Adenomyoepithelioma of the breast. A spectrum of biologic behavior.

Adenomyoepitheliomas of the breast have been considered to have limited metastatic potential; axillary node metastasis has been reported, but there has been no report of distant metastasis. We report six cases, including two malignant adenomyoepitheliomas, one of which metastasized to the lung and brain. Patient age ranged from 26 to 63 years (mean 46). Primary tumors were solitary and measured 0.9-3.5 cm (mean 1.7). Five of six tumors presented as palpable masses. Two patients treated by local resection have no evidence of disease at 5 and 18 months' follow-up. Two patients treated by local resection had recurrences, one at 48 the other at 60 months. The fifth patient had a spindle-cell type adenomyoepithelioma diagnosed as malignant because of high mitotic rate and cytologic atypicality of the myoepithelial component. This patient was treated by mastectomy and has no evidence of disease at 18 months. The sixth patient, initially treated by local excision, had six local recurrences over 52 months treated by reexcisions, mastectomies, and radiation. A lung metastasis was resected at 54 months and brain metastases were identified at 60 months with death occurring at 64 months. Both malignant adenomyoepitheliomas had high mitotic rates [11-14/10 high-power fields (HPF)] diffusely throughout the tumors and foci of cytologically malignant cells. The malignant adenomyoepithelioma that metastasized had an infiltrative growth pattern that increased with successive local recurrences. The four other tumors had only isolated areas of mitotic activity (maximum 1-9/10 HPF) and minimal cytologic atypia. Immunohistochemistry performed on five of six cases confirmed dual epithelial/myoepithelial cell populations in all tumors examined, including the metastasis. Electron microscopic examination of the malignant adenomyoepithelioma that metastasized also confirmed dual epithelial/myoepithelial cell populations in a local recurrence and the lung metastasis. We conclude that there is a spectrum of behavior for breast adenomyoepitheliomas with potential for local recurrence and, rarely, distant metastasis.     

Myoepithelial Neoplasms of Soft Tissue: An Updated Review of the Clinicopathologic,Immunophenotypic, and Genetic Features

Myoepithelial tumors in skin and soft tissue are uncommon but have been increasingly characterized over the past decade. Men and women are equally affected across all age groups and lesions arise most frequently on the extremities and limb girdles. Approximately 20 % of cases occur in pediatric patients, in whom they are frequently malignant. Similar to their salivary gland counterparts, myoepithelial tumors of soft tissue demonstrate heterogeneous morphologic and immunophenotypic features. Tumors are classified as mixed tumor/chondroid syringoma, myoepithelioma, and myoepithelial carcinoma; in soft tissue, tumors having at least moderate cytologic atypia are classified as malignant. Mixed tumor and myoepithelioma show a benign clinical course, with recurrence in up to 20 % (typically secondary to incomplete excision), and do not metastasize. In contrast, myoepithelial carcinoma shows more aggressive behavior with recurrence and metastasis in up to 40–50 % of cases. The majority of myoepithelial neoplasms typically coexpress epithelial antigens (cytokeratin and/or EMA) and S-100 protein; GFAP and p63 are frequently positive and a subset of malignant neoplasms lose INI1 expression. Up to 45 % of myoepitheliomas and myoepithelial carcinomas harbor EWSR1 gene rearrangements, unlike mixed tumor/chondroid syringoma which is characterized by PLAG1 gene rearrangement. While mixed tumor/chondroid syringoma are likely related to primary salivary myoepithelial tumors, soft tissue myoepithelioma and myoepithelial carcinoma appear to be pathologically distinct neoplasms.     

