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Revista Española de Cirugía Oral y Maxilofacial

versão On-line ISSN 2173-9161versão impressa ISSN 1130-0558

Rev Esp Cirug Oral y Maxilofac vol.30 no.2 Madrid Mar./Abr. 2008

 

CASO CLÍNICO

 

Malignant oropharyngeal fibrous histiocytoma. Resection and radial reconstruction with fasciocutaneous flap

Histiocitoma fibroso maligno de orofaringe. Resección y reconstrucción con colgajo fasciocutáneo radial

 

 

L. García Monleón1, J. Acero Sanz2, Á. del Amo Fernández de Velasco1, S. Ochandiano Caicoya2, J. López de Atalaya2, C. Navarro Vila3.

1 Médico Residente.
2 Médico Adjunto.
3 Jefe de Servicio.
Servicio de Cirugía Oral y Maxilofacial. Hospital General Universitario Gregorio Marañón. Madrid, España

Correspondence

 

 


ABSTRACT

Malignant fibrous histiocytoma is recognized as the most common soft-tissue sarcoma in adults, although its location in the pharynx is unusual. Only 6 cases of the pharynx have been reported in the scientific literature. We report the case of a patient with a large malignant fibrous histiocytoma in the oropharynx. The tumor was resected with generous margins and the defect was reconstructed with a radial fasciocutaneous flap. The epidemiology, clinical manifestations, incidence of metastases, histopathology, prognostic factors and treatment of malignant fibrous histiocytoma are discussed in relation to this case.

Key words: Malignant fibrous histiocytoma; Maxillofacial; Head and neck; Oropharynx; Reconstruction; Radial flap.


RESUMEN

El histiocitoma fibroso maligno se reconoce como el sarcoma de tejidos blandos más frecuente de la edad adulta, aunque su localización en faringe no es usual habiéndose recogido en la literatura científica tan solo 6 casos en esta localización. Presentamos el caso de un paciente con un histiocitoma fibroso maligno en orofaringe de gran tamaño, al que se le realizó una resección con amplios márgenes y la reconstrucción del defecto con un colgajo fasciocutáneo radial. Discutiremos a raíz del caso la epidemiología, manifestaciones clínicas, incidencia de metástasis, histopatología, factores pronósticos y tratamiento de este tipo de tumores.

Palabras clave: Histiocitoma fibroso maligno; Maxilofacial; Cabeza y cuello; Orofaringe; Reconstrucción; Colgajo radial.


 

Introduction

O’Brien and Stout 1 described malignant fibrohistiocytoma in 1964. Since then, it has been recognized as the most frequent sarcoma of soft tissues in adults. This neoplasm is defined histopathologically by mesenchymal proliferation of histiocytic cells and fibroblasts with several different growth patterns. MFH must be differentiated from other neoplasms that exhibit marked pleomorphism, such as pleomorphic liposarcoma, pleomorphic rhabdomyosarcoma, pleomorphic carcinoma, and leiomyosarcoma.2,3

It is located most frequently in the limbs, retroperitoneum and abdominal cavity, occurring in the head and neck in only 1-7% of cases.2,4-6 It is rarely located in the soft tissues of the oral cavity and very few cases occur in the oropharynx, as in our present case. Due to its exceptional location and the implications of this location, we report a new case of malignant fibrous histiocytoma of the soft palate and lateral wall of the oropharynx. The literature on this pathology is reviewed.

 

Clinical case

We report the case of a 54-year-old man who was seen in our clinic for dysphagia, dyslalia, and a foreign body sensation in the oral cavity of 2 months’ evolution. The patient was a regular smoker. Intraoral examination revealed a large, fleshy, exophytic mass, 7 cm maximum diameter, that appeared to be pediculated from the soft palate and occupied the oropharyngeal lumen (Fig. 1). No cervical lymph nodes were palpated. Cervicofacial CAT disclosed a softtissue lesion of large size and well defined margins that seemed to arise from the soft palate, growing exophytically and occupying the oropharyngeal lumen (Fig. 2). No pathological lymph node enlargement was observed. The rest of the physical examination and thoracoabdominal CAT found no tumoral spread. An incisional biopsy revealed a fusocellular neoplasm constituted by a population of fibrohistiocytic cells with a storiform growth pattern, which was compatible with malignant fibrohistiocytoma (Fig.3). As upper airway obstruction was developing rapidly, the patient required tracheostomy before definitive surgery. He underwent surgery via medial labiomandibulotomy and the lesion was removed with safety margins. This involved resection of the entire soft palate, amygdalar pillars, and the left wall of the oropharynx. The procedure concluded with immediate reconstruction using a left radial fasciocutaneous flap (Figs 4-7). The histopathologic study of the piece revealed a fusocellular neoplasm constituted by a population of cells of fibrohistiocytic appearance with pleomorphic oval nuclei and fusiform cytoplasm, which alternated with cells of epithelioid appearance. They were arrayed forming imbricated bundles and adopting a storiform growth pattern in areas, which confirmed the initial diagnosis. Frequent mitotic figures were identified, some of them atypical. Staining for vimentine and CD68 protein was positive. The neoplasm was removed surgically with ample resection margins. Due to the large size of the lesion, complementary irradiation was given. The patient has remained free of local or distant recurrence until now. The aesthetic and functional results are excellent, with normal swallowing and phonation.

