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Massive localised lymphoedema: a clinicopathological study of 22 cases and review of the literature.
J Clin Pathol. 2009 Sep; 62(9):808-11.JC

Abstract

BACKGROUND

Massive localised lymphoedema (MLL) is a rare, relatively recently described pseudosarcoma most often occurring in morbidly obese patients.

AIM

To perform a retrospective review of all cases diagnosed as MLL.

METHODS AND RESULTS

Clinical information was obtained. 22 morbidly obese adults (mean patient weight 186 kg) presented with unilateral, large soft tissue lesions of longstanding duration. Most lesions involved the thigh, but also occurred in the posterior calf and lower leg. Clinically, most lesions were regarded as representing benign processes, including pedunculated lipoma, lymphocoele or recurrent cellulites, although soft tissue sarcoma was also suspected in two cases. Grossly, all masses showed markedly thickened skin with a "cobblestone" appearance, and were ill-defined, unencapsulated, lobulate, and very large (mean size 31 cm, range 15-61.5 cm, mean weight 3386 g, range 1133-10,800 g). Histologically, all 22 cases showed striking dermal fibrosis, expansion of the fibrous septa between fat lobules with increased numbers of stromal fibroblasts, lymphatic proliferation and lymphangiectasia. Multinucleated fibroblastic cells, marked vascular proliferation, moderate stromal cellularity and fascicular growth raised concern among referring pathologists for atypical lipomatous tumour/well differentiated liposarcoma, angiosarcoma, and a fibroblastic neoplasm such as fibromatosis in 10, 2 and 1 case, respectively.

CONCLUSION

The diagnosis of MLL continues to be challenging, in particular for pathologists. Awareness of this entity, clinical correlation and gross pathological correlation are essential in the separation of this distinctive pseudosarcoma from its various morphological mimics.

Authors+Show Affiliations

Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota 55905, USA.No affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article
Review

Language

eng

PubMed ID

19734477

Citation

Manduch, M, et al. "Massive Localised Lymphoedema: a Clinicopathological Study of 22 Cases and Review of the Literature." Journal of Clinical Pathology, vol. 62, no. 9, 2009, pp. 808-11.
Manduch M, Oliveira AM, Nascimento AG, et al. Massive localised lymphoedema: a clinicopathological study of 22 cases and review of the literature. J Clin Pathol. 2009;62(9):808-11.
Manduch, M., Oliveira, A. M., Nascimento, A. G., & Folpe, A. L. (2009). Massive localised lymphoedema: a clinicopathological study of 22 cases and review of the literature. Journal of Clinical Pathology, 62(9), 808-11. https://doi.org/10.1136/jcp.2009.066555
Manduch M, et al. Massive Localised Lymphoedema: a Clinicopathological Study of 22 Cases and Review of the Literature. J Clin Pathol. 2009;62(9):808-11. PubMed PMID: 19734477.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Massive localised lymphoedema: a clinicopathological study of 22 cases and review of the literature. AU - Manduch,M, AU - Oliveira,A M, AU - Nascimento,A G, AU - Folpe,A L, PY - 2009/9/8/entrez PY - 2009/9/8/pubmed PY - 2009/12/16/medline SP - 808 EP - 11 JF - Journal of clinical pathology JO - J. Clin. Pathol. VL - 62 IS - 9 N2 - BACKGROUND: Massive localised lymphoedema (MLL) is a rare, relatively recently described pseudosarcoma most often occurring in morbidly obese patients. AIM: To perform a retrospective review of all cases diagnosed as MLL. METHODS AND RESULTS: Clinical information was obtained. 22 morbidly obese adults (mean patient weight 186 kg) presented with unilateral, large soft tissue lesions of longstanding duration. Most lesions involved the thigh, but also occurred in the posterior calf and lower leg. Clinically, most lesions were regarded as representing benign processes, including pedunculated lipoma, lymphocoele or recurrent cellulites, although soft tissue sarcoma was also suspected in two cases. Grossly, all masses showed markedly thickened skin with a "cobblestone" appearance, and were ill-defined, unencapsulated, lobulate, and very large (mean size 31 cm, range 15-61.5 cm, mean weight 3386 g, range 1133-10,800 g). Histologically, all 22 cases showed striking dermal fibrosis, expansion of the fibrous septa between fat lobules with increased numbers of stromal fibroblasts, lymphatic proliferation and lymphangiectasia. Multinucleated fibroblastic cells, marked vascular proliferation, moderate stromal cellularity and fascicular growth raised concern among referring pathologists for atypical lipomatous tumour/well differentiated liposarcoma, angiosarcoma, and a fibroblastic neoplasm such as fibromatosis in 10, 2 and 1 case, respectively. CONCLUSION: The diagnosis of MLL continues to be challenging, in particular for pathologists. Awareness of this entity, clinical correlation and gross pathological correlation are essential in the separation of this distinctive pseudosarcoma from its various morphological mimics. SN - 1472-4146 UR - https://www.unboundmedicine.com/medline/citation/19734477/Massive_localised_lymphoedema:_a_clinicopathological_study_of_22_cases_and_review_of_the_literature_ L2 - http://jcp.bmj.com/cgi/pmidlookup?view=long&pmid=19734477 DB - PRIME DP - Unbound Medicine ER -