Granular cell tumor is a

tumor A neoplasm () is a type of abnormal and excessive growth of tissue. The process that occurs to form or produce a neoplasm is called neoplasia. The growth of a neoplasm is uncoordinated with that of the normal surrounding tissue, and persists ...

that can develop on any

skin Skin is the layer of usually soft, flexible outer tissue covering the body of a vertebrate animal, with three main functions: protection, regulation, and sensation. Other animal coverings, such as the arthropod exoskeleton, have different ...

or mucosal surface, but occurs on the

tongue The tongue is a Muscle, muscular organ (anatomy), organ in the mouth of a typical tetrapod. It manipulates food for chewing and swallowing as part of the digestive system, digestive process, and is the primary organ of taste. The tongue's upper s ...

40% of the time. It is also known as Abrikossoff's tumor, granular cell myoblastoma, granular cell nerve sheath tumor, and granular cell schwannoma. Granular cell tumors (GCTs) affect females more often than males.

Pathology

Granular cell tumors are derived from neural tissue, as can be demonstrated by

immunohistochemistry Immunohistochemistry is a form of immunostaining. It involves the process of selectively identifying antigens in cells and tissue, by exploiting the principle of Antibody, antibodies binding specifically to antigens in biological tissues. Alber ...

and ultrastructural evidence using

electron microscopy An electron microscope is a microscope that uses a beam of electrons as a source of illumination. It uses electron optics that are analogous to the glass lenses of an optical light microscope to control the electron beam, for instance focusing i ...

. These lesions characteristically consist of polygonal cells with bland nuclei, abundant cytoplasm and fine eosinophilic cytoplasmic granules. The tumor cells stain positively for S-100 as they are of Schwann cell origin. Both malignant and benign versions of the tumor exist, where malignant tumors are characterized histologically by features such as spindling, high nuclear to cytoplasmic ratios, pleomorphism, and necrosis. Multiple granular cell tumors may seen in the context of '' LEOPARD syndrome'', due to a mutation in the ''

PTPN11 Tyrosine-protein phosphatase non-receptor type 11 (PTPN11) also known as protein-tyrosine phosphatase 1D (PTP-1D), Src homology region 2 domain-containing phosphatase-2 (SHP-2), or protein-tyrosine phosphatase 2C (PTP-2C) is an enzyme that in hu ...

gene In biology, the word gene has two meanings. The Mendelian gene is a basic unit of heredity. The molecular gene is a sequence of nucleotides in DNA that is transcribed to produce a functional RNA. There are two types of molecular genes: protei ...

''. These tumors, on occasion, may appear similar to neoplasms of renal (relating to the kidneys) origin or other soft tissue neoplasms.

Treatment

The primary method for treatment is surgical, not medical. Radiation and

chemotherapy Chemotherapy (often abbreviated chemo, sometimes CTX and CTx) is the type of cancer treatment that uses one or more anti-cancer drugs (list of chemotherapeutic agents, chemotherapeutic agents or alkylating agents) in a standard chemotherapy re ...

are not needed for benign lesions and are not effective for malignant lesions. Benign granular cell tumors have a recurrence rate of 2% to 8% when

resection margin A resection margin or surgical margin is the edge or "margin" of apparently non-tumorous tissue around a tumor that has been surgically removed, called " resected", in surgical oncology. The resection is an attempt to remove a cancer tumor so th ...

s are deemed clear of tumor infiltration. When the resection margins of a benign granular cell tumor are positive for tumor infiltration the recurrence rate is increased to 20%. Malignant lesions are aggressive and difficult to eradicate with surgery and have a recurrence rate of 32%.

Epidemiology

Granular cell tumors can affect all parts of the body; however, the head and neck areas are affected 45% to 65% of the time. Of the head and neck cases 70% of lesions are located intraorally (

oral mucosa The oral mucosa is the mucous membrane lining the inside of the mouth. It comprises stratified squamous epithelium, termed "oral epithelium", and an underlying connective tissue termed '' lamina propria''. The oral cavity has sometimes been des ...

hard palate The hard palate is a thin horizontal bony plate made up of two bones of the facial skeleton, located in the roof of the mouth. The bones are the palatine process of the maxilla and the horizontal plate of palatine bone. The hard palate spans ...

). The next most common location that lesions are found in the head and neck area is the

larynx The larynx (), commonly called the voice box, is an organ (anatomy), organ in the top of the neck involved in breathing, producing sound and protecting the trachea against food aspiration. The opening of larynx into pharynx known as the laryngeal ...

(10%). Granular cell tumors are also found in the internal organs, particularly in the upper aerodigestive tract. Vaginal granular cell tumors are generally rare. Breast granular cell tumors arise from intralobular breast stroma and occurs within the distribution of the cutaneous branches of the supraclavicular nerve. As they do follow the innervation of the skin, they may demonstrate skin changes like contraction or shrinkage. Unlike traditional

breast cancer Breast cancer is a cancer that develops from breast tissue. Signs of breast cancer may include a Breast lump, lump in the breast, a change in breast shape, dimpling of the skin, Milk-rejection sign, milk rejection, fluid coming from the nipp ...

s, granular cell tumors are mostly found in the upper inner quadrant of the breast. They can be erroneously diagnosed as an invasive ductal carcinoma via

ultrasound Ultrasound is sound with frequency, frequencies greater than 20 Hertz, kilohertz. This frequency is the approximate upper audible hearing range, limit of human hearing in healthy young adults. The physical principles of acoustic waves apply ...

and

mammography Mammography (also called mastography; DICOM modality: MG) is the process of using low-energy X-rays (usually around 30 kVp) to examine the human breast for diagnosis and screening. The goal of mammography is the early detection of breast cance ...

, therefore, it is necessary to consider a diagnosis of invasive ductal carcinoma. The usual presentation is of a slow growing behavior, forming a polygonal accumulation of secondary lysosomes in the

cytoplasm The cytoplasm describes all the material within a eukaryotic or prokaryotic cell, enclosed by the cell membrane, including the organelles and excluding the nucleus in eukaryotic cells. The material inside the nucleus of a eukaryotic cell a ...

. Granular cell tumors are typically solitary and rarely larger than three centimeters. However, proliferative growth and development of an ulcer indicates likely malignancy as this type of tumor can be either

benign Malignancy () is the tendency of a medical condition to become progressively worse; the term is most familiar as a characterization of cancer. A ''malignant'' tumor contrasts with a non-cancerous benign tumor, ''benign'' tumor in that a malig ...

malignant Malignancy () is the tendency of a medical condition to become progressively worse; the term is most familiar as a characterization of cancer. A ''malignant'' tumor contrasts with a non-cancerous benign tumor, ''benign'' tumor in that a malig ...

. Malignancy is rare and constitutes only 2% of all granular cell tumors.Fanburg-Smith JC, Meis-Kindblom JM, et al. Malignant granular cell tumor of soft tissue: diagnostic criteria and clinicopathologic correlation. Am J Surg Pathol. Jul 1998;22(7):779-94.

References

External links

{{DEFAULTSORT:Granular Cell Tumor Oral neoplasia Dermal and subcutaneous growths

Pathology

Treatment

Epidemiology

See also

References

External links