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Byline: Hiroshi Hojo (1), Sayuri Suzuki (1), Atushi Kikuta (2), Masaki Ito (2), Masafumi Abe (1) Keywords: Key words: neuroblastoma, sarcoid reaction, lymphocyte phenotype, immunohistochemistry Abstract: We present a unique case of a 9-month-old infant with a left adrenal neuroblastoma with sarcoid reaction, detected by mass screening. There was no clinical evidence indicating systemic sarcoidosis or pulmonary mycobacterial infection. Histological examination of the resected adrenal tumor revealed many noncaseating epithelioid granulomas with lymphocytic infiltrate, composed of epithelioid cells and few giant cells, arising in tumor parenchyma and fibrovascular stroma. Most of the lymphocytes in the granulomas were CD3- or CD45RO-positive T cells, with fewer being CD20-positive B cells. The lymphocytes in the epithelioid granulomas expressed CD4 or CD8, but not CD56 and CD57. CD4-positive cells were observed more within the granulomas (internal area) than in the surrounding area (external area) of the same granulomas, while most of the CD8-positive cells were seen consistently at the outer margin of the granulomas (marginal zone). CD45RA-positive T cells were observed predominantly in the external area. The results of immunostaining demonstrated that lymphocytes in granulomas of this case showed the same distribution pattern as that seen in systemic sarcoidosis. Although the sarcoid reaction is a phenomenon known to be associated with the region of cancer, granuloma within the primary neuroblastoma is extremely rare. The sarcoid reaction in the present case of neuroblastoma may be associated with a delayed-type hypersensitivity reaction, and its significance and relevance still remain obscure. Author Affiliation: (1) 1st Department of Pathology, School of Medicine, Fukushima Medical University, 1 Hikariga-oka, Fukushima, 960-1295, Japan, JP (2) Department of Pediatrics, School of Medicine, Fukushima Medical University, 1 Hikariga-oka, Fukushima, 960-1295, Japan, JP Article note: Received September 9, 1998 accepted October 21, 1999.

Byline: Stephen J. Qualman (1), Cheryl M. Coffin (2), William A. Newton (1), Hiroshi Hojo (3), Timothy J. Triche (4), David M. Parham (5), William M. Crist (6) Keywords: Key words: rhabdomyosarcoma, embryonal, botryoid, spindle cell, alveolar, anaplasia Abstract: Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma of childhood, and 75% of such cases in the United States are reviewed at the Pathology Center for the Intergroup Rhabdomyosarcoma Study Group (IRSG). The first four generations of IRSG therapeutic trials (IRS I--IV) and supportive pathologic studies have generated a new International Classification of Rhabdomyosarcoma (ICR) that offers new morphologic concepts to the practicing pathologist. The objective of this report is to clearly define emerging histopathologic categories of RMS as defined by the ICR, and to emphasize correlative immunohistochemical or molecular studies. Emerging ICR variants of RMS place the patient in widely divergent prognostic categories (superior, botryoid or spindle cell variants poor, solid alveolar or diffusely anaplastic variants). The cardinal histopathologic features of the ICR combined with results of studies of fusion genes seen with t(1 13) and t(2 13) will help delineate therapeutic subgroups of RMS for the fifth generation (IRS V) of IRSG studies. Consequently, it is imperative for the practicing pathologist to be familiar with the practical workup and diagnosis of RMS in childhood. Author Affiliation: (1) Department of Laboratory Medicine and IRSG Pathology Center, Children's Hospital and Ohio State University School of Medicine, 700 Children's Drive, Columbus, OH 43205, USA , US (2) Department of Pathology, Division of Pediatric Pathology, University of Utah School of Medicine and Primary Children's Medical Center, 100 North Medical Drive, Salt Lake City, UT 84113, USA , US (3) Department of Pathology, Fukushima Medical College, 1 Hikariga-Oka, Fukushima 960-12, Japan , JP (4) Department of Pathology, Children's Hospital Los Angeles and University of Southern California School of Medicine, 4650 Sunset Boulevard, M543, Los Angeles, CA 90027, USA , US (5) Department of Pathology, Arkansas Children's Hospital and University of Arkansas Medical School, 800 Marshall Street, Slot 820, Little Rock, AR 72202-3591, USA , US (6) Department of Pediatric and Adolescent Medicine, Mayo Clinic, Desk East 9 (Peds), 200 First Street, SW, Rochester, MN 55905-0001, USA , US