0
We're unable to sign you in at this time. Please try again in a few minutes.
Retry
We were able to sign you in, but your subscription(s) could not be found. Please try again in a few minutes.
Retry
There may be a problem with your account. Please contact the AMA Service Center to resolve this issue.
Contact the AMA Service Center:
Telephone: 1 (800) 262-2350 or 1 (312) 670-7827  *   Email: subscriptions@jamanetwork.com
Error Message ......
Special Feature |

Pathological Case of the Month FREE

[+] Author Affiliations

Section Editor: Walter W. Tunnessen, MD

More Author Information
Arch Pediatr Adolesc Med. 2001;155(3):412. doi:10.1001/archpedi.155.3.411.
Text Size: A A A
Published online

DENOUEMENT AND DISCUSSION: KI-1–POSITIVE ANAPLASTIC LARGE CELL LYMPHOMA INVOLVING MUSCLE

Figure 1. Swelling with overlying erythema on the anterior aspect of the left upper arm.

Figure 2. Computed tomographic scan of the upper limbs showing a tumor arising from the left biceps muscle.

Figure 3. Cohesive sheets of large pleomorphic cells displaying vesicular nuclei with peripheral condensation of chromatin are seen in the dermis. Some have prominent irregular nucleoli and amphophilic or vacuolated abundant cytoplasm. Several mitotic figures are also noted (hematoxylin-eosin, original magnification ×400).

Figure 4. Lymphoma cells display strong staining of cell membranes for Ki-1 antigen CD30 (Bcr-H2 monoclonal antibody, peroxidase-antiperoxidase, original magnification ×400).

The CT scan of the thigh showed a tumor arising from the left rectus femoris muscle. Examination of the bone marrow and cerebrospinal fluid did not show any metastases. The lymphoma was classified as stage 3 (Murphy classification), and the child was treated with chemotherapy using the French LMB-89 protocol. She has completed therapy and is currently healthy with resolution of all the swellings.

Large cell lymphomas constitute approximately 20% of all childhood non-Hodgkin lymphomas.1 Ki-1–positive anaplastic large cell lymphoma (Ki-l+ ALCL) is a tumor that expresses the antigen CD30, which is detected by the monoclonal antibody Ki-1. It is associated with the translocation t(2;5).2 Seventy percent of ALCL are of T-cell lineage, as in this case.3 The usual presentation of Ki-1+ ALCL is lymphadenopathy. Common extranodal sites include skin, soft tissue, lung, and bone. Bone marrow and central nervous system involvement is uncommon.4

Primary skeletal muscle lymphoma is rare. In a large retrospective series, only 8 (0.1%) of 7000 cases had primary muscle lymphoma, with the thigh and upper arm being the most common sites of involvement.5 Previous reports in the literature have been mainly in adults, particularly the elderly population.6

In children with advanced-stage ALCL, CD30 positivity is associated with a better survival than CD30 negativity.7 Overall survival rates for children with Ki-1+ ALCL treated with chemotherapy are approximately 70% to 80%, regardless of whether a B-cell or T-cell protocol is used.8 Our patient responded well to a protocol for B-cell lymphomas.

Accepted for publication November 24, 1999.

Reprints: Bina S. Menon, MRCP (UK), No. 53, Lorong 5/4 H, 46000 Petaling Jaya, Selangor, Malaysia.

Readett  DRJGerrard  MP Lymphoma. Curr Pediatr. 1998;892- 97
Link to Article
Bitter  MAFranklin  WALarson  RA  et al.  CD30-positive non-Hodgkin's lymphoma is correlated with clinical features and the presence of a unique chromosomal abnormality, t(2;5) (p23;q35). Am J Surg Pathol. 1990;14305- 316
Link to Article
Kadin  ME Primary Ki-1 positive anaplastic large-cell lymphoma: a distinct clinicopathologic entity. Ann Oncol. 1994;525- 30
Link to Article
Shad  AMagrath  I Malignant non-Hodgkin's lymphomas in children. Pizzo  PAPoplack  DGeds.Principles and Practice of Pediatric Oncology Philadelphia, Pa Lippincott-Raven1997;545- 587
Travis  WDBanks  PMReiman  HM Primary extranodal soft tissue lymphoma of the extremities. Am J Surg Pathol. 1987;11359- 366
Link to Article
Yi  KKLiang  R Report of a case of primary skeletal muscle lymphoma and review of the literature. Acta Haematol. 1996;96184- 186
Link to Article
Sandlund  JTPui  CHSantana  VM  et al.  Clinical features and treatment outcome for children with CD30+ large cell non-Hodgkin's lymphoma. J Clin Oncol. 1994;12895- 898
Patte  C Non-Hodgkin's lymphoma. Eur J Cancer. 1998;34359- 362
Link to Article

Tables

References

Readett  DRJGerrard  MP Lymphoma. Curr Pediatr. 1998;892- 97
Link to Article
Bitter  MAFranklin  WALarson  RA  et al.  CD30-positive non-Hodgkin's lymphoma is correlated with clinical features and the presence of a unique chromosomal abnormality, t(2;5) (p23;q35). Am J Surg Pathol. 1990;14305- 316
Link to Article
Kadin  ME Primary Ki-1 positive anaplastic large-cell lymphoma: a distinct clinicopathologic entity. Ann Oncol. 1994;525- 30
Link to Article
Shad  AMagrath  I Malignant non-Hodgkin's lymphomas in children. Pizzo  PAPoplack  DGeds.Principles and Practice of Pediatric Oncology Philadelphia, Pa Lippincott-Raven1997;545- 587
Travis  WDBanks  PMReiman  HM Primary extranodal soft tissue lymphoma of the extremities. Am J Surg Pathol. 1987;11359- 366
Link to Article
Yi  KKLiang  R Report of a case of primary skeletal muscle lymphoma and review of the literature. Acta Haematol. 1996;96184- 186
Link to Article
Sandlund  JTPui  CHSantana  VM  et al.  Clinical features and treatment outcome for children with CD30+ large cell non-Hodgkin's lymphoma. J Clin Oncol. 1994;12895- 898
Patte  C Non-Hodgkin's lymphoma. Eur J Cancer. 1998;34359- 362
Link to Article

Correspondence

CME
Meets CME requirements for:
Browse CME for all U.S. States
Accreditation Information
The American Medical Association is accredited by the Accreditation Council for Continuing Medical Education to provide continuing medical education for physicians. The AMA designates this journal-based CME activity for a maximum of 1 AMA PRA Category 1 CreditTM per course. Physicians should claim only the credit commensurate with the extent of their participation in the activity. Physicians who complete the CME course and score at least 80% correct on the quiz are eligible for AMA PRA Category 1 CreditTM.
Note: You must get at least of the answers correct to pass this quiz.
You have not filled in all the answers to complete this quiz
The following questions were not answered:
Sorry, you have unsuccessfully completed this CME quiz with a score of
The following questions were not answered correctly:
Commitment to Change (optional):
Indicate what change(s) you will implement in your practice, if any, based on this CME course.
Your quiz results:
The filled radio buttons indicate your responses. The preferred responses are highlighted
For CME Course: A Proposed Model for Initial Assessment and Management of Acute Heart Failure Syndromes
Indicate what changes(s) you will implement in your practice, if any, based on this CME course.
Submit a Comment

Multimedia

Some tools below are only available to our subscribers or users with an online account.

Related Content

Customize your page view by dragging & repositioning the boxes below.

Articles Related By Topic
Related Collections
PubMed Articles