Malignant Adenomyoepithelioma of the Breast with Lung Metastases: Report of a Case

Adenomyoepithelioma of the breast is a rare lesion, and has a bicellular pattern of epithelial and myoepithelial cells which are regularly distributed in the tubular structures based on the histologic and ultrastructural features. It is thought to be a benign or a low-grade malignant disease. We herein describe a case of malignant adenomyoepithelioma of the breast with lung metastases in an 86-year-old woman. A primary massive tumor in the left breast grew rapidly within a short period of time. A simple mastectomy with sampling of the axillary lymph nodes was performed. The obtained lymph nodes did not include any metastatic lesions. Malignancy was evidenced by the presence of a high mitotic rate and severe nuclear atypia. Three months after the operation, radiology showed multiple lung metastases, and the patient died 2 weeks thereafter. Reviewing the literature, nine similar cases were reported, and the prognosis of malignant adenomyoepithelioma of the breast with distant metastases was very poor with the time of recurrence varying after initial treatments. Malignant adenomyoepithelioma should be followed up with careful screening for distant metastases. Received: November 16, 2000 / Accepted: May 15, 2001     

Adenomyoepithelial Adenosis of the Breast: Clinical, Radiological, and Pathological Findings for Differential Diagnosis

BACKGROUND: Myoepithelial cells are widely present in the breast, and their hyperplasia may result in a spectrum of disease ranging from myoepitheliosis to myoepithelial carcinoma. CASE REPORT: A 46-year-old woman presented with a palpable mass in her right breast. Mammography and ultrasonography showed a lesion in the upper quadrant of the right breast with spiculated borders and shape. Excisional biopsy showed adenomyoepithelial adenosis. CONCLUSIONS: Although considered benign, adenomyoepithelial lesions tend to recur due to inadequate excision. Therefore, possibility of recurrence and even metastasis should be considered during follow-up of patients with a diagnosis of adenomyoepithelial lesions.     

Solitary, late metastatic recurrence of renal cell carcinoma: Two extraordinary cases

Late recurrence of renal cell carcinoma (RCC) has been well documented in the literature. We present two extraordinary cases of solitary, late metastatic recurrence of RCC. The first is a case of a solitary, adrenal metastasis excised 38 years after nephrectomy and the second is a case in which two solitary metastatic deposits were resected 14 and 26 years after excision of the primary tumor. In each of these patients the solitary metastases were initially believed to be primary tumors at other sites; however, on histological examination they were found to be metastatic RCC recurrences. In patients with a previous history of RCC presenting with apparently new solitary lesions, metastatic RCC must first be excluded.     

Myoepithelial Carcinoma: A Rare Neoplasm of the Breast

BACKGROUND: Malignant myoepitheliomas of the breast are extremely rare. There has been a limited number of published reports of myoepithelial carcinomas originating from the breast. CASE REPORT: We describe a malignant myoepithelioma of the breast in a 56-year-old woman. Histological examination showed polygonal epithelioid cells and spindle cells with moderate to marked nuclear atypia. Immunohistochemistry showed reactivity in the spindle cells for smooth muscle actin, cytokeratin (AE1/AE3), and p63, indicating a myoepithelial cell lineage of tumor cells. The patient underwent radical surgical excision of the lesion and axillary lymph node dissection. She demonstrated no evidence of recurrence over an 11-month follow-up. CONCLUSIONS: We suggest myoepithelial carcinomas of the breast be managed with appropriate surgical clearance. A multidisciplinary approach is usually required.     

Surgical treatment of ductal carcinoma in situ of the breast. 10- to 20-year follow-up.

Between 1967 and 1977, 36 patients received treatment at the Virginia Mason Medical Center in Seattle, Wash, for ductal carcinoma in situ of the breast. Twenty-five patients had modified radical mastectomies, 10 had radical mastectomies, and one had a simple mastectomy. Twenty-seven patients have been followed up for at least 10 years and are without known recurrence (mean follow-up, 17.7 years; range, 8 to 24 years), eight patients died without known recurrence (mean follow-up, 10.6 years; range, 6 to 14 years), and one patient with a prior contralateral mastectomy for infiltrating cancer of the breast had a recurrence in the scalene nodes on the side of the infiltrating cancer and died of metastatic cancer. No patients with ductal carcinoma in situ had local recurrences in the ipsilateral breast or chest wall, and no patients developed cancers in the contralateral breast; one patient had axillary metastasis. Twenty-eight (78%) of 36 patients had multicentric ductal carcinoma in situ in their mastectomy specimens. Twenty-three (88%) of 26 patients with comedocarcinoma-type ductal carcinoma in situ had multicentric lesions. Conversely, patients with low-grade nuclear papillary ductal carcinoma in situ did not have multicentric lesions. Five (14%) of 36 patients had incidental microinvasion discovered in the mastectomy specimens; all had comedocarcinoma. In summary, our study of patients with ductal carcinoma in situ revealed that (1) mastectomy provided excellent local and systemic control; (2) cancer in the contralateral breast was infrequent; (3) axillary metastasis was rare; and (4) histologic features of tumors markedly affected the frequency of multicentricity and chance for microinvasion.     

