Malignant fibrous histiocytoma belongs to the group of soft tissue sarcomas and is rare in persons under 20 years of age. It most commonly occurs in the lower limbs

Malignant Fibrous Histiocytoma belongs to the group of soft tissue sarcomas and is one of the most frequent tumours in the 50-70 age group

It is very rare in people under the age of 20. It prefers the male sex.

It consists of histiocytic and fibroblastic cells with a typical and characteristic ‘storiform’ arrangement, i.e. arranged in irregularly intertwined spirals, similar to that of a straw mat.

Malignant Fibrous Histiocytoma can also affect bone as well as soft tissue with a certain frequency

There are five histological subtypes: ‘storiform-pleomorphic’, ‘myxoid’, ‘giant cell’, ‘inflammatory’, ‘angiomatoid’.

Of these, ‘storiform-pleomorphic’ is the most common, accounting for more than 70% of cases.

The myxoid variant is the second most common, accounting for 20% of cases.

The cause of the development of this disease, like the other soft tissue sarcomas, remains unknown.

In exceptional cases, a familial recurrence of soft tissue sarcomas has been observed.

Genetic studies are currently underway to attempt to define a possible genetic susceptibility in these families.

Malignant Fibrous Histiocytoma can develop in any part of the body but most commonly in the lower limbs, especially the thigh

Other common localisations are the upper limbs and retroperitoneum, as well as intra-abdominal and intrathoracic sites, although these are rarer.

Patients often complain of a swelling that grows over a short period of time, ranging from weeks to months.

It is quite common for patients to report trauma to the affected area, which is not the cause but rather the event that allows the tumour to be discovered.

The mass usually does not cause pain unless it compresses an adjacent nerve structure.

Symptoms such as weight loss and fatigue are not typical but may occur in patients with an advanced stage of disease.

Tumours of the retroperitoneum may become very large before manifesting themselves through symptoms such as anorexia or increasing pressure in the abdomen.

In the presence of a mass suspected to be sarcoma, a fragment of the tumour must be taken and analysed by the pathologist (possibly an expert in this type of lesion), who is the specialist able to make the diagnosis.

There are also further investigations to assess the characteristics and extent of this tumour:

• Nuclear magnetic resonance imaging (MRI) and/or computed tomography (CT) of the involved region, which provides very important information including the size of the lesion, exact location and proximity to neuro-vascular structures;
• Radiography or computed tomography of the chest, bone scintigraphy and/or positron emission tomography-PET for the evaluation of any distant secondary lesions.
• In specific situations, exploration of any suspicious lymph nodes, satellites of the lesion, may be necessary.

The type of treatment for this tumour depends mainly on the site and characteristics of the tumour, as well as its extent.

Surgery is the mainstay of treatment, and in cases where removal of the lesion is radical, it constitutes the only treatment.

In cases where excision is incomplete, an adjunct to local treatment with radiotherapy may be necessary.

The role of chemotherapy is not entirely clear, as tumours are poorly sensitive to chemotherapeutic drugs.

The drugs that have been most widely used are ifosfamide and adriamycin, which have not shown a significant advantage in terms of improving survival.

Chemotherapy is therefore almost exclusively reserved for patients with remotely recurrent lesions.

Prognosis in patients with Malignant Fibrous Histiocytoma depends on factors related to both tumour and patient

These include tumour size and site, histological subtype, resectability, and patient age.

Age over 60 years, lesions larger than 5 cm, non-radically resectable, non-mixoid histological subtypes, as well as the presence of distant repetitive lesions represent unfavourable prognostic factors.

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Source

Bambino Gesù