Multiple childhood osteosarcomas in an american indian family with erythroid macrocytosis and skeletal anomalies

John J. Mulvihill, Harvey R. Gralnick, Jacqueline Whang‐Peng, Brigid G. Leventhal

Research output: Contribution to journalArticle

22 Citations (Scopus)

Abstract

Three of nine children of possibly consanguineous American Indian parents developed typical osteosarcoma in a 2‐year period. Etiologic investigations detected limb anomalies and elevated mean corpuscular volumes (98‐109 μm3) in the surviving tumor patient, several of her sibs, and her father. Limb anomalies included simple clinodactyly with brachymesophalangy, absence of one digital ray of the foot, and bilateral radioulnar synostosis. The red cell macrocytosis was not accompanied by anemia or explained by the usual causes. No unusual environmental exposures were found and screening for possible oncogenic viruses by culture, electron microscopy, and serology was negative. All family members had elevated antibody titers to Epstein‐Barr viral antigens. The proband and her father had excessive chromosomal breaks in the bone marrow. This unusual familial pattern of osseous malignancy and malformation and defective erythropoiesis, tentatively called OSLAM syndrome, may represent impaired regulation of bone development. Cancer 40:3115‐3122, 1977.

Original languageEnglish
Pages (from-to)3115-3122
Number of pages8
JournalCancer
Volume40
Issue number6
DOIs
Publication statusPublished - Jan 1 1977
Externally publishedYes

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North American Indians
Osteosarcoma
Fathers
Extremities
Chromosome Breakage
Neoplasms
Oncogenic Viruses
Erythrocyte Indices
Viral Antigens
Erythropoiesis
Bone Development
Environmental Exposure
Serology
Foot
Anemia
Electron Microscopy
Parents
Bone Marrow
Antibodies

ASJC Scopus subject areas

  • Oncology
  • Cancer Research

Cite this

Multiple childhood osteosarcomas in an american indian family with erythroid macrocytosis and skeletal anomalies. / Mulvihill, John J.; Gralnick, Harvey R.; Whang‐Peng, Jacqueline; Leventhal, Brigid G.

In: Cancer, Vol. 40, No. 6, 01.01.1977, p. 3115-3122.

Research output: Contribution to journalArticle

Mulvihill, John J. ; Gralnick, Harvey R. ; Whang‐Peng, Jacqueline ; Leventhal, Brigid G. / Multiple childhood osteosarcomas in an american indian family with erythroid macrocytosis and skeletal anomalies. In: Cancer. 1977 ; Vol. 40, No. 6. pp. 3115-3122.
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