Cagri Karaciklar Benign Soft Tissue Tumors

Cagri KARACIKLAR
16 April 2020
Benign Soft Tissue Tumors

Soft tissue is defined as the supportive tissue of various organs and the non-epithelial, extraskeletal
structures exclusive of lymphohematopoietic tissues. It includes fibrous connective tissue, adipose tissue,
skeletal muscle, blood/lymph vessels, and the peripheral nervous system. Embryologically, most of it is
derived from mesoderm, with a neuroectodermal contribution in the case of peripheral nerves.
Soft tissue tumors develop in connective tissue other than bone such as the skeletal muscle, fat, tendon,
fibrous tissue and nerve and blood vessel (neurovascular) tissue.
Soft tissue tumors can occur anywhere in the body but are most frequent in the lower extremities, trunk
and abdomen and upper extremities. The cause is unknown. Trauma may bring attention to a previous
existing tumor.
Benign soft tissue tumors rarely metastasise. They are categorised according to their behaviour to be
aggressive or not, ranging from inactive, to active, to aggressive.
In adults, the most common benign soft tissue tumor is a lipoma. In children, popliteal (Baker's) cysts,
lipomas and hemangiomas are common. Benign tumors may be very large and deep but are usually soft -
except for the fibrous tumors which may feel firm.
Etiology
Genetic conditions
Good evidence exists suggesting that certain genetic disorders and gene mutations
are predisposing factors for some benign and malignant soft-tissue tumors. The
NF1 gene in neurofibromatosis is a classic example, predisposing patients to
multiple neurofibromas with a proclivity for malignant transformation. Many tumor suppressor genes,
oncogenes, and cytogenetic defects are now associated with various soft-tissue sarcomas. Other clinical risk
factors account for a small proportion of soft tissue malignancies.
Radiation
Chronic lymphedema
Environmental carcinogens
Infection
Trauma
BENIGN SOFT TISSUE TUMORS 1

Types of Benign Soft Tissue Tumors
Lipomas
The most common benign soft tissue tumor made of fat and is usually large and soft. They are located on
the back, shoulders, abdomen and upper and lower extremities. They usually do not cause symptoms unless
they begin to push on nearby nerves. Lipomas usually grow slowly with little change over many years. Many
patients seek medical attention for cosmetic reasons when a lipoma grows too large. An angiolipoma which
involves blood vessels usually occurs in children located deep in the muscle and is tender because it involves
the vessels. On the MRI scan, the lipoma is seen as a bright, well defined mass. Treatment includes removing
(excising) the mass or observing it if it is not bothersome. Recurrences are unusual.
Hemangioma
A benign vascular (involving blood vessels) soft tissue tumor. It is the most common soft tissue tumor in
children. It can develop in the top skin (superficial) layer or deep within the muscle. They occur more often in
the lower extremities than the upper extremities. Hemangiomas can range from being well-defined,
noninvasive and involving small vessels (capillaries) to less defined, invasive and involving large vessels. The
most common type of hemangioma is made of both large and small vessels. The MRI scan is the best type
of imaging to show the location and extent of the tumor. Angiograms may also be done to evaluate the large
blood vessels.

Treatment is not necessary unless they cause pain. Aspirin and compression stockings may be quite successful
in relieving symptoms. Surgical excision is sometimes necessary if symptoms persist. Although hemangiomas
are vascular, they do not spread to other areas or turn malignant. Occasionally for large tumors that can not
be removed, radiation therapy and embolization (a procedure to plug/stop the blood supply in the tumor
area preventing tumor growth) has been attempted to treat hemangiomas with little success. Recently,
treatment has also included injections with alcohol into the tumor (sclerotherapy).
Fibroma & Fibromatosis

