Aggressive Fibromatosis: Government Royapettah Hospital Experience

INTRODUCTION: Aggressive Fibromatosis also known as Desmoid tumors is a monoclonal disorder affecting the musculoaponeurotic tissues. It is relatively a rare neoplasm with a frequency of about < 3% of all soft tissue tumors and annual incidence of about 0.2 to 0.5 per 100000 population. Exact etiology of aggressive fibromatosis is currently unknown. It is probably multifactorial with hormonal, genetic and trauma playing their parts. They are locally infiltrative and never metastasize but have a tendency for multiple recurrences. The morbidity caused by this lesion is due to local destruction of tissues and occasional death has been reported. Aggressive Fibromatosis are heterogenous group of tumors which share similar clinical, histological and molecular genetic feature. But each type has some subtle and unique feature which distinguishes it from others. Due to the relative rarity of this tumor and their enigmatic clinical behavior, treatment for fibromatosis has not been optimized. In this article we will be discussing about our experience with deep fibromatosis. AIM OF THE STUDY: 1. To study the epidemiological characteristics of the disease in India. 2. To analyze the surgical data and present the outcome. 3. Describe the pattern of recurrence and salvage modality for recurrence. 4. To find out the optimal management strategy for this rare disease. MATERIALS AND METHODS: A retrospective analysis of our data over a period of 13 yrs (between 1998 and 2010) was done. There were 33 patients with a diagnosis of deep fibromatosis in our records which included abdominal, intra-abdominal and extra abdominal. In 28 patients a wide excision of the lesion was performed with curative intent. Adjuvant radiotherapy was given for 4 patients and systemic therapy in the form of tamoxifen was given for 4 patients. Information regarding epidemiological characteristics of the disease, surgical procedure performed postoperative margin status on histopathological examination, adjuvant treatment given, recurrences in the follow-up period, pattern of recurrence and salvage modality for recurrence were collected for analysis. OBSERVATION AND ANALYSIS: The disease and its natural course to some extent were described 170 years ago. Although numerous researchers have dedicated their time to acquire more knowledge about this disease, every aspect of this disease beginning form etiology to management is full of controversies. The enigma of fibromatosis will continue to haunt the researchers for some more time as new findings (like ERβ) lead to more questions than answering the older ones. In this analysis we will concentrate on the Indian perspective about the disease and explore the lacunae in the literature. CONCLUSION: Demographic trend in India might be different from what is reported in the western literature with more incidences in male sex and pediatric age group. Recurrences after surgery alone can be quite high (40% in our series). Positive margin & recurrent tumors were found to be adverse prognostic factor for recurrence in our series. Even in negative margin the recurrence rate was high this we believe is due to multicentricity of the lesion. Addition of radiation provides good local control but should be used with diligence as most of the recurrences are surgically salvageable and these tumors occur in young patients with long life expectancy. The role of systemic therapy including Tamoxifen needs further evaluation. Aggressive fibromatosis is an enigma occupying the twilight zone between benign and malignant behavior. There are considerable lacunae in our knowledge regarding the natural course of the disease. Due to the rarity and slow growing nature of the disease there had not been many studies with adequate numbers and follow-up to optimize treatment protocol for this tumor. There had never been a randomized control study comparing different treatments undertaken anywhere in the world. Multi institutional prospective randomized control trials may optimize the treatment protocol for this rare and enigmatic disease