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granular cell tumor

Tuesday 16 June 2009

Abrikossoff tumor, Granular cell myoblastoma, Granular cell nerve sheath tumor, Granular cell schwannoma

Definition: Slow-growing, usually benign tumor, of debatable histogeneses, that often involves the skin, mucosa, or connective tissue, composed of large cells with highly granular cytoplasm due to accumulation of lysosomes.

Images

Granular cell tumor and mastocytosis

https://twitter.com/Levelsx5/status/736700850295308288

Esophageal granular cell tumor with pseudoepitheliomatous hyperplasia (differential : squamous cell carcinomas )

https://twitter.com/Konzult_/status/739536733432389633

cutaneous granular cell tumor

https://twitter.com/NMacagnoMD/status/1068226203268583424

Digital slides

HPC:73 (HPC:73) : Mammary granular cell tumor
HPC:63 (HPC:63) : Gingival granular cell tumor (Neonatal epulis)
HPC:369 : Tracheal invasive granular cell tumor
UI:340 - Granular cell tumor
pathxchange.org #3106 : Esophageal granular cell tumor
JRC:5333 : Mammary granular cell tumor

Granular cell tumor is a tumor that can develop on any skin or mucosal surface, but occurs on the tongue 40% of the time.

The formation of a granular cell tumor is a neoplastic process and the lesions formed are of neural derivation, as supported by immunophenotypic and ultrastructural evidence.

The usual presentation is of slow growing behavior, forming a polygonal accumulation of secondary lysosomes in the cytoplasm.

Granular cell tumors are typically solitary and are rarely larger than three centimeters.

Localization

The neoplasm 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 intra-orally (tongue, oral mucosa, hard palate). The next most common location that lesions are found in the head and neck area is the larynx (10%).

Granular cell tumors are also found in the internal organs, particularly in the upper aerodigestive tract.

tongue
skin
vulva
breast
larynx
bronchus
esophagus
stomach
appendix
rectum
anus
bile ducts
pancreas
urinary bladder
uterus
brain
pituitary gland
soft tissue

Epidemiology

May be multiple (particularly if black patient)
Occasionally congenital (most in gingiva: neonatal epulis)
some with systemic involvement
Usually benign clinical course
Few cases with distant metastases

Macroscopy

Usually small

up to 5 cm diameter

Hard consistency
Ill-defined margins
Sometimes ulcerate if larger and cutaneous
can look like a malignant neoplasm
Rarely polypoid

Microscopy

large cells:
large cytoplasm
highly granular cytoplasm (granular cell tumors)
The cells contain innumerable fine cytoplasmic granules as well as scattered larger eosinophilic globules.
granules

mostly small and regular
alternate with larger round droplets with homogeneous eosinophilic appearance and stronger PAS positivity

stroma

often elastosis

Secondary epithelial hyperplasia:

if near an epithelial surface in sites such as skin, vulva, larynx
may be incorrectly diagnosed as carcinoma
- Pseudoepitheliomatous hyperplasia in squamous epithelium overlying granular cell tumor.

Special Stains

Immunohistochemistry

Cytoplasmic granules: contain large amounts of hydrolytic enzymes such as acid phosphatase, consistently positive for Luxol fast blue

At least some lesions positive for:

S-100 protein
laminin
calretinin
α subunit of inhibin
HLA-DR:
myelin basic protein:
CEA

Differential diagnosis

schwannoma
neurofibroma
alveolar soft part sarcoma
make diagnosis only when entire lesion granular
designate other cases according to basic component, noting presence of focal granular changes

Ultrastructure

cytoplasmic granules have appearance of lysosomes
second cell population with ‘angulated bodies’ resulting in Gaucher cell-like appearance
replicated basal lamina material around granular cells, suggesting repeated cycles of cellular injury and repair

Management

The primary method for treatment is surgical, not medical.

Radiation and chemotherapy are not needed for benign lesions and are not effective for malignant lesions.

Prognosis

This type of tumor has been found to be both benign and malignant, although malignancy is rare and comprises only 2% of all granular cell tumors.