 

Discussion

As a result of the description by O’Brien and Stout of malignant fibrous histiocytoma (MFH) as an independent histologic type of softtissue sarcoma, large series of MFH have been published. Today it is accepted as the most frequent soft-tissue sarcoma in adults2,4-9 and one of most frequent in the maxillofacial territory.10, 11

Malignant fibrohistiocytoma may occur at any age, although it is uncommon before the age of 40 years and extremely rare in childhood. A peak incidence is observed in the seventh decade and it is more frequent in men than in women.2,5-7 It may be located in soft tissues or appear as a primary intraosseous tumor. The most frequent sites are the limbs (60%) and retroperitoneum or abdominal cavity (20%), the least common site being the maxillofacial area.2, 4-7,12,13 The most frequent craniofacial site is the nasosinusal tract; other sites are the facial skeleton, larynx, soft tissues of the neck and major salivary glands.3,14 While the location in the oral cavity is uncommon, 12,14-18 pharyngeal tumors are even rarer. Only 6 cases of malignant fibrous histiocytoma have been reported in the pharynx in the scientific literature.19-24

The gross tumor had a gray-colored, fleshy, multilobate appearance. Although it appears circumscribed, in most cases there is microscopic spread to adjacent tissue.2 Microscopically, the neoplasm has various morphologies, sometimes similar to histiocytes and other times similar to fibroblasts. The histologic pattern is highly variable from one area to another and between different tumors. It is classified, based on appearance and predominant cell population, into different subtypes: storiform-pleomorphic, myxoid, giant-cell, inflammatory, and angiomatoid. The pleomophic-storiform type is the most common and is characterized as containing groups of spindle-shape neoplastic cells in a storiform pattern around blood vessels, in addition to histiocyte-like round cells that frequently exhibit a certain degree of pleomorphism (Fig.3). This type tends to be poorly differentiated and more frequently grade 2 or 3. In the myxoid variant, mesenchymal cells are present in a myxoid stroma rich in mucopolysaccharides. The myxoid variant also exhibits a certain degree of cellular pleomorphism, but much less than the previous subtype. It has less tendency to metastasize at a distance and thus has a better prognosis. In the giant-cell subtype, what is peculiar is the presence of a mixture of malignant and benign multinucleate cells and, in particular, osteoclastic type giant cells associated with stromal cells that resemble mononuclear histiocytes with variable degrees of anaplasia. The inflammatory variant is characterized by the presence of abundant neutrophils, plasma cells, lymphocytes and, occasionally, foam cells in poorly collagenized stroma. The angiomatoid type predominates in subjects under the age of 40 years. This type usually is low-grade, with areas of bleeding and hemosiderin deposits, together with a pleomorphic fibrohistiocytic population and high mitotic activity. 2,6,7 Our case pertained to the storiform variant.

As for other types of sarcomas, radiation-induced cases of malignant fibrohistiocytoma have been observed after a period of latency ranging from 5 to 45 years.6, 25-28 In 1999, Patel et al.25 studied a series of 10 patients with radiationinduced sarcoma of the head and neck and observed that MFH was the most frequent subtype (4/10). In the same article, they reviewed radiation-induced head and neck sarcomas published in the scientific literature up until that time and found that the most frequent diagnosis was also MFH (36%). Previous irradiation was not found in our case.

This tumor usually occurs as a painless mass with progressive growth that habitually has a history of less than six months. When the tumor is located in cavities like the abdomen or retroperitoneum, the patient may present symptoms of progressive abdominal distention, such as a progressive increase in abdominal girth and the appearance of hernias and varicoceles. Intraabdominal and retroperitoneal cases may be accompanied by constitutional syndrome, but at other sites it is not a habitual clinical manifestation.2, 7 The special location of the tumor in the case presented here cause rapid and extensive airway obstruction, which made tracheostomy necessary before definitive surgery.

Remote metastatic extension is not uncommon, being found in about 40% of cases. The most typical location of remote spread is the lung (80-90%), followed by bone and liver. In different series, regional lymph node involvement is found in 10-32%.2,3,5,6,29 With regard to the risk of metastasis, Le Doussal et al.6 encountered more metastasis in patients with grade 3 histology and tumor size greater than 8 cm. Other authors find that the depth of the tumor correlates directly with the percentage of metastases; in contrast, the percentage of inflammatory cells present in the lesion seems to have a protective effect against metastasis.2 In our case, the study of the extension found no regional or distant metastases and, given that, in general, it is not considered to be a highly lymphophilic tumor, prophylactic removal of cervical lymph nodes is not thought to be indicated.

