Spindle Cell Carcinoma of the Breast

Spindle cell carcinoma of the breast is a rare sub-type of breast cancer, that falls within the general category of metaplastic breast carcinoma. There are really no clinical symptoms that would distinguish this particular cancer from other types of breast cancers. Thus diagnosis is dependent on the histological evaluation of tissue samples from a biopsy.

As the name suggests, these type of breast tumors tend to have spindle shape cells along with some other component of either

The working definition of spindle cell cancer of the breast is still a little bit vague, which is not unusual in a tumor that features a mixture of various elements.

There is also a lack of clarity as to the genetic origin of the spindle cell component in these breast cancers. Some researchers feel that malignant spindle cells are of a myoepithelial cell origin, while others maintain that malignant spindle cells originate in epithelial cells that have somehow genetically evolved into spindle cells.

Just to let you know that this page is getting somewhat old. So we have a more up-to-date page on information about types of breast cancer including Spindle cell cancer. However, this page still has some great information.

What is Spindle Cell Breast Cancer?

Doctors also refer to spindle cell breast cancer as:-

squamous carcinoma with spindle metaplasia
pseudosarcoma
sarcomatoid carcinoma
carcinosarcoma

This is in itself evidence of the ambiguous and varied presentation of this sub-type of breast cancer.

Researchers think that spindle cells generally evolve from mesenchymal cells, which are the building blocks of connective and muscle tissue. Whereas epithelial cells generally are in the membranes and linings.

Myo-epithelial cells are more-or-less a combination of the two and give strength and resilience to the breast duct lining. But more and more evidence suggests that spindle-cell breast cancer is a unique subcategory of metaplastic breast cancer.

So spindle cell breast cancer has its own differential diagnosis, prognosis and treatment strategies. A clinical finding of a large and well-circumscribed tumor, especially with accompanying cyst formation, would be strongly suggestive of spindle cell.

There are a wide variety of spindle cell tumors

spindle-cell-breast-carcinoma-ultrasound

Within the category of spindle cell breast tumors, there is still a wide spectrum of variability, with cellular features ranging from overly malignant to mildly atypical.

The majority of these tumors contain a spindle cell component greater than 80%. So, conventional invasive ductal carcinoma cells make up the remaining 20% or so of the tumor. A predominate spindle cell tumor with even a small amount of ductal carcinoma in situ will usually also be referred to as spindle cell cancer.

Features of Spindle Cell Breast Cancer

Spindle cell tumors of the breast tend to be:-

grossly nodular
hard
well-circumscribed

frequently with one or more cysts.

There is also some data to suggest that spindle cell tumors tend to be a little bit larger than other breast cancer tumors. Spindle cell cancer is actually more common in the oral cavity and the larynx than in the breast.

The incidence rate for spindle cell breast cancer is actually very low. Indeed, research estimates the rate to be between 0.2% and 0.5% of all breast cancers.

Spindle cell breast cancer tends to affect post-menopausal women, but not exclusively. The average age for developing spindle cell cancer of the breast is around 68 years of age. However, there is quite a wide age range.

The average tumor size tends to be between 4 cm and 5 cm at the time of diagnosis.

The prognosis for spindle cell cancer varies, depending mostly on the grade of the tumor, but even low-grade spindle cell tumors have shown some potential to metastasize. However, the tendency for the tumor to metastasize is probably related to the amount of conventional invasive ductal carcinoma component present.

Histological aspects of spindle cell breast cancer

Microscopically, spindle cell breast cancer tumors tend to be characterized by sheets of spindle cells which often contain squamous epithelial islands.

Spindle cell breast cancer tumors frequently show numerous cystic spaces lined by epidermoid carcinoma (the ductal carcinoma component) or benign-appearing squamous epithelium. In almost all cases, spindle cell breast carcinoma tumors will show ‘areas of transition’ from the malignant spindle cells to the regions of squamous epithelium or epidermoid carcinoma.

The ratio of spindle cell vs epithelial cell elements is variable

The ratio of the spindle cell component to the ductal-carcinomatous component is variable. Sometimes they are in a 1:1 ratio, ranging right up to an 8:1 ratio or higher.

However, if the ductal carcinoma element is higher than 50%, the tumor might be named differently. Even though elements of ductal, lobular and squamous patterns are often present, sometimes there is a pure spindle cell pattern in spindle cell carcinoma tumors.

When this is the case, differential diagnosis become more difficult and more important. Pure spindle-cell presentations must be differentiated from:-

primary sarcoma
nodular fasciitis
myofibroblastic lesions
Phyllodes tumor
inflammatory pseudotumors.

