[Abdominal wall repair after total resection of desmoid fibroma in a patient with diabetes mellitus type 1]. / Rekonstruktsiya perednei bryushnoi stenki posle radikal'noi rezektsii desmoidnoi fibromy na fone sakharnogo diabeta 1-go tipa.

Desmoid fibroma (DF) is a rare connective tissue tumor comprising about 0.03-0.13% of all neoplasms. DF has a low potential for malignant transformation, but it is characterized by aggressive course and unfavorable prognosis. The main contingent of patients consists of women of reproductive age. Despite the nearly two-century history of study, there are certain unsolved problems including endocrine problems associated with this disease. We report a 30-year-old female with DF and diabetes mellitus type 1. Total resection of the affected right rectus abdominis muscle was performed in a single block with aponeurotic sheath and peritoneum after normalization of carbohydrate metabolism. Muscular aponeurotic defect 27�10 cm was closed after separation of abdominal wall structures and implantation of polypropylene prosthesis. There are no X-ray and clinical signs of DF recurrence or postoperative hernia after 8 months.

Muscular dystrophy associated with extra-abdominal desmoid tumor showing aberrant chromosome 1 [46,XX,add(1)(p36)].

We report on a 2-year-old girl with probable limb-girdle muscular dystrophy associated with an extra-abdominal desmoid tumor of the right mandible. This association is previously undescribed. The tumor was totally removed. Cytogenetic analysis of the tumor showed a clonal karyotypic abnormality: 46,XX,add(1)(p36) in 3 of 20 cells analyzed. Since an association of a neoplasm with limb-girdle muscular dystrophy has previously been reported in 3 cases, the two abnormalities are likely related causally. The chromosome abnormality in our patient may play a role in the occurrence of her desmoid tumor.

Aggressive fibromatosis in the head and neck region: Benign tumor with often mutilating effects.

BACKGROUND: Aggressive fibromatosis (AF) or desmoid tumor of the head and neck region is a rare, usually unresectable, benign soft tissue tumor with locally aggressive behavior. METHODS AND RESULTS: A 31-year-old woman presented with a progressive trismus, a swelling in the retromandibular area, as well as loss of sensibility of the maxillary and mandibular branch of the trigeminal nerve. MRI of the head and neck revealed an infiltrative mass involving the masticator, parapharyngeal, and prevertebral and paravertebral space on the left with intracranial extension through the orbital fissure. After the fifth biopsy, 15 months after presentation, the diagnosis of AF was made. The tumor was unresectable, so intensity-modulated radiotherapy was given with curative intent using a total dose of 60 Gy in 30 fractions of 2 Gy. After 16 months, she showed progressive disease, for which tamoxifen 40 mg twice daily was started with a good response for 2 years. After that, she started with sorafinib, on which she has stable disease now. CONCLUSION: The often long delay in proper diagnosis and the treatment challenges of a desmoid tumor are illustrated in this case. Furthermore, this article reviews the literature concerning AF, especially of the head and neck region.

Three siblings with juvenile hyaline fibromatosis.

Juvenile hyaline fibromatosis (JHF) is an extremely rare hereditary genetic disease of autosomal recessive transmission that is characterized by large cutaneous tumors commonly involving the scalp, papulonodular skin lesions, flexural joint contractures, gingival hyperplasia, and osteolytic bone lesions. JHF is usually diagnosed in young infants and in children younger than 5 years, and the lesions characteristic of this disorder consist of fibrous tissue and homogenous amorphous eosinophilic hyaline material. We report the case of a 9-year-old girl with severe gingival hyperplasia, nasal enlargement, mild osteoporosis, and multiple papulonodular skin lesions. Her two brothers (7 and 13 years of age, respectively) were also diagnosed as having JHF. In the patient described in this report, the maintenance of oral hygiene after gingivectomy enabled the continued resolution of gingival hyperplasia, although skin lesions recurred and nasal overgrowth persisted.

Tumor desmoide extraabdominal: descripción de un caso y revisión de la bibliografía / Extraabdominal desmoid tumor: case description and review of the literature

Objetivo: presentar un caso clínico de tumor desmoide de maxilar inferior en un niño de 4 años, analizar las características histológicas y el comportamiento clínico e informar el diagnóstico y tratamiento. Caso clínico: un varón de 4 años de edad, sin antecedentes patológicos, fue atendido por presentar tumoración en región mandibular izquierda de 7 meses de evolución. Se realizaron ecografía, tomografía axial computarizada y resonancia magnética, las cuales describieron masa ocupante de espacio sólida, de límites escasamente definidos con resorción perióstica. La biopsia informó neurofibroma submandibular. Se efectuó la extirpación quirúrgica que confirmó el diagnóstico de tumor desmoide extraabdominal. Conclusiones: el tumor desmoide es de histología benigna pero infiltrante, con una tasa de recurrencia alta, por lo que el tratamiento indicado es la resección quirúrgica amplia. La radioterapia puede controlar las lesiones irresecables.