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massive_localized_lymphedema

Massive Localized Lymphedema

What is massive localized lymphedema?

I first became aware of massive localized lymphedema after one of our members posted on this huge “growth's she had at the top of one of her legs. Her doctors were apparently clueless as to what it was. In their ignorance, some told her that what ever it was, it could not be removed; that it would eventually kill her, on and on.

I started searching every term I could think of in trying to find out information for her, and it was then I came accross these clinical abstracts on the subject.

These abstracts below represent the totality of information in the medical literature.

Pat O'Connor

Massive localized lymphedema: A soft tissue process that may present to dermatologists

Am J Dermatopathol. 2010 Jun

Wang NS, Walters RF, Warren SJ.

Department of Dermatology, New York University School of Medicine, New York, 10016, USA. nadiaswang@hotmail.com

*Abstract*

Massive localized lymphedema (MLL) is a clinically and histologically distinct entity seen in morbidly obese patients. We describe two obese patients with MLL in the lower abdomen and suprapubic area. Biopsies showed characteristic histologic features of an expanded dermis with lymphangiectases, fibrotic septae and focal fat necrosis. One patient had long-standing hypothyroidism, an association previously reported. This disease is frequently complicated by recurrent cellulitis and may be amenable to surgery. Patients with MLL may present to dermatologists, and this disease has characteristic histopathologic findings that may mimic liposarcoma to the unaware dermatopathologist.

PubMed

Massive localized lymphedema - a rare entity

Decker P, Gortz M, Sigmund G, Kriegsmann J, Decker D.

Chirurgische Klinik I der Krankenanstalten Mutterhaus der Borromaerinnen Trier. decker@mutterhaus.de

Massive localized lymphedema is a rare disease. Only a few cases have been described in the literature. These monstrous pseudotumors of the subcutis are mostly localized in the inguinal region or at the lower extremity. These tumors often show a slow growth for many years. Besides hernias, lipomatous tumors must be distinguished. The therapy of choice is the excision of the tumor. Relapse is not uncommon in the few cases described in the literature so far. The diagnostic procedure and therapy of an 48-year-old women with a massive localized lymphedema weighing about 22 kg are demonstrated and discussed.

PubMed

Massive Localized Lymphedema With Unusual Presentations : Report of Two Cases and Review of the Literature.

Int J Surg Pathol. 2008 Jul 8

Bogusz AM, Hussey SM, Kapur P, Peng Y, Gokaslan ST.

Massive localized lymphedema is a benign soft tissue lesion that usually presents as a large mass in morbidly obese adults. The diagnosis may be challenging as it can mimic other lesions, including well-differentiated liposarcoma. We report 2 cases of massive localized lymphedema with unusual presentation. The first case is a recurrent massive localized lymphedema in the right thigh of a 40-year-old morbidly obese woman. In addition to typical massive localized lymphedema features such as prominent edema and vascular proliferation in the adipose_tissue, we observed prominent and abundant multinucleated stromal floret-like giant cells, arborizing network of capillaries, and areas of hyalinized collagen. Our second case is in a rare location (scrotum extending into penile soft tissue) in an overweight 55-year-old male. This lesion exhibits striking smooth muscle hyperplasia. Lack of staining by antibodies against murine double minute 2 protein and cyclin dependent kinase 4 and absence of high mobility group AT- hook 2 transcription factor rearrangement by fluorescence in situ hybridization support our diagnosis of massive localized lymphedema in both cases.

Sage Journals

Localized lymphedema (elephantiasis): a case series and review of the literature

J Cutan Pathol. 2008 Jun 17

Lu S, Tran TA, Jones DM, Meyer DR, Ross JS, Fisher HA, Carlson JA. Department of Pathology and Laboratory Medicine, Albany Medical College, Albany, NY, USA.

Background: Lymphedema typically affects a whole limb. Rarely, lymphedema can present as a circumscribed plaque or an isolated skin tumor.

Objective: To describe the clinical and pathologic characteristics and etiologic factors of localized lymphedema.

Methods: Case-control study of skin biopsy and excision specimens histologically diagnosed with lymphedema and presenting as a localized skin tumor identified during a 4-year period. Results: We identified 24 cases of localized lymphedema presenting as solitary large polyps (11), solid or papillomatous plaques (7), pendulous swellings (4), or tumors mimicking sarcoma (2).

