Concurrent giant tumoral pseudoangiomatous stromal hyperplasia necessitating bilateral mastectomy

Abstract

Pseudoangiomatous stromal hyperplasia (PASH) is an uncommon benign mesenchymal tumor of the breast. Majority occur as diffuse lesions, and diagnosis is often incidental or in a background of other breast pathologies. Bilaterality with multiple tumoral masses in giant breasts is a rarity. We report a 34-year-old nonlactating female with 2-year history of rapid progressive painless bilateral enlargement of the breasts following surgical excision of ill-defined breast lumps which were not subjected to histopathological evaluation a year earlier. Examination revealed bilateral nontender giant breasts extending to the umbilical area with masses which were not attached to overlying skin, Grade 2 pressure ulcers on the lateral posterior breast aspects bilaterally and peau d’orange. There were no other palpable masses or lymph nodes. A clinical assessment of bilateral gigantomastia was made. Bilateral mastectomy revealed giant PASH which was confirmed with positive immunohistochemical reactivity for CD34 and vimentin. No other breast pathologies were seen with extensive sectioning. Diffuse multiple breast lesions with incomplete excision are associated with rapid growth in PASH as seen in this case. The presence of concurrent bilateral giant tumoral masses without any underlying breast pathology is a novelty. The mainstay of treatment in this case is mastectomy despite its benign nature.

Treatment of Pseudoangiomatous Stromal Hyperplasia of the Breast

“…Then the patient was lost to follow-up until she was seen 10 years later with no disease progression and some breast postpartum involution, although nodules were still palpable. The appearance of the patient after 10 years reinforced the original treatment decision for this form of presentation of the disease, although some would have advocated for bilateral mastectomies and reconstruction 9, 12–18 . Pseudoangiomatous stromal hyperplasia has been associated with malignant disease.…”

“…The appearance of the patient after 10 years reinforced the original treatment decision for this form of presentation of the disease, although some would have advocated for bilateral mastectomies and reconstruction. 9,[12][13][14][15][16][17][18] Pseudoangiomatous stromal hyperplasia has been associated with malignant disease. However, almost all of the time, it is an incidental finding on pathologic examination of mastectomy or lumpectomy specimens.…”

Análise de receptores hormonais de estrógeno e progesterona em hipertrofia mamária versus normomastia, avaliação de presença e densidade com uma nova proposta de classificação das hipertrofias mamárias

“…: PASHhiperplasia pseudoangiomatosa Ex. : estromal hipertrofia virginal benigna 45,[47][48][49] Farmacológica Neothetazone, tamoxifeno 46 , ciclosporina, estrógeno, bucilamina, penicilamina 14 , prednisolona 50 Tumor benigno Lipoma, fibroadenoma 35 Cystosarcoma 47 Tumor phylloids 47 , miofibroblastoma 51 Tumor maligno Linfoma 37 A grande parte dos estudos nesta área é baseada em relatos de casos 14 , série de casos ou opiniões de especialistas (nível de evidência C e D) 55 . 57 .…”

“…Já em outro estudo, a apresentação clássica com nódulos 45 ocorre em 97% 74 a 97,6% 75 dos casos e as formas proliferativas difusas sem nódulos 69 foram 2,4% 75 a 3% 74 dos casos. Variando novamente a estatística, neste estudo, a apresentação pode ser focal em 34% 74 das pacientes ou difusa por toda mama 74 em 66% dos casos.…”

“…Entretanto, quando se almejam esclarecimentos sobre este assunto, os estudos publicados não são claros, em sua maioria, com pouca relevância por serem estudos com padrão de evidência fracos, e baixo "N". Classificações históricas possíveis são idiopática 23 , associada à obesidade 14 , gestacional 22,23,25 , distúrbio hormonal 14,34 , tumores benignos 35,36 , tumores malignos 37 , juvenil [38][39][40] , hiperplasia pseudoangiomatosa estromal (Pseudoangiomatous Stromal Hyperplasia -PASH) [41][42][43][44][45] e farmacológica 14,46 .…”