Granular Cell Tumor of the breast
A granular cell tumor, sometimes also called breast granular cell myoblastoma, is an uncommon neoplastic cell growth than originates in the Schwann cells of the peripheral nervous system. Most of the time, these tumors grow in the head and neck region, but they occur in the breast in about 6% of cases. Breast granular cell tumors are usually benign, but there are some rare examples of malignant presentation.
I just want to let you know that I have created a newer version of this page with more up-to-date information on Granular Cell Breast Tumors, because well, this page is getting sort of a little out-of-date, and in need for improving – but you can still use it!
Granular cell breast tumors can take a while to diagnose, and it is usually quite a relief when they are confirmed because they can mimic breast cancer. Mammographic findings are typically suspicious-looking, and these breast tumors can also simulate carcinoma due to their fibrous consistency, fixation to the pectoral fascia, and common skin retraction and ulceration. The average age of diagnosis for breast granular cell tumors is about 40, but they can actually happen at any age.
Granular cell breast tumors may occur in both sexes
Granular cell breast tumors are generally 3cm or smaller. They usually have well-circumscribed margins, but can on occasion show infiltrative margins, which is more suggestive of a malignant breast cancer lesion. They were originally discovered in 1926 by Abrikossoff, who gave it the original name of ‘granular cell myoblastoma‘.
Breast granular cell tumors can also simulate breast cancer on a clinical breast examination because they can be fixed to the skin, and are frequently ‘rock hard‘. Granular cell breast tumors tend to be slow growing, and solitary, though they occur multiply about 10% of the time. Granular cell tumors, particularly multiple tumors, also tend to be slightly more common in persons with black skin. Granular cell tumors of the breast may occur in both sexes, but they are slightly more common in women.
Microscopic and histological aspects of breast granular cell tumors
Histological analysis of granular cell breast tumors typically reveals sheets and nests of polygonal cells. Granular eosinophilic cytoplasm is abundant, and cells usually have distinct borders. The granularity of these breast tumors is due to an accumulation of secondary lysosomes in the cytoplasm.
As a result, granular cell breast tumors will tend to stain positive for the S-100 protein, which also supports the idea that they derive from Schwann cells (Breast schwannoma and breast neurofibroma also derive mostly from Schwann cells, which are also found in malignant peripheral nerve sheath tumors of the breast). Immunohistochemical analysis of granular cell breast tumors tends to include reactivity for periodic acid-Schiff, CD68, and S100 and negative reactivity for cytokeratin.
Histological features of granular cell breast tumors can resemble apocrine carcinoma of the breast, but a main difference is the absence of mitotic figures in granular cell tumors. Granular cell breast tumors also tend to be negative for estrogen receptivity.
Features of breast granular cell tumors suggestive of malignancy
Histological features of granular cell breast tumors which are more suggestive of malignancy include a larger size tumor (greater than 5cm), cellular and nuclear pleomorphism, increased mitotic activity, prominent nucleoli, and the presence of necrosis. (A granular cell tumor which recurs locally after having been excised would also tend to indicate a malignant situation).
The only truly reliable measure of malignancy in a breast granular cell tumor would be evidence of metastasis. It must be remembered, however, that malignant granular cell breast tumors are very rare, totally less than 2% of all granular cell tumors. Malignancy is most often encountered with ‘deep-seated‘ lesions in older patients, with an average age of 50.
Diagnostic imaging of breast granular cell tumors tends to be suspicious of breast tumors
Breast cancer screening mammograms have the advantage of being highly sensitive (showing a suspicious density of mass) but not very specific (giving a clear indication of what the mass or density actually is). Breast granular cell tumors are rare, and mostly benign, but mammographic images tend to be highly suspicious of malignancy.
Mammograms tend to show a suspicious, spiculated mass, possibly with infiltrative margins. They can frequently also be microcalcifications associated with granular cell breast tumors, which, depending on their ‘pattern‘, can be highly suggestive of ductal carcinoma in situ. Breast ultrasounds of granular cell breast tumors typically reveal an irregularly defined hypoechoic mass (solid) with posterior shadowing (sometimes called ‘dorsal‘ shadowing).
Treatment of breast granular cell tumors
Wide local excision tends to be the way that granular cell breast tumors are treated, whether they are malignant or not. They have a tendency to recur so margins must be wide and completely free of tumor cells.
