Cystosarcoma phyllodes: Epidemiology, pathohistology, pathobiology, diagnosis, therapy, and survival

Abstract

Cystosarcoma phyllodes is a very rare tumor which maybe difficult to diagnose clinically. The epidemiology and pathobiology are different from those of breast carcinoma. Risk factors, multicentricity, bilaterality, as associated with breast carcinoma, are not observed in patients with cystosarcoma phyllodes. Although the term "sarcoma" indicates a malignant tumor, only 10%-30% of cystosarcomas are histologically diagnosed as malignant; clinical diagnosis of malignancy does not exceed 10%. Axillary node involvement is rare, but hematogenous spread of cystosarcoma occurs into lung, pleura, bone, and liver. Clinically, cystosarcoma is a large (usually 3-5 cm in diameter) painless tumor with sudden growth acceleration especially during pregnancy. Cystosarcoma is usually circumscribed, containing firm and soft areas. The differential diagnosis has to include fibroadenoma, fibrocystic disease, mastitis, abscess, and medullary carcinoma. Neither clinical, mammographic or sonographic signs exist to predict a benign or malignant tumor. Therapy of cystosarcoma is not uniformly agreed upon. Radical, modified-radical, and simple mastectomy and tumorectomy are typical treatments; therapeutic results are the same for each treatment modality. For histologically diagnosed malignant cystosarcoma, the relative 5-year survival rate is about 80%. Clinically, malignant metastatic cystosarcoma is incurable; radiotherapy, endocrine treatment, and polychemotherapy are all ineffective. Because of the specific tumor pathobiology of cystosarcoma and its rarity, evaluation of treatment modalities and comparison of survival rates are difficult.

Gigantinis piktybinis filoidinis krūties navikas: klinikinio atvejo aprašymas ir literatūros apžvalga

“…The main options of local PT treatment are breast preserving operation with excision of normal breast tissue around the tumor (to obtain of at least a 1 cm margin [1975][1976][1977][1978][1979][1980][1981][1982][1983][1984][1985][1986] the bloodstream, the lymph node metastases rates are very low (<5%). Thus, axillary dissection is unnecessary during the initial surgical intervention [10,11,12]. Radiotherapy is recommended for individuals with a high local recurrence risk.…”

“…Tumor size is an important factor in predicting effect of local treatment [11]. The main options of local PT treatment are breast preserving operation with excision of normal breast tissue around the tumor (to obtain of at least a 1 cm margin [1975][1976][1977][1978][1979][1980][1981][1982][1983][1984][1985][1986] the bloodstream, the lymph node metastases rates are very low (<5%).…”

“…Chemotherapy is used to treat patients with a high systemic metastatic risk. The real role of adjuvant irradiation and chemotherapy is undefined and should be tested in prospective, randomized trials [10,11,12,15,16].…”

“…[10]. Using pooled cancer registry data, Vorherr et al estimated an overall five year survival rate of 80% for malignant PT [11]. The five-year survival for benign, borderline or malignant tumors is of 95.7% for benign tumors, 73.7% for borderline tumors, and 66.1% for malignant tumors [10].…”

Phyllodes tumor of the breast: Wolf inside the sheep’s skin!

“…[21] One possible explanation for such a disparity may be the low incidence of PT, resulting in small patient groups observed in individual studies. Moreover, different workers have used different histological criteria for subclassifying these tumors.…”