reviewing 3725 lymphomas diagnosed in our institution from 1999 to 2010, we found eight cases (0.21%) of primary lymphoma in soft tissue with comprehensive histologic and immunohistochemical studies. Of the eight cases of primary lymphoma in soft tissue, two patients were male and six were female. The mean age was 54.8 years with range from 23 to 76 years. The greatest diameter of tumor ranged from 1.3 to 20 cm with an average diameter of 7.5 cm. Six cases were diffuse large B-cell lymphoma (DLBCL), subclassified as three DLBCL non-germinal center B-cell phenotype, one DLBCL germinal center B-cell phenotype, and two (Epstein-Barr Virus) EBV-positive DLBCL of the elderly; one case was anaplastic large cell lymphoma, ALK-positive; and one case was peripheral T cell lymphoma, not otherwise specified. Follow-up was available for four patients. One patient who refused further therapy died of the disease at 2 months after diagnosis. The other three patients with follow-up were alive without recurrence and metastatic disease at 9, 36, and 48 months, respectively, after excisional biopsy combined with appropriate chemotherapy and/or radiation therapy.

Clinicopathologic features of primary lymphoma in soft tissue.

Primary involvement of skeletal muscle is a very rare event in ALK-1 positive anaplastic large cell lymphoma (ALCL). We describe a case of a 10-year old boy presenting with a three week history of pain and a palpable firm swelling at the dorsal aspect of the left thigh. Histological examination of the lesion revealed a tumoral and diffuse polymorphic infiltration of the muscle by large lymphoid cells. Tumor cells displayed eccentric, lobulated "horse shoe" or "kidney-shape" nuclei. The cells showed immunohistochemical positivity for CD30, ALK-1, CD2, CD3, CD7, CD8, and Perforin. Fluorescence in situ hybridization analysis revealed a characteristic rearrangement of the ALK-1 gene in 2p23 leading to the diagnosis of ALK-1 positive ALCL. Chemotherapy according to the ALCL-99-NHL-BFM protocol was initiated and resulted in a complete remission after two cycles. This case illustrates the unusual presentation of a pediatric ALCL in soft tissue with a good response to chemotherapy.

/pdf/case-report-a-unique-pediatric-case-of-a-primary-cd8-5bjx9mw1vp.pdf

Case report: a unique pediatric case of a primary CD8 expressing ALK-1 positive anaplastic large cell lymphoma of skeletal muscle

Anaplastic large cell lymphoma (ALCL) represents approximately 10 to 30% of all childhood non-Hodgkin lymphomas. It frequently involves both lymph nodes and extranodal sites whereas primary or secondary muscular involvements are quite uncommon. We describe a case of an 8-year-old boy presented with one month progressively swelling right buttock mass without association of lymphadenopathy or skin extension. Biopsy of the lesion showed large anaplastic cells with voluminous and abundant cytoplasm as well as folded nuclei. The tumour cells were positive for CD30, CD3, EMA and ALK-1. Chemotherapy resulted in durable remission status. This case emphasizes the occurrence of anaplastic large cell lymphoma in the soft tissue and the favourable outcome of ALK-positive anaplastic large cell lymphoma.

Primary CD30/ALK-1 positive anaplastic large cell lymphoma of the skeletal muscle in a child.

Summary Background Bloom syndrome is a rare disease characterized by chromosomal instability and increased risk of developing lymphoma. Observation We report on a case of Bloom syndrome in a 5-year-old boy with Burkitt lymphoma. The diagnosis was suspected by growth retardation, repeated respiratory infections, facial telangiectasia, and a low immunoglobulin level, then confirmed cytogenetically by sister chromatid exchanges. Chemotherapy was poorly tolerated, which required reducing the doses. Unfortunately, it was not sufficient to control the neoplasm and the patient died 14 months after diagnosis. Conclusions Cancers in Bloom syndrome are a challenge since the potentially life-threatening side effects of the chemotherapy may require modifications in standard treatment such as dose reduction, which can compromise the tumor prognosis.

Burkitt lymphoma in a child with Bloom syndrome

Resume Le rhabdomyosarcome botryoide des voies biliaires extrahepatiques est une cause rare d’ictere cholestatique chez l’enfant. Il est de tres mauvais pronostic et rarement diagnostique en preoperatoire. Nous rapportons un cas de rhabdomyosarcome botryoide du choledoque chez un nourrisson de 22 mois. Les explorations radiologiques etaient en faveur d’un lymphangiome kystique. Une resection de la vesicule biliaire, du canal cystique, du choledoque et de la tete du pancreas a ete faite suivie d’une chimiotherapie postoperatoire. Le diagnostic a ete porte par l’examen anatomopathologique. L’evolution etait favorable apres un recul de 22 mois.

Une choléstase causée par un rhabdomyosarcome botryoïde du cholédoque chez un nourrisson de 22 mois

Resume Le sarcome a cellules claires du rein est une tumeur maligne rare de l’enfant La frequence des metastases osseuses et son mauvais pronostic ont conduit a son individualisation hors du groupe des tumeurs de Wilms Nous rapportons deux nouveaux cas survenus chez deux garcons âges de quatre ans et de 12 mois, explores pour une masse abdominale Un traitement chirurgical suivi d’une chimiotherapie a ete realise dans les deux cas Le diagnostic a ete porte par l’examen histologique L’evolution etait favorable dans les deux cas avec, respectivement, un recul de dix et sept mois

Sarcome à cellules claires du rein. À propos de deux cas pédiatriques

Le Nephroblastome bilateral (NBb) est rare, il represente environ 5% de l'ensemble des nephroblastomes de l'enfant. Il pose un probleme therapeutique en l'absence de protocoles codifies. L'objectif est de guerir en epargnant le maximum de tissu renal fonctionnel. Dans le but d'analyser les particularites cliniques et les resultats therapeutiques des NBb, nous avons etudie retrospectivement 12 observations colligees sur une periode de 13 ans et pris en charge a l'hopital d'Enfant de Tunis et au CHU de Monastir. Il s'agit de 9 garcons et 3 filles âges de 6 a 72 mois. L'âge moyen est de 17 mois. 11 patients avaient une NBb synchrone et dans un seul cas metachrone . Tous nos patients ont recu une chimiotherapie preoperatoire sauf un seul opere d'emblee sans contexte d'urgence. Celle-ci etait faite d'une association de Vincristine - Actinomycine dans 7 cas, Actinomycine-Vincristine et Adriamycine dans 2 cas, Etoposide - Carboplatine- Cyclophosphamide dans 1 cas. Neuf patients ont ete operes : une nephrectomie totale unilaterale et une nephrectomie partielle polaire controlaterale dans 3 cas, une enucleation tumorale bilaterale en 2 temps dans 1 cas. Deux patients sont decedes de toxicite medicamenteuse par septicemie. Le recul moyen etait de 58 mois (8-15 ans). Un patient a eu une rechute tumorale locale et metastatique. La survie est de 83,3 pour cent. L'evolution de la fonction renale etait normale dans tous les cas avec un recul moyen de 87 mois.

Le nephroblastome bilateral : prise en charge diagnostique et therapeutique. a propos de 12 cas

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