Vascular tumors of the breast. V. Nonparenchymal hemangiomas of mammary subcutaneous tissues

Nine patients with vascular lesions of the mammary subcutaneous tissue were studied. All were women who ranged in age from 20 to 76 years (average 53 years). The lesions were angiolipomas (three cases), cavernous hemangiomas (three cases), and one example each of juvenile hemangioma, venous hemangioma, and papillary endothelial hyperplasia. The average size of these tumors was 1.8 cm (range, 0.8-3.2 cm). Only one of the excisional biopsy specimens contained breast tissue, and in this case the hemangioma was also extra parenchymal. With follow-up available for seven patients ranging from 5 months to just over 5 years (average 29 months), there were no recurrences. Subcutaneous hemangiomas of the breast do not differ appreciably in their clinical or pathological features from comparable lesions at other anatomic sites. Although mammary subcutaneous hemangiomas are benign and are not prone to recurrence or progression to angiosarcoma, complete excision is recommended to exclude the possibility of an underlying angiosarcoma.     

Follow‐up outcomes of benign vascular lesions of breast diagnosed on core needle biopsy: A study of 117 cases

Benign vascular lesions of breast are uncommon findings on core biopsy and surgical excision has been recommended to rule out a more serious lesion. However, a recent study suggested that excision may be spared for vascular lesion without atypia. The aim of this study was to assess the follow‐up outcomes in lesions yielding benign vascular lesions on core biopsy. We retrospectively reviewed 117 patients with diagnosis of hemangioma (106 patients) and atypical hemangioma (11 patients) on core biopsy at our institution over an 18‐year period. Majority of benign vascular lesions were followed‐up clinically and/or radiologically. Surgical excision was performed on 18 patients (16.9%) with benign hemangiomas and all 11 patients (100%) with atypical hemangiomas. Upon excision, the majority of patients (82.8%, 24/29) retained benign final pathology and five patients (17.2%) were atypical hemangioma. There was no upgrade on excision. All patients had a benign course regardless whether the lesions were excised or not. Our findings support the recent study that benign vascular lesion of breast may not require surgical excision.     

Vascular Lesions of the Breast

Vascular lesions represent a minority of tumors originating in the breast. The most common entities are benign and include hemangiomas and angiolipomas. Malignant vascular lesions (angiosarcomas) are rare and may be primary or secondary to radiation. Also appreciated in association to radiotherapy is the development of cutaneous atypical vascular lesion affecting the skin of the breast. The relationship of the latter to radiation-associated angiosarcoma is controversial and remains to be elucidated. This article reviews the most likely encountered vascular lesions in the breast, with emphasis on key pathologic diagnostic features and potential diagnostic pitfalls.     

Vascular tumors of the breast. IV. The venous hemangioma

Recent studies have documented the diversity of vascular tumors of the breast. In addition to angiosarcoma, it has been possible to define several categories of benign lesions. This report describes four patients whose vascular tumor consisted of a circumscribed disorderly vascular proliferation composed largely of venous channels. These vascular tumors of the breast correspond most closely to soft-tissue lesions that have been classified as venous hemangiomas. The patients ranged in age from 24 to 59 years. Each presented with a mass; the tumors measured 1.0-5 cm in diameter. Three were treated by excisional biopsy and one by mastectomy. All have remained well. The patient with the longest follow-up (11 years) reported that her tumor had been present for 13 years before diagnosis. A 38-year-old woman had coexistent lobular carcinoma in situ. She remains well less than 1 year after excisional biopsy. Thus far there is no evidence that this apparently benign lesion is likely to develop into angiosarcoma. Based on the information at hand, complete local excision appears to be adequate treatment. Longer follow-up and the study of other cases will provide a more complete assessment of the clinical course of this vascular tumor.     