Treatment of Pure Tubular Carcinoma of the Breast

Abstract: A retrospective study of 63 cases of tubular breast carcinoma was performed to address issues of optimum therapy; in particular, the extent of surgery, the need for axillary lymph node dissection, and the need for postoperative radiation therapy. The patient's mean age at presentation was 57 years. The primary lesions were treated by local excision in 61% of cases and by some form of mastectomy in the remainder. Twenty-one patients were treated with radiation therapy after local excision. Axillary lymph node dissection was performed on 39 patients. The primary lesions ranged in size from 3 to 21 mm (mean 8 mm). Fifteen lesions were larger than 10 mm. Two patients had axillary lymph node metastases (5% of those were treated by axillary lymphadenectomy). These patients were 2 of 15 that had lesions larger than 10 mm. Therefore 13.3% of patients with tumors 10 mm or larger metastasized to axillary lymph nodes. In those two cases, the size of the primary tumor was 11 mm and 14 mm. All patients were free of disease at last follow-up (mean follow-up 4 years, 9 months; median follow-up 4 years). The study demonstrates excellent prognosis for patients with tubular breast carcinoma, regardless of the extent of the surgery, performance of axillary lymph node dissection, or administration of postoperative radiation therapy. Local excision may be adequate to control the primary disease and axillary lymph node dissection may not be necessary in patients with small lesions.     

Results of conservative treatment of upper urinary tract transitional cell carcinoma

BACKGROUND: The treatment preserving the kidney for upper urinary tract tumor is still controversial. The indications and results of conservative treatment remain to be elucidated. Experiences of this type of treatment are reported. METHODS: Between April 1981 and March 1998, 14 patients with upper urinary tract transitional cell carcinoma were treated with renal preserving methods. Five were elective and nine were imperative cases. Treatments performed were partial nephrectomy, partial ureterctomy with or without adjuvant chemotherapy, endoscopic tumor resection and topical bacillus Calmette-Guerin instillation in one, 10, two and one patient, respectively. RESULTS: Crude and cause-specific 5 year-survival rates were 91.7 and 100%, respectively. Of 14 patients, five had bladder recurrences, but ipsilateral local recurrence developed in only one patient. Two patients died from metastasis of transitional cell carcinoma 61 and 89 months after initial treatment. The lesions of carcinoma in situ were well controlled with topical bacillus Calmette-Guerin therapy. CONCLUSION: The results of conservative treatment for upper urinary tract tumor were satisfactory and local excision can be indicated for low grade, solitary tumors located in the distal ureter.     

Local excision of urothelial cancer of the upper urinary tract

In 9 of 93 patients (9.7%) with urothelial cancer of the upper tract (7 renal pelvis tumors, 3 ureteral tumors), conservative surgery was employed using a free peritoneal autotransplant for replacement of the renal pelvis in 5 kidneys. Absolute indications for conservative surgery were solitary kidneys/nonfunctioning contralateral kidneys in 4 patients and bilateral tumors in 1 patient. Local recurrences developed 1-3 years after operation in 4 of 6 kidneys (3 patients), 3 of which had grade-2 and grade-3 primary lesions. All patients were treated successfully by repeated local excision. In the presence of a normal contralateral kidney, local tumor excision was done electively in 4 patients (3 low-grade/low-stage lesions, 1 high-risk patient), none of these patients developed recurrences. Two patients died without evidence of tumor recurrence, 7 patients are free of tumor at an average follow-up of 23 months (range 5-65 months). Local excision of urothelial cancer should be considered not only for solitary kidneys, bilateral tumors and cases with renal failure, but also for low-stage/low-grade localized tumors, leaving the patient better prepared for later treatment of a possible recurrence due to the well recognized chance of a multiplicity of tumors in time and space.     

