[Desmoid Tumor Treated by Wide Resection of the Anterior Chest Wall] [Article in Japanese]

We report the case of a 72-year-old man with a desmoid tumor arising from the anterior chest wall which invaded neighboring organs extensively. The patient complained of dyspnea on exertion and appetite loss and was referred to our hospital. Chest computed tomography revealed an anterior chest wall tumor 12 cm in diameter adjacent to the right lung, diaphragm, and sternum. An ultrasound-guided biopsy was conducted, and the tumor was diagnosed as a desmoid tumor. He underwent right-sided anterior chest wall resection with combined resection of the right lung, diaphragm, and sternum body. The chest wall defect was reconstructed using an expanded polytetrafluorethylene mesh (dualmesh). Although initial active surveillance has recently been recommended for asymptomatic patients with non-progressing desmoid tumors, our patient underwent resection because of his symptoms.

The aim of this retrospective study was to analyze clinical presentation, therapy, and outcomes in a consecutive series of four patients with desmoid-type fibromatosis (DF) at the foot. From 1994 to 2014, four patients had been surgically treated. The resection margin was marginal or even intralesional in all. One patient already had local recurrence at first presentation. The end point was either local recurrence or progression of residual disease.The mean patient age was 27 years. In one patient, marginal excision healed the disease. In another patient, local recurrence after marginal resection necessitated distal phalanx amputation. Two other patients showed stable disease after either adjuvant radiotherapy or treatment with nonsteroidal anti-inflammatory drugs and tamoxifen. If surgery is necessary, operative margins are less important than keeping function for the patient. Radiotherapy might be an option to avoid major amputation. The role of adjuvant radiotherapy is controversially discussed. A watchful wait-and-see policy seems to be justified by the published data but may be difficult for DF at the foot.

Gastrointestinal Stromal Tumor (GIST) is the most common mesenchymal tumor of the gastrointestinal tract and most commonly affects the stomach, while fibromatosis is a rare locally aggressive fibrous tissue neoplasm. There have been reports of GIST and fibromatosis occurring in same individual and in most of them fibromatosis occurs within the abdomen. In 75% of patients fibromatosis occurs after the diagnosis of GIST while in 20% it is seen synchronously. Here we report a case of axillary fibromatosis with synchronous GIST of the gastroesophageal junction in a 45-year-old male treated with surgery and now on adjuvant imatinib.

We report a case of desmoid tumor that was initially diagnosed as metastasis from renal cell carcinoma. A follow-up computed tomographic (CT) scan after nephrectomy for renal cell carcinoma in a 64-year-old man revealed a right retroperitoneal tumor 3.5 cm in diameter. Though the CT density of the tumor was similar to that of muscles, we first suspected metastasis from renal cell carcinoma or other cancer because the tumor growth was relatively fast. We resected the tumor. The pathological diagnosis was desmoid type fibromatosis. We also review six cases of desmoid tumors after nephrectomy for renal cell carcinoma reported in Japan. Careful followup is required for the patients with desmoid tumors because of frequent recurrence.

A 28-year-old pregnant woman was diagnosed with Gardner syndrome in whom an intra-abdominal tumor was found a year after undergoing a laparoscopic total colectomy due to family adenomatous polyposis. At 32 weeks gestation, she presented to our department for the third time complaining upper abdominal pain caused by the giant abdominal mass about 21 × 12 cm2 in size. After multidisciplinary consultation and discussion, the decision of fetal preservation treatment was made. After the delivery of a baby girl, abdominal mass resection was performed, and pathological examination revealed a fibrous adenoma. The patient was discharged after a week and was uneventful in the follow-up for half a year. In our case, we selected surgery to remove the intra-abdominal desmoid tumor after the natural delivery of the fetus and no abnormalities were observed during the 6 months follow-up. Women during pregnancy have an increased risk for the development of desmoid tumors, likely with the sex hormone to be one of the triggers. Therefore, we suggested that when a patient with Gardner syndrome desire to conceive again, they should go to the hospital for a regular review at least once every 3 months.

A 32-year-old woman presented with progressive pain and swelling of the left wrist for 6 months. Physical examination revealed a firm, tender, oval swelling over the left wrist. X-rays showed a pressure effect on the distal radius and ulna. Magnetic Resonance Imaging (MRI) revealed a well-defined, asymmetrical, dumbbell-shaped soft-tissue lesion involving the interosseous region of the distal forearm and extending until the distal radioulnar joint (DRUJ). Core needle biopsy confirmed the diagnosis of desmoid tumour. Marginal excision of the tumour was done. At the 2-year follow-up, the patient was doing well and had painless and improved left wrist motion. Desmoid tumour involving the DRUJ has not been previously reported. We, through this case, report new observation and discuss the epidemiology, investigation of choice, treatment modalities, and the need for a regular follow-up for appendicular desmoid tumours.