Massive Vulval Edema Secondary to Obesity and Immobilization: A Potential Mimic of Aggressive Angiomyxoma

Abstract

We report 2 cases of surgically resected vulval masses in women aged 27 and 40 years. One patient was wheelchair bound and the other was obese, both presented with bilateral vulvar swelling. One specimen measured 45 cm in maximum dimension and the other 5 cm and were described as grossly edematous or gelatinous. Histologically, in both cases, there was edema of the skin overlying the lesion. The lesion itself consisted of markedly edematous connective tissue with widely separated bland spindle-shaped cells and numerous dilated vascular channels, sometimes surrounded by cuffs of lymphocytes and plasma cells. In the larger of the 2 specimens, underlying edematous adipose tissue was present. To some extent, the appearances, especially the morphological features, mimicked aggressive angiomyxoma because of the presence of a mass, the lack of circumscription, the hypocellular, edematous appearance, and the presence of numerous vascular channels. However, a combination of clinical and pathological features, including bilateralism, lack of a true myxoid stroma, the presence of perivascular cuffs of lymphoid cells, and lack of staining with estrogen receptor, is against aggressive angiomyxoma. The appearances were interpreted as those of massive edema. In one case, there was recurrence of the mass after surgery. There has been a single previous report of a similar vulvar case in a quadriplegic female patient and of similar cases involving the upper and lower extremities of obese patients. Clinicians and pathologists should be aware of the existence of this lesion, which is likely due to lymphatic obstruction and lymphedema secondary to immobilization and obesity.

Massive localized lymphedema: a clinicopathologic study of 46 patients with an enrichment for multiplicity

“…The other differential diagnoses can include desmoid-type fibromatosis, lipomatosis, angiomatosis, lymphangiomatosis, angiosarcoma, angiomyoma, lipoma, and lipomatosis. 1,2,8 However, careful attention to the clinical history and prototypic histomorphology will facilitate prompt recognition and avoid major mishaps.…”

“…Massive localized lymphedema displays a predilection for the inner thigh, but other sites have been reported, including the lower abdominal wall, suprapubic region, mons pubis, vulva, inguinoscrotal, penis, popliteal fossa, lower leg, and arm. [1][2][3][4][5][6][7][8][9] The etiology and pathogenesis of massive localized lymphedema is unclear but might be multifactorial. In the original paper of Farshid and Weiss, lymphatic obstruction due to massive adipose tissue was proposed as the main etiological factor.…”

Vulvar Lymphoedematous Pseudotumours Mistaken for Aggressive Angiomyxoma: Report of Two Cases

“…This feature as a potential problem for misdiagnosis of aggressive angiomyxoma has been dis- cussed in 2 previous reports that presented cases with similar findings. In all of these cases, the first hypothesis of vulvar soft tissue tumour was supported by the clinical impression of a neoplastic mass, particularly in our case, due to the presence of a unilateral lesion and grossly due to the gelatinous cut surface with poorly circumscribed margins (see table 2 ) [4,5] . Histologically, the massive oedema, the lack of circumscription and the presence of thin-walled vascular channels are other potential histological pitfalls for the differential diagnosis.…”

“…Vang et al and McCluggage et al have proposed that regional trauma resulting in lymphatic obstruction is a possible aetiology. Immobility and marked obesity have also been shown to be predisposing factors [4,5] . These possibilities were not confirmed in our cases because the patients had no history of regional trauma, previous surgery in the anogenital regional and skin diseases, and they were not disabled patients; however, the lesions presented here were unilateral and localised exclusively on the right labia.…”

Reactive Fibroblastic and Myofibroblastic Proliferation of the Vulva (Cyclist's Nodule)

“…6 Another pseudoneoplastic lesion that rarely occurs at this site is massive vulval edema. 2,12,20 Predisposing factors to this uncommon condition include marked obesity, immobilization, paraplegia, and earlier surgery. 2,12,20 Massive vulval edema is characterized histologically by poor circumscription, edematous connective tissue and adipose tissue, dilated vascular channels, and a perivascular inflammatory infiltrate; because of marked edema, which may suggest myxoid change, the histologic features can mimic aggressive angiomyxoma.…”