Primary breast angiosarcoma is a rare form of breast cancer, accounting for only 0.04% of all malignant breast tumours.
PRESENTATION OF CASE
A 28-year-old woman presented with a progressive lump in the right breast for one month. Fine needle aspiration cytology (FNAC) was highly suspicious of angiosarcoma, and this diagnosis was confirmed on core needle biopsy. Modified radical mastectomy was performed as sole treatment.
Primary breast angiosarcoma is a rare type of breast malignancy, usually occurring in the third to fourth decade and only reported in women. The histological features of angiosarcoma of the breast are conventionally graded I, II or III. Total mastectomy appears to be the only treatment conferring benefit, chemotherapy and radiation therapy being of little proven value to date. The 5-year disease-free survival for grade I tumours can be as high as 76%, and up to 70% for grade II tumours whereas for grade III tumours it is reportedly about 15%.
Primary angiosarcoma of the breast is a rare malignancy with a poor prognosis, even after complete resection. Surgery is the mainstay of treatment with a limited role for chemotherapy and radiotherapy.
Primary angiosarcoma; Core needle biopsy; Modified radical mastectomy
Here we report a case of primary epithelioid angiosarcoma (eas) of the breast occurring in a 30-year-old woman. Following fine-needle asspiration cytology (fnac) and tru-cut biopsy, the patient was initially diagnosed with mammary carcinoma and thereafter underwent modified radical mastectomy. Postoperative histopathologic examination and immunohistochemistry revealed a diagnosis of primary epithelioid angiosarcoma of the breast. The patient received postoperative radiotherapy to the chest wall and was started on adjuvant thalidomide. Preoperatively, eas can be mistaken for carcinoma because it is difficult to appreciate the typical morphology on fnac or tru-cut biopsy. Indeed, this is an area of potential diagnostic error because, nowadays, neoadjuvant therapy is often instituted after core biopsy of a breast mass. This case is being reported not only for its diagnostic difficulty, but also because of its rarity in English literature.
Breast; primary epithelioid angiosarcoma
We report a young lady with bilateral angiosarcoma of breast because of its rarity. A 29 year old unmarried female presented with bilateral breast lump. She underwent bilateral mastectomy with axillary dissection histopathology of which showed low grade angiosarcoma of left breast and high grade angiosarcoma of right breast. CT Thorax revealed right hilar and right mediastenal lymph nodes. She was treated with radiotherapy and chemotherapy postoperatively.
Bilateral angiosarcoma; breast; young lady
Angiosarcoma is an aggressive tumor that most commonly presents on the scalp or face of elderly patients; however, it can develop in pateints with breast cancer following radiation and breast-conserving therapy, complicating 0.1 to 0.2 percent of such cases. Mammography and fine-needle aspiration, though often very useful in evaluating for breast carcinomas and cytological features, are often negative in early stages of angiosarcoma and difficult to interpret. We present the case of a 49-year-old Caucasian woman with a history of stage II invasive ductal carcinoma of the left breast who presented with a two-month history of increased firmness and tenderness in her left breast. On exam, her left breast had significant firmness and hardening of breast tissue with slight erythema of skin. Punch biopsy initially suggested angiosarcoma, but subsequent biopsies instead diagnosed an atypical vascular proliferation. This case represents a patient who presented with an atypical lesion concerning for angiosarcoma. Repeated biopsies were necessary to obtain the correct diagnosis. One biopsy may not be sufficient for distinguishing atypical vascular proliferation from angiosarcoma. We present this case to increase awareness of the difficulty in making this distinction.
Secondary angiosarcoma of the breast is a rare but severe long-term complication of breast cancer treated with breast-conserving surgery and radiotherapy. We characterized a population-based cohort of patients with secondary angiosarcomas from two tertiary hospitals to investigate this complication with respect to surgical treatment and outcome.
We identified 35 patients with a history of radiation for breast cancer that developed angiosarcoma in the irradiated field from 1990 to 2009. Of these, 31 underwent surgery and were included for analysis.
Angiosarcoma developed after median 7 years (range 3–25 years). R0 resection was obtained in 23 of 31 patients after primary treatment. Local recurrence developed in 19 patients after median 6 months (range 1–89 months). Regional and distant metastases occurred in 13 patients after median 17 months (range 2–50 months); nine which also had local recurrence. Patients whose local recurrence could be operated on had a better survival after treatment than those who were not considered for surgical treatment, median 34 months (range 6–84 months) compared with 6 months (range 5–24 months). The median disease-free survival and disease-specific survival was 16 and 37 months, respectively.
