Bardají Bofill Manel

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Soft tissue Tumors of the head and neck

Soft tissue tumors of the head and neck are neoplasms that arise from the tissues of the facial and cervical region and do not originate in the osteocartilaginous skeleton, nor in the trachea of the esophagus, the larynx, the thyroid, the parathyroids and the salivary glands. They represent 10% of all soft tissue tumors. Other exclusions are skin tumors, central nervous system tumors, lymph node metastases. Including the derivatives of the vascular endothelium and excluding congenital processes such as lymphangioma and teratoma.

100 benign and 50 malignant entities have been described, with very different behaviors. Its frequency is estimated at 2/100 000 inhabitants/year, they are more frequent in children (6.5% in children and 1% in adults. The benign/malignant ratio varies according to the series from 2/1 to 100/1.The most common form of presentation is that of a mass that produces compressive symptoms in contiguous neurovascular structures. Occasionally, lesions can be located on the skin, such as angiosarcomas and dermatofibrosarcoma protuberans. Benign lesions have a slow and gradual growth, although in other benign lesions such as nodular fasciitis this is rapid and infiltrative. Despite this exception, a well-defined surface mass, less than 5 cm., of slow growth presents a high possibility of being benign; While if it is deep, it grows quickly, has a peritumoral infiltrative appearance and a size greater than 5 cm. it is more likely to be malignant.

Benign soft tissue tumors of the large neck may have compressive symptoms that depend on the location of the tumor and contiguous structures. In general, the most striking are the neurological symptoms (paresthesias, paralysis, neuralgia), which may coincide with a tumor originating from the peripheral nerve. In this case it is not possible to move the mass in the direction of the axis of the nerve, although it is possible in its perpendicular axis. Rarely, paralysis is caused by compression outside the cervical region, as described in cervical lipomatosis, which infiltrates the mediastinum and produces bilateral paralysis of the recurrent, Other times the predominance of symptoms is vascular (larte mass, hypertensive crises, hemorrhagic diathesis) seems to indicate a vascular tumor. In the case of settling on the carotid axis, it is only possible to move it laterally.

The same neurovascular symptomatology can be observed in malignant tumors, although by infiltative mechanism. In advanced lesions or high degree of malignancy, inflammatory reactions, hemorrhages, necrosis and ulcerations that presume their character are also produced.

In case of suspicion, an FNA is required, which can guide but is not always the definitive diagnosis. It has the advantage of avoiding the dissemination that would cause the incisional biopsy, which should only be considered in case of inoperability of the tumor or in the same surgical act, proceeding then to its complete removal.

Excisional biopsy is recommended in benign lesions, well encapsulated, provided that their size allows it. It is not the appropriate procedure for sarcomas, especially if they are larger than 3 cm., although they have a good plane of detachment.

Once the tissue diagnosis has been established, the extent of the tumor must be known, as well as the secondary involvement of the great vessels and the upper respiratory and digestive tract, using imaging techniques such as CT and MRI; As well as investigate the possibility of metastasis in other organs (lungs, liver and bones).

Type and approximate frequency of cervicofacial soft tissue tumors.

Tissue	Tumor Benigno	Malignant tumor
Adipose	Lipoma (27%)	Liposarcoma (8%)
Fibrous Connective	Fibroma (2%) Fascitis nodular (15%)	Fibrosarcoma (10%) Fibrohistiocitoma(20%) Dermatofibrosarcoma P.
Nerves	Neurofibroma(7%) Schwanoma(4%)	Neurogenic sarcoma(1%) Malignant schwanoma (10%)
Vascular	Hemangioma (11%) Paraganglioma(1%) Linfangioma	Hemangiosarcoma(7%)
Smooth muscle	Leiomyoma	Leiomiosarcoma
Striated muscle	Rabdomioma	Rhabdomyosarcoma
Synovial	Giant cell tumor	Synovial sarcoma

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Soft Tissue Sarcoma
The evolution of soft tissue sarcomas at first compress the surrounding tissue creating a reactive zone of circumcised tissue creating a reactive zone of edematous tissue without vessels that originates a pseudocapsule. This false barrier does not prevent the tumor from spreading along the facial septums and between the muscle fascicles in the form of small tentacles outside the central point, presenting a multinodular configuration. The tumor progresses and infiltrates different tissues, nerve and muscle fibers reaching the adventitia of the vessels, where the process of distant metastasis begins. Patients with sarcoma in the neck have up to 40% of metastases has distance. Its production mechanism is related to the size and depth of the tumor. Intrinsic aggressiveness, treatment performed and the body's own immune response. The treatment is surgical, although individualized according to the cases, and then chemotherapy.
Lipoma
Lipomas are the most frequent benign tumors of mesenchymal origin and 20% are located in the head and neck. They affect more males (70%) and between the fourth and sixth decade.

