Tumeur desmoïde de la glande Bartholin: à propos d'un cas

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Abstract

Les tumeurs desmoides sont les tumeurs rares, bénignes mais fréquemment agressives d'origine mésenchymateuse, ils sont extrêmement rares en localisation Vulvaire. Nous rapportons le cas d'une patiente âgée de 32 ans présentant un fibrome desmoïde de la glande de Bartholin prise pour un simple kyste au début. Le traitement consiste en une excision chirurgicale de la lésion et nous discutons la possibilité de traitement adjuvent pour éviter une éventuelle récidive post-opératoire.

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The desmoid syndrome. New aspects in the cause, pathogenesis and treatment of the desmoid tumor.

T. M. Scheinin, J J Reitamo, P J Häyry (1986)

Based on a detailed clinical and laboratory investigation of 89 patients with histologically verified desmoid tumor and the pertinent medical literature, we have reviewed the etiologic factors, clinical characteristics, and results of treatment of this rare disorder. The incidence of the tumor in the Finnish population is low, 2.4 to 4.3 new cases per 10(6) inhabitants per year. The age distribution profile demonstrated four distinct peak periods: the juvenile period, the fertile period, the middle-age period and the old-age period. The juvenile desmoid tumor is an extraabdominal tumor found in young girls, the fertile variety is an abdominal tumor found in women, the middle age variety is also overwhelmingly abdominal but the sex ratio approaches equality, whereas in the old age group, both abdominal and extraabdominal tumors are equally frequent and the sex ratio is equal. In all male patients, the growth rate was low. A low growth rate was also recorded in young girls. A growth rate of twice that speed was seen in fertile women and four times that speed in the middle age group. In the old age group, a low growth rate, equal to that of male patients, was a rule. The fertile female patients with desmoid tumor had a significant predisposition to estrogen dominance and deviation from progesterone dominance. The direct relationship of the growth rate to the level of endogenous estrogen in the female patients and the demonstration of significant amounts of estradiol but not progesterone receptors in the tumor cytosol further suggest that the growth rate of desmoid tumor is regulated by steroid sex hormones. A significant number of patients with an abdominal desmoid tumor had a history of surgical trauma in the region of subsequent tumor growth. A very high number of the patients demonstrated multiple minor malformations of the bony skeleton. An increased frequency of these malformations was also recorded in the families of the patients and the distribution of the malformations among the family members was compatible with an autosomally dominant pattern of inheritance. After operation, the frequency of recurrence was not statistically different, regardless of whether the tumor was completely removed or not. A combination of operation and radiotherapy did not reduce the frequency of recurrences; in fact, it doubled it.(ABSTRACT TRUNCATED AT 400 WORDS)

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Abdominal desmoid tumors.

G H Sakorafas, George Peros, Christos Nissotakis (2007)

Desmoid tumors are rare, benign, fibromatous lesions that are the result of abnormal proliferation of myofibroblasts. Desmoid tumors can be classified as extra-abdominal and abdominal. Abdominal desmoid tumors are either superficial or intraabdominal. These tumors are associated with a high recurrence rates, even if their microscopic characters indicate a benign disease; their biologic behavior often indicates rather a "malignant" disease, which can cause even the death. Intraabdominal desmoid tumors can engulf surrounding viscera and vessels, thereby greatly complicating their surgical treatment. Management is multidisciplinary. Simple observation is a reasonable management option for asymptomatic patients; spontaneous regression of these tumors may be observed. Complete excision is the treatment of choice for tumors causing symptoms or complications. Surgery should be minimized as much as feasible, while at the same time achieving free margins. Adjuvant therapy should be considered in selected cases; the role of other management options (including gene transfer therapy) is currently under intensive investigation.

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Desmoplastic fibroma of the bone. A report of two patients, review of the literature, and therapeutic implications.

S Kröber, M Laniado, A Greschniok … (1996)

Desmoplastic fibroma (DF) is an extremely rare bone tumor. The recommendations for therapy are often based on limited personal experience, and the rate of local recurrence in the published cases is very high. Therefore, an analysis of treatment results of published cases was performed. Furthermore, DNA analysis of the tumors from two patients was also performed. The clinical, radiologic, and histologic data of two patients with DF of the long bones are presented. DNA flow cytometry was performed on both DFs, three cases of abdominal fibromatosis, and three cases of extraabdominal fibromatosis. One hundred eighty-nine patients analyzed in the literature and our own 2 patients were evaluated with regard to epidemiologic, clinical, and histologic data, with particular emphasis on treatment results. DNA analysis of the locally infiltrating tumors revealed indices of proliferation between 21.5% and 24%, noticeably elevated values in comparison with extraosseous desmoid tumors (8.04%). Magnetic resonance imaging (MRI) was most valuable for imaging the intraosseous and extraosseous extent of DF. The evaluation of 191 patients (189 from the literature, 2 of the authors) showed the numbers of males and females to be equivalent, with a mean age of 23 years. DF has been reported in almost all bones, with a tendency to occur in the mandible and the long bones. Approximately 12% of patients presented with a pathologic fracture (20 of 161 patients). Infiltrative growth in the soft tissue was documented in 48% of patients. Three patients developed metastases after local recurrence. Analyzing the treatment results, the authors found a recurrence rate of 55-72% after nonresection procedures, and 17% after resection. No recurrences are reported after resection with wide surgical margins. The recurrence rate of tumors of the extremities was 55%, and 25% of these patients eventually required an amputation. Considering the "semimalignant" character of this entity and the poor treatment results in patients with recurrent tumors, marginal or wide resection for primary treatment is recommended. The superior imaging quality of MRI greatly facilitates preoperative planning.

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Journal

Journal ID (nlm-ta): Pan Afr Med J

Journal ID (iso-abbrev): Pan Afr Med J

Journal ID (publisher-id): PAMJ

Title: The Pan African Medical Journal

Publisher: The African Field Epidemiology Network

ISSN (Electronic): 1937-8688

Publication date (Electronic): 18 September 2015

Publication date Collection: 2015

Volume: 22

Electronic Location Identifier: 49

Affiliations

[1 ]Service de Gynécologie Obstétrique, Hôpital Militaire d'Instruction Mohamed V, Rabat, Maroc

[2 ]Service d'Anatomie Pathologique, Hôpital Militaire d'Instruction Mohamed V, Rabat, Maroc

Author notes

[& ]Corresponding author: Ihssane Hakimi, Service de Gynécologie Obstétrique, Hôpital Militaire d'Instruction Mohamed V, Rabat, Maroc

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Publisher ID: PAMJ-22-49

DOI: 10.11604/pamj.2015.22.49.5259

PMC ID: 4662525

SO-VID: a4a595c4-6c65-4c87-9c50-52b2b0de4375

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The Pan African Medical Journal - ISSN 1937-8688. This is an Open Access article distributed under the terms of the Creative Commons Attribution License which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

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Tumeur desmoïde de la glande Bartholin: à propos d'un cas Translated title: Desmoid tumor of the Bartholin gland: about a case

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Most cited references 8

The desmoid syndrome. New aspects in the cause, pathogenesis and treatment of the desmoid tumor.

Abdominal desmoid tumors.

Desmoplastic fibroma of the bone. A report of two patients, review of the literature, and therapeutic implications.

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