Pleomorphic hyalinizing angiectatic tumor of the vulva: literature review based on a rare presentation

Pleomorphic hyalinizing angiectatic tumor (PHAT) of soft tissues is a rare, non-metastatic tumor of unknown etiology and uncertain behavior, which may recur locally. There are few reports on this condition, and due to the rarity of the disease, its lineage has not yet been fully elucidated. The present study aims to report the case of an unusual entity observed for the first time in vulval topography. A female patient, 83 years old, presented with a tumor in the vulvar region that had evolved for approximately 4 months. Magnetic resonance imaging showed an expansive perineal formation of 8.5 × 3.5 cm, and a hemivulvectomy with a flap rotation was performed. The review of the slides revealed a mesenchymal lesion without significant atypia, which was richly vascularized. In the areas of interest, the immunohistochemical (IHC) study demonstrated positivity for CD34, estrogen, and progesterone receptors; it was negative for the other tested markers. Morphological findings associated with the IHC staining panel supported the diagnosis of PHAT. The main morphological features of PHAT are clusters of ectatic vessels of different sizes that show deposits of subendothelial and intraluminal fibrin. Fusiform and pleomorphic cells randomly arranged in leaves or long fascicles intermingle these vessels. It is essential to recognize this entity and consider it among the differential diagnoses of a mesenchymal lesion, given the wide variety of entities that comprise this group of lesions.

Giant lactating adenoma

Lactating adenoma is a rare benign breast lesion that most often presents as a small (up to 3 cm), solid, well-circumscribed, solitary, painless, mobile, lobulated mass. The highest incidence occurs in primiparous women (20 to 40 years old) during the third trimester of pregnancy. However, in the rare case presented herein, in addition to its giant size (more than 10 centimeters on palpation), this lactating adenoma is distinctive due to the presence of multiple nodules, poorly defined ultrasonographic margins, worrisome radiologic features, growth since early pregnancy, presence of infarction and association with chronic mastitis. From the clinical-radiologic perspective, the differential diagnoses included abscess associated with puerperal mastitis, phyllodes tumor, and galactocele. Biopsy was performed, and pathologic examination revealed the classic characteristics of lactating adenoma with multiple infarcted areas, leading to an unexpected confirmed case of giant lactating adenoma.

Pulmonary artery aneurysm rupture

Pulmonary artery aneurysm is a disorder of varying etiology and should be diagnosed early for appropriate interventions. A 45-year-old man was hospitalized for chest pain, dyspnea, cough, chills, diarrhea, and vomiting, which had started 3 weeks before admission. Physical examination indicated a reduced vesicular murmur in the right hemithorax. A chest x-ray performed indicated a pneumothorax and pulmonary abscess in the right hemithorax. Thoracostomy released abundant purulent and fetid fluid. Direct examination of the pleural fluid using saline revealed structures similar to Trichomonas. Non-contrast chest computed tomography revealed right pneumothorax along with an irregular cavitation located at the pleuropulmonary interface of the posterior margin of the right lower lobe. A pleurostomy was performed. On the second postoperative day, the patient suffered a sudden major hemorrhage through the surgical wound and died on the way to the operating room. The autopsy revealed an abscess and ruptured aneurysm of the lower lobar artery in the lower right lung. Microscopic examination revealed extensive liquefactive necrosis associated with purulent inflammation and the presence of filamentous fungi and spores. This case can be characterized as a severe disorder that requires early diagnosis to achieve a good therapeutic response and to avoid fatal outcomes.

Síndrome de Fournier: 10 anos de avaliação / Fournier´s syndrome: a 10-year evaluation study", "_e": "n

INTRODUÇÃO: A gangrena de Fournier é uma fasciite necrosante sinérgica do períneo e parede abdominal, que tem origem no escroto e pênis, no homem, e vulva e virilha, na mulher. O processo inicialmente foi descrito como idiopático, mas atualmente sabe-se que se trata de grave afecção causada por bactérias Gram positivas, Gram negativas ou anaeróbios, que pode levar a comprometimento sistêmico importante e, eventualmente, morte. MÉTODO: Foi realizado estudo retrospectivo, baseado na análise de prontuários médicos de 23 pacientes portadores de síndrome de Fournier, no período de janeiro de 2002 a janeiro de 2012. O tratamento dos pacientes incluiu intervenção precoce, com antibioticoterapia de largo espectro e desbridamentos consecutivos. O procedimento terapêutico empregado para a reconstrução variou desde a aproximação das bordas com sutura simples até uso de retalhos e enxertos, nas lesões extensas. RESULTADOS: As técnicas de reparação cutânea foram eficientes e a reparação escrotal foi também efetiva em todos os casos, obtendo-se bons resultados estéticos. Houve 3 (13%) óbitos, 2 deles em pacientes com doenças pregressas e portadores de comorbidades. CONCLUSÕES: Apesar da reconhecida gravidade da Síndrome de Fournier, as medidas terapêuticas adotadas, como rápida intervenção, desbridamento precoce e antibioticoterapia de amplo espectro, juntamente com abordagem multidisciplinar, demonstraram-se bastante eficazes no controle da doença, permitindo reconstrução cirúrgica das áreas atingidas, com baixa mortalidade.

BACKGROUND: Fournier's gangrene is a synergistic necrotizing fasciitis of the perineum and abdominal wall that develops in the scrotum and penis in men and the vulva and groin in women. This disease was initially believed to be idiopathic; however, recent studies have indicated that it is a serious condition caused by gram-positive, gram-negative, or anaerobic bacteria. It is often associated with severe and fatal systemic involvement. METHODS: A retrospective study involving a chart review of 23 patients with Fournier's syndrome treated between January 2002 and January 2012 was conducted. Prompt treatment consisted of broad-spectrum antibiotic administration and serial debridement. The reconstruction techniques ranged from edge approximation via simple sutures to the use of flaps and grafts in cases with extensive lesions. RESULTS: The skin and scrotal reconstruction techniques were effective in all cases, yielding satisfactory aesthetic results. Three (13%) patients died, including 2 patients who had previous illnesses and comorbidities. CONCLUSIONS: Although Fournier's syndrome is a serious disease, therapeutic measures such as prompt intervention, with early debridement and broad-spectrum antibiotic therapy, in a multidisciplinary approach is very effective for controlling the disease, enabling surgical reconstruction of the affected areas with a low mortality rate.