Aggressive Angiomyxoma with Lymphangitis a Rare Entity - Case Report.

Sir,

Aggressive angiomyxoma is a rare but locally invasive mesenchymal tumor occurring usually in women of reproductive age. It is a slow-growing vulvovaginal mesenchymal neoplasm with a marked tendency for local recurrence, but with a low tendency to metastasize. It was first described by Steeper and Rosai in 1983.[1] It presents as a vulval polyp clinically and is diagnosed on histology. Estrogen and progesterone receptors are commonly found in AAM.[2] It is thus likely to grow during pregnancy and respond to hormonal manipulation. It involves mainly the pelvis, vulva, perineum, vagina and urinary bladder in adult women in the reproductive age. Incomplete excision is common because of the infiltrating nature of the neoplasm and absence of a definite capsule. Non-surgical interventions like hormonal manipulation, radiotherapy, arterial embolization have been tried with variable success rates.[3]

A 28-year-old female with asymptomatic slow growing mass over pubic area since 8 years which increased in size after her pregnancy 6 years back and with unilateral swelling over limb since childhood. Local examination showed multiple skin colored to hyperpigmented polypoidal to pedunculated growth with smooth surface [Figure 1] and spongy and soft in consistency and non-tender. Her inguinal and axillary lymphnodes were not enlarged. Unilateral swelling over left foot extending until knee non-pitting in nature was observed. Viral markers were negative. Unilateral swelling over left foot extending up to knee was pitting in nature. Later punch biopsy with excisional biopsy was performed from one of the pedunculated mass, which showed findings suggestive with fibrous growth. We referred her to surgeon who performed wide local excision of the mass, which was en mass again sent for histopathology, which confirmed the diagnosis of aggressive angiomyxoma.

Figure 1

(1) Shows multiple skin colored to hyperpigmented polypoidal to pedunculated growth over pubis and lower abdominal wall. (2) Postoperative (day 20) surgical wound in healing phase

Aggressive angiomyxoma (AAM) is a rare mesenchymal neoplasm arising primarily in soft tissue of pelvis and perineum of adults. It occurs predominantly in women of reproductive age, with a peak incidence in the fourth decade of life and an age range of 11 to 77 years.[4] AAM is often mistaken for more-common, superficial lesions, such as vaginal or labial cysts and lipomas, and the frequently large, bulky size and extent of the deep tissue involvement is not appreciated until radiographic imaging and surgical resection are performed. These tumors often have a smooth surface, partially or completely encapsulated with finger-like projection on adjacent soft tissue and cut surface gives a glistening, gelatinous appearance, bluish grey in color, with areas of hemorrhage and congestion.[3] In our case macroscopically external surface showed presence of multiple pedunculated growth, grey brown in color [Figure 1] and was soft and spongy in consistency with unilateral left foot non-pitting edema [Figure 2] since childhood. Therapeutic surgical excision was performed and sent for histopathology. Histological examination on H and E stained section showed presence of small spindle and stellate-shaped cells in a myxoid background. The tumor cells showed pink eosinophilic cytoplasm [Figure 3]. There was no nuclear pleomorphism and mitosis. There was also presence of numerous prominent dilated thick walled vessels (H and E, x100) [Figure 4]. Immunohistochemical staining of the tumor reveals high positivity for desmin, vimentin, ER, and PR receptor and negative for S-100 protein.[5] We also performed immunohistochemistry (IHC) for confirming the diagnosis. IHC stained section showed presence of focal Smooth Muscle Antigen (SMA) positivity (H and E, x400) [Figure 5]. Since our hospital lacks facility for ER, PR receptor staining we couldn’t test for the same. Surgical resection is the main treatment modality, but local recurrence rates are high. The reported recurrence rate varies from 35% to 72%, even with clear surgical margins.[4]

Figure 2

Shows unilateral non-pitting edema over left foot extending up to knee

Figure 3

Tumor mass composed of small spindle and stellate-shaped cells in a myxoid matrix with presence of numerous dilated thick walled vessels (H and E, x40)

Figure 4

Tumor mass composed of small spindle and stellate-shaped cells in a myxoid matrix (pointed arrows) on left side and presence of numerous dilated thick walled vessels on right side (H and E, x100)

Figure 5

IHC showing focal positivity for SMA (IHC x400)

Radiation therapy and chemotherapy are less-suitable options due to low mitotic activity. Hormonal manipulation with tamoxifen, raloxifene and gonadotropin-releasing hormone analogues reduce the tumor size and help to make complete excision feasible in large tumors. Angiographic embolization shrinks tumor and makes it easier for excision.[6] In our case, AAM with lymphangitis was found. Even after extensive literature search, relation between AAM and lymphangitis has been found to be rare, which is found in our case. Further study is required to find out any association between AAM and lymphangitis. Also post-treatment examination for remission of lymphangitis couldn’t be done as there was lost to follow-up. Hence AAM being rarely reported entity extensive reporting of AAM with associated abnormality is required.

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Conflicts of interest

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