Patients were 18 females and 6 males with a mean age of 41 years (range 16-74). Anogenital involvement was most frequent (75%) - mostly vulva (58%), followed by eyelid (13%), thigh (8%) and breast (4%). Causative factors included injury due to trauma, surgery or childbirth (54%), chronic inflammatory disease (rosacea, Crohn's disease) (8%), and bacterial cellulitis (12%). Eighty-five percent of these patients were either overweight (50%) or obese (35%). Compared with a series of 80 patients with diffuse lymphedema, localized lymphedema patients were significantly younger (41 vs. 62 years old, p = 0.0001), had no history of cancer treatment (0% vs. 18%, p = 0.03), and had an injury to the affected site (54% vs. 6%, p = 0.0001).

Histologically, all cases exhibited dermal edema, fibroplasia, dilated lymphatic vessels, uniformly distributed stromal cells and varying degrees of papillated epidermal hyperplasia, inflammatory infiltrates and hyperkeratosis. Tumor size significantly and positively correlated with history of cellulitis, obesity, dense inflammatory infiltrates containing abundant plasma cells, and lymphoid follicles (p < 0.05).

A history of cellulitis, morbid obesity, lymphoid follicles and follicular cysts predicted recurrent or progressive swelling despite excision (p < 0.05).

Conclusions: Localized lymphedema should be considered in the etiology of skin tumors when assessing a polyp, plaque, swelling or mass showing dermal edema, fibrosis and dilated lymphatics on biopsy.

A combination of lymph stasis promoting factors (trauma, obesity, infection and/or inflammatory disorders) produces localized elephantiasis.

Blackwell Synergy

Pseudosarcoma: massive localized lymphedema of the morbidly obese.

Obes Surg. 2006 Jan;16(1):88-93

Goshtasby P, Dawson J, Agarwal N. Department of General and Trauma Surgery, York Hospital, York, PA 17405, USA. parvizmd75@yahoo.com

Massive localized lymphedema is a term used to describe a benignovergrowth of lymphoproliferative tissue in morbidly obese patients, which is characterized by fibrotic and edematous fibroadipose tissue. Because of its large size and similar appearance to sarcomas, it has often been termed a pseudosarcoma. Patients tend to seek treatment only when the masses reach a sufficient size to alter their activities of daily living or have problems with excoriation or wound breakdown. Resection is indicated in these cases or if there is any question as to underlying malignancy of the lesion. Although recurrence is common, overall prognosis is good, with only anecdotal reports of transformation to angiosarcoma in the literature. We report a patient afflicted with this unique disorder.

PMID: 16417764 [PubMed - indexed for MEDLINE]

PubMed

Massive localized lymphedema in the morbidly obese : A histologically distinct reactive lesion simulating liposarcoma

Author(s) FARSHID G. (1) ; WEISS S. W. (1) ; Affiliation(s) du ou des auteurs / Author(s) Affiliation(s) (1) Department of Pathology, University of Michigan Hospitals, Ann Arbor, Michigan, ETATS-UNIS

Abstract

We report 14 cases of a soft tissue lesion in the limbs of morbidly obese adults that presents as a large mass and histologically simulates well-differentiated liposarcoma (WDL). Based on its distinctive clinical setting and morphologic identity to diffuse lymphedema we have termed this process massive localized lymphedema (MLL). All cases occurred in morbidly obese adults (mean weight 372 Ibs; mean age 47 years). Women predominated (9 women: 5 men). The lesions affected the proximal medial aspect of the extremities (12 thigh : 2 arm) and were unilateral in all but two patients.

Etiologically significant antecedent events include ipsilateral axillary lymphadenectomy in both patients with arm lesions, chronic lymphedema resulting from vein-stripping 10 years prior in one patient, inguinal lymphadenectomy for anal carcinoma in another patient, and significant blunt trauma to the inner thigh during a motor vehicle accident in a third patient. The tumors were long standing (1-10 years) and extremely large (mean size 33.4 cm. 7408 g). Clinically, they were diffuse, ill-defined masses that histologically consisted of lobules of mature fat interrupted by expanded connective tissue septa.

The constituents of the septa were fine, fibrillary collagen, edema fluid, and uniformly distributed fibroblasts. Clusters of capillaries were frequently found at the interface between fat and connective tissue. The widened septa simulated the fibrous bands of sclerosing WDL, but MLL lacks the degree of nuclear atypia seen in the former.

