Extra-abdominal desmoid tumours

McDougall, Andrew; McGarrity, G

doi:10.1302/0301-620x.61b3.479262

Cited by 49 publications

(20 citation statements)

References 1 publication

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“…However, the recommendation to treat with anti-estrogens is based on a limited number of studies reporting positive staining for ER α [2,[36][37][38][39][40][41]. In our patient collective staining for ER β was positive in only three cases, supporting data from previously conducted studies of our institution in which positive staining was found Pediatric Hematology and Oncology Pediatr Hematol Oncol Downloaded from informahealthcare.com by Gazi Univ.…”

Section: Estrogen Progesterone and Androgen Receptorssupporting

confidence: 84%

Is Wide Resection Obsolete for Desmoid Tumors in Children and Adolescents? Evaluation of Histological Margins, Immunohistochemical Markers, and Review of Literature

Woltsche

Gilg

Fraissler

et al. 2014

Pediatric Hematology and Oncology

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Desmoid fibromatosis is a benign fibroblastic neoplasm with high recurrence rates predominantly observed in pediatric and adolescent patients. The use of wide resection margins has been discussed controversially in literature. In addition, data on non-surgical treatment is limited as phase III studies are still missing. Nineteen patients under the age of 18 years were identified. Tumor location, surgical treatment for primary or recurrent tumors, resection margins, medical neo-/adjuvant treatment, time to recurrence as well as immunohistochemical markers (estrogen receptor, ER α and β, progesterone and androgen receptors, somatostatin, Ki-67, c-kit, platelet-derived growth factor receptors, PDGFRs, α and β, β-catenin) were evaluated. The mean age at diagnosis was 6.6 years, with a mean follow-up of 114 months. Recurrences were detected in four out of nineteen patients. Surprisingly, the recurrence rate was not influenced by type of resection used (R0, R1/2). All samples were tested negative for ER α, somatostatin, and progesterone receptor. In contrast, a majority of tumors showed positive results for PDGFR α and β and β-catenin. No correlation between positive immunohistochemical markers and tumor recurrences was detectable. In conclusion, recurrence rates are not depending on resection type and immunohistochemical markers seem to behave differently in children and adolescents in contrast to adult patients.

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Section: Estrogen Progesterone and Androgen Receptorssupporting

confidence: 84%

Is Wide Resection Obsolete for Desmoid Tumors in Children and Adolescents? Evaluation of Histological Margins, Immunohistochemical Markers, and Review of Literature

Woltsche

Gilg

Fraissler

et al. 2014

Pediatric Hematology and Oncology

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“…Swelling may reappear after extensive procedures, but it is not always due to recurrence and may disappear totally or partially, as has also been noted by Enzinger and Rock. Tumours in the popliteal fossa, supraclavicular fossa, buttock, forearm and pelvis were the most difficult to cure [15]. Although tumours located in the posterior thigh and leg have a tendency to recur, wide procedures controlled all but one of our patients.…”

Section: Discussionmentioning

confidence: 74%

Surgical treatment of extra-abdominal desmoid tumours (aggressive fibromatoses)

Higaki

Tateishi

Ohno

et al. 1995

International Orthopaedics

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Extra-abdominal desmoid tumours (EADT) are benign lesions but difficult to cure because of their infiltrative nature and tendency to recur. Among many treatments recommended in the past, wide excision has been successful, even in difficult cases. We have analyzed retrospectively 41 cases of histologically confirmed EADTs. A total of 98 operations were performed on these patients: 29 wide excisions on 22 patients, 52 intra-lesional excisions with wide margins on 16 patients, and 17 incomplete excisions on 3 patients. One patient, with intra-pelvic lesions, died of a massive haemorrhage 3 days after surgery. Forty patients were followed from between 3 and 29 years. One, who had a multicentric EADT for 21 years, died from the disease. The significant factors concerning local recurrence after wide procedures were an unsatisfactory initial wide local excision, disease affecting 4 or more muscles and the invasive nature of the recurrences. We recommend wide local excision of these tumours in all anatomical areas that allow this procedure. When major nerves and vessels are involved, we recommend an intralesional excision with wide margins in order to preserve limb function. Radiation therapy should be confined to cases in which wide local procedures are not feasible. Overall, 37 of our patients (90%) were cured of the disease, 2 had their disease controlled, and 2 died.

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“…The treatment of desmoid tumors with progesterone has also been reported by several authors (Lipschutz and Grismail 1944;Nadel 1950;Dahn et al 1963;Lanari 1983). Clinically, pregnancy has been well-known to ameliorate the course of abdominal desmoid tumors (Ober et al 1955;McDougall and McGarrity 1979;Way and Culham 1999). This clinical observation has raised questions concerning the most appropriate hormonal treatment for these tumors.…”

Section: Discussionmentioning

confidence: 99%

“…Progesterone has been considered to inhibit the proliferation of desmoid tumors but further investigations are required to clarify the detailed involvement of progesterone in the biological behavior of desmoid tumors, because extra-abdominal desmoid tumors most frequently occur in the anterior abdominal wall, typically in females of childbearing age when serum progesterone levels were elevated (Musgrove and McDonald 1948;Dahn et al 1963;McDougall and McGarrity 1979;Reitamo et al 1982). In our present study, PR immunoreactivity was detected only in desmoid tumors, which suggests that PR expression is more pronounced in desmoid than in reactive fibrosis.…”

Section: Discussionmentioning

confidence: 99%