Abstract YO29
Case summary
Desmoid fibromatoses are rare, benign neoplasms of myofibroblast origin that lack metastatic potential but can be locally invasive. Diagnosis and management are challenging as these are based on a multitude of factors such as symptomatology, tumor location, risk of recurrence, and the possibility of treatment-induced morbidity. A healthy 48-year-old Filipino woman presents with an 2x3 cm incidental jejunal mesenteric mass found intraoperatively during the excision of her choledochal cyst. Histopathology of the core-needle biopsy revealed desmoid fibromatosis. An initial watchful waiting approach was employed since the patient was asymptomatic and resection would cause significant bowel loss. After three months, her abdominal CT scan shows that the mass had progressed, to 4.7 x 4.5 x 4.7cm. Anti-hormonal therapy with high-dose Tamoxifen (120mg daily) was initiated due to its limited toxicity and low cost. The patient is currently asymptomatic, on her sixth month of treatment. The tumor remained stable at 4.5 x 4.6 x 4.7cm, on repeat abdominal imaging. Desmoid fibromatosis represents a benign histology with a malignant and unpredictable course. It warrants a multidisciplinary team approach upfront. Watchful waiting is an appropriate first line option, especially in asymptomatic tumors and those found in non-critical locations. In case of progression, management should be site specific and via a stepwise approach. Therapeutic toxicities, such as significant intestinal loss upon resection, should be carefully weighed before choosing treatment. Preferred management for patients with intra-abdominal desmoid tumors that progress after observation has shifted towards non-surgical approaches or systemic therapies, including anti-estrogens, NSAIDs, tyrosine-kinase inhibitors (sorafenib, pazopanib and imatinib) and chemotherapy (methotrexate and vinblastine, and vinorelbine). A stepwise escalation of therapy from less to more toxic agents are a reasonable approach. It is also possible to discontinue treatment and resume watchful waiting for patients not progressing for two years.
Clinical trial identification
Editorial acknowledgement
Resources from the same session
YO10 - BRCA2 Mutated Recurrent Adult Granulosa Cell Tumor Treated with PARP Inhibitor
Presenter: Debapriya Mondal
Session: Poster viewing 06
YO11 - Satisfactory response after Rituximab-Bendamustin in Poor Performance Status Geriatric with Relapsed DLBCL
Presenter: Yasjudan Putra
Session: Poster viewing 06
YO12 - Allogeneic hematopoietic stem cell transplant in a case of acute myeloid leukaemia with end stage renal disease
Presenter: DEVYANI SURANGE
Session: Poster viewing 06
YO13 - Rosai-Dorfman Disease Presenting with Multiple Contiguous Lymphadenopathy: A Case Report
Presenter: Lean Marx Maaño
Session: Poster viewing 06
YO15 - Nasopharyngeal carcinoma in pregnancy: A Case Report
Presenter: Mary Antonette Ong
Session: Poster viewing 06
YO16 - New onset SIADH masquerading progression in esthesioneuroblastoma
Presenter: Muthu Manikandan
Session: Poster viewing 06
YO17 - Olfactory Neuroblastoma with Cervical Lymph Node Metastases in a 21- Year old Filipino Woman: A Case Report
Presenter: Mary Antonette Ong
Session: Poster viewing 06
YO18 - Challenges in Provision of Patient centered care with a Practical approach in a Resource-limited setting - Steven Johnson Syndrome – Toxic Epidermal Necrolysis (SJS-TEN) overlap related to Phenytoin use and review of logistical challenges related to care provision - a Case Report.
Presenter: Rahul D. Arora
Session: Poster viewing 06
YO19 - Posterior Reversible Encephalopathy Syndrome associated with Pharmacological management of Bowel obstruction at the end of life – a review of two cases separated by 5 years
Presenter: Rahul D. Arora
Session: Poster viewing 06
YO20 - A case of drug-induced large vessel vasculitis with hoarseness as the chief complaint caused by pegfilgrastim during chemotherapy
Presenter: Takuya Sato
Session: Poster viewing 06