Despite R0 resection, two-thirds of the patients developed a local recurrence. Survival among those with local recurrence was better if the patient could be treated with surgery. Overall, the prognosis was dismal and median DSS was just over 3 years.
Background. Primary breast angiosarcoma is a rare entity. Case. Initial diagnosis was a benign hemangioma at core biopsy. Wide local excision was performed, with positive margins. Pathology after surgery reported a moderately differentiated angiosarcoma. Tumor was finally treated using mastectomy and radiations. She developed a second angiosarcoma in contralateral breast, with an initial diagnosis on core biopsy of an atypical vascular lesion and was again treated using mastectomy and radiations. She developed bones and lung metastases. Conclusion. Primary breast angiosarcoma is a rare entity often difficult to diagnose on core biopsy, and a benign differential diagnosis is frequent. A highly vascular breast mass should always be considered malignant until proven otherwise. Surgical treatment seems to be the best course of action. There is a lack of data proving efficacy of adjuvant chemotherapy and radiation therapy.
We performed this study to detect preoperative axillary metastases with ultrasound (US)-guided fine needle aspiration biopsy (FNAB), to eliminate the need for time-consuming and costly sentinel lymph node (SLN) scintigraphy and biopsy steps in the treatment of breast cancer patients, and in that of with suspicious US findings, and to evaluate the accuracy of preoperative US-guided FNAB for patients with suspicious lymph node metastases on US.
Patients with a suspicious breast lump or histopathologically proven breast cancer underwent breast-axillary US. Increase in lymph node size, cortical thickening, non-hilar cortical flow, and hilar changes were evaluated with gray scale-color Doppler US. FNAB was performed if US results were suspicious for malignancy.
Thirty-eight axillary lymph nodes (ALN) underwent FNAB. ALN dissection, SLN scintigraphy, and biopsy steps were bypassed in 23 axillas with positive ALN FNAB (60.5%). The sensitivity of ALN FNAB was 88.46%; specificity and positive predictive value were 100%; and negative predictive value was 66.6% (inadequate cytology included; 76.7%, 100%, 100%, 53.3%, respectively). Asymmetrical cortical thickening, non-hilar cortical flow, and increase in hypoechogenity were only detected in metastatic nodes. Cortical thickening, and lymph node and breast mass size was higher in the metastatic group.
By performing FNAB on suspicious lymph nodes, the routine, high-cost SLN scintigraphy and intraoperative gamma probe steps may be skipped, and axilla dissection can be performed directly. This leads to the elimination of the need for SLN investigation in more than half of the patients. The assessment of ALN metastases with preoperative US-guided FNAB is a cost-effective method with high specificity, that eliminates the need for costly and time-consuming SLN scintigraphy and biopsy steps, and helps in preoperative staging.
Axilla; Breast neoplasms; Fine needle aspiration biopsy; Lymph node; Metastasis
Breast is an uncommon and rare site for metastasis. Primary and secondary tumors of the breast need to be differentiated as management is different. We present a 67 year old female patient with two breast lumps and an axillary lymph node, 5 years after nephrectomy for Renal Cell Carcinoma (RCC). Mammogram report showed a dense spiculated mass at right upper outer quadrant and a retroareolar mass associated with clustered micro calcifications. Fine Needle Cytology and trucut biopsy were inconclusive. Computer Topography (CT) abdomen did not show evidence of RCC recurrence. After discussion with the patient, she underwent mastectomy with axillary clearance and the final histopathology report was consistent with metastasis from RCC. The management of this case is discussed.
Extrammammary breast metastasis; Renal cell carcinoma; Post radical nephrectomy
Dermatofibrosarcoma protuberans is a rare neoplasm of soft tissues and its location in the breast is extremely uncommon. Confusion is possible with other primary breast lesions.
