Childhood asymmetry labium majus enlargement (CALME) is a disctinctive clinicopathologic entity of pre- and early puberty first described in 2005. It is defined as an expansion of normal soft tissues of the vulva. Although CALME is not a rare lesion, it has been called lipoma, fibroma, hamartoma, and fibrous hyperplasia. CALME is not a true neoplasm and is a physiologic growth in response to hormone. It may tend to resolve spontaneously and recur after surgical resection. We report four cases of CALME with a review of the literature. To the best of the knowledge, this is the first Korean report.
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Keep CALME (childhood asymmetry labium majus enlargement) and follow up Andrea Sechi, Annalisa Patrizi, Giulio Vara, Rita Golfieri, Iria Neri JDDG: Journal der Deutschen Dermatologischen Gesellschaft.2021; 19(9): 1276. CrossRef
Bei CALME (childhood asymmetry labium majus enlargement): die Ruhe bewahren und nachverfolgen Andrea Sechi, Annalisa Patrizi, Giulio Vara, Rita Golfieri, Iria Neri JDDG: Journal der Deutschen Dermatologischen Gesellschaft.2021; 19(9): 1276. CrossRef
Childhood Asymmetry Labium Majus Enlargement (CALME): Description of Two Cases Cristina Salvatori, Ilaria Testa, Marco Prestipino, Maria Elena Laurenti, Sara Riccioni, Giuseppe Di Cara, Nicola Principi, Susanna Esposito, Mirko Bertozzi International Journal of Environmental Research and Public Health.2018; 15(7): 1525. CrossRef
Childhood asymmetrical labium majus enlargement sonographic and MR imaging appearances Ami Gokli, Jeremy Neuman, Ruby Lukse, June Koshy, Fanyi Kong, Tal Laor Pediatric Radiology.2016; 46(5): 674. CrossRef
Angiomyofibroblastoma of the vulva is a rare mesenchymal tumor. It has been diagnostically confused with aggressive angiomyxoma which has a somewhat different clinical course and therapy. Herein we report a case of angiomyofibroblastoma of the vulva in a 46-year-old female.
Microscopically, there were alternating hypercellular and hypocelluar edematous zones in which abundant capillary blood vessels were irregularly distributed. Spindle, plump spindle, and oval stromal cells were concentrated around the blood vessels, or loosely dispersed in the hypocellular area. Immunohistochemically, the stromal cells were positive for desmin, vimentin, muscle-specific actin and weakly positive for S-100 protein. Ultrastructural studies showed well developed rough endoplasmic reticulum, abundant intermediate filaments, and pinocytic vesicles in the stromal cells.
Angiomyofibroblastoma is a distinctive, hitherto uncharacterized, benign soft tissue tumor of the vulva with histology similar to an aggressive pelvic angiomyxoma. It can be distinguished from an aggressive angiomyxoma by its circumscribed borders, higher cellularity, more numerous blood vessels, the frequent presence of plump stromal cells, minimal stromal mucin, and rarity of erythrocyte extravasation. We experienced a case of angiomyofibroblastoma of the vulva occurring in a 45-year-old woman. The lesion was a well-defined but not encapsulated mass, 4.5x4.2 cm. Histologically the mass was characterized by alternating hypercellular and hypocellular edematous zones in which abundant blood vessels were irregularly distributed. Immunohistochemically, the spindled, plump spindled, and oval stromal cells were reactive for vimentin and desmin, but not for cytokeratin, or S-100 protein.
Angiomyofibroblastoma is a rare, benign mesenchymal tumor of the vulva. Since it was described in 1992 by Fletcher, 15 cases have been reported in literature. We recently experienced a recurred angiomyofibroblastoma of the vulva.
A 45-year-old woman was presented initially in 1991 with a mass of labium major and local excision of tumor mass had been performed. A histologic diagnosis was made of angiomyxoma, but this diagnosis was revised to angiomyofibroblastoma by the authors. The recurred mass was well circumscribed, measuring 2.5x1.6x1.5cm in dimensions.
Microscopically the tumor was characterized by high cellularity, numerous blood vessels(which lack prominent hyalinization), and plump stromal cells.
Immunohistochemically, the stromal cells were reactive for vimentin and desmin, but not alpha-smooth muscle actin, or S-100 protein. We thought that this case was a recurred angiomyofibrblastoma of the vulva due to incomplete surgical excision.
Cellular angiofibroma is a recently described, distinctive soft tissue tumor of the vulvovaginal region which is characterized by small, well-circumscribed tumors with fibroblastic differentiation. We report three cases of cellular angiofibroma of the vulva in middle-aged women. All three patients presented with painless swelling in the labium majora. The age of the three patients ranged from 43 to 56 years old (mean: 48 years old) and the size of the tumor ranged from 2 to 5 cm. The microscopic appearance was characterized by a cellular, well-circumscribed mass composed of uniform, bland, spindle stromal cells, numerous thick-walled, hyalinized vessels, and a scarce component of mature adipocytes. Immunohistochemical stains of the tumor cells show positivity for vimentin but negativity for smooth muscle actin, S-100 protein, desmin, factor VIII-related antigen and epithelial membrane antigen. The tumor should be differentiated from aggressive angiomyxoma and angiomyofibroblastoma because of its different clinicopathologic features, cells of origin and immunohistochemical findings.
A vestibular adenoma arising in mucin-secreting glands of the vulva is described. Grossly, the adenoma was a firm, well-demarcated solid mass with mucoid cut surface, measuring 3.5x3x3 cm, which was associated with an adjacent Bartholin's duct cyst. Microscopically, the solid mass was composed of proliferated mucous acini separted by fibromuscular septa and ducts lined by mucin-secreting columnar epithelium, transitional epithelium or metaplastic squamous epithelium. To our knowledge, less than 20 cases of such cases have been reported in the English literatures.
However, whether the nature of proliferation is neoplastic or non-tumorous is still unclear.
In 1947, Liver described adenoid squamous cell carcinoma under the title of "adenoacanthoma of sweat gland". This tumor is not rare, but reported cases in literatures were relatively few. The tumor were frequently found in the sun-exposed skin; such as face, ear, neck etc.. The cases involving non-sun-exposed area, such as oral and labial mucosa, nosopharynx and vulva etc., were also reported.
Therefore, the relation between the pathogenesis of adenoid squamous cell carcinoma and sun damage has been still debated. Immunohistochemical studies have recently suggested that this tumor is squamous cell carcinoma undergoing acantholysis. The authors experienced a case of adenoid squamous cell carcinoma of the vulva in a seventy-one year old female patient. She had suffered from itching sensation on vulva for 10 years, and palpable mass on vulva for 1 month. On gross examination, a pale gray pigmentation and erosion, was noted at the right labia major, the minor extending left labia major and minor. On microscopic examination, nests of atypical epithelial cells extending into the cornium showed an adenoid architecture. These were composed of a peripheral layers of cuboidal cell in a lacy pattern. And the glandular spaces contained dyskeratotic acantholytic tumor cells in central portion. Therefore we diagnosed this case as adenoid squamous cell carcinoma of the vulva.