Benign soft tissue tumors made of fibrous tissue come in many different forms. Congenital fibromatosis
occurs in infants and usually presents as a solitary mass. Fibrous tumors in adolescents and adults include
fibromas, extra-abdominal desmoid tumors, palmer fibromatosis and nodular fasciitis. Fibromatosis (desmoid
tumor) refers to a benign but aggressive tumor usually located in the lower extremity. Fibromatoses are very
aggressive at the original site and invade the nearby muscle, soft tissue and bone but do not metastasize
(spread to other sites). MRI scans or CT scans are done preoperatively. Plain x-rays will rule out any bone
involvement under the soft tissue mass, and a bone scan will show any other bony invasion.
Treatment includes removal (excision) of the soft tissue mass. In

some cases the surgical margin (area around the tumor) is not large
enough because the tumor extends microscopically beyond the main
mass. Thus, there is a high rate of recurrence. These tumors should
be treated aggressively with wide margin excisions. Radiation
therapy is also used for local control.
Neurofibroma & Neurofibromatosis

Benign soft tissue tumors that are made of nerve tissue. They are also called nerve sheath tumors or
schwannomas because the tumor tissue has cells like the nerve sheath (envelope around the nerve fibers), or
Schwann cell. Neurofibromas may occur as a single tumor or many tumors and may be found anywhere on
the body. They range from simple, small masses to gross enlargement of an extremity (elephantiasis), to
masses causing bony erosion and deformities of the spine. Since neurofibromas come from nerve roots, those
located in the spine can press on the spinal cord causing neurologic symptoms.

Most often they are observed and not excised unless they
are growing or painful. If a sarcoma develops, the patient
must undergo staging studies to rule out metastatic
disease. Pre- or post-operative radiation therapy is usually
included in the treatment plan, and sometimes
chemotherapy.
Numerous cutaneous neurofibromas (A.) and a large

plexiform neurofibroma (B.).
Granular cell tumors

These are usually benign tumors in adults that often start in the tongue but can be found almost anywhere in
the body.
Schwannomas
These are benign tumors of the cells that coat nerves.
Myositis Ossificans
A non-tumorous soft tissue mass that occurs after a blunt trauma. The soft tissue reacts to the trauma by
forming a bony mass. Patients present with a painful or painless mass which is increasing in size. The tumor
is usually located in the upper arm, thigh or buttock. X-rays show a round ossified (bony) mass which may be
read as a sarcoma. However, the myositis ossificans has a distinct margin of ossification (bone formation) with
an active centre containing immature cells.
Ganglion or Synovial Cyst
A benign soft tissue mass 'cyst like' in nature and comes from the capsule of the joint, synovial membrane
(lining) or tendon sheath (covering). It usually develops on the top of the wrist in adults between 25 and 45
years of age, and are more common in women.

References
1. Conrad EU 3rd, Bradford L, Chansky HA. Pediatric soft-tissue sarcomas. Orthop Clin North Am. 1996
Jul.
2. Ludwig JA. Personalized therapy of sarcomas: integration of biomarkers for improved diagnosis,
prognosis, and therapy selection. Curr Oncol Rep. 2008 Jul. 10
3. Ordóñez JL, Martins AS, Osuna D, Madoz-Gúrpide J, de Alava E. Targeting sarcomas: therapeutic
targets and their rational. Semin Diagn Pathol. 2008 Nov. 25
4. Dal Cin P. Soft tissue tumors: an overview. Atlas of Genetics and Cytogenetics in Oncology and
Haematology. Available at http://AtlasGeneticsOncology.org/Tumors/softissuTumID5042.html. July 2013
5. Mankin HJ, Mankin CJ, Simon MA. The hazards of the biopsy, revisited. Members of the
Musculoskeletal Tumor Society. J Bone Joint Surg Am.

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Cagri KARACIKLAR

Benign Soft Tissue Tumors

BENIGN SOFT TISSUE TUMORS 1

BENIGN SOFT TISSUE TUMORS 2

Fibroma & Fibromatosis

Treatment includes removal (excision) of the soft tissue mass. In

Neurofibroma & Neurofibromatosis

BENIGN SOFT TISSUE TUMORS 3

Numerous cutaneous neurofibromas (A.) and a large

Granular cell tumors

BENIGN SOFT TISSUE TUMORS 4

BENIGN SOFT TISSUE TUMORS 5

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