A 75-year-old Caucasian woman presented with a mass in her left breast 21 years after being diagnosed with invasive ductal carcinoma of the right breast, treated by a right mastectomy and axillary dissection followed by radiotherapy and breast reconstruction. Mammography revealed a dish-shaped skin nodule formation in the upper outer quadrant of her left breast. Echography confirmed the presence of a lesion measuring 1.4 × 0.8 cm. Based on imaging, the diagnosis was a probable angiosarcoma. Due to the presence of a pacemaker for cardiac arrhythmia and full anticoagulation therapy for a pulmonary embolism, magnetic resonance imaging and a biopsy were not done. We proceeded directly to a quadrantectomy and the final diagnosis revealed a dermatofibrosarcoma protuberans, 1. 8 cm in its greatest microscopic dimension, located 0.1 cm from the upper surgical margin. To ensure the wide resection margins required for this type of neoplasm, a re-excision was performed.
A dermatofibrosarcoma protuberans of the breast is an uncommon discovery. The aim of this case report is to highlight the importance of the surgical procedure in cases of the discovery of dermatofibrosarcoma protuberans. Re-excision may be necessary to ensure adequate resection margins.
Secretory carcinoma is a rare form of breast carcinoma which has a predilection for juveniles and young adults (usually less than 30 years of age), becoming progressively less common with advancing age. It is a low grade breast carcinoma which shows distinct features at histology. Diagnosis of this carcinoma on fine needle aspiration cytology (FNAC) is difficult. We report a case of a 62-year-old woman diagnosed to have secretory carcinoma of breast on FNAC. Histopathological examination confirmed the diagnosis. The recognition of secretory carcinoma is important because the cytological findings can be confused with those of lactating breast. Preoperative diagnosis is essential for appropriate surgical therapy. Secretory carcinoma is reported to have good prognosis, but surgical therapy with an axillary node dissection is recommended, since axillary metastases have been found in approximately 30% of the recorded cases.
Breast carcinoma; lactating breast; secretory carcinoma; fine needle aspiration cytology
Angiosarcoma is an uncommon malignancy, which spread out from the endothelial cells of vessels. Scalp angiosarcoma with cervical lymph node metastasis is particularly rare. This article describes a rare case of angiosarcoma of the scalp, presenting as neck inflammation. Imaging procedures such as computed tomography (CT), magnetic resonance image (MRI) and ultrasonography (US) were not sufficient to diagnose this case. A needle biopsy provided an effective and accurate diagnosis of cervical lymph node metastasis. Additional observation and physical examination was required to diagnose the origin of the primary cancerous lesion. Once the angiosarcoma diagnosis was confirmed histologically, sequential weekly and monthly docetaxel (DTX) treatment was effective in preventing reoccurrence. Nonetheless, the optimization of angiosarcoma treatment remains a future goal. Although patients generally describe pain and swelling at the primary lesion site, this patient complained only of painful neck inflammation, without any indication of pain or swelling of the scalp. A revised diagnostic protocol should note that cervical lymph node metastasis of unknown primary origin may result from angiosarcoma of the scalp.
angiosarcoma; docetaxel; neck inflammation; needle biopsy; scalp
The standard treatment of the axilla in breast cancer used to be an axillary lymph node dissection. An axillary lymph node dissection is known to give substantial risks of morbidity. In recent years the sentinel node biopsy has become common practice. Future randomized study results will determine whether the expected decrease in morbidity can be proven.
Before the introduction of the sentinel node biopsy, we conducted a study in which 180 women of 50 years and older with T1/T2 cN0 breast cancer were treated with breast conserving therapy. Instead of an axillary lymph node dissection regional radiotherapy was given in combination with tamoxifen (RT-group). The study group was compared with 341 patients, with the same patient and tumour characteristics, treated with an axillary lymph node dissection (S-group).
The treatment groups were comparable, except for age. The RT-group was significantly older than the S-group. The median follow up was 7.2 years. The regional relapse rates were low and equal in both treatment groups, 1.1% in RT-group versus 1.5% in S-group at 5 years. The overall survival was similar; the disease free survival was significant better in the RT-group.
Regional recurrence rates after regional radiotherapy are very low and equal to an axillary lymphnode dissection.
The significance traditionally attached to regional lymph node metastases has been questioned following a retrospective review of 922 patients with early breast cancer.
Conservative surgical removal of axillary lymph node metastases and conservative irradiation of internal mammary lymph node metastases did not prejudice the five- and 10-year survival rates of patients so treated.
