Low-grade fibromyxoid sarcoma, a deceptively benign tumor in a 5-year-old child.
Summary of "Low-grade fibromyxoid sarcoma, a deceptively benign tumor in a 5-year-old child."
Low-grade fibromyxoid sarcoma (LGFMS) is very uncommon in the pediatric population with only 20% of reported cases under the age of 18. The youngest reported case to date has been in a 4-year-old child. Lesions are usually slow growing and asymptomatic, and locations described in children have included paravertebral, thigh and intrathoracic. Although benign in appearance, these lesions can behave aggressively, with local recurrence and distant metastases primarily to lungs. These lesions can be resistant to the usual chemotherapy and radiotherapy with surgical resection being the treatment of choice. We report a case of a 5-year-old boy who presented with a mass in the left buttock.
Affiliation
Department of Surgery, The Children's Hospital at Westmead, Sydney, Australia, seema.menon@health.nsw.gov.au.
Journal Details
This article was published in the following journal.
Name: Pediatric surgery international
ISSN: 1437-9813
Pages:
Links
- PubMed Source: http://www.ncbi.nlm.nih.gov/pubmed/22130782
- DOI: http://dx.doi.org/10.1007/s00383-011-3024-z
Medical and Biotech [MESH] Definitions
Carcinoma, Mucoepidermoid
A tumor of both low- and high-grade malignancy. The low-grade grow slowly, appear in any age group, and are readily cured by excision. The high-grade behave aggressively, widely infiltrate the salivary gland and produce lymph node and distant metastases. Mucoepidermoid carcinomas account for about 21% of the malignant tumors of the parotid gland and 10% of the sublingual gland. They are the most common malignant tumor of the parotid. (From DeVita Jr et al., Cancer: Principles & Practice of Oncology, 3d ed, p575; Holland et al., Cancer Medicine, 3d ed, p1240)
Endometrial Stromal Tumors
Neoplasms of the endometrial stroma that sometimes involve the MYOMETRIUM. These tumors contain cells that may closely or remotely resemble the normal stromal cells. Endometrial stromal neoplasms are divided into three categories: (1) benign stromal nodules; (2) low-grade stromal sarcoma, or endolymphatic stromal myosis; and (3) malignant endometrial stromal sarcoma (SARCOMA, ENDOMETRIAL STROMAL).
Mucoepidermoid Tumor
A malignant epithelial tumor of glandular tissue, especially the salivary glands, characterized by acini with mucus-producing cells and by the presence of malignant squamous elements. Most mucoepidermoid tumors are low-grade lesions readily cured by adequate excision. They may appear in any age group. They grow slowly. If high-grade, they behave aggressively, widely infiltrating the salivary gland and producing lymph node and distant metastases. (Dorland, 27th ed; from DeVita Jr et al., Cancer: Principles & Practice of Oncology, 3d ed, p575)
Child Mortality
Number of deaths of children between one year of age to 12 years of age in a given population.
Gilbert Disease
A benign familial disorder, transmitted as an autosomal dominant trait. It is characterized by low-grade chronic hyperbilirubinemia with considerable daily fluctuations of the bilirubin level.
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