Adjuvant radiation therapy may be given, but typically only if the tumor is malignant. Unfortunately, removal of granular cell breast tumors tends to be a fairly major surgery, because they have a tendency to attach themselves to adjacent muscles and other structures.
Metastasis rate is quite high for granular cell breast cancer
Because there is a tendency for local recurrence and the remote possibility of distant metastasis, follow-up is a crucial aspect of treatment and management. The local recurrence rate of benign granular cell breast tumors ranges between 2% and 8%, while the recurrence rate for malignant forms is much higher, at around 35%. Local recurrence is typically rapid, however, usually within 1 year of the original surgery.
Distant metastasis of malignant granular cell breast tumors is quite high, at just over 60%. For unknown reasons, recurrence of malignant breast granular cell tumors is much more common for women than for men. However, bear in mind that for benign granular cell breast tumors, surgical excision is usually a complete cure.
For further reading, I suggest you visit this page with some information on fibrocystic disease of the breast.
- Tavassoli FA: Pathology of the Breast. Elsevier, New York, 1992
- Miller, JA., ; Karcnik, TJ.,Karimi, S; Granular cell tumor of the breast: definitive diagnosis by sonographically guided percutaneous biopsy. Journal of clinical ultrasound. 2000-Feb; vol 28 (issue 2) : pp 89-93
- Page DL, Anderson TJ: Diagnostic Histopathology of the Breast. New York, Churchill Livingstone, 1987
- Al-Ahmadie H, Hasselgren PO, Yassin R, Mutema G. Colocalized granular cell tumor and infiltrating ductal carcinoma of the breast.Arch Pathol Lab Med 2002 Jun;126(6):731-3
- Barkan GA, Paulino AF., Are epidermal growth factor and transforming growth factor responsible for pseudoepitheliomatous hyperplasia associated with granular cell tumors? Ann Diagn Pathol 2003 Apr;7(2):73-7
- Ray, S., Jukic, DM., Cutaneous granular cell tumor with epidermal involvement: a potential mimic of melanocytic neoplasia.J Cutan Pathol. 2007 Feb;34(2):188-94.
- Franzblau MJ, Manwaring M, Plumhof C, Listrom MB, Burgdorf WH. Metastatic breast carcinoma mimicking granular cell tumor.J Cutan Pathol 1989 Aug;16(4):218-21
- Scheithauer BW, Woodruff JM, Erlandson RA. Benign granular cell tumors and malignant granular cell tumors. In: Tumors of the Peripheral Nervous System, Atlas of Tumor Pathology. 3rd ed. Washington, DC: Armed Forces Institute of Pathology; 1997::248, 259, 358-65.
- Osipov, VO., Shidham, VB., Rao, N. Granular Cell Tumor. emedicine.medscape.com/article/282430-overview
- 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
- Mazur MT, Shultz JJ, Myers JL. Granular cell tumor. Immunohistochemical analysis of 21 benign tumors and one malignant tumor. Arch Pathol Lab Med. Jul 1990;114(7):692-6
- Montagnese MD, Roshong-Denk S, Zaher A et al. Granular cell tumor of the breast. Am Surg 2004;70:5254
- Abrikossoff, AI., Über myome, ausgehend von der quergestreiften willkürlichen Muskulatur. Virchows Archiv für pathologische Anatomie und Physiologie und für klinische Medizin, 1926, 260: 215-233.
- Sposto, MR>, Navarro, CM., de Andrade, CR., Granular cell tumour (Abrikossoffs tumour): Case series. Oral Oncology Extra Volume 42, Issue 5, May 2006, Pages 194-197
- Fanburg-Smith,JC., Meis-Kindblom,JM., Fante, R., and Kindblom,LG. Malignant granular cell tumour of soft tissue: diagnostic criteria and clinicopathologic correlation, Am J Surg Pathol 22 (1998) (7), pp. 779794.
- Strong EW, McDivitt RW, Brasfield RD. Granular cell myoblastoma. Cancer. Feb 1970;25(2):415-22
- Green, DH., Clark, AH. Case Report: granular cell myoblastoma of the breast, a rare benign tumor mimicking breast carcinoma. Clin Radiol 1995; 50:799.