Tumeur fibreuse solitaire parautérine: à propos d’un cas

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Abstract

Les tumeurs fibreuses solitaires du tractus génital féminin sont extrêmement rares. Nous rapportons le cas d’une patiente âgée de 78 ans qui a présenté une masse pelvienne. L’exploration chirurgicale a montré une tumeur parautérine. L’examen anatomopathologique a conclu à une tumeur fibreuse solitaire avec des signes de malignité. Les suites ont été marquées par le décès de la patiente. Il est important de connaître ces tumeurs dont l’évolution peut être péjorative. Un suivi au long cours doit être recommandé pour les tumeurs résécables.

Translated abstract

Solitary fibrous tumors of the female genital tract are extremely rare. We report the case of a 78-year old patient presenting with a pelvic mass. Surgical exploration showed parauterine tumor. Anatomo-pathological examination confirmed the diagnosis of malignant solitary fibrous tumor. The evolution was marked by the death of the patient. It is important to be able to detect these tumors whose evolution can be poor. Long-term follow-up of patients with resectable tumor should be recommended.

Most cited references 14

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Atypical and malignant solitary fibrous tumors in extrathoracic locations: evidence of their comparability to intra-thoracic tumors.

S. Dry, A-V Decouvelaere, Paul C. Fletcher (1998)

Solitary fibrous tumor (SFT), first described as a pleural lesion, has been reported at numerous extrathoracic sites over the past 10 years. About 10% to 15% of intrathoracic SFTs are histologically or clinically malignant, but such cases have very rarely been described at other locations. Among 92 cases of extrathoracic SFT in our files, we identified 10 that either had recurred (2 cases) or had a least one atypical histologic feature (8 cases). The ten tumors occurred in five men and five women, 32 to 81 years old (median 56), measured 1.9 cm to 20 cm (median 11.5 cm), and were located in the abdomen/pelvis (4 cases), retroperitoneum (3 cases), groin, trunk, and upper arm. Nuclear atypia (8 cases), markedly increased cellularity (6 cases), areas of necrosis (4 cases), and greater than 4 mitoses/10 HPFs (3 cases) were seen in addition to the typical histologic features of SFT. Six tumors had at least two of these atypical histologic features. Nine cases were positive for CD34, six were positive for O-13, and one was focally positive for smooth muscle actin. Eight were excised completely. Subsequent follow-up revealed tumor relapse in eight cases (follow up 6-180 months, median 24). Four patients had local recurrence at 12 to 168 months. Distant metastasis developed at 1 to 6 years in five cases with spread to lung (2 cases), liver (4 cases), and bone. Metastasis or local recurrence developed within 2 years in five patients. To date, no patient has died of their tumor. These findings demonstrate that nuclear atypia, hypercellularity, greater than 4 mitoses/10 HPFs, and necrosis may be seen in up to 10% of extrathoracic SFTs, and are associated with, but are not by themselves predictive of, aggressive clinical behavior. In addition, our findings confirm that the behavior of extrathoracic SFTs is unpredictable, entirely comparable to that of their better known pleural counterparts, and confirm that patients with SFTs in all locations require careful, long-term follow up. It is probably unwise to regard any such lesion as definitely benign.

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Extrathoracic solitary fibrous tumors: their histological variability and potentially aggressive behavior.

S Hirohashi, T Shimoda, Y Matsuno … (1999)

The histological variability of solitary fibrous tumors may contribute to the difficulty in diagnosing these neoplasms, especially when they arise in extrathoracic sites. Like intrathoracic lesions, the behavior of extrathoracic solitary fibrous tumors is currently unpredictable because these types of tumor have only recently been recognized. This study therefore was undertaken to examine the clinical behavior and histological, immunohistochemical, and ultrastructural features of 24 extrathoracic solitary fibrous tumors with long-term follow-up. The patients comprised 10 men and 14 women, between 30 and 85 years of age (mean, 51 years). Ten tumors were located in the retroperitoneum or pelvis, 5 in the trunk, 4 in the extremities, 2 in the orbital region, and 1 each in the kidney, uterine cervix, and meninges. All of the tumors showed a classic morphological appearance, diffuse and strong immunoreactivity for both vimentin and CD34, and variable reactivity for bcl-2. All 7 cases examined ultrastructurally contained fibroblasts and myofibroblasts. Six tumors contained multinucleated giant cells, and in 4 cases these lined pseudovascular spaces with mononuclear cells, thus resembling giant cell angiofibroma and giant cell fibroblastoma. Other potentially similar spindle cell neoplasms mixed with adipose tissue, such as dendritic fibromyxolipoma, lipomatous hemangiopericytoma, cellular angiofibroma, and spindle cell lipoma, were considered in the differential diagnosis. One tumor displayed atypical histological features in the form of increased cellularity and nuclear pleomorphism, but this patient has remained free of disease for 14 years. Another 2 patients developed local recurrences at 6 months and 5 years, and a further patient developed pulmonary metastases that were diagnosed after 7 years. These tumors lacked any atypical histological features in the primary lesions. No patient has so far died of the disease. In conclusion, most extrathoracic solitary fibrous tumors appear to pursue a benign course, although, because some have the potential to recur or metastasize, careful long-term follow-up is necessary for all patients.

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Dermatofibrosarcoma protuberans is a unique fibrohistiocytic tumour expressing CD34.

N Tabata, H Ootani, S Aiba … (1992)

Dermatofibrosarcoma protuberans (DFSP) is a slow growing, locally invasive tumour whose differentiation from other fibrohistiocytic tumours sometimes poses serious diagnostic problems. We investigated CD34 expression immunohistologically in various fibrohistiocytic tumours including dermatofibroma, DFSP, malignant fibrous histiocytoma (MFH), infantile myofibromatosis, fibrosarcoma, hypertrophic scar and keloid. Among these, DFSP was unique in that tumour cells themselves expressed CD34, whereas in other tumours. CD34 expression was observed only on vascular endothelial cells amongst the tumour cells. Until now, there have been no reports of useful immunohistological markers for DFSP. CD34 expression by the tumour cells can be an extremely useful marker in establishing a definitive diagnosis of DFSP.

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Author and article information

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): 21 November 2016

Publication date Collection: 2016

Volume: 25

Electronic Location Identifier: 180

Affiliations

[1 ]Service de Gynécologie-Obstétrique A, Hôpital Charles Nicolle, Tunis, Tunisie

[2 ]Faculté de Médecine de Tunis, Tunisie

Author notes

[& ]Corresponding author: Olfa Slimani, Hopital Charles Nicolle, service Gynécologie-obstétrique A, Tunis, Tunisie

Article

Publisher ID: PAMJ-25-180

DOI: 10.11604/pamj.2016.25.180.8184

PMC ID: 5326071

PubMed ID: 28292142

SO-VID: 55eaaee7-2040-4a81-be37-6dcc8c7ed97b

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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 fibreuse solitaire parautérine: à propos d’un cas Translated title: Parauterine solitary fibrous tumor: about a case

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Abstract

Translated abstract

Most cited references 14

Atypical and malignant solitary fibrous tumors in extrathoracic locations: evidence of their comparability to intra-thoracic tumors.

Extrathoracic solitary fibrous tumors: their histological variability and potentially aggressive behavior.

Dermatofibrosarcoma protuberans is a unique fibrohistiocytic tumour expressing CD34.

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