Benign granular cell tumors have a recurrence rate of 2% to 8% when resection margins 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%.

Pathogenesis

Histogenesis controversial
most favor Schwann’s cell origin
but sometimes no evidence of Schwann’s cell participation
indistinguishable changes in neoplastic and non-neoplastic smooth muscle cells and in tumoral ameloblasts
may therefore be expression of a degenerative change resulting in cytoplasmic accumulation of lysosomes in a variety of cell types whether:

previously normal
part of a benign or a malignant neoplasm such as:
- MPNST
- leiomyosarcoma
- angiosarcoma

See also

Tumors

References

Garancis JC, Komorowski RA, Kuzma JF. Granular cell myoblastoma. Cancer. 1970;25:542–550.

Garancis JC, Komorowski RA, Kuzma JF. Granular cell myoblastoma. Cancer. 1970;25:542–550.Lack EE, Worsham GF, Callihan MD, Crawford BE, Klappenbach S, Rowden G, et al. Granular cell tumor. A clinicopathologic study of 110 patients. J Surg Oncol. 1980;13:301–316.

Garancis JC, Komorowski RA, Kuzma JF. Granular cell myoblastoma. Cancer. 1970;25:542–550.McSwain GR, Colpitts R, Kreutner A, O’Brien PH, Spicer S. Granular cell myoblastoma. Surg Gynecol Obstet. 1980;150:703–710.

Localization

Chandrasoma P, Fitzgibbons P. Granular cell tumor of the intrapancreatic common bile duct. Cancer. 1984;53:2178–2182.

Copas P, Dyer M, Hall DJ, Diddle AW. Granular cell myoblastoma of the uterine cervix. Diagn Gynecol Obstet. 1981;3:251–254.

Demay RM, Kay S. Granular cell tumor of the breast. Pathol Annu. 1984;19(Pt 2):121–148.

Garancis JC, Komorowski RA, Kuzma JF. Granular cell myoblastoma. Cancer. 1970;25:542–550.Johnston J, Helwig EB. Granular cell tumours of the gastrointestinal tract and perianal region. A study of 74 cases. Dig Dis Sci. 1981;26:807–816.

Garancis JC, Komorowski RA, Kuzma JF. Granular cell myoblastoma. Cancer. 1970;25:542–550.Sakurama N, Matsukado Y, Marubayashi T, Kodama T. Granular cell tumour of the brain and its cellular identity. Acta Neurochir (Wien). 1981;56:81–94.

Garancis JC, Komorowski RA, Kuzma JF. Granular cell myoblastoma. Cancer. 1970;25:542–550.Moscovic EA, Azar HA. Multiple granular cell tumors (“myoblastomas”). Case report with electron microscopic observations and review of the literature. Cancer. 1967;20:2032–2047.Vance SF, Hudson RP. Granular cell myoblastoma. Clinicopathologic study of 42 patients. Am J Clin Pathol. 1969;52:208–211.

Garancis JC, Komorowski RA, Kuzma JF. Granular cell myoblastoma. Cancer. 1970;25:542–550.Moscovic EA, Azar HA. Multiple granular cell tumors (“myoblastomas”). Case report with electron microscopic observations and review of the literature. Cancer. 1967;20:2032–2047.Seo IS, Azzarelli B, Warner TF, Goheen MP, Senteney GE. Multiple visceral and cutaneous granular cell tumors. Ultrastructural and immunocytochemical evidence of Schwann cell origin. Cancer. 1984;53:2104–2110.

Garancis JC, Komorowski RA, Kuzma JF. Granular cell myoblastoma. Cancer. 1970;25:542–550.Moscovic EA, Azar HA. Multiple granular cell tumors (“myoblastomas”). Case report with electron microscopic observations and review of the literature. Cancer. 1967;20:2032–2047.de la Monte SM, Radowsky M, Hood AF. Congenital granular-cell neoplasms. An unusual case report with ultrastructural findings and a review of the literature. Am J Dermatopathol. 1986;8:57–63.

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