Most are subcutaneous and located in the back of the neck, although they are also seen in the anterior part. The only clinical manifestation is a painless mass with slow growth or stationary, not usually exceeding 5 cm. of maximum diameter. They can be multiple, usually in family cases. The diagnosis is established by its clinical appearance and it is possible to confirm it by FNA. The radiological appearance of lipomas is very characteristic. In the TAC they are presented as well-defined masses with the same density as fat. MRI shows hyperintense in T1 and hypointense in T2. A variant is multiple lipomatosis or Madelung's disease, characterized by gradual deposit of fatty tissue in superficial location of the neck and in the retroauricular, suboccipital and parotid regions, producing a characteristic aesthetic deformity. It occurs in middle-aged men with a history of alcoholism or liver disease. The tanks are not encapsulated and are poorly defined with deep extensions which makes it difficult to extract them

Another variety is infiltrating lipomas that originate in the intermuscular fasciae and grow between the muscle fascicles and muscle fibers infiltrating as they grow. They can be confused with low-grade liposarcomas, however no malignant degeneration of lipomas has been demonstrated. Clinically they are asymptomatic and give symptoms if the growth of the tumor comes to produce compression of neighboring structures. Surgical treatment is of choice.
Lymphangioma
Lymphangiomas are developmental disorders of the lymphatic system, representing 2 to 5% of benign congenital cervical masses. Despite being considered congenital disorders, they can occur at any age, although 90% occur before 2 years of age.

The most frequent types of lymphangiomas are the cavernous, formed by dilated lymphatic ducts, and the cystic hygroma, composed of large cystic spaces containing clear, whitish or hemorrhagic liquid, and it is common for both types to coexist in the same lesion. Cavernous lymphangiomas have a greater tendency to infiltration and poorly defined edges, so their complete excision is difficult.

Children often have significant functional problems resulting from obstruction of the aerodigestive tract, while adults often have asymptomatic masses. These are soft, lobed, depressible masses fixed to deep planes, but not painless skin on palpation. They are not pulsatile, nor are they modified with the valsalva, they are translucent in transillumination. Quite often the patient reports rapid and progressive growth of the mass over the course of a few weeks and sometimes suddenly by a respiratory infection or intracystic hemorrhage. They have been described most frequently in the posterior triangle of the neck after sternocleidomastoid.

On CT, lymphangiomas appear as cystic masses, with homogeneous content of low density and poorly defined thin walls that show increased signal after administration of intravenous contrast. MRI shows a more characteristic of low intensity in T1 and high intensity in T2, which reflects the high free water content of the cysts.

Different forms of treatment were tried, such as esclesrosant injection, electrocoagulation and cryotherapy, but surgical removal is still the most ideal, although complete resection is difficult, although small islets of lymphangioma tend to involute.
Hemangioma
They are lesions that are usually present at birth, being in the head and neck area where they settle up to 65%. Being the most common cause of increased parotid in the newborn. Hemangiomas are composed of a proliferation of blood vessels with differentiated endothelial characteristics. Hemangiomas develop during the first years of life, returning up to 50% of them, involuting towards 4 or 5 years, in contrast to vascular malformations that do not remit with age.

Clinically they are a diffuse cutaneous mass or a deep soft mass. On the skin there is a purple, soft, poorly defined color that pales to compression, having a worm sack touch. Radiologically they present phlebolitos in 30%, and in the MRI hyperintense in T2, however the scintigraphy is the best diagnosis using red blood cells labeled with TC99, specific to hemangiomas. x

It is recommended to postpone the surgical intervention, at least until five years waiting for a remission, otherwise, excision by total parotidectomy with preservation of the facial nerve will be indicated.

Nerve sheath tumors

This type includes two types of peripheral nerve tumors, Schawnomas or neurinomas and neurofibromas. 35% of these injuries are found in the head and neck. The most common location excluding the Schwanoma of the VIII pair, is the lateral cervical chain, being lesions that derive from the brachial or cervical plexus, those that affect the parapharyngeal space are fundamentally of origin in the vagus or spinal chain. Clinically they are asymptomatic masses of years of evolution, over time and according to their location they will present compressive symptoms such as dysphagia or dysphonia, neurological symptoms are rare except in large tumors. FNA helps diagnose, but does not differentiate between malignant and benign tumors, and it is even difficult to differentiate histologically. Its treatment is surgical, and variable depending on the place and size of the lesion.

Derivatives of gill arches

Bow	Skeletal derivative	Nerves	Arterial Derivative
1º Mandibular	Mandibula Masticatory muscles Belly ANT Digastric Mylohyoid	Mandibular V3 String Eardrum	Disappears
2º Hioid	Hyoid Top Platysma, Facial Expression Muscles	Facial VII	Art. Estapedial
3º Arc	Hyoid Body major horn Upper and Middle Constrictor of the pharynx	Glosofaringeo	Carotid Int.Carotid Ext.
4º Arc	Thyroid Cartilage	Upper laryngeal	Cayado Aortic
6º Arc	Cricoides and Aritenoids	Recurrent	Ductus Arteriosus

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Centro Médico Teknon

Bardají Bofill Manel

Consultorios Marquesa (despacho 34)

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