Axillary lymph node recurrences had an ominous prognosis and occurred more commonly in the conservatively treated patients, yet survival rates were the same as those following radical mastectomy. Many axillary lymph node recurrences occurred more than five years after primary therapy, or with or after other evidence of reactivation of the breast cancer.
It is suggested that breast cancer patients do not do poorly because they have regional lymph node metastases, but rather they have these metastases when they do poorly.
Our case report pertains to a 32-year-old woman initially presenting with left flank pain and gross haematuria throughout her urinary stream. CT of her kidney/ureter/bladder (CT KUB) revealed ureteric dilatation to the level of the bladder without evidence of renal calculus and subsequently a stent was inserted. She represented a month later with contralateral flank pain, and a transuretheral resection of bladder tumour was performed. Histopathological diagnosis was epithelioid angiosarcoma. Further imaging (MRI pelvis) revealed that the tumour arose from the posterior bladder wall with local invasion and regional lymph node metastasis. Ifosfamide and epirubicin chemotherapy with single-fraction radiotherapy induced significant reduction in tumour bulk, although this initial response was followed by the development of symptoms suggestive of disease progression. She died 19 months after initial diagnosis with persistent pulmonary and vertebral metastases although no autopsy was performed.
A case of single breast metastasis from colon adenocarcinoma, with omolateral axillary micrometastasis, is reported with a brief review of the pertinent literature. The originality of the oncological concept of metastasis from metastasis, through lymphatics penetration, is discussed in the setting of a rare condition of breast metastasis from a colorectal carcinoma.
The demonstration of axillary lymph node micrometastasis has been possible because fine needle aspiration cytology of the breast nodule was suspicious, but not conclusive for metastasis from colon cancer, so lumpectomy with sentinel node biopsy was planned.
Although no disseminated nodal metastases were evident on computerized tomography scan and ultrasonography before breast surgery, the patient developed brain metastases and deteriorated rapidly; she died 16 months after presenting with the breast mass.
In conclusion, solid cancers are able to further metastasize, via well-known pathways also recognized in primary cancers such as neoplastic cell invasion of peritumoral lymphatics.
Angiosarcomas are rare aggressive neoplasms that can occur secondary to chronic lymphedema (Stewart-Treves syndrome). Although secondary angiosarcomas are commonly described after-mastectomy and/or after-radiotherapy, few cases have been reported in association with chronic lymphedema of congenital origin. We report the clinical, pathological, and cytogenetic findings in a case of cutaneous epithelioid clear cells angiosarcoma that occurred in a 21-year-old woman with hemibody congenital lymphedema. Surgical biopsies of the tumor mass revealed diffuse epithelioid proliferation of clear atypical cells, for which immunophenotyping highlighted the vascular differentiation. Despite en bloc resection of the tumor, the patient died of metastatic disease three months after diagnosis. This case illustrates the clinical and pathology characteristics of angiosarcoma that is a rare entity secondary to chronic lymphedema. It is the first reported case for which the c-MYC amplification status was assessed. The diagnostic value of this amplification should be further evaluated in this specific context.
Paget's disease is a rare form of breast cancer often associated with an underlying ductal carcinoma in situ or invasive cancer. A 47-year-old female patient presented with bleeding from the left nipple since 4 months. Imprint smears from the lesion showed pleomorphic malignant epithelial cells in singles and in small clusters, many of them containing dark brown cytoplasmic pigment granules. There was no palpable breast lump or axillary lymph node enlargement. Based on the physical examination and cytological features, a diagnosis of malignant melanoma with a differential of Paget's disease of the nipple was made. Biopsy showed features of Paget's disease and immunohistochemistry was positive for HER-2/neu, negative for HMB-45 and S-100, thus confirming the diagnosis. This case has been reported to emphasize the fact that Paget's cells can contain imbibed brown cytoplasmic pigment and should not be mistaken for melanoma cells on cytology smears.