Vascular tumors of the breast. III. Angiomatosis

This report describes three female patients who were treated for diffuse vascular lesions of the breast. The lesion was congenital in one patient. Two were adults (40 and 59 years old). Each presented with a mass within the breast. Microscopically, the tumors were composed of vascular channels growing diffusely in breast parenchyma. The vessels were lined by flat, inconspicuous endothelium with sparse supporting mural tissue virtually devoid of smooth muscle. The lesions consisted of hemangiomatous (erythrocyte-containing) and lymphangiomatous (seemingly empty) channels with lymphoid aggregates. This combination of a mixed pattern of vessels and diffuse growth is similar to that seen in lesions that have been called angiomatosis in other anatomic sites. Because the microscopic distinction between very orderly angiosarcomas and angiomatosis may be difficult, complete excision of the tumor is necessary. For bulky lesions, this may require mastectomy. In one patient congenital angiomatosis has persisted and recurred twice in the mammary region over 39 years. The other two patients remain well 3 years after local excision and 2 years and 4 months after mastectomy, respectively. Angiomatosis of the breast has not exhibited clinically malignant behavior since no metastases have been observed.     

A series of vascular tumors and tumorlike lesions of the bladder

Vascular tumors of the bladder are rare and a subject of small series and case reports. We retrospectively identified vascular tumors of the urinary bladder from the consultation files from one of the authors. We identified 13 lesions that included 3 hemangiomas, 3 intravascular papillary endothelial hyperplasias (Masson vegetant hemangioendotheliomas), 2 arteriovenous malformations (AVMs), 1 epithelioid hemangioendothelioma (EHE), and 4 angiosarcomas. One of the angiosarcomas was associated with conventional high-grade urothelial carcinoma (sarcomatoid carcinoma). All patients were adults with a range in age from 18 to 85 years old (mean 63.3). There was no statistical difference among the various lesions in terms of age, although angiosarcomas tended to arise in older patients (mean 71 y vs. 60 y of the remainder). Hematuria was the most common presentation of both benign and malignant lesions. Other symptoms included voiding irritation, pelvic pain, and obstruction. Histologically, benign and malignant lesions were similar to their counterparts in other organ systems. Two hemangiomas were of the capillary type and a third one of the cavernous subtype. They measured 1.1, 2.4, and 3.2 cm. Both AVMs were clinically large broad-based masses measuring 5.5 and 5.8 cm in greatest diameter. One of the AVMs was associated with pseudoepitheliomatous hyperplasia of the urothelium. All 3 patients with Masson lesion had history of radiation therapy for other causes. These presented as raised lesions and were all <1.0 cm. Patients with hemangiomas, papillary endothelial hyperplasias, and AVM had an invariably benign prognosis and needed no further therapy. These benign lesions had consistent involvement of the submucosa and spared the muscularis propria of the organ. All cases of angiosarcoma and EHE involved the muscularis propria. Two of four patients with angiosarcoma had a history of prior radiation therapy and all 4 were dead of disease at 6 months. Angiosarcomas measured 3, 4.5, 5, and 5.8 cm in greatest diameter at cystoscopy. The patient with EHE had a single nodule treated by transurethral resection of the bladder and no evidence of disease at 4 years of follow-up. None of the patients experienced marked gross hematuria that resulted in morbidity or mortality. A wide spectrum of benign, intermediate malignant, and malignant vascular lesions primarily involved the bladder. Despite the potential for marked hemorrhage, none of the tumors resulted in marked hematuria. Papillary endothelial hyperplasia occurs in the bladder and must be differentiated from angiosarcoma, which has a rapidly fatal outcome.     

Large plaque-type blue nevus with subcutaneous cellular nodules

Unusual or atypical melanocytic nevi can be confused with malignant melanoma. The authors present two cases of an unusual variant of blue nevus that were misdiagnosed initially as malignancy. Both lesions were asymptomatic and characterized clinically by childhood onset, with slow enlargement during adolescence and subsequent nodule formation. One lesion, which measured 24 cm in greatest dimension, was located on the anterior chest wall of a 53-year-old woman. The other lesion, which measured approximately 15 cm in greatest dimension, was located on the lateral abdominal wall of a 20-year-old man. Both lesions were characterized by a multifocal dermal and subcutaneous proliferation of fusiform and dendritic pigmented melanocytes. The histologic appearance of individual foci ranged from dermal melanocytosis to common blue nevus and cellular blue nevus. The cellular foci were located in the subcutis and involved, in one patient, the stroma of the breast. The cells were immunoreactive for S-100 protein, gp100 (HMB-45), and Melan-A (A103). Ultrastructural analysis revealed melanocytes typical of blue nevus. The woman underwent complete excision of the lesion, and the man underwent only partial excision of the lesion. On clinical follow-up of 32 and 19 months, respectively, both patients are alive and well with no evidence of recurrence or progression. Because the lesions presented clinically as large plaques and were diagnosed histologically as blue nevi with subcutaneous foci of cellular blue nevus, we term this rare variant of blue nevus large plaque-type blue nevus with subcutaneous cellular nodules. Recognition of this lesion enhances our knowledge of the morphologic spectrum of melanocytic tumors and helps to avoid confusion with malignant melanoma.     

