Aggressive angiomyxoma: A clinicopathological and immunohistochemical study of 11 cases with long-term follow-up

Leiden University, Leyden, South Holland, Netherlands
Archiv für Pathologische Anatomie und Physiologie und für Klinische Medicin (Impact Factor: 2.65). 03/2005; 446(2):157-63. DOI: 10.1007/s00428-004-1135-9
Source: PubMed


To report the clinicopathological and immunohistochemical features and longer term biological behaviour of aggressive angiomyxoma, an uncommon mesenchymal neoplasm occurring predominantly in the pelvi-perineal region of adults. Using immunohistochemistry, possible overexpression of CDK4 and MDM2 was analysed, which might point to (cyto)genetic alteration(s) in chromosome region 12q13-15, an area reported to be altered in this tumour entity.
Cases (n=11) of aggressive angiomyxoma were retrieved from the consultation files of the Comprehensive Cancer Centre of the Middle Netherlands (IKMN) panel for soft tissue tumours. Clinical and follow-up information were obtained, and immunohistochemical analysis was performed using antibodies directed against vimentin, cytokeratin AE1/AE3, desmin, alpha-smooth-muscle actin, CD34, S-100 protein, oestrogen receptors, CDK4 and MDM2. Five patients were female (age range 24-47 years; median 39 years), and six patients were male (age range 36-69 years; median 44.5 years). Of 11 cases, 10 arose in the pelvi-perineal area and 1 arose in the abdominal cavity in close relation to the bladder. Morphology was consistent with previous reports of this entity. Immunohistochemically, 8 of 11 cases were desmin positive (5 of 5 positive in females; 3 of 6 positive in males), 6 of 11 cases were positive for alpha-smooth-muscle actin, 5 of 11 cases were CD34 positive, 11 of 11 cases, irrespective of gender, were positive for oestrogen receptors and 3 of 11 cases were positive for cytokeratin AE1/AE3. Strong, diffuse nuclear positivity for CDK4 expression was present in all 6 cases tested, while only 1 of 11 cases tested for MDM2 showed weak focal positivity. Clinical follow-up in all cases (range 1-216 months; median 72 months) showed one local recurrence (9%) after 36 months. No metastases or tumour-related deaths were noted.
The sex distribution of cases reported in this study was roughly equal, in contrast to previous reports emphasising the predominance of this tumour in females. Our study confirms the local aggressive nature of aggressive angiomyxoma, although our local recurrence rate is lower than previous reports (9% versus 36-72%); no metastases and/or disease-related patient deaths were documented. All cases arising in females were positive for desmin, while three of the six cases arising in males were negative for desmin, supporting previous findings and indicating that the lesion may be somewhat different in males. The strong diffuse positivity for CDK4 in all six cases tested goes some way in implicating CDK4, either directly or indirectly, in tumourigenesis. The negative immunostaining for MDM2 would argue against functional amplification of this gene.

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Available from: Pancras C W Hogendoorn, Sep 08, 2015
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    • "The main theory for AA pathogenesis is that a primitive multipotent mesenchymal cell of the lower female genital tract that has the capability of differentiating by various ways gives rise to the lesion. The latter is mainly supported by immunohistochemical expression of desmin and, in some cases, a smooth muscle actin along with desmin by tumour cells [8]. "
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    ABSTRACT: Introduction. Aggressive angiomyxoma is a rare, slowly growing, and benign tumour of mesenchymal origin, which affects women of reproductive age and is associated with a high risk of local recurrence. Case Presentation. A case of a 47-year-old white female is presented herein, with a large polypoid, gelatinous mass on the right labia majora, measuring 26 × 21 × 6 cm. Histopathologically, the lesion was composed of spindle and stellate-shaped cells embedded in a myxoid matrix. Another specific feature was the presence of variable-sized thin-walled capillaries and thick-walled vascular channels. The patient underwent wide local excision of the tumour with clear margins and developed local recurrence 18 months later. Discussion. Aggressive angiomyxoma of the vulva needs to be distinguished from benign myxoid tumors with a low risk of local recurrence as well as from malignant myxoid neoplasms. Usually wide local excision with tumour-free margins and occasionally hormonal manipulation is the treatment of choice.
    Case Reports in Oncological Medicine 05/2015; 2015:1-4. DOI:10.1155/2015/292304
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    • "AAM, characterized by components including myxoid stroma and scattered spindle-shaped cells, is believed to be of fibroblast or myofibroblast origin by ultrastructural analysis. According to a recent immunohistochemical profile, the response of relevant tumor cells is positive for vimentin; variable for desmin,5 muscle-specific actin,1,5,6 and CD34;5 and negative for S-100 protein.5 "
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    ABSTRACT: Close examination of a 67-year-old Japanese man, who complained of persistent nocturia, revealed that a semitransparent polypoid tumor had developed from the bladder neck to the prostatic urethra obstructing the internal urethral meatus, which resulted in excessive urinary retention and post-renal dysfunction. The tumor was resected by a transurethral procedure and a pathological examination of specimens revealed aggressive angiomyxoma (AAM) of the prostate. AAM usually develops in the intrapelvic and perineal organs of females. So far as we know, this is the second case of primary prostatic AAM reported in the English literature, and is the first case where the patient encountered urethral obstruction.
    Rare tumors 03/2010; 2(1):e15. DOI:10.4081/rt.2010.e15
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