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Malignant fibrous histiocytoma of the face: report of a case
- László Seper1Email author,
- Richárd Schwab†2,
- Sirichai Kiattavorncharoen†1, 3,
- Andre Büchter†1, 4,
- Ágnes Bánkfalvi†5, 6,
- Ulrich Joos†1,
- József Piffkó†1 and
- Birgit Kruse-Loesler†1
© Seper et al; licensee BioMed Central Ltd. 2007
Received: 20 October 2006
Accepted: 18 October 2007
Published: 18 October 2007
Soft tissue sarcomas in the head and neck region are rare and often present a difficult differential diagnosis. The aim of our presentation is to point out the complexity of the diagnosis, treatment and follow up.
An eighty-seven year old female patient was referred to our unit with a fast growing brownish lump on the face. Four months beforehand, a benign fibrous histiocytoma (BFH) had been removed from the same location by excision biopsy with wide tumour-free resection margins. Excision biopsy of the recurrent lesion revealed a malignant fibrous histiocytoma (MFH). Radical tumour resection was completed by extended parotidectomy and neck dissection; the skin defect was covered by a regional bi-lobed flap. No adjuvant radio- or chemotherapy was administered. Full functional and cosmetic recovery was achieved; follow-up has been uneventful more than two years postoperatively.
Malignant transformation of BFH is extremely rare and if so, extended radical surgery may give a fair chance for a favourable outcome even in patients with advanced age.
Soft tissue tumours in the head and neck region sometimes display borderline pathological features regarding benign or malignant behaviour. Despite similar histological patterns the clinical outcome is often different and difficult to predict.
Malignant fibrous histiocytoma [MFH] is a primitive, often pleomorphic sarcoma consisting of partly fibroblast-like, partly histiocyte-like cells. It has been classified as a distinct histopathological entity since the nineteen-sixties . Nowadays, it is one of the most common soft tissue sarcomas of late adult life, with a male predominance since all neoplasms of mesodermal origin previously regarded as sarcomas of uncertain origin are now included in the category of MFH 
Changing of the histological picture during progression of the disease and transformation of borderline-benign lesions to malignant phenotype has been described. Reported incidence of malignant transformation lies around 1% .
Benign fibrous histiocytoma [BFH] is the most common benign neoplasm in practically all localizations affecting the epidermis. The proliferative, atypical or borderline type is a cell-dense variant mostly growing faster and greater in size, with characteristically frequent local recurrences, reported incidence lying at 3–5%. Both BFH and its borderline variant are known not to give either haematogenous or loco-regional metastases to lymph-nodes .
The aim of our presentation is to point out the complexity of the diagnosis, treatment and follow up of patients with soft tissue tumours of borderline character.
Benign fibrous histiocytomas (BFH) are frequently found in sun-exposed skin of the extremities and of the head and neck. They are among the most common soft tissue lesions of the skin . Their biological nature, in particular whether they are neoplastic or reactive, has long been disputed. Recent studies have found cytogenetic evidence of clonal chromosomal abnormalities in a part of lesions, which support their neoplastic origin . In addition to the common histological pattern, a number of variants are recognized, some of which can be confused with sarcoma. Of the cellular and atypical types, about 20% are localised in the head and neck region, they usually grow faster and are greater in size than the other subtypes, and tend to recur locally (up to 26%) . Reported incidence of malignant transformation is around 1% .
In contrast, malignant fibrous histiocytoma (MFH) is a sarcoma with both fibroblastic and histiocytic features. It has been classified as a distinct histopathological entity since the nineteen-sixties . Nowadays, it is one of the most commonly diagnosed sarcomas of late adulthood, since all neoplasms previously regarded as sarcomas of uncertain origin are now included in this category . MFH typically arises in the soft tissues of the extremities and retroperitoneum, the head and neck region is seldom involved. In superficial sites such as the skin, MFH may behave in a benign fashion despite high-grade looking and fast growing tumour cells.
The primary treatment of MFH consists of radical excision with wide safety margins and dissection of the loco-regional lymph nodes . Post- or sometimes even preoperative radio- or chemotherapy may be an adjuvant option of treatment, however their indications and efficacy remain controversial [2, 6]. In the present case, we have disregarded adjuvant oncological treatment because of the age of the patient and the lack of clear-cut evidence for indication.
In conclusion, predicting biological behaviour on the base of cellular features in cutaneous fibrohistiocytic tumours is sometimes very difficult. It is well illustrated by the present case, which showed malignant transformation in recurrence despite the bland histopathological appearance of the primary lesion. It is therefore mandatory that patients with such lesions are closely monitored postoperatively, so as to be able to act promptly in case of local recurrences. Patients' management requires close collaboration between dermatologists, maxillo-facial surgeons, radiologists and pathologists, which may result in sufficient clinical outcome.
Written consent for publication was obtained from the patient or their relative.
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