The consistent clustering of reactive vessels at the interface between the fat and fibrous tissue also contrasted with WDL. Six patients experienced persistent or recurrent lesions within 10 months to 10 years. No aggressive growth or histologic progression was observed during this time, however. Awareness of the features of MLL is important to avoid misclassification of this reactive lesion with WDL.

Revue / Journal Title

The American journal of surgical pathology (Am. j. surg. pathol.) ISSN 0147-5185 CODEN AJSPDX

Source / Source 1998, vol. 22, no10, pp. 1277-1283 (4 ref.)

http://cat.inist.fr/?aModele=afficheN&cpsidt=2412582

Massive localized lymphedema : Additional locations and association with hypothyroidism

Author(s)

WU Debbie ; GIBBS John ; CORRAL David ; INTENGAN Marilyn ; BROOKS John J. ; Affiliation(s) du ou des auteurs / Author(s) Affiliation(s) Department of Pathology, SUNY at Buffalo, Buffalo, NY, ETATS-UNIS Department of Surgery, Section of Soft Tissue/Melanoma, Section of Urology, and Department of Pathology and Laboratory Medicine, Roswell Park Cancer Institute, Buffalo, NY, ETATS-UNIS

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.

Revue / Journal Title

Human pathology (Human pathol.) ISSN 0046-8177 CODEN HPCQA4 Source / Source

2000, vol. 31, no9, pp. 1162-1168 (29 ref.)

http://cat.inist.fr/?aModele=afficheN&cpsidt=1510894

Massive Localized Lymphedema of the Morbidly Obese

Authors: Goshtasby, Parviz1; Dawson, John1; Agarwal, Nikhilesh1

Source: Obesity Surgery, Volume 16, Number 1, January 2006, pp. 88-93(6)

Publisher: FD Communications Inc.

Abstract:

Massive localized lymphedema is a term used to describe a benign overgrowth of lymphoproliferative tissue in morbidly obese patients, which is characterized by fibrotic and edematous fibroadipose tissue. Because of its large size and similar appearance to sarcomas, it has often been termed a pseudosarcoma.

Patients tend to seek treatment only when the masses reach a sufficient size to alter their activities of daily living or have problems with excoriation or wound breakdown. Resection is indicated in these cases or if there is any question as to underlying malignancy of the lesion.

Although recurrence is common, overall prognosis is good, with only anecdotal reports of transformation to angiosarcoma in the literature. We report a patient afflicted with this unique disorder.

Keywords: LYMPHEDEMA; MORBID OBESITY; FIBROMA; LOWER EXTREMITY; PSEUDOSARCOMA; LIPOMA; LIPOSARCOMA

Document Type: Case report

DOI: 10.1381/096089206775222014

Affiliations: 1: Department of General and Trauma Surgery, York Hospital, York, PA, USA

http://www.ingentaconnect.com/content/fd/os/2006/00000016/00000001/art00017

Massive Localized Lymphedema (MLL) in Bariatric Candidates

Authors: Modolin, Miguel L.A.1; Cintra, Wilson1; Paggiaro, André O.1; Faintuch, Joel2; Gemperli, Rolf1; Ferreira, Marcus C.1

Source: Obesity Surgery, Volume 16, Number 9, September 2006, pp. 1126-1130(5)

Publisher: FD Communications Inc.

Abstract:

Background: Mild lymphedema of lower limbs and eventually abdomen is not exceedingly rare in morbid obesity. However, few large symptomatic masses have been reported. In a consecutive series of patients, all requiring resection of the lesion before bariatric treatment, clinical features and surgical findings are described, aiming to clarify the nature of this intricate problem.

Methods: Subjects (n=4, 50% females, age 34.0±13.7 years (19-53), BMI 56.4±10.5 kg/m2 (44.1-73.1) displayed lesions on the anteromedial aspect of the thigh (n=3) and hypogastrium (n=1). All reported episodes of intertrigo of local skin-folds in the preceding years, managed by local care and antibiotics. The mass was described as a serious nuisance, impairing walking, dressing and personal hygiene.

Results: The mass was surgically removed without requirement for blood transfusion except in the case of one huge mass. Complications were relatively minor and consisted of partial skin dehiscence and lymph leakage for 2-3 weeks. Histologically, a complex pattern was observed including skin hypertrophy, edema, fibrosis, foci of microabscesses and dilated blood vessels, along with the pathognomonic lymphangiectasia. On follow-up to 6 months, improvement or restoration of the ability to walk occurred, with no additional skin infection and no recurrence.