Surgical treatment of hemangiomas of soft tissue

Hemangioma is one of the most common soft tissue tumors comprising 7% of all benign tumors. The etiology is unclear. Many treatment modalities for the symptomatic deep subdermal or intramuscular hemangioma have been used, but surgical excision is the preferred treatment. During the past 20 years, 89 patients with soft tissue hemangiomas were treated by surgical excision at the authors' institution. This study was done to define the clinical characteristics of pathologically proven hemangiomas and to evaluate the outcome of the operative procedures. Intralesional or marginal excision for symptomatic hemangiomas yields satisfactory results for pain relief, functional recovery, and avoidance of recurrence. According to the data a hemangioma of the soft tissues is a benign lesion in which more aggressive surgery (wide or radical excision) or other modalities such as radiation usually are not warranted.     

Infantile hemangiomas: differential diagnosis and associated anomalies

Infantile hemangiomas are frequent benign vascular tumors that are often easily recognized. However, the diagnosis between infantile hemangiomas and other vascular tumors, whether benign or malignant, may be difficult. This chapter describes the different clinical presentations of hemangiomas and details the investigations that are needed to confirm the nature of the lesion and to diagnose the potentially associated anomalies. Knowledge on differential diagnosis enables clinicians to detect hemangiomas that can lead to complications and that necessitate a multidisciplinary approach.     

Quiescent Volcano—Chest Wall Hemangioma

Chest wall hemangiomas are rare tumors that may originate within the soft tissue or from the ribs. Intramuscular hemangioma is infrequent, representing less than 1 % of all hemangiomas, and the localization in the chest wall is even less frequent. They are typically cutaneous in location, large, and poorly circumscribed and can be locally destructive. We present a case of a 34-year-old lady presented with firm lump 3 × 3 cm in left upper and inner quadrant of left breast well defined borders, non-pulsatile and restricted mobility. Sono-mammogram was suggestive of ill-defined lesion at 10 o’clock position. CT chest was conclusive of chest wall hemangioma. The patient underwent excision of the lump. HPE was suggestive of cavernous hemangioma. Cavernous hemangioma typically manifest at birth or before the age of 30 years. CT is more sensitive than plain radiography in detecting phleboliths, which are present in approximately 30 % of cavernous hemangiomas. Surgical excision would be treatment of choice. In this case, the site of the lesion was in the breast clinically mimicking that of a fibroadenoma which warrants hemangioma as a differential diagnosis.     

Angiosarcoma of the breast

The clinical histories, operative reports, and pathologic slides of 20 patients seen between 1907 and 1984 with diagnoses of angiosarcoma, hemangiosarcoma, and hemangioendothelioma were reviewed. The mean age of the 19 female patients and one male patient was 40.05 years (range, 16 to 67 years). Ten patients had the right breast involved, and five patients had contralateral breast involvement. Presenting symptoms included a mass in 20 patients, pain in ten patients, and breast discoloration in six patients. Before referral, ten patients had the misdiagnosis of a benign breast lesion. Because of the deceptively benign appearance of many angiosarcomas, these lethal tumors are often misdiagnosed as benign hemangiomas. Simple mastectomy is the treatment of choice, while adjuvant therapy remains to be evaluated.     

Enucleation of giant hemangiomas of the liver. Technical and pathologic aspects of a neglected procedure.

Cavernous hemangiomas are the most common benign tumors of the liver. Giant cavernous hemangiomas, defined as those larger than 4 cm in diameter, can reach enormous proportions. Newer imaging modalities, although often demonstrating characteristic features that strongly suggest the diagnosis, should not be augmented by biopsy because of the risk of hemorrhage. Elective surgical resection may be indicated for symptomatic giant lesions and for those with an atypical appearance where the diagnosis is in doubt. Between October 1986 and May 1991, we treated 10 patients with giant hemangiomas by enucleation or enucleation plus resection. Median operative blood loss was 800 mL (range, 200 to 3000 mL). One patient required reoperation for control of postoperative hemorrhage. Detailed pathologic examination has demonstrated an interface between hemangiomas and the normal liver tissue that allows enucleation. Enucleation is an underused procedure that if carefully performed allows resection of giant hemangiomas with a reduced blood loss and the preservation of virtually all normal hepatic parenchyma.     