Cell necrosis is commonly seen

The spindle cells may appear benign, low-grade or they may have a high-grade sarcoma-like appearance.

Malignant spindle cells will likely show atypical nuclei and evidence of high mitotic activity. Evidence of necrosis is evident in almost all spindle cell breast carcinoma tumors. Furthermore, there may be cysts that frequently contain elements of hemorrhage, necrosis and exudates. Cysts within spindle cell breast carcinoma tumors are often partially surrounded by tumor cells and granulation tissues.

Immunohistochemical reactivity typical of spindle cell breast cancer tumors

In terms of the immunohistochemical staining profile for spindle cell breast carcinoma, virtually all of them will show reactivity for keratin.

Around half of all spindle cell breast cancers will be reactive for S-100. Many spindle cell breast cancer tumors will exhibit immunoreactivity for:-

smooth muscle actin
vimentin
CD10
cytokeratin 14

This leads some researchers to conclude that these breast cancers are of a myoepithelial cell origin. This, however, is still in dispute amongst medical specialists. Despite the sarcomatous features of spindle cell breast carcinoma, most researcher still believe that the spindle cells are likely to be derived from epithelial cells of mammary glands.

Spindle cell breast cancer tumors are almost uniformly negative for ER, PR and Her-2/neu receptors.

Spindle cell breast cancers often show reactivity for p63.

Immunohistochemical staining with a broad spectrum cytokeratin is crucial for the diagnosis of spindle cell carcinoma of the breast. The use of nuclear antigens that specifically aim to identify p63 are now in use in histological studies of spindle cell breast carcinoma.

Research shows that P63 is present in 40% of spindle cell breast cancer tumors and probably more. P63 is also called ‘transformation-related protein 63′ and is a member of the p53 family of transcription factors.

P63 is a member of the p53 gene family, based on structural similarities, but was discovered 20 years after p53. Overexpression of the p53-related genes tends to be involved in the tumorigenesis of both components (spindle cell and ductal epithelial) of spindle cell breast cancers. However, some researchers now suggest that the spindle cell component tends to show a higher degree of proliferative activity than the carcinomatous component, which is why p63 staining is so beneficial in diagnosis.

Treatment for spindle cell breast cancer

First of all, diagnosis of spindle cell breast carcinoma usually requires a wide excisional biopsy because fine needle aspiration biopsies are unreliable. This is normal considering the mixed-element presentation of spindle cell breast tumors. It would be very easy to ‘miss‘ one of the elements and come up with a wrong diagnosis.

The treatment for most spindle cell breast carcinomas is either by surgical excision or mastectomy. Axillary node dissection is usually necessary for evaluative purposes.

Medics will utilize chemotherapy in most treatments of spindle cell breast cancer. In addition, adjuvant endocrine therapy with Taximofen is also useful. Post operative radiation therapy is used in most cases in the hopes of preventing local recurrence or metastasis.

Prognosis for women with spindle cell breast cancer

Spindle cell breast cancers seem to grow expansively within their circumscribed boundary and the rate of lymph node metastasis is actually comparatively low. Spindle cell breast carcinoma tumors tend to be a bit larger than other typical breast cancers, but the malignant potential is lower than one might expect based upon tumor size.

The prognosis for spindle cell breast cancer is basically similar to the prognosis for other more common breast carcinomas, though at times it can be a highly aggressive neoplasm. Studies suggest the cumulative 5-year survival rate for spindle cell carcinoma of the breast is around 65%. This rate is a little better than general survival rates for most metaplastic breast cancers.

Higher ratios of spindle cell components generally leads to a better prognosis

However, the relative amounts of spindle cell versus ductal carcinoma elements can have a bearing on the outlook. Spindle cell breast carcinoma tumors which consist almost entirely of spindle cells (‘pure‘ spindle cell carcinoma) exhibit a significantly lower rate of nodal metastases than conventional ductal and lobular breast carcinomas.

However, spindle cell breast cancer tumors with a more even split of spindle cell and ductal carcinoma elements have a higher rate of extranodal metastases and a poorer prognosis.

The rate of metastasis for spindle cell breast carcinoma shows no consistent statistical trend at this time. Some studies show the rate of metastasis as high as 46%, with the most common sites being to the lungs, then the bone and the liver.

The rate of lymph node metastasis of spindle cell breast carcinoma is actually quite low, with estimates around 6%. However, a very high percentage of women who develop lymph node metastasis of spindle cell breast cancer, eventually die from the disease.