Conclusions: 1) Surgical treatment was effective. 2) Functional rehabilitation was achieved. 3) No recurrence was observed within the follow-up period.

Keywords: LYMPHEDEMA; MASSIVE LOCALIZED LYMPHEDEMA; PSEUDOSARCOMA; ELEPHANTIASIS NOSTRAS; BARIATRIC SURGERY; MORBID OBESITY

Document Type: Research article

DOI: 10.1381/096089206778392185

Affiliations: 1: Plastic Surgery, Hospital das Clinicas, São Paulo, SP, Brazil 2: Gastroenterology Service, Hospital das Clinicas, São Paulo, SP, Brazil

http://www.ingentaconnect.com/content/fd/os/2006/00000016/00000009/art00003;jsessionid=8m5jl9n42t4lp.alice

Unusual case of lymphoedema in a morbidly obese patient.

Australas J Dermatol. 2007 May

Weston S, Clay CD. Department of Dermatology, Royal Perth Hospital, Perth, Western Australia, Australia.

A morbidly obese 57-year-old woman presented with dermatological complications of obesity including cellulitis and severe localized lymphoedema of the right leg. There were two large pedunculated masses on the right lateral thigh with early involvement of the left and overlying skin changes of chronic lymphoedema. Our patient's condition is clinically consistent with a new entity recently described in the surgical pathology literature as massive localized lymphoedema.

PMID: 17535201 [PubMed - indexed for MEDLINE]

http://www.blackwell-synergy.com/doi/abs/10.1111/j.1440-0960.2007.00349.x

Massive localized lymphedema: a reactive lesion simulating liposarcoma

Cesk Patol. 2002 Jul

Adamicová K, Vána J, Celec J, Haluska P. Ustav patologickej anatómie Martinskej fakultnej nemocnice a Jesseniovej lekárskej fakulty, Martin. adamicova@jfmed.uniba.sk

The authors present a case-history of massive localized lymphedema in a 54 year old female patient (height 167 cm, weight 113 kg). The history of the lymphedema lasted about 1 year. Its growth was not accompained with subjective complaints. It was diagnosed as a pendulous tumor of soft tissues in the thigh, 70 x 65 cm large. In preoperative diagnosis it was classified as a liposarcoma. The tumor lesion was removed and sent for bioptic examinations. Both histological and immunohistochemical biopsies denied benign or malignant nature of the soft tissue tumors and confirmed the diagnosis of a massive localized lymphedema.

PMID: 12325478 [PubMed - indexed for MEDLINE]

http://www.ncbi.nlm.nih.gov/sites/entrez?Db=pubmed&Cmd=ShowDetailView&TermToSearch=12325478&ordinalpos=8&itool=EntrezSystem2.PEntrez.Pubmed.Pubmed_ResultsPanel.Pubmed_RVDocSum

Massive localised lymphoedema: a clinicopathological study of 22 cases and review of the literature.

J Clin Pathol. 2009 Sep

Manduch M, Oliveira AM, Nascimento AG, Folpe AL. Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota 55905, USA.

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.

Full text version: Journal of Clinical Pathology

Massive Localized Lymphedema: Review of an Emerging Problem and Report of a Complex Case in the Mons Pubis.

May 2011

Brewer MB, Singh DP.

Source

From the Division of Plastic Surgery, Department of Surgery, University of Maryland Medical Center, Baltimore, MD.

Abstract

Massive localized lymphedema (MLL) is an emerging complication of the obesity epidemic. Caused by the obstruction of lymphatics, MLL presents as a giant swelling, with characteristic skin changes, and often lymphatic weeping. MLL has also been called “pseudosarcoma” because of its morphologic and pathologic similarity to sarcoma. Left untreated, MLL can degenerate into angiosarcoma. We present a case of MLL of the mons pubis in a 40-year-old man with a body mass index of 69. The literature is reviewed and an additional 40 cases of MLL are described. We found a female predominance of 1.6 to 1, an average weight of 421 lbs, and a 58% majority of cases in the thigh.

PubMed

A Patient with Massive Localized Lymphedema. Nov. 2011

Lymphedema People Resources

massive_localized_lymphedema.txt · Last modified: 2012/10/16 14:40 (external edit)