Case Report

A 27 year old male presented with decreased vision in the right eye lasting for the past two years, swelling and bulging of the right eye, and severe pain for the past five months. He had a history of trauma to the right frontal area about seven months prior to the consultation. On examination, there was proptosis in the right eye. The conjunctiva was dry and swollen and there was corneal opacity. Neurological examination showed absence of vision in the right eye. The radiological examination of the chest was normal. A CT scan showed a heterogeneous enhancing space occupying lesion with calcification in the right frontal bone, right temporal bone, greater wing of sphenoid, medial wall of the right orbit infiltrating the right lateral rectus, superior rectus, and superior oblique, destroying the medial wall of the right ethmoid sinus and ethmoid septae. Involvement of the soft tissue extending to the upper and lower eyelids and infiltration of the right eye wall was also present. Inferiorly, extension in the intraconal compartment of the right orbit was seen. Superiorly intracranial extension was present (fig 1).

Figure 1

Figure 1: CT scan showing tumor extension to involve the frontal bone with orbital and cranial spread

The differential diagnosis was a primary skull tumors such as an osteosarcoma, a Ewing sarcoma, and a chondrosarcoma, or a metastatic lesion. The possibility of MFH was not considered before surgery. The patient’s age and the absence of a primary tumor on full staging work-up made the possibility of a metastatic tumor less likely. An open biopsy of the lesion disclosed MFH. Right craniotomy with extensive, en bloc resection of the temporal region infiltrated by tumor and enucleation of the right eye was performed .

On pathological examination, the cut surface grossly showed myxoid areas with small solid white areas. Microscopically, the section showed an infiltrating malignant neoplasm with both spindle cell and bony areas. The tumor was predominantly made of cells with loose pale background with scattered spindle cells showing slender nuclei with focal nuclear pleomorphism. Interspersed foci of increased cellularity were present with compactly arranged fascicles of spindle cells showing hyperchromatic nuclei and moderate amount of bipolar cytoplasm. Fair number of mitoses were evident in the cellular areas. Foci of necrosis and haemorrhage with lymphatic infiltration were also present. Vascular proliferation was evident (fig 2 a and b).

Figure 2

Figure 2: (a) H & E stained section showing a spindle cell neoplasm with hypocellular myxoid areas and (b) tumor invading bony lamellae 125 x digital magnification

Figure 3

The patient received two cycles of adjuvant chemotherapy with ifosfamide and doxorubicin, followed by 5600 cGy in 28 fractions followed by four more cycles of the same chemotherapy. The patient has completed the treatment and is presently in follow up.

Discussion

Myxofibrosarcoma, also known as the myxoid variant of MFH, is one of the most common sarcomas ^{in the extremities of elderly people and occurs more frequently in the dermal and subcutaneous tissues than in deeper soft tissues. It is relatively rare in the head and neck region. The maxilla and the mandible are the most frequently involved sites. MFH originating in the cranial bone rarely invades the brain tissues (₃,₄,₅). Our case presented with a large defect in the right frontotemporal bone intracranial extension.}

In recent years, the concept of MFH has come under discussion ^{(₆,₇,₈). This tumor is now believed to show fibroblastic rather than histiocytic differentiation. MFH is divided into four subtypes based on histological appearance; storiform–pleomorphic, myxoid, giant cell, and inflammatory (₁,₉). The myxoid type, also known as a myxofibrosarcoma, is the second most common type comprising 20% of all MFH. The myxofibrosarcoma is thought to be a variant, featuring less cellularity and a rich matrix; it also tends to be associated with a better prognosis than its more cellular counterparts (₁₀). The rate of metastases of myxofibrosarcomas decrease as their myxoid components increases. The myxomatous change in these tumors does not merely represent degenerative change but is regarded as a form of differentiation areas in which the tumor cells multiply more slowly and produce an abundant mucoid matrix thus explaining the better prognosis (₁₀,₁₁).}

MFH has been reported in irradiated areas or traumatized bone, in bone infarction, or in association with Paget disease and fibrous dysplasia. Mentzel et al. (₁₂) analyzed clinicopathological features of 75 cases of myxofibrosarcoma which had myxoid areas in at least 10% of the whole tumor. These lesions varied in appearance from a hypocellular, mainly myxoid and purely low-grade spindle cell to high-grade, pleomorphic (MFH-like) neoplasms with multinucleated giant cells, high mitotic activity and areas of necrosis. There was local recurrence in 33 of 60 (54%) cases between 2 and 72 months after surgery independently of the histological grade. Thirteen patients had metastases and 13 tumor-related deaths occurred. Most metastasizing lesions were high-grade.

Mentzel et al. (₁₂) classified myxofibrosarcoma into two groups: superficial (dermal/subcutaneous) and deep (intramuscular/mainly subfascial). The first group tends to infiltrate while the second group tends to form a single discrete mass. The first group is characterized by lesions composed of gelatinous or firmer nodules that often spread extensively in a longitudinal manner. Sometimes these tumors extend to the deep fascia, losing their nodular appearance and demonstrating a more infiltrative growth pattern. Differential diagnoses in our case included various sarcomas of the skull as well as metastatic lesions. Clinical, radiological and histological distinguishing features are listed in Table 1 (₁₃, ₁₄, ₁₅, ₁₆).

Figure 4

Table 1: Differential Diagnosis of Head and Neck Sarcomas

Recurrent myxofibrosarcoma have usually higher histological grades. The higher-grade neoplasms tend to metastasize. Primary low and intermediate grade lesions can be treated by wide resection. Higher grade lesions and recurrences may be treated by radiation or chemotherapy, however due to the small numbers of cases in the head and neck region this modality has not been well established. In a significant number of patients, local recurrence or metastases develop within 2 years (₁₅).

Correspondence to

Pragya Shukla, M.D. Department of Radiotherapy CSMMU, Lucknow Email: pragya_upadhyay@yahoo.co.in Phone number - +91- 510-2471553