Primary malignant lymphoma arising in postmastectomy lymphedema. Another facet of the Stewart-Treves syndrome

In rare cases, primary malignant lymphomas may arise in the soft tissues. Only one previous case has arisen in the context of chronic lymphedema. Because of the clinical appearance of such lesions, which resemble violaceous nodular or plaquelike tumors, they may be confused clinically with lymphedema-associated angiosarcomas occurring after radical mastectomy (Stewart-Treves syndrome). Furthermore, the histologic appearance of some lymphomas and angiosarcomas may also be similar. We studied two women with primary postmastectomy lymphedema-related malignant lymphoma in the soft tissues of the upper arm. These tumors arose 11 and 30 years, respectively, after radical removal of ductal mammary carcinomas. Histologically, one neoplasm mimicked metastatic carcinoma or epithelioid angiosarcoma; whereas the other was initially confused with a variety of pathologic entities, including vasculitis, epithelioid hemangioma, and malignant fibrous histiocytoma. The lymphoid nature of both lesions was confirmed by immunoreactivity for leukocyte common antigen in addition to the B-lymphocyte marker, L26. Conversely, vascular and epithelial determinants were absent. One patient's disease pursued an indolent course; she died of unknown causes but with no evidence of lymphoma at last follow-up. The second patient is currently in remission on chemotherapy. Awareness of the existence of lymphedema-related malignant lymphoma and familiarity with methods used for its distinction from epithelioid vascular sarcomas should prevent unnecessary surgery.     

Sarcoma of the breast. A study of 32 patients with reappraisal of classification and therapy.

A retrospective clinicopathologic review of 32 patients with mammary sarcoma exclusive of angiosarcoma or lymphoma was performed. For 25 patients with previously untreated lesions, the median tumor diameter was 4 cm and 14 patients had high or intermediate grade lesions. One of 22 patients treated by mastectomy and one of three patients treated by local excision died of sarcoma yielding an actuarial 5-year survival of 91%. None of the 25 patients had received adjuvant chemotherapy and only one treated by mastectomy had post-operative radiation therapy. Seven other patients were referred for treatment of recurrent mammary sarcoma. In this group, median size of the primary tumor was 6 cm and four had high or intermediate grade histology. Tumor control was achieved for only one of five patients with local recurrence and neither of the two with distant metastases. Median survival was 6 months following initiation of treatment for recurrence. Whenever possible breast sarcomas should be classified according to histologic cell type and grade. For lesions not readily classified, the terms unclassified or anaplastic sarcoma should be used. The diagnosis of stromal sarcoma is best reserved for those infrequent sarcomas that can be traced to the specialized periductal and perilobular stroma of the breast. Total mastectomy is recommended for most patients with postoperative radiation therapy indicated when the adequacy of the margin is in doubt. The role of adjuvant chemotherapy in the primary management of mammary sarcoma is yet to be determined.     

Mediastinal hemangiomas

Hemangiomas of the mediastinum are rare tumors. Fewer than 100 have been reported. This study reviews the cases of 15 patients with mediastinal hemangiomas confirmed pathologically. We analyzed and evaluated the presentation, evaluation, and treatment. Follow-up is available for 14 of the 15 patients and ranges from 15 months to 15 years. Eight of the 15 patients were seen with signs and symptoms related to the tumor. This presentation correlated with invasion of contiguous mediastinal structures by the tumor. Six patients underwent total excision and 6, subtotal excision. During follow-up, residual tumor did not spread, become symptomatic, or show evidence of malignant degeneration. Based on this analysis, we believe that in patients in whom total excision of this tumor can be accomplished only by hazardous resection, a subtotal resection should be performed.     

Osteosarcomatous differentiation in phyllodes tumors.

