Table of Contents

2015 Month : January Volume : 4 Issue : 2 Page : 303-307

AGRRESSIVE ANGIOMYXOMA: A RARE SOFT TISSUE TUMOR

Ritu Sharma1, Sunny Goyal2, Apoorva Sarawat3, H. B. Saxena4, Nikhil Bansal5

1. Resident, Department of Pathology, Mahatma Gandhi Medical College and Hospital, Sitapura, Jaipur.
2. Resident, Department of Radiodiagnosis, Mahatma Gandhi Medical College and Hospital, Sitapura, Jaipur.
3. Resident, Department of Pathology, Mahatma Gandhi Medical College and Hospital, Sitapura, Jaipur.
4. Professor, Department of Pathology, Mahatma Gandhi Medical College and Hospital, Sitapura, Jaipur.
5. Resident, Department of Radiodiagnosis, Mahatma Gandhi Medical College and Hospital, Sitapura, Jaipur

CORRESPONDING AUTHOR:
Dr. Ritu Sharma,
Department of Pathology,
MGMCH, Jaipur.
E-mail: drritzsharma@gmail.com

ABSTRACT: Introduction-Aggerssive angiomyxoma is a rare soft tissue tumor, typically occuring in the female pelvis and carrying a high risk of local infiltration and relapse. Surgery remains the first line of treatment.1 Material and Methods- We describe a case of perineal mass in a 20-year old woman who underwent surgical excision of the tumor. Diagnosis was made on the histopathological examination and positive immunohistochemical staining with estrogen and progesterone receptors in Pathology department of Mahatma Gandhi Medical College & Hospital, Jaipur. Discussion: Almost exclusively, AAM involves women of childbearing age; although very rare cases have been diagnosed in perimenopausal women.1 It usually exhibit a slow, insidious growth pattern, a capacity for local infiltration and a marked tendency to repeated local recurrence. Preoperative diagnosis of AAM is often difficult because of its rarity. Misdiagnosis is common, occurring in > 80% of cases.2 Histopath-ologically, differentiation may be difficult between the following: Liposarcoma, myxoma; myxoid neurofibroma.3 The methods to establish a final diagnosis is pathological analysis and immunohistochemical stain.Background: Aggressive anigomyxoma is rare mesenchymal tumor that affects women in more than 90% of cases.4 It is most commonly arises in the vulvovaginal region, region. Other sites in womens are perineum, pelvis, buttock and retroperitoneum.1 The term aggressive emphasizes the often infiltrative nature of the tumor and its frequent association with local recurrence.5

 

How to cite this article

Ritu Sharma, Sunny Goyal, Apoorva Sarawat, H. B. Saxena, Nikhil Bansal5 Aggressive Angiomyxoma: A Rare Soft Tissue Tumor. Journal of Evolution of Medical and Dental Sciences 2015; Vol. 4, Issue 02, January 05; Page: 303-307, DOI: 10.14260/jemds/2015/47

CASE REPORT: A 25 year old woman, presented with a complain of swelling in perineal region since 8-9 months.

CLINICAL PRESENTATION: Patient presents with pelvic fullness and with a visible perineal or vulval mass that is discovered during routine pelvic examination or with radiographic imaging. No significant past history present.

ON EXAMINATION: General examination only pallor present. Systemic examination: Large mass measuring 22x10x10 cm is present in perineal region. Biochemical examination: Normal.

RADIOLOGIC FEATURES: Well-defined, homogeneous mass that is hypodense relative to muscle, or it may be predominantly cystic with solid components. Characteristic appearances. On CECT include hypointensity on T1- weighted images and hyperintensity on T2-weighted images.

MRI OF PELVIS (PLAIN AND CONTRAST): Shows 20 x10 x 9.2cm sized (CCx AP x Tr), large, well marginated, lobulated, heterogeneously enhancing mass lesion with central non-enhancing / necrotic area seen in per- sacral region / pelvis with extent as described above. Findings S/o Neoplastic mass lesion.

 

 

(T1 and T2- weighted axian view of a MRI scan) MRI of Pelvis


    

MRI of Pelvis sagittal view T1 & T2


PATHOLOGIC FEATURES: GROSS FEATURES –Formalin fixed specimen consist Grey-white to Grey-brown, non-encapsulated, oval to rounded mass measures 20 x 9 x 9cm in size, soft to rubbery in consistency with a glistening, pink or reddish-tan external surface. Cut surface is gelatinous, homogenous gray-white with focal areas of hemorrhage seen.

 

FIGURE SHOWING: Glistening, pink or reddish-tan external surface


MICROSCOPIC FEATURES: H and E section studied shows sparsely cellular tumor composed of pale to eosinophilic stroma studded with numerous haphazardly arranged blood vessels that stand out against the myxoid background and range in size from thin walled capillaries and venules to large muscular arteries. Paucicellular neoplasm with large areas of myxoid stroma with no necrosis is seen and very scanty mitotic activity. Overall histomorphology suggestive of: AGRRESSIVE ANGIOMYXOMA.