Cytology; melanoma; nipple; Paget's disease; pigment
Sjögren's syndrome (SS) is an autoimmune disease that chronic inflammation and lymph node proliferation. Patients with SS carry a greater risk of developing lymphoproliferative malignancy. In addition to other organ cancers, breast cancer may also occur in these patients. Considering these, breast cancer in patients with SS can be misdiagnosed as being in an advanced stage particularly in the presence of axillary lymphadenopathy. Here, we report a rare case of a 45-year-old woman with SS who presented with a breast mass. Radiology showed a 4 cm solid lesion and conglomerates of axillary lymphadonepathy. A breast biopsy revealed ductal carcinoma in situ. A modified radical mastectomy was performed; however, no axillary metastases were detected. Clinicians should remain vigilant to the possibility that a false clinical impression of axillary metastasis may occur in such patients with breast cancer. Therefore, axillary node status should be verified first.
Breast neoplasms; Lymphatic diseases; Overstaging; Sjögren's syndrome
For patients with early breast cancer and lymph node metastasis, axillary treatment is widely recommended. This is either surgical removal of the axillary lymph nodes, or axillary radiotherapy. The rationale for axillary treatment is that it will reduce the risk of recurrence in the axilla, and may improve survival. However, both treatments are associated with adverse effects, such as lymphedema, pain and sensory loss, and are costly to the health services and to patients. With improvements in adjuvant therapy, routine axillary treatment may no longer offer any overall advantage.
To assess the short and long term benefits and adverse effects of routine axillary treatment (axillary lymph node clearance or axillary radiotherapy) for patients with lymph node positive early-stage breast cancer.
Criteria for potentially eligibility for the study will be that the participants are men and women with early breast cancer and lymph nodes with metastasis. The study compares either axillary treatment with no axillary treatment, or axillary node clearance with axillary radiotherapy, and the study is a randomized trial. Primary outcomes are axillary recurrence, disease-free and overall survival. Secondary outcomes include breast or chest wall recurrence, distant metastasis, time to axillary recurrence, axillary recurrence-free survival, arm morbidity, quality of life and health economic costs. The search strategy will include the Cochrane Central Register of Controlled Trials, MEDLINE, EMBASE and WHO International Clinical Trials Registry Platform (ICTRP) search portal. Two independent reviewers will assess studies for inclusion in the review, assess study quality and extract data. Characteristics of included studies will be described. Meta-analysis will be conducted using ReVman software.
This review addresses an important clinical question, and results will inform clinical practice and health care policy.
A 67 year old woman presented with a right breast lump which proved to be a grade 2 invasive ductal carcinoma with axillary lymph node metastasis. She had a five year history of CD5 positive chronic lymphocytic leukaemia, which never required treatment. Immunoperoxidase stains for CD5, using the monoclonal antibody NCL-CD-54C7, showed that there was extensive infiltration of axillary lymph nodes with CD5 positive B lymphocytes. Strong staining for CD5 was also seen in the carcinoma cells within the breast and lymph node metastases. It has recently been suggested that there is a tumour suppresser locus in chronic lymphocytic leukaemia at 13q12.3 near or at the BRCA2 locus. Deletion of regions on chromosome 13q containing the BRCA2 and RB1 genes has also been reported in sporadic breast cancers. These observations suggest that there may be a link between these two diseases acting through chromosome 13, but amplification of several microsatellite repeat markers failed to show any loss of heterozygosity or repeat instability at either these or several other loci on chromosome 13. Examination of additional such cases is needed to perform a more comprehensive study of the significance of positive CD5 staining of breast carcinoma.
We report a case involving a 45-year-old woman, who presented with an axillary mass 10 years after bilateral cosmetic augmentation mammaplasty. A lump was detected in the left axilla, and subsequent mammography and magnetic resonance imaging demonstrated intracapsular rupture of the left breast prosthesis. An excisional biopsy of the left axillary lesion and replacement of the ruptured implant was performed. Histological analysis showed that the axillary lump was lymph nodes containing large amounts of silicone. Silicone lymphadenopathy is an obscure complication of procedures involving the use of silicone. It is thought to occur following the transit of silicone droplets from breast implants to lymph nodes by macrophages and should always be considered as a differential diagnosis in patients in whom silicone prostheses are present.