Preliminary results of conservative treatment of early breast cancer with tumourectomy, axillary dissection and postoperative radiotherapy. A retrospective review of 107 patients

A consecutive series of 107 patients with early breast cancer treated by tumourectomy, axillary dissection and postoperative radiotherapy was retrospectively reviewed. The average age at presentation was 48, range 30-79. Only 23.4% of women were post-menopausal. Average follow-up time was 48 months, range 19-94 months. Eight-three per cent had palpable lesions, the remaining 16.8% had needle localization. Ninety-four patients (88.7%) had lesions less than 2 cm in diameter clinically. One patient had a lesion greater than 3 cm in diameter clinically. Seventy-two per cent had invasive duct carcinoma. Twenty (18.9%) had invasive disease at the margins and fifteen were re-excised. Of the 20 patients who had invasive disease at the margins, three developed local recurrence. True loco-regional recurrence rate (i.e., loco-regional recurrence rate without distant metastasis) was 2.8%. All had level I and II axillary dissections and 26.4% were up-staged from clinical stage 1 to pathological stage II. Five patients died, three with recurrences and two with unrelated disease. Tumourectomy, axillary dissection and postoperative radiotherapy is an acceptable regimen for early carcinoma of the breast.     

A rare and unusual case of bilateral, axillary, metachronous apocrine carcinoma

Apocrine carcinoma is a rare sweat gland neoplasm with very few cases reported in the published literature. We report a case of primary axillary apocrine carcinoma with later recurrences in both axillae. A 55-year-old man was clinically diagnosed with hydradenitis suppurativa in the right axilla, and after excision of lesion, histology showed metastatic adenocarcinoma of probable breast origin. However, no primary focus was found after extensive work-up except for metastatic lymph nodes in the ipsilateral axilla treated with axillary clearance. After 4 years, the patient developed metastatic lymph nodes in the contralateral axilla and had surgery. He had a further recurrence in the right axilla and was treated with surgery and radiotherapy. He continues to do well at this time, with no evidence of local or distant metastases.     

Do basal cell carcinomas recur after complete conventional surgical excision?

For 1378 patients treated in the 11 years 1988-1998 by conventional excision of 1635 basal cell carcinomas, 1516 first index lesions were histologically completely excised. All patients having more than one BCC excised were identified from the data base from 1988 to 2003 to give minimum 5 years follow for last treated primary lesions in 1998. Measured clearance margins around the initial lesions at or near sites of presumptive recurrent lesions were noted and the lesions recorded photographically. All incompletely excised lesions whether or not re-excised were excluded. The median age for all patients was 70 years. Over minimum 5 years follow up, six patients developed nine subsequent lesions contiguous with the scar or graft repair of primary index lesion excision site (probable recurrences). The median interval to recurrence was 41 months (4 months-8 years 10 months), with median lateral clearance margin around the primary tumour of 2 mm (0.3-6.8 mm). A further nine patients developed 11 new lesions near (within 1cm of) the scar or graft of primary index lesion excision site (possible recurrences). The median interval to recurrence was 59 months (1 year-8 years 6 months). The median lateral clearance margin around the primary tumour was 4.1 mm (0.8-5.8 mm). For the two groups combined the maximum recurrence rate expressed as a percentage of index lesions was 1.3% (20/1516). Two thirds of possible and probable recurrences occurred in the temple and forehead, although these sites represented only 22% of all lesions, which may rather suggest new lesions in an area of field change as opposed to residual disease. The measured clearance margins reported here perhaps suggest that some original lesions may well have been completely excised primarily and many 'recurrences' were new primaries. These figures indicate there is a low order of probability for the incidence of recurrent basal cell carcinoma during minimum 5 years follow period after conventional surgical excision and conventional histological assessment of tumour resection margins.     