Osteosarcomatous differentiation in phyllodes tumors is uncommon. The clinicopathologic features of 22 such cases in our files were retrospectively reviewed to evaluate the prognostic significance of this rare neoplasm. All patients were women between 40 and 83 years of age (mean, 60 years). Most (73%) presented with a palpable mass. None had prior irradiation to the breast or chest region. Patients were treated with excisional biopsy (N = 4), partial mastectomy (N = 1), or mastectomy (N = 17). All axillary nodes, dissected in 11 patients, were free of tumor. Two patients had extramammary spread at diagnosis. The neoplasms measured 1.9-15 cm (mean, 6.4 cm); 54% were grossly circumscribed or multilobulated. The osteosarcomatous component was classified as fibroblastic (N = 11), osteoclastic (N = 6), or osteoblastic (N = 5) and occupied a variable percentage of the phyllodes' stroma ranging from -25% to essentially 100% of the neoplasm. Of 21 patients with available follow-up, 11 (52%) were alive at a median follow-up of 44 months. Nine patients (43%) developed locally recurrent (N = 1) or metastatic (N = 8) disease. Metastases were clinically apparent within 1 year of diagnosis in all eight patients; seven died within 12 months of detection of initial metastasis. By univariate analysis, gross tumor size and osteosarcoma subtype significantly correlated with prognosis. In a multivariate analysis, neither of these factors were independent prognosticators. Phyllodes tumors with an osteosarcomatous component are potentially aggressive neoplasms, particularly when large (>5 cm) or associated with an osteoclastic or osteoblastic osteosarcoma. Complete excision without axillary dissection is advised.     

Diagnosis and treatment of breast fibroadenomas by ultrasound-guided vacuum-assisted biopsy

HYPOTHESIS: Ultrasound-guided vacuum-assisted biopsy (UGVAB) can serve as an efficient tool for the diagnosis and excision of breast fibroadenomas. DESIGN: Patients with a clinically and radiographically suspected breast fibroadenoma were prospectively referred for UGVAB to confirm the diagnosis and to attempt to excise the lesion. PATIENTS: Fifty-two female patients, aged 19 to 68 years, were included in the 2-year study. All had at least 1 suspected fibroadenoma. The procedure was performed for a total of 56 lesions. INTERVENTIONS: Imaging modalities prior to biopsy to confirm the clinical suspicion included Doppler ultrasound and mammography or Doppler ultrasound alone. Tumor size and volume were recorded. Ultrasound-guided vacuum-assisted biopsy was performed in all cases, with guidance using the 11-gauge Mammotome handheld vacuum-assisted biopsy system (Ethicon Endo-Surgery Inc, Cincinnati, Ohio). MAIN OUTCOME MEASURES: Major end points included diagnosis compatibility rate, excision rate, complications, and short-term follow-up. RESULTS: A tissue diagnosis was obtained in all cases and was compatible with the clinical diagnosis of fibroadenoma. Complete excision was achieved in all lesions less than or equal to 1.5 cm (mean volume, 0.25 mL). All lesions greater than 2 cm (mean volume, 1 mL) were incompletely excised. Of the 20 lesions measuring 1.5 to 2.0 cm, 11 (55%) were completely excised. The volume of all completely excised lesions was less than 0.9 mL. Four lesions with a volume less than 0.9 mL were incompletely excised due to bleeding. Ten of the 13 cases with incomplete excision were confident enough with the diagnosis to choose imaging follow-up instead of surgery. Two patients (16%) were referred by the radiologist for surgical excision. Only 1 patient with incomplete removal (8%) felt uncomfortable with the remnant lump and requested surgical excision. CONCLUSIONS: Although the breast fibroadenoma is a common benign breast tumor, the treatment and follow-up of these lesions is still debatable. We suggest that UGVAB, which has a well-documented role in the diagnosis of breast lesions, may provide an option for the definitive treatment of breast fibroadenomas.     

Spontaneous resolution of multiple hemangiomas of the brain. Case report

A case of multiple intracerebral hemangiomas is reported. The lesions were angiographically hypervascular and accompanied by marked perifocal brain edema. After removal of the main lesion and postoperative steroid administration, the rest of the lesions resolved spontaneously. Histologically, the lesion was composed of capillaries and fibromyxoid stroma. The distinctive clinical, radiological and histological features of this lesion are discussed in contrast to several other types of intracranial vascular tumors.