Keywords
sarcoma - malignant fibrous histiocytoma - neoplasms of soft tissue neoplasms ENT
Introduction
Of the malignant tumors of the adult population only 1% are soft tissue sarcomas (SPM)[1]. Of these, 5 to 16% occur in the head and neck[2]
[3]. The malignant fibrous histiocytoma (MFH) is a rare tumor and there are few cases
described in the literature, and thus difficult to define prognosis and treatment[4]. It was described in the 60s as a distinct type of SPM, and has been the most common
histological type diagnosed since. The atypical fibroxanthoma (FA) is one of its variants.
The SPM exhibit aggressive behavior, tendency to invasion and metastatic spread early.
We present a case of probable recurrence of AF after two years, surgical treatment
and a brief review of the literature.
Case Report
Female patient, 63 years old, came to us complaining of swelling in the face (cheek),
left, with six months of evolution and rapid growth in the past two months. She reported
no other symptoms and disorders. However, reported history of resection of a skin
lesion on the same location for about two years. Whose histopathology was inconclusive
on histological lineage, however, assured clear margins and no additional study was
conducted for a conclusive diagnosis.
Physical examination revealed subcutaneous swelling in the left side of fibro-elastic
consistency, adjusting, measuring approximately four by two centimeters, movable with
respect to the depth and apparently fixed to the overlying skin, where scar visualized
approximately three inches, corresponding prior to surgery. He had no palpable cervical
lymph nodes. Computed tomography (CT) revealed an increase in volume in the subcutaneous
tissue of the left face, with contrast medium uptake and cleavage plane with the left
parotid gland deeply. We performed fine needle aspiration biopsy that showed only
atypical cells. During this period there was an increase in lesion volume and apparent
infiltrating the epidermis.
Due to suspicion of malignancy with rapid evolution, we opted for surgical resection
of the lesion accompanied by frozen section intraoperative examination. This has not
been able to define the histological lineage, but confirmed that it was invasive malignancy.
Resection was performed with safety margins and included the overlying skin. Primary
closure was performed and postoperatively the patient developed salivary fistula,
treated with pressure dressings and healing after 15 days.
Pathology showed undifferentiated malignant neoplasm characterized by proliferation
of spindle cells, pleomorphic epithelioid and some involving the dermis and subcutaneous
tissue. Observing occasional mitotic figures. The surgical margins were free. The
immunohistochemical study revealed for CD10 expression, favoring the diagnosis of
atypical fibroxanthoma (malignant fibrous histiocytoma surface). The tumor was staged
as N0 M0 pT1a - stage IIB (UICC 2006). It was chosen to carry out radiotherapy or
chemotherapy. The patient had no signs of local recurrence or distant metastasis when,
after four months was detected on clinical examination new tumor in the topography
of the mandibular body left in an area that had no contact with the resected lesion.
A biopsy of the tumor showed that it was FA. The patient was referred to a tertiary
hospital to give the sequel treatment, since it required at this time a more extensive
resection followed by reconstruction of the mandible. Which was done so as radiotherapy.
Discussion
The SPM represent only 1% of all cancers of the head and neck[5]. They are grouped together because of similarities in their appearance pathological,
clinical presentation and natural history, although they have different cellular origins[3]
[6]. MFH is a rare tumor, but it can be in the region of the head and neck should be
part of the differential diagnosis of primary cervical lesions. One of its variants
is the FA. The clinical behavior is characterized by the tendency to invasion of adjacent
soft tissues and early metastatic dissemination[7]. Occasionally these tumors are associated with genetic syndromes or prior radiation.
Most often there is no clear etiology[2].
Men are more affected than women (3:2). The most common presentation is as painless
swelling[5]. Many symptoms may occur depending on the location of the tumor[2]. Physical examination usually reveals sub mucosal or subcutaneous tumor with distortion
or destruction of adjacent structures. CT and magnetic resonance imaging (MRI) scans
are the choice in most cases and provide information about bone involvement, extension
and lymph node metastasis in addition to size and location[2]. These tests can be used in a complementary way, especially in surgical planning[8]. However, for a histological diagnosis in addition to the microscopic characteristics,
it is necessary to perform immunohistochemical analysis[9].
The prognostic factors are histology grade, location, size and lymph node involvement[7]. MFH is generally considered a lesion of high-grade malignancy[10]
[11]. Regarding location in the head and neck, is considered a worse prognosis with higher
recurrence rates and lower disease-specific survival[12]. Tumors larger than three centimeters in diameter negatively affect the prognosis.
Literature data show that surgical safety margin is among the most important prognostic
factors affecting local control, although its significance as a determinant of survival
remains uncertain[13]. In the study of Belal et al.[3], positive microscopic margins were associated with increased local recurrence. This
author also found a significant correlation between adequate margins and disease-free
survival and overall survival. In the head and neck high rates of positive margins
are reflective of the closeness of relationships between adjacent structures. Local
recurrence occurs in approximately 20% of cases, but the biggest cause of death is
distant metastasis. The survival rate is 40% in the largest series, ranging from 19
to 75%[5]. The authors differ if the local recurrence itself is a cause of distant metastases
and increased mortality[5]
[14].
The mainstay of treatment is wide surgical resection with clear margins[1.3]. Some authors consider elective neck dissection is not necessary since regional
metastases are uncommon. Postoperative radiotherapy is essential for banks affected[3]. It is also recommended for unresectable tumors and margins slim. The role of adjuvant
chemotherapy is controversial[3], its primary indication is for cases of metastatic disease[15].
In the case of AF patients had a facial tumor with no metastases and that probably
it was a local recurrence of resected lesion two years ago, but without histopathological
diagnosis. We opted for surgical treatment, associated with intraoperative frozen
due to great suspicion of malignancy, diagnostic difficulty and rapid progression
at the time of preoperative investigation. The intraoperative frozen was not able
to define the histological diagnosis, as this can only be determined after immunohistochemistry.
After the histological diagnosis of AF (HFM) neck dissection was not performed due
to lack of commitment to regional clinical and radiological evaluations. Also we decided
not to radiotherapy of believing in a small possibility of recurrence due to tumor
staging and the wide margins of resection (minimum of 1 cm) secured by microscopic
analysis. The literature is unclear as to the minimum margin of safety. Chemotherapy
was not used in view of the controversies reported in the literature. But after four
months of surgery was the emergence of new tumor in the left side, which after biopsy
showed that it was FA. The patient was referred to a tertiary hospital for treatment
to follow.
Conclusion
The FA is a rare and difficult histological diagnosis. Surgery with wide resection
of the lesion is the mainstay of treatment. The identification of tumor histology
is of fundamental importance, as well as the postoperative follow-up. In this case
the histological diagnosis of the primary lesion would provide better preoperative
planning and early surgical intervention after local recurrence.
Figure 1. Tumor in the subcutaneous tissue of the left cheek. It is observed cleavage plane
with glula to deep left.