Salivary gland-type tumors with myoepithelial differentiation arising in pulmonary hamartoma: report of 2 cases of a hitherto unrecognized association

Reported is a hitherto unrecognized association of pulmonary hamartomas with salivary gland-type tumors showing myoepithelial differentiation, namely, a case of myoepithelioma arising in a otherwise classic hamartoma with cartilage predominance, and a case of malignant mixed tumor arising in a predominantly fibrous hamartoma resembling müllerian adenofibroma. The tumors occurred in middle-aged female patients of 35 and 44 years, respectively, and presented as 7 cm (treated with lobectomy) and 13 cm (treated with pneumonectomy) masses of the right upper lobe showing a short clinical history of cough, dyspnea, and wheezing. Both lesions did not present regional lymph node metastases after mediastinal lymphadenectomy. The myoepithelioma patient was well with no signs of recurrent disease at 6-month clinical control, but she was then lost to follow-up; the malignant mixed tumor patient is alive and well after 6 months since operation. Both tumors presented with morphologic and immunohistochemical features of myoepithelial cells, and we interpret them as being derived from a myoepithelial-like stromal cell population found within the hamartomatous areas, which is also consistently detected in classic pulmonary hamartoma. The lack of individual cell necrosis, mitotic activity, cell atypia, and pulmonary parenchyma infiltration supported a diagnosis of benign or unproven malignant potential tumor for the myoepithelioma, whereas the reverse held true for the other tumor in which the diagnosis of malignant mixed tumor of the lung was rendered. Their main importance of recognizing this association lies in separating these tumors histologically from other monophasic or biphasic tumors, either primary or secondary, such as pulmonary sarcomatoid carcinomas or true sarcomas, and metastatic salivary gland tumors, spindle cell carcinomas, melanomas, and soft tissue and visceral sarcomas.     

Villous tumors of the duodenum.

Five cases of villous tumors of the duodenum are reported, all of which involve the ampulla of Vater. Three of the five lesions contained either infiltrating carcinoma or carcinoma in situ. Although preoperative endoscopic biopsy was performed on all tumors no malignancy was identified. Frozen sections done at the time of operation on the three patients with carcinoma also failed to identify malignancy. One patient underwent pancreaticoduodenectomy and four patients had local excision of the tumor. Three of the patients treated with local excision developed recurrence and two subsequently had pancreaticoduodenectomy. Because of the difficulty in making an accurate diagnosis and the chance of recurrence when local excision is employed, strong consideration should be given to pancreaticoduodenectomy as the initial form of treatment of these lesions.     

Low-grade adenosquamous carcinoma. A variant of metaplastic mammary carcinoma

The prognostic significance of metaplasia in mammary carcinoma has not been well defined. Pseudosarcomatous metaplasia reportedly has an unfavorable impact on survival. Squamous metaplasia may have less effect on outcome except for the high-grade spindle cell form, which is particularly aggressive. This report describes 11 patients with a low-grade variant of adenosquamous metaplastic carcinoma which has a distinctive combination of glandular and squamous differentiation. The average age of the patients was 59 years (range 42-76). The tumors measured 1.5-3.4 cm (average 2.3) and were grossly hard, tan-yellow, and infiltrative. Follow-up in seven cases treated at least 1 year ago averages 3.5 years (range 1-6 years). Four of eight patients treated by local excision alone developed recurrence in the breast in 1-3.5 years. Negative lymph nodes were found in five axillary dissections. There have been no recurrences after mastectomy performed as primary therapy or for recurrence in seven cases. These findings suggest that patients with the low-grade adenosquamous variant of metaplastic carcinoma have a favorable prognosis. They may be good candidates for breast conservation therapy, but this will require adequate excision followed by primary radiotherapy.     

Tubular carcinoma of the breast. Clinical pathologic correlations based on 100 cases.

