A giant desmoid-type fibromatosis of the abdominal cavity

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Desmoid-type fibromatosis (DTF) is a rare, soft-tissue tumour. These tumours do not metastasize, but their local aggressive tumour growth and unpredictable behaviour can have a significant impact on health-related quality of life (HRQoL). Little is known about which DTF patients are particularly affected by an impaired HRQoL. The objectives of this study were to assess HRQoL among different groups of DTF patients and to investigate which socio-demographic and clinical characteristics were associated with DTF-specific HRQoL. A cross-sectional study was conducted among DTF patients from the United Kingdom and the Netherlands. HRQoL was assessed using the European Organization for Research and Treatment of Cancer Quality of Life Questionnaire Core 30 (EORTC QLQ-C30), accompanied by the DTF-QoL to assess DTF-specific HRQoL. The scores were compared amongst subgroups, based on the socio-demographic and clinical characteristics of DTF patients. Multiple linear regression analyses with a backward elimination were conducted to identify the factors associated with DTF-specific HRQoL. A total of 235 DTF patients completed the questionnaires. Female patients, patients with more than two comorbidities, or patients who received treatment other than only active surveillance (AS) or surgery scored significantly worse on the subscales of both the EORTC QLQ-C30 and DTF-QoL. Patients that were ≥ 40 years scored significantly worse on the physical functioning scale of the EORTC QLQ-C30, while younger patients (18-39 years) scored significantly worse on several DTF-QoL subscales. Differences in the DTF-QoL subscales were found for tumour location, time since diagnosis and the presence of recurrent disease. Furthermore, treatments other than AS or surgery only, female sex, younger age and the presence of comorbidities were most frequently associated with worse scores on the DTF-QoL subscales. This study showed that (DTF-specific) HRQoL differs between groups of DTF patients. Awareness of these HRQoL differences could help to provide better, personalised care that is tailored to the needs of a specific subgroup.

Desmoid-type fibromatosis (DTF) is a rare locally aggressive soft tissue neoplasm which occurs in children and adults, with a peak incidence in young adults. For the majority of the patients DTF is a chronic and symptomatic disease which affects health-related quality of life. Systemic treatment regimens tend to differ for patients treated by pediatric oncologists compared to medical oncologists. This systematic review identified 14 clinical trials in children and adults with DTF. Tumor response and progression-free survival rates varied widely between studies and study populations. Treatment choices for patients with DTF are based on a paucity of (randomized) trials. Treatment principles of DTF are similar in pediatric and adult oncology, but the treatment itself is different. This seems mostly driven by a lack of TKI accessibility in pediatric oncology. An insufficient number of studies examined patient-reported outcomes, which are extremely important for patients with a chronic disease like DTF.

Desmoids are a rare type of tumor with an unpredictable natural history. The annual incidence in the general population is estimated at 2.4-4.3 cases per million. This rare case is considered a diagnostic challenge. Therefore, the emergence of knowledge of CT features and other findings is an important aspect in the diagnosis. In this case study, we present a 57-year-old female patient with a 2-year history of chest pain in the right upper chest. To establish a proper diagnosis a chest X-ray was performed and showed opacities in the right hemithorax, and computed tomography revealed a solid pleural mass measuring 4.4 cm × 4 cm × 3 cm. Along with imaging results, histopathological examination and immunohistochemical analysis of an open biopsy revealed a desmoid tumor.

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Fibromatosis is an extremely rare tumor that arises from the myofascial tissue and has high rates of infiltration and recurrence. Fibromatosis of the chest wall is even less common, and wide surgical resection is the preferred treatment alternative to radiation therapy, chemotherapy, and systemic treatments. We report the case of a 3-year-old boy with radiologically and pathologically confirmed fibromatosis of the periscapular region who underwent wide resection. We discuss diagnosis, treatment options, and technical pearls for a desmoid tumor of the chest wall. The resection should go through healthy tissue because of the infiltrative nature of the tumor. Although visceral involvement did not occur in this case, surgeons should be aware of its possibility, and surgery should be performed by a multidisciplinary team, including a pediatric orthopedic surgeon, a thoracic surgeon, and an anesthesiologist.