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Print version ISSN 0365-0596On-line version ISSN 1806-4841
An. Bras. Dermatol. vol.79 no.5 Rio de Janeiro Sept./Oct. 2004
Malignant fibrous histiocytoma of the extremity: a case report*
Roberta Cardoso de SiqueiraI; Márcio Lobo JardimII; Valdir BandeiraIII; Rosana Maria de Cássia X. das Chagas FerreiraI; Luciano Tavares MontenegroIII; Patrícia GuimarãesI; Virgínia BatistaI
IIHead of Dermatology Department UFPE
IIIProfessor of Pathology, UFPE
Malignant fibrous histiocytoma is the most common soft tissue sarcoma found in young adults. It is rarely confined exclusively to the skin and subcutis. In most cases it is only diagnosed after excision and analysis of the tumor. It is aggressive, presenting a high degree of local recurrence and metastasis. This article reports a case of malignant fibrous histiocytoma on an extremity of a young adult patient.
Key words: dermatofibroma; neoplasms; sarcoma.
Malignant Fibro-Histiocytoma (MFH) or malignant fibrous histiocytoma is an aggressive soft tissue sarcoma, that most frequently occurs in the skeletal muscle of the extremities and abdominal or retroperitoneal cavity of young adults.1-6
Most MFH are located below the fascia. It is seldom confined to the skin and subcutaneous tissue.2,5,7
It is rarely diagnosed before excision and anatomicopathological exam, and has an unfavorable prognostic in some cases.1,4
This work reports the case of a young adult with cutaneous MFH and underscores the importance of the early diagnosis of this neoplasia.
A male, 26-year-old patient, merchant, light-skinned, natural of and resident in Recife, was seen at the Dermatology clinic of UFPE in March 1999. He presented a history of ulcerated lesion in the fifth right finger, onset 11 months previously and without tendency to cicatrization despite all the measures of local antisepsis. He also reported that, one year prior to this, he had been submitted to papular exeresis in the same site as the current ulcerated lesion. At that time, the patient was informed he had "dermatofibroma", however anatomicopathological exam of the excised tumor was not performed. Soon after the surgery there was cicatrization of the lesion with progressive retraction between the medial and proximal phalanges.
At clinical exam, the lesion presented as an ulcer with infiltrated borders, a granular base, vivid red, very friable, measuring approximately two centimeters in diameter, located in the middle third of the fifth right finger (Figure 1), suggesting a clinical diagnostic hypothesis of epidermoid carcinoma.
Based on an incisional biopsy from the border of the lesion, the histopathological exam showed a tumor formed at the expense of pleomorphic cells and partly fusiform or polygonal cells. Nuclei were irregularly configured, hyperchromatic and with presence of mitoses. Rich vascularization in bursts of vessels, infiltrated by mononuclear cells and presence of rare foamy cells. Scarce presence of collagen (Figures 2 and 3).
Immunohistochemical exam demonstrated localized pleomorphic cells in the dermis, distributed in diffuse manner with cytoplasmatic immunophenotyping positive for the Lysozyme (Dako, California, USA) and Vimentin (Dako, California, USA) primary antibodies showed by the Streptoavidin-Biotin method (Dako, California, USA), thereby demonstrating the presence of histiocytes and fibroblasts, respectively, in the tumor (Figures 4 and 5). Primary antibodies were also used for melanocytic cells, HMB-45 (Dako, California, USA), and for epithelial cells, Cytokeratin AE1-AE3 (Dako, California, USA), both with negative results.
X-ray of the right hand showed deformation of the fifth left finger, loss of soft parts from the projection of the proximal interphalangeal articulation and absence of bone alterations.
Bone scintilography showed a focal area with a marked degree of hyperfixation of the radiotracer in the fifth proximal interphalangeal articulation of the right hand (Figure 6). A focal concentration of the marker in the right side of the jaw was observed (interpreted by the nuclear medicine team at UFPE as probably due to the alveolar-dental inflammatory process).
Blood count, urine summary, transaminases, bilirubin, urea, creatinine computed tomography of skull, chest x-ray and ultrasound scan of total abdomen were normal.
The patient was referred to the Institute of Medicine and Cancer (IMEC) in Pernambuco, where radical treatment was decided upon with disarticulation of the involved finger and the patient was followed up.
Three years after the surgery there were no signs of local recurrence or metastasis.
MFH has been recognized since 1963. It is a sarcoma with a high degree of polymorphism and capacity for producing collagen, but without other defined characteristic.8 Approximately two thirds of these tumors are located in the skeletal muscle. Few cases of cutaneous origin, as described in this work, have been reported.9,10 Exceptional locations have been described, such as the conjunctiva,11 traumatic ulcer of the leg, inferior lip in place of an epidermoid carcinoma previously treated with radiotherapy, scars from prior surgery,9 scalp7 eand burn scars.2,12,13
According to their location they can be classified into superficial and profound. The superficial form is very rare and is confined to the skin and to the subcutaneous tissue, it may be adhered to the fascia. The profound form extends from the skin along the fascia until the muscle, or it can be located entirely within the muscle.4
The precise histogenesis of this tumor has not been established. Five different histological subtypes have been recognized: pleomorphic, mixoid, gigantic cells, inflammatory and angiomatoid.1,14 Recent ultrastructural studies have demonstrated the presence of histiocytic and mesenchymal cells.15
From a clinical and histopathologic perspective they can resemble malignant melanoma, epidermoid carcinoma, dermatofibrosarcoma protuberans, atypical fibroxanthoma and leiomyosarcoma. Diagnosis is possible through clinicopathological correlation and use of additional criteria from the histology and immunohistochemistry. A correct interpretation is important, since the prognostic and treatment vary significantly among those entities.1,4
Ample excision is recommended, due to the high degree of local recurrence (approximately 44%) and metastasis, most commonly in the lungs (approximately 42% of cases).2,5,7,8 The presence of metastasis is usually associated with a poor prognostic. Primary MFH of the skin can have a more favorable prognostic than homologous tumor originated in more profound and retroperitoneal soft tissue.15
Mohs micrographic surgery has been showing better results than conventional excision in terms of local recurrence.4
This work demonstrates a case of localized MFH in the digital extremity of a young patient, clinically suggestive of epidermoid carcinoma. However, anatomicopathological and immunohistochemical studies showed that it was a malignant, ulcerated, infiltrative, poorly differentiated neoplasia based on histiocytes and fibroblasts. The fact that it was extremely difficult to diagnose this tumor before microscopic exam underscores the importance of "good documentation".
Disarticulation of the digit was performed due to the great extension of the lesion, involving the greater part, and according to reports in the literature due to the high risk of local recurrence and metastasis.2,4,7,9,11,12 Three years after the surgery there were no clinical or laboratory signs of recurrence of the tumor or distant lesions, reaffirming that radical excision of this type of lesion is imperative. Nevertheless, in this case it might have been possible to avoid amputation of the whole finger if, at the first attempt to excise the lesion, the surgeon had forwarded part of tumor for anatomicopathological exam. This would have increased the probability of diagnosing the tumor in an earlier stage and a more conservative excision could have been indicated.
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Roberta Cardoso de Siqueira
Av. Agamenon Magalhães, 4775 - SL. 201
CEP 50070-160 - Ilha do Leite , Recife - PE
Tel.: (81) 3222-2602 / 3231-0180 - Fax: (81) 2125-7402
Received on December 05, 2002.
Approved by the Consultive Council and accepted for publication on October 07, 2003.
* Work done at the Clinicas Hospital, Federal University of Pernambuco.