One hundred tubular carcinomas were reviewed, analyzed and compared with previously reported studies in the literature. Our cases were subdivided into five groups, according to the proportion of the carcinoma which was tubular. In the case of pure or almost pure tubular carcinomas (76% or more), tumor size was small, no metastases were found, no recurrences developed, and there were no deaths attributable to the carcinoma. As the proportion of the carcinoma which was tubular decreased, the size and biologic aggressiveness of the tumor increased; this it is likely that tubular carcinoma may represent an early form of carcinoma. A right-sided preponderance was found of the "pure tubular" carcinomas; and lesions of the central sector of the breast were rare in all five groups. The incidence of bilateral cancer was greater than that expected for breast cancer in general and included three patients with bilateral tubular carcinomas. On the basis of our findings, we suggest that lesions which are composed 90% or more of tubular carcinoma may be treated by simple mastectomy, and that axillary dissection is not necessary. For all carcinomas with a lower proportion of tubular elements, at least total mastectomy and axillary dissection are indicated, although the safest treatment probably is radical mastectomy.     

Adenomyoepithelioma: clinical, histologic, and immunohistologic evaluation of a series of related lesions

Adenomyoepithelioma, strictly defined, is a proliferation of both epithelial and myoepithelial elements. The broad range of lesions that may fall under this umbrella, however, may be quite diverse. The diagnostic confusion surrounding this entity and its prognostic implications have led to a diagnosis by default as malignant and to overtreatment of some patients. We evaluated available material from a series of 35 women whose slides were seen in consultation and who were diagnosed with adenomyoepithelioma or a closely related lesion. This comprehensive review of the varied histology of adenomyoepithelioma and similar lesions and their immunohistochemical properties will assist general pathologists in evaluating these sometimes difficult lesions. Follow-up and treatment information demonstrates their benignity. Architecture and histologic features should be combined with immunohistochemistry when determining categorization.     

Breast adenomyoepithelioma and adenomyoepithelioma with carcinoma (malignant adenomyoepithelioma) with associated breast malignancies: A case series emphasizing histologic,radiologic, and clinical correlation

The 2012 World Health Organization (WHO) classification of breast tumors distinguishes adenomyoepitheliomas (AMEs) as benign tumors composed of a biphasic proliferation of phenotypically variable myoepithelial cells around small epithelial lined spaces. Many AMEs have demonstrated benign behavior and are often cured with excision with negative margins, but some have exhibited malignant transformation of the myoepithelial cells, ductal epithelial cells, or both. When one of the components is histologically malignant, it is termed AME with carcinoma. Due to the rarity, the literature correlating imaging, histology, and clinical outcome is limited. A retrospective review was undertaken. A review of an institutional pathology database identified 14 cases with AME or malignant AME. Most AMEs had nonspecific imaging findings and were categorized as Bi-Rads 4. Histologic features of AME did not correlate with prior or concurrent breast malignancies or any radiographic features. Clinical follow up could be obtained for all but one case (mean follow up time = 75 months). 5 cases had no known treatment post-biopsy and 5 patients received mastectomy. No recurrences were noted. 3/13 cases of benign AME had associated breast malignancies including invasive ductal adenocarcinoma and ductal carcinoma in-situ. 1 case of malignant AME had a synchronous separate malignant phyllodes tumor. Given the unclear and unpredictable propensity for malignant transformation, conservative excision with negative margins currently seems appropriate.     

Solitary enchondroma at the hand. Long-term follow-up study after operative treatment

Twenty-one patients (17 women and four men) who underwent operative treatment for a solitary enchondroma of the hand were examined at a follow-up of between 2 and 18 years (mean, 9 years). Radiographs showed normal cancellous bone at the site of surgery in 11 cases, three had recurrent enchondroma and seven had bone defects so that recurrence could not be excluded. Two of the three recurrences underwent reoperation. Previous studies have regarded persistent bony defects as evidence of complete excision without recurrence. However, in view of the slow asymptomatic growth of this tumour this opinion is incorrect. As shown in this study, recurrences may occur in these defects many years after excision surgery and go undetected until they cause widening or cortical erosion. We recommend periodical radiological re-examination for asymptomatic recurrences before weakness of bone leads to pathological fracture.