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Radiology Case Reports Oct 2021Malignant myoepithelioma of the scrotum is extremely rare. We report the case of a 51-year-old man with malignant myoepithelioma of the scrotum, wherein computed...
Malignant myoepithelioma of the scrotum is extremely rare. We report the case of a 51-year-old man with malignant myoepithelioma of the scrotum, wherein computed tomography and magnetic resonance imaging revealed a lobulated soft tissue mass with calcification, cystic component, and solid component with gradual contrast enhancement on dynamic contrast-enhanced scans. The patient presented with scrotal induration, and there was no elevation of tumor markers and no evidence of a metastatic lesion on computed tomography and magnetic resonance imaging. Histopathological examination of the resected scrotal specimen confirmed a well-circumscribed solid tumor with septa, a small area of hemorrhage, and necrosis. The subsequent diagnosis was malignant myoepithelioma of the scrotum. This case shows that scrotal malignant myoepithelioma might appear as a well-defined lobulated mass with cystic regions. We conjecture that the enhancement pattern and apparent diffusion coefficient values can be potential markers for scrotal myoepithelial tumors.
PubMed: 34401034
DOI: 10.1016/j.radcr.2021.07.013 -
Advances in Therapy Aug 2019A vast increase in knowledge of numerous aspects of malignant salivary gland tumours has emerged during the last decade and, for several reasons, this has not been the... (Review)
Review
INTRODUCTION
A vast increase in knowledge of numerous aspects of malignant salivary gland tumours has emerged during the last decade and, for several reasons, this has not been the case in benign epithelial salivary gland tumours. We have performed a literature review to investigate whether an accurate histological diagnosis of the 11 different types of benign epithelial salivary gland tumours is correlated to any differences in their clinical behaviour.
METHODS
A search was performed for histological classifications, recurrence rates and risks for malignant transformation, treatment modalities, and prognosis of these tumours. The search was performed primarily through PubMed, Google Scholar, and all versions of WHO classifications since 1972, as well as numerous textbooks on salivary gland tumours/head and neck/pathology/oncology. A large number of archival salivary tumours were also reviewed histologically.
RESULTS
Pleomorphic adenomas carry a considerable risk (5-15%) for malignant transformation but, albeit to a much lesser degree, so do basal cell adenomas and Warthin tumours, while the other eight types virtually never develop into malignancy. Pleomorphic adenoma has a rather high risk for recurrence while recurrence occurs only occasionally in sialadenoma papilliferum, oncocytoma, canalicular adenoma, myoepithelioma and the membranous type of basal cell adenoma. Papillomas, lymphadenoma, sebaceous adenoma, cystadenoma, basal cell adenoma (solid, trabecular and tubular subtypes) very rarely, if ever, recur.
CONCLUSIONS
A correct histopathological diagnosis of these tumours is necessary due to (1) preventing confusion with malignant salivary gland tumours; (2) only one (pleomorphic adenoma) has a considerable risk for malignant transformation, but all four histological types of basal cell adenoma can occasionally develop into malignancy, as does Warthin tumour; (3) sialadenoma papilliferum, oncocytoma, canalicular adenoma, myoepithelioma and Warthin tumour only occasionally recur; while (4) intraductal and inverted papilloma, lymphadenoma, sebaceous adenoma, cystadenoma, basal cell adenoma (apart from the membranous type) virtually never recur. No biomarker was found to be relevant for predicting recurrence or potential malignant development. Guidelines for appropriate treatment strategies are given.
Topics: Adenoma; Adenoma, Pleomorphic; Adult; Aged; Aged, 80 and over; Female; Humans; Male; Middle Aged; Salivary Glands
PubMed: 31209701
DOI: 10.1007/s12325-019-01007-3 -
Frontiers in Oncology 2022Among sarcomas, which are rare cancers with an incidence of <6 per 100.000/year cases, ultra-rare sarcomas have an incidence of approximately ≤1/1,000,000/year cases...
BACKGROUND
Among sarcomas, which are rare cancers with an incidence of <6 per 100.000/year cases, ultra-rare sarcomas have an incidence of approximately ≤1/1,000,000/year cases and altogether account for ~20% of all soft tissue sarcomas (STS) and bone sarcomas. The Italian Sarcoma Group has recently performed a non-interventional, retrospective TrObs study with data from 512 anthracycline-pretreated patients with advanced multiple STS histologies and treated with trabectedin (Palmerini, 2021; ClinicalTrials.gov Identifier: NCT02793050).
METHODS
A analysis of case series to evaluate the efficacy and safety of trabectedin on patients with ultra-rare and other rare translocation-related sarcomas included in TrObs study was performed. Main outcomes comprised investigator-assessed overall response rate (ORR), disease control rate (DCR), progression-free survival (PFS) and safety.
RESULTS
Thirty-six patients (18 women) with ultra-rare and other rare sarcoma and a median age of 53.0 years (range: 22-81) were included. Most patients had solitary fibrous tumor (SFT; n=11) followed by epithelioid sarcoma (n=5), malignant peripheral nerve sheath tumor (MPNST; n=4), extraskeletal myxoid chondrosarcoma (EMC; n=3), desmoplastic small round cell tumor (DSRCT; n=3), and alveolar soft part sarcoma (ASPS), rhabdomyosarcoma and clear cell sarcoma (n=2 each). Thirty-five patients had metastatic disease and 23 patients received trabectedin as a second-line treatment. Among 35 patients evaluable for response, two patients with SFT and ASPS had a partial response and one patient with DSRCT obtained a complete response, reaching an ORR of 8.6% (95% CI: 2.8-23.4%). Among patients with an ORR, 6-months PFS was 100% in patients with ASPS, 45.7% in patients with SFT and 33.3% in those with DSRCT. Two patients with epithelioid sarcoma and myoepithelioma had disease stabilization lasting >24 months. Nine patients had at least one grade 3/4 adverse event, mostly being bone marrow toxicity (n=6).
CONCLUSIONS
Trabectedin has some anti-tumor activity in some ultra-rare and other rare sarcomas, particularly translocation-related sarcomas, with the well-known manageable safety profile.
PubMed: 36568164
DOI: 10.3389/fonc.2022.1042479 -
The British Journal of Radiology Jul 2010We report an extremely rare case of malignant myoepithelioma presenting as