Cases reported • Fibromatosis, Abdominal

1/5. abdominal wall and foot reconstruction after extensive desmoid tumor resection with free tissue transfer.

BACKGROUND: Desmoid tumors are rare connective tissue tumors, also referred to as deep, aggressive fibromatosis. Although histologically benign, they show an invasive growth behavior and have a high local recurrence rate. methods: The treatment of choice is surgical resection with wide negative margins, while the use of radiotherapy remains controversial. Wide resection of greater desmoid tumors may result in considerable defects and functional impairment. Few papers discuss different options for defect coverage after desmoid tumor resection. Two cases of extensive desmoid tumors, one at the trunk, one at the foot, both with compromised wound margins due to multiple previous surgeries, are presented. To achieve a stable and functional soft tissue cover, the defects were treated with microvascular soft tissue transfer (one free latissimus dorsi, one free radial forearm flap). RESULTS: Both flaps healed uneventfully and patients regained full function of the abdominal wall and foot, respectively. CONCLUSIONS: The presented cases demonstrate the efficacy of free flap coverage as an ultimate therapeutic option in selected cases of critical defects after extra-abdominal desmoid tumor resection. Furthermore, free flaps provide a well vascularized ground for adjuvant radiotherapy.

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2/5. pregnancy as an autologous tissue expander for closure of an abdominal-wall defect.

We report the reconstruction of a complex abdominal-wall defect using expanded skin from pregnancy. Wound closure was achieved using a vertical abdominoplasty.

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ranking = 1.25
keywords = defect
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3/5. Desmoid tumour involving the abdominal rectus muscle: report of a case.

The desmoid tumour (DT) is a quite rare soft tissues neoplasm that lacks metastatic potential. Though it is characterized by a local infiltrating growth that involves frequent relapses after surgical excision. The presented case report refers to a young female in her childbearing age, who underwent the radical excision of a large DT infiltrating the left rectus muscle of the abdomen. The only radical treatment of this tumour is still the surgical resection carried out far from the tumour borders into the healthy tissues. Nevertheless, when these tumours arise in the abdominal wall, their resection causes wide muscle-fascial defects involving the whole thickness wall and determining serious reconstructive problems. These problems are connected to the use of wide prosthesis that are exposed both to the possible development of visceral adhesions and to the abdominal wall rigidity due to the inclusion in the fibrosis of high quantities of totally non-absorbable material. These remarks induced to employ a new composite prosthesis presenting the double advantage of having a non-adherent visceral surface and part of its network made of absorbable material. Its application in filling the wide abdominal muscle-fascial gap gave very satisfying mechanical and esthetical results.

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ranking = 0.25
keywords = defect
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4/5. Deletion (5q) in a desmoid tumor of a patient with Gardner's syndrome.

Desmoid tumors are associated with as many as 20% of cases of familial adenomatous polyposis (FAP) and Gardner's syndrome. In the present study, four specimens from different regions of a massive intraabdominal desmoid tumor from a 23-year-old white male with Gardner's syndrome were analyzed cytogenetically. Two different clonal abnormalities were observed. Two of the four specimens analyzed showed a del(5)(q14q31), which involves the region q21-->22 where the familial adenomatous polyposis gene is localized. In the two other specimens, a balanced translocation involving chromosomes 3 and 4 and an inv(4) was detected. Our findings confirm previous reports about the importance of chromosome defects on 5q in development of desmoid tumors, particularly in patients with Gardner's syndrome.

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keywords = defect
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5/5. melorheostosis with associated minimal change nephrotic syndrome, mesenteric fibromatosis and capillary haemangiomas.

melorheostosis is a benign, rare, congenital disorder of hyperostosis of one or more bones. Associated soft tissue abnormalities are often observed. A case of melorheostosis with minimal change nephrotic syndrome, mesenteric fibromatosis and capillary haemangiomas is reported. A congenital mesenchymal defect occurring early in embryonic life is suggested.

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keywords = defect
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