Local recurrence has a great impact on survival. Previous studies report a MFH recurrence rate ranging from 26 to 51%.2, 5-7, 14, 29 In a recent study, Sabesan et al.3 find that the recurrence rate is directly related with the extent of resection. For MFH of the head and neck, the recurrence rate is 86% in marginal resections (with less than 3 cm of surgical margin) versus 27% in radical resections. This study supports the results obtained by other authors for MFH in other sites, such as Shinozaki et al., who observed local recurrence in 100%, 72%, 17% and 0%, respectively, of intralesional, marginal, or extensive resections and amputation.30

Various factors seem to influence the evolution of malignant fibrohistiocytoma. In a study of 227 cases of MFH, Pezzi et al.5 found that the size of the lesion and its histologic grade were the most important prognostic factors. In this study, a mean 5-year survival for tumors smaller than 5 cm in diameter and intermediate grade malignancy of 93% was observed. For tumors larger than 10 cm and high-grade malignancy, the 5-year survival was only 41%. In a study of prognostic factors in patients with localized MFH, Le Doussal et al.6 found that disease-specific survival was inversely and significantly related with stage, presence of residual disease, tumor depth, and nonmyxoid histology. They also observed that postoperative radiotherapy and surgical margins free of tumoral infiltration seemed to increase local control of the disease. However, other authors like Kearney et al.,7 in a study of 167 MFH, agree that tumor depth and location seem to affect survival, but tumor size and histologic subtype are not considered to be independent risk factors. Roser et al. 9 also found factors like tumor depth, histologic grade, lesion size, and storiform-pleomorphic subtype to be indicative of worse prognosis.

Among the sarcomas of the head and neck, MFH presents a longer survival than other tumors of the same group, being 50-74% at 5 years depending on the series.3,5,6,9,29,31 Wanebo et al.,31 in a study of 214 head and neck sarcomas, found that the mean survival of MFH at 5 years was 72%, which is higher than the survival for other, more aggressive types of sarcoma, such as angiosarcoma, osteosarcoma, and rhabdomyosarcoma.

With respect to the prognosis of MFH of the head and neck versus the limbs and trunk, the 2004 study of Sabesan et al.3 of 131 patients with MFH disclosed 54 in the head and neck and 77 in other locations. These authors reported a longer 5-year survival for MFH of the limbs and trunk (77% of patients alive at 5 years and 65% disease-free versus 48% and 41%, respectively, for MFH of the head and neck). This could be explained by two factors: a larger percentage of high-grade tumors of the head and neck in this series (78% vs. 55%) and a higher percentage of extended and radical resections in the limbs and trunk.

The treatment of choice is resection with wide margins because these tumors have a strong high tendency to local recurrence. This seems to be directly related with the extension of the resection3,30 and presence or absence of residual disease.6 MFH is not considered a highly lymphopilic tumor, so prophylactic cervical resection is not indicated. Cervical dissection is contemplated only in the presence of clinically or radiologically positive metastatic lymph nodes. Although the role of irradiation is not well established, different prognostic factors (age, sex, tumor location, stage, size, depth, positive surgical margins, irradiation and the dose administered) have been analyzed in studies like that of Hsu et al.29 on 76 patients with localized MFH. These authors found that postoperative irradiation was the only factor that improved local disease control, although without increasing mean survival time (local control at 5 years of 85% for irradiated patients vs. 44% of non-irradiated patients). The role of chemotherapy is well established for some types of soft-tissue sarcoma, like rhabdomyosarcoma, osteosarcoma and Ewing’s sarcoma. However, this factor is debated in other sarcomas of adults, as in the case of MFH. A meta-analysis published by the SMAC (Sarcoma Meta- Analysis Collaboration) on the individual data of 1568 patients treated with doxorubicin-based chemotherapy schedules is noteworthy regarding this point.32 They found that chemotherapy treatment apparently had a significant influence on local and distant recurrence and that it increased the disease-free survival of patients with localized soft-tissue sarcoma. Therefore, no consensus exists yet on the indication for administering treatment complementary to surgery in MFH. Prospective randomized studies are needed, but given the low incidence of this type of tumor, they would be very difficult to conduct.

 

Conclusions

Malignant fibrohistiocytoma of the head and neck is an aggressive tumor. Inadequate resection is related to a higher local recurrence rate and worse prognosis. For that reason, the primary treatment of this type of tumor is resection as extensive as possible. In case of tumors in which adequate margins are not possible due to their location or large size, postoperative irradiation may improve control of the disease.

Radial fasciocutaneous flaps produce excellent results in the reconstruction of large defects of the oropharynx, including the palate.

 

 

Correspondence:
Laura García Monleón
Calle Antonio Zapata, nº 14, 2º B
28002 Madrid, España
Email: lauragmonleon@gmail.com

Recibido: 29.11.06
Aceptado: 14.01.08

 

 

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