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Lymphology 36 (2003) 69-73
LIPOBLASTOMA-LIPOBLASTOMATOSIS ASSOCIATED WITH UNILATERAL LIMB HYPERTROPHY: A CASE REPORT IN A NEWBORN
C.M. Papendieck, L. Barbosa, P. Pow, C. Vanelli, D. Braun, A. Iotti
Universidad del Salvador, Catedra de Cirugia Pediatrica. ISNA, Instituto Argentino de Diagn6stico y Tratamiento. Buenos Aires, Argentina
ABSTRACT normal growth of toes and similar length of feet and limbs (Fig. 1a,b). There was no other Lipoblastoma (1) is a benign mesenchymal tumor in other parts of the body. Magnetic tumor ofembryonic adipose tissue, uncommon resonance imaging (MRI) showed a lobulated in infancy. Multicentricity, absence ofa capsule architecture and a peripheral capsule (Fig. 1c). and histopathology best defines a lipoblas Plain x-rays disclosed abnormal growth of the toma (2,3). Synonyms for this lesion are (4) 4th metatarsal with continuous periosteum, embryonic lipoma, fetal lipoma, lipoblastic hypertrophic tarsus and metatarsus in the tumor, and congenital lipomatoid tumor. involved foot (Fig. 1d). Lipoblastoma is more common in males Initial treatment was an extended but not (-80%) (5), is usually located in the subcuta radical excision of the tumor. Postoperative neous soft tissue (benign lipoblastoma) or in function and sensitivity of the foot was intact. the deep interstitium (benign lipoblastomatosis), At operation, the lesion was a lobulated or sometimes in all corporeal segments. fat tumor, infiltrating the spaces between the Primary treatment is complete excision. bones but not specific tissues on the foot, and Relapse is between 14-25%, many of which was pseudoencapsulated with difficulty in are more "mature," and difficult to differen determining the limits between normal and tiate from lipoma. The differential diagnosis abnormal fat tissue. Hemostasis was includes liposarcoma, which is rare under ten excellent, there were no grossly abnormal years (6). Radical excision in children is blood vessels and no damage to what recommended with relapses, especially with appeared to be a normal blood circulation. lipoblastomatosis (7). Chromosomal markers Total volume of the resected specimen help discriminate between liposarcoma and was 200 mg. There was proliferation of lipoblastoma (8). adipocytes in different stages of maturity, confirming a well-lobulated tumor. Lipoblasts CASE REPORT mixed with well-differentiated adipocytes (Fig. 2). The fat tumor involved all other A one-month old female baby presented structures without infiltration (9), and the with an asymptomatic soft tissue tumor on final diagnosis was lipoblastomatosis (10). the bottom of the left foot (three times bigger The post-operative course was without volume than a contralateral unaffected foot) complications. Over the next several months, without specific signs, but with otherwise there was increased discrepancy of the length
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Fig. 1: (a) Pre-operative image ofboth feet; (b) lateral view of the affected foot; (c) MR1 without specific signs of lipoblastoma; (d) comparative plain x-ray offeet. Nonspecific lesion ofthe 4th metatarsal bone in its metaphysis (arrow). and volume of the lower limb (hypertrophy) challenge. Diagnosis before operation is not on the affected side, with increased size always possible. An example is lipoblastoma. (recurrence) of the tumor on the sole and This report requires other considerations. both malleolar regions (pseudohypertrophy). First, the peculiar bone lesion which is At the age of eight months, reoperation was unclear was not progressive. Second, undertaken with attempt at complete hypertrophy (increase of the bone growth in resection (Fig. 3a). At age three years, there proportion to the increase of the soft tissue was discrepancy of limb length of -2 em. The enlargement) is not easily explained in abnormal growth of the 4th metatarsal bone comparison with the healthy side. This is still seen (Fig. 3b). association may be coincidental or the confluence of three derangements namely, DISCUSSION lipoblastomatosis, hypertrophy, and dysmorphogenic bone growth. Each aspect Soft tissue tumors in the newborn and can have an established explanation the first year of life are a clinical and surgical separately. Dysmesenchymallipogenesis, in
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Fig. 2: Histology of resected tissue. Proliferating lobulated adipocytes in different stages of maturation; lipoblasts mixed with well differentiated adipocytes. Mitotic activity not prominent. Low and high magnification. [H&E, (a) 200x and (b) lOOOx]
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Fig. 3: (a) Lateral view of both feet after resection oftumor recurrence on the sole and maleolar areas. (b) Plain x-ray showing increase ofgrowth ofbones and soft tissue (hypertrophy) on the involved left side.
Permission granted for single print for individual use. Reproduction not permitted without permission of Journal LYMPHOLOGY . 73 the context of lipodystrophy, in combination and a review of the literature. Cancer 52 with limb hypertrophy, may be related to (1983),567-574. angiodysplasias (11-12). In this patient after 7. Mahour, GH, BJ Bryan, H Isaass: Lipoblastoma and lipoblastomatosis. A report reoperation, no direct or indirect signs of of six cases. Surgery 104 (1988),577-579. recurrence have thus far appeared. 8. Willen, H, et al: Comparison of chromosomal patterns with clinical features in 165 lipomas: A report of the CHAMP Study Group. Cancer REFERENCES Genet. Cytogenet. 102 (1998), 46-49. 9. Enzinger, FM, SN Weiss: Soft Tissue Tumors. S1. Louis: CV Mosby, 1983,214-221. 1. Bertana, S, GP Parigi, M Giuntoli, et al: 10. Chung, EB, FM Enzinger: Benign Lipoblastoma and lipoblastomatosis in chil lipoblastomatosis. An analysis of 35 cases. dren. Minerva Pedi
Permission granted for single print for individual use. Reproduction not permitted without permission of Journal LYMPHOLOGY .