Massive localized lymphedema: Additional locations and association with hypothyroidism
Identifieur interne : 006424 ( Istex/Curation ); précédent : 006423; suivant : 006425Massive localized lymphedema: Additional locations and association with hypothyroidism
Auteurs : Debbie Wu ; John Gibbs ; David Corral ; Marilyn Intengan ; John J. BrooksSource :
- Human Pathology [ 0046-8177 ] ; 2000.
Abstract
We report the second series of a new entity called “massive localized lymphedema in morbidly obese patients” (MLL), recently described in medical literature. Our 6 cases present additional locations as well as an association with hypothyroidism. Huge masses, of longstanding duration ranging from 9 months to 8 years, afflicted the thigh, popliteal fossa, scrotum, suprapubic and inguinal region, and abdomen of morbidly obese adults. Although clinical impressions were generally of a benign process, including lipoma and recurrent cellulitis, the possibility of a malignant neoplasm could not be eliminated. Poorly defined and non-encapsulated, these skin and subcutaneous lesions were most remarkable for their sheer size, measuring 50.6 cm in mean diameter (range, 38-75 cm) and weighing a mean of 6764.5 g (range, 2,060-12,000 g) The overlying skin exhibited the induration and peau d'orange characteristic of chronic lymphedema. Grossly and histologically, a prominent marbled appearance, rendered by fibrous bands intersecting lobules of adipose tissue, simulated sclerosing well differentiated liposarcoma. However, the absence of atypical stromal cells, atypical adipocytes, and lipoblasts precluded the diagnosis of well differentiated liposarcoma. Instead, reactive features, encompassing lymphatic vascular ectasia, mononuclear cell infiltrates, fibrosis, and edema between the collagen fibers, as well as ischemic changes including infarction and fat necrosis, established the diagnosis of MLL. Although the pathogenesis of MLL may be as simple as obstruction of efferent lymphatic flow by a massive abdominal pannus and/or prior surgery, the presence of hypothyroidism in 2 of our patients suggests an alternative pathogenesis. Recognition of this entity by both clinicians and pathologists should avert a misdiagnosis as a low-grade liposarcoma. HUM PATHOL 31:1162-1168.
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DOI: 10.1053/hupa.2000.17987
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Debbie Wu<affiliation><mods:affiliation>From the Department of Pathology, SUNY at Buffalo, Buffalo, NY; the Department of Surgery, Section of Soft Tissue/Melanoma, Section of Urology, and Department of Pathology and Laboratory Medicine, Roswell Park Cancer Institute, Buffalo, NY.</mods:affiliation><wicri:noCountry code="subField">NY.</wicri:noCountry></affiliation><affiliation><mods:affiliation>From the Department of Pathology, SUNY at Buffalo, Buffalo, NY; the Department of Surgery, Section of Soft Tissue/Melanoma, Section of Urology, and Department of Pathology and Laboratory Medicine, Roswell Park Cancer Institute, Buffalo, NY.</mods:affiliation><wicri:noCountry code="subField">NY.</wicri:noCountry></affiliation><affiliation><mods:affiliation>From the Department of Pathology, SUNY at Buffalo, Buffalo, NY; the Department of Surgery, Section of Soft Tissue/Melanoma, Section of Urology, and Department of Pathology and Laboratory Medicine, Roswell Park Cancer Institute, Buffalo, NY.</mods:affiliation><wicri:noCountry code="subField">NY.</wicri:noCountry></affiliation><affiliation><mods:affiliation>From the Department of Pathology, SUNY at Buffalo, Buffalo, NY; the Department of Surgery, Section of Soft Tissue/Melanoma, Section of Urology, and Department of Pathology and Laboratory Medicine, Roswell Park Cancer Institute, Buffalo, NY.</mods:affiliation><wicri:noCountry code="subField">NY.</wicri:noCountry></affiliation><affiliation><mods:affiliation>From the Department of Pathology, SUNY at Buffalo, Buffalo, NY; the Department of Surgery, Section of Soft Tissue/Melanoma, Section of Urology, and Department of Pathology and Laboratory Medicine, Roswell Park Cancer Institute, Buffalo, NY.</mods:affiliation><wicri:noCountry code="subField">NY.</wicri:noCountry></affiliation>Le document en format XML