Augmentation; breast prostheses; implant; mammaplasty; silicone lymphadenopathy; ruptured breast implant
Several randomized trials have shown that breast-conserving therapy (BCT) is as effective as mastectomy and should be a standard treatment for early-stage breast cancer. Recently, there has been an increase in reports of angiosarcoma (AS) after BCT. Herein, we report a case of AS which developed after BCT and a case of Stewart-Treves syndrome with a focus on lymphedema. Chronic lymphedema is the primary risk factor for AS, which was first described in 1948 by Stewart and Treves [Cancer 1948;1:64–81]. Radiation therapy secondarily tends to induce the development of AS, since radiation therapy induces fibrosis and proliferation of lymphatic vessels via cytokines such as vascular endothelial growth factor, which is followed by subclinical chronic edema. It is suggested that axillary lymph node dissection predisposes patients to the development of AS, since it is closely associated with lymphedema. Breast surgeons and radiologists should be aware of skin changes in order to improve the early detection of AS during the follow-up of patients who have undergone BCT, and especially those treated with axillary lymph node dissection.
Angiosarcoma; Breast-conserving therapy; Radiation; Breast cancer; Lymphedema; Stewart-Treves syndrome; Axillary lymph node dissection; Breast cancer surgery
Malignant vascular tumors are rare and angiosarcomas of the breast in patients under 21 years of age are exceedingly uncommon. In this report an angiosarcoma in the breast of a 14-year-old girl is described. She died nine months after mastectomy with recurrent disease in the bones and the contralateral breast. The etiology of most primary angiosarcomas is unknown. Secondary angiosarcomas can develop after radiotherapy and chronic lymphedema. The histology of this angiosarcoma is illustrated.
angiosarcoma; infancy; breast.
A 73-year-old woman was found to have a 1.7 cm axillary mass, for which a core needle biopsy was performed. The specimen revealed fragmented squamous epithelium surrounded by lymphoid tissue consistent with a squamous inclusion cyst in a lymph node, but a metastatic squamous cell carcinoma could not be excluded. Within one month, the lesion enlarged to 5 cm and was excised. Touch preparation cytology during intraoperative consultation displayed numerous single and sheets of atypical epithelioid cells with enlarged nuclei and occasional mitoses, suggesting a carcinoma. However, multinucleated giant cells and neutrophils in the background indicated reactive changes. We interpreted the touch preparation as atypical and recommended conservative surgical management. Permanent sections revealed a ruptured squamous inclusion cyst in a lymph node with extensive reactive changes. Retrospectively, the atypical epithelioid cells on touch preparation corresponded to reactive histiocytes. This is the first case report of a rapidly enlarging ruptured squamous inclusion cyst in an axillary lymph node following core needle biopsy. Our case demonstrates the diagnostic challenges related to a ruptured squamous inclusion cyst and serves to inform the readers to consider this lesion in the differential diagnosis for similar situations.
The aim of this study was to assess the accuracy of 18F-fluorodeoxyglucose (FDG) positron emission tomography (PET)/CT to visualize lymph node metastases before the start of neoadjuvant chemotherapy and to determine how often the visualization is sufficiently prominent to allow monitoring of the axillary response.
Thirty-eight patients with invasive breast cancer of >3 cm and/or lymph node metastasis underwent FDG PET/CT before neoadjuvant chemotherapy. The results of the FDG PET/CT were compared with those from ultrasonography with fine-needle aspiration (FNA) cytology or sentinel node biopsy. Patients suitable for response monitoring of the axilla were defined as having either a maximum standardized uptake value (SUVmax) ≥ 2.5 or a tumour to background ratio ≥5 in the most intense lymph node.
The sensitivity and specificity of FDG PET/CT in detecting axillary involvement were 97 and 100%, respectively. No difference existed between the SUVmax of the primary tumour and that from the related most intense lymph node metastasis. Moreover, the mean tumour to background ratio was 90% higher in the lymph nodes compared to the primary tumour (p = 0.006). Ninety-three per cent of the patients had sufficient uptake in the lymph nodes to qualify for subsequent response monitoring of the axilla. A considerable distinction in metabolic activity was observed between the different subtypes of breast cancer. The mean SUVmax in lymph node metastases of oestrogen receptor (ER)-positive, triple-negative and human epidermal growth factor receptor 2 (HER2)-positive tumours was 6.6, 11.6 and 6.6, respectively.
The high accuracy in visualizing lymph node metastases and the sufficiently high SUVmax and tumour to background ratio at baseline suggest that it is feasible to monitor the axillary response with FDG PET/CT, especially in triple-negative tumours.
Breast cancer; Axillary lymph node metastasis; FDG-PET/CT; Neoadjuvant chemotherapy