The lymph node metastasis of spindle cell breast carcinoma is mostly related to the ‘ductal carcinoma’ component of the tumor and not the spindle cell component. Local recurrence is not nearly such an ominous sign, as over 70% of women who experience local recurrence of spindle cell breast carcinoma still manage to survive the disease.

If metastasis occurs, survival rates are less positive

A comparison of various case studies suggests that the mortality rate for spindle cell breast carcinoma ranges between 40% and 50%. The average survival time after diagnosis is between 11 and 18 months.

Higher tumor grade tends to correlate with poorer survival for spindle cell carcinoma of the breast with an absence of complete microscopic circumscription and the size of tumors which eventually recur, as significant negative prognostic indicators.

Some women even with low grade tumors succumb to spindle cell breast carcinoma. However, studies also show that as many as 33% of women with spindle cell breast cancer may survive completely free of metastatic or recurrent disease.

Here are a few important Q&A’s:

Spindle cell cancer treatment

Breast Cancer Surgery
Cryotherapy
Radiotherapy
Hormone therapy
Chemotherapy

The choice of treatment depends on the location and the size of the tumor. The treatments may also be in combination for better results.

What causes spindle cell carcinoma?

Injury and inflammation in patients that are already thought to be predisposed to such tumors.

Spindle cell carcinoma symptoms

fatigue
malaise
mild dizziness

What are spindle cell tumors? How to treat them?

They are going to receive the same treatment as other types of breast cancer and the prognosis is similar.

References

Carter MR, Hornick JL, Lester S, Fletcher CD. Spindle cell (sarcomatoid)
carcinoma of the breast: a clinicopathologic and immunohistochemical analysis of 29 cases. Am J Surg Pathol. 2006 Mar;30(3):300-9.
Gersell DJ, Katzenstein AL. Spindle cell carcinoma of the breast. Aclinicopathologic and ultrastructural study. Hum Pathol 1981; 12: 550–61.
Wargotz ES, Deos PH, Norris HJ. Metaplastic carcinomas of the breast. II. Spindle cell carcinoma. Hum Pathol 1989; 20: 732–40.
Al-Bozom IA, Abrams J.Spindle cell carcinoma of the breast, a mimicker of benign lesions: case report and review of the literature.Arch Pathol Lab Med. 1996 Nov;120(11):1066-8.
Maemura, M., Iino, Y., Oyama, T., Hikino, T., Yokoe, T., Takei, H., Horiguchi, J. Ohwada, S., Nakajjioma, T., Morishita, Y., Spindle Cell Carcinoma of the Breast. Japanese Journal of Clinical Oncology, Volume 27, number 1.,
p. 46-50.
Ellis IO, Bell J, Ronan JE, Elston CW, Blamey RW. Immunocytochemical investigation of intermediate filament proteins and epithelial membrane antigen in spindle cell tumors of the breast. J Pathol 1988;154:157-65.

More References

Bauer TW, Rostock RA, Eggleston JC, Baral E. Spindle cell carcinoma of the breast: four cases and review of the literature. Hum Pathol 1984;15:147-52.
El All, HSA., Breast spindle cell tumours: about eight cases., Diagnostic Pathology 2006, 1:13
Carter MR, Hornick JL, Lester S, Fletcher CD: Spindle cell (sarcomatoid) carcinoma of the breast: a clinicopathologic and immunohistochemical analysis of 29 cases. Am J Surg Pathol 2006 , 30:300-309.
Nakayama Y, Iwasaki H, Iwanaga S, Nakamura H, Shiroshita T, Kikuchi M, Jozaki H, Hashimoto M, Ikeda S: Spindle cell carcinoma of the breast: a case report and an immunohistochemical study including p53 and Ki-67 expression. Pathol Int 1997 , 47:404-411.
Khan HN. Wyld L. Dunne B. Lee AH. Pinder SE. Evans AJ. Robertson JF. Spindle cell carcinoma of the breast: a case series of a rare histological subtype. European Journal of Surgical Oncology. (sept. 2003) 29(7):600-3.
Yang A, Kaghad M, Wang Y, Gillett E, Fleming MD, Dötsch V, Andrews NC, Caput D, McKeon F (September 1998). “p63, a p53 homolog at 3q27-29, encodes multiple products with transactivating, death-inducing, and dominant-negative activities”. Mol. Cell 2 (3): 305–16.
Tan M, Bian J, Guan K, Sun Y (February 2001). “p53CP is p51/p63, the third member of the p53 gene family: partial purification and characterization”. Carcinogenesis 22 (2): 295–300.