<record><TEI wicri:istexFullTextTei="biblStruct"><teiHeader><fileDesc><titleStmt><title xml:lang="en">Massive localized lymphedema: Additional locations and association with hypothyroidism</title><author><name sortKey="Wu, Debbie" sort="Wu, Debbie" uniqKey="Wu D" first="Debbie" last="Wu">Debbie Wu</name><affiliation><mods:affiliation>From the Department of Pathology, SUNY at Buffalo, Buffalo, NY; the Department of Surgery, Section of Soft Tissue/Melanoma, Section of Urology, and Department of Pathology and Laboratory Medicine, Roswell Park Cancer Institute, Buffalo, NY.</mods:affiliation><wicri:noCountry code="subField">NY.</wicri:noCountry></affiliation></author><author><name sortKey="Gibbs, John" sort="Gibbs, John" uniqKey="Gibbs J" first="John" last="Gibbs">John Gibbs</name><affiliation><mods:affiliation>From the Department of Pathology, SUNY at Buffalo, Buffalo, NY; the Department of Surgery, Section of Soft Tissue/Melanoma, Section of Urology, and Department of Pathology and Laboratory Medicine, Roswell Park Cancer Institute, Buffalo, NY.</mods:affiliation><wicri:noCountry code="subField">NY.</wicri:noCountry></affiliation></author><author><name sortKey="Corral, David" sort="Corral, David" uniqKey="Corral D" first="David" last="Corral">David Corral</name><affiliation><mods:affiliation>From the Department of Pathology, SUNY at Buffalo, Buffalo, NY; the Department of Surgery, Section of Soft Tissue/Melanoma, Section of Urology, and Department of Pathology and Laboratory Medicine, Roswell Park Cancer Institute, Buffalo, NY.</mods:affiliation><wicri:noCountry code="subField">NY.</wicri:noCountry></affiliation></author><author><name sortKey="Intengan, Marilyn" sort="Intengan, Marilyn" uniqKey="Intengan M" first="Marilyn" last="Intengan">Marilyn Intengan</name><affiliation><mods:affiliation>From the Department of Pathology, SUNY at Buffalo, Buffalo, NY; the Department of Surgery, Section of Soft Tissue/Melanoma, Section of Urology, and Department of Pathology and Laboratory Medicine, Roswell Park Cancer Institute, Buffalo, NY.</mods:affiliation><wicri:noCountry code="subField">NY.</wicri:noCountry></affiliation></author><author><name sortKey="Brooks, John J" sort="Brooks, John J" uniqKey="Brooks J" first="John J." last="Brooks">John J. Brooks</name><affiliation><mods:affiliation>From the Department of Pathology, SUNY at Buffalo, Buffalo, NY; the Department of Surgery, Section of Soft Tissue/Melanoma, Section of Urology, and Department of Pathology and Laboratory Medicine, Roswell Park Cancer Institute, Buffalo, NY.</mods:affiliation><wicri:noCountry code="subField">NY.</wicri:noCountry></affiliation></author></titleStmt><publicationStmt><idno type="wicri:source">ISTEX</idno><idno type="RBID">ISTEX:D5D63BB5EE7AD054A4B197FFD3EAC3E6B0813B03</idno><date when="2000" year="2000">2000</date><idno type="doi">10.1053/hupa.2000.17987</idno><idno type="url">https://api.istex.fr/document/D5D63BB5EE7AD054A4B197FFD3EAC3E6B0813B03/fulltext/pdf</idno><idno type="wicri:Area/Istex/Corpus">006424</idno><idno type="wicri:explorRef" wicri:stream="Istex" wicri:step="Corpus" wicri:corpus="ISTEX">006424</idno><idno type="wicri:Area/Istex/Curation">006424</idno></publicationStmt><sourceDesc><biblStruct><analytic><title level="a" type="main" xml:lang="en">Massive localized lymphedema: Additional locations and association with hypothyroidism</title><author><name sortKey="Wu, Debbie" sort="Wu, Debbie" uniqKey="Wu D" first="Debbie" last="Wu">Debbie Wu</name><affiliation><mods:affiliation>From the Department of Pathology, SUNY at Buffalo, Buffalo, NY; the Department of Surgery, Section of Soft Tissue/Melanoma, Section of Urology, and Department of Pathology and Laboratory Medicine, Roswell Park Cancer Institute, Buffalo, NY.</mods:affiliation><wicri:noCountry code="subField">NY.</wicri:noCountry></affiliation></author><author><name sortKey="Gibbs, John" sort="Gibbs, John" uniqKey="Gibbs J" first="John" last="Gibbs">John Gibbs</name><affiliation><mods:affiliation>From the Department of Pathology, SUNY at Buffalo, Buffalo, NY; the Department of Surgery, Section of Soft Tissue/Melanoma, Section of Urology, and Department of Pathology and Laboratory Medicine, Roswell Park Cancer Institute, Buffalo, NY.</mods:affiliation><wicri:noCountry code="subField">NY.</wicri:noCountry></affiliation></author><author><name sortKey="Corral, David" sort="Corral, David" uniqKey="Corral D" first="David" last="Corral">David Corral</name><affiliation><mods:affiliation>From the Department of Pathology, SUNY at Buffalo, Buffalo, NY; the Department of Surgery, Section of Soft Tissue/Melanoma, Section of Urology, and Department of Pathology and Laboratory Medicine, Roswell Park Cancer Institute, Buffalo, NY.</mods:affiliation><wicri:noCountry code="subField">NY.</wicri:noCountry></affiliation></author><author><name sortKey="Intengan, Marilyn" sort="Intengan, Marilyn" uniqKey="Intengan M" first="Marilyn" last="Intengan">Marilyn Intengan</name><affiliation><mods:affiliation>From the Department of Pathology, SUNY at Buffalo, Buffalo, NY; the Department of Surgery, Section of Soft Tissue/Melanoma, Section of Urology, and Department of Pathology and Laboratory Medicine, Roswell Park Cancer Institute, Buffalo, NY.</mods:affiliation><wicri:noCountry code="subField">NY.</wicri:noCountry></affiliation></author><author><name sortKey="Brooks, John J" sort="Brooks, John J" uniqKey="Brooks J" first="John J." last="Brooks">John J. Brooks</name><affiliation><mods:affiliation>From the Department of Pathology, SUNY at Buffalo, Buffalo, NY; the Department of Surgery, Section of Soft Tissue/Melanoma, Section of Urology, and Department of Pathology and Laboratory Medicine, Roswell Park Cancer Institute, Buffalo, NY.</mods:affiliation><wicri:noCountry code="subField">NY.</wicri:noCountry></affiliation></author></analytic><monogr></monogr><series><title level="j">Human Pathology</title><title level="j" type="abbrev">YHUPA</title><idno type="ISSN">0046-8177</idno><imprint><publisher>ELSEVIER</publisher><date type="published" when="2000">2000</date><biblScope unit="volume">31</biblScope><biblScope unit="issue">9</biblScope><biblScope unit="page" from="1162">1162</biblScope><biblScope unit="page" to="1168">1168</biblScope></imprint><idno type="ISSN">0046-8177</idno></series></biblStruct></sourceDesc><seriesStmt><idno type="ISSN">0046-8177</idno></seriesStmt></fileDesc><profileDesc><textClass></textClass><langUsage><language ident="en">en</language></langUsage></profileDesc></teiHeader><front><div type="abstract" xml:lang="en">We report the second series of a new entity called “massive localized lymphedema in morbidly obese patients” (MLL), recently described in medical literature. Our 6 cases present additional locations as well as an association with hypothyroidism. Huge masses, of longstanding duration ranging from 9 months to 8 years, afflicted the thigh, popliteal fossa, scrotum, suprapubic and inguinal region, and abdomen of morbidly obese adults. Although clinical impressions were generally of a benign process, including lipoma and recurrent cellulitis, the possibility of a malignant neoplasm could not be eliminated. Poorly defined and non-encapsulated, these skin and subcutaneous lesions were most remarkable for their sheer size, measuring 50.6 cm in mean diameter (range, 38-75 cm) and weighing a mean of 6764.5 g (range, 2,060-12,000 g) The overlying skin exhibited the induration and peau d'orange characteristic of chronic lymphedema. Grossly and histologically, a prominent marbled appearance, rendered by fibrous bands intersecting lobules of adipose tissue, simulated sclerosing well differentiated liposarcoma. However, the absence of atypical stromal cells, atypical adipocytes, and lipoblasts precluded the diagnosis of well differentiated liposarcoma. Instead, reactive features, encompassing lymphatic vascular ectasia, mononuclear cell infiltrates, fibrosis, and edema between the collagen fibers, as well as ischemic changes including infarction and fat necrosis, established the diagnosis of MLL. Although the pathogenesis of MLL may be as simple as obstruction of efferent lymphatic flow by a massive abdominal pannus and/or prior surgery, the presence of hypothyroidism in 2 of our patients suggests an alternative pathogenesis. Recognition of this entity by both clinicians and pathologists should avert a misdiagnosis as a low-grade liposarcoma. HUM PATHOL 31:1162-1168.</div></front></TEI></record>Pour manipuler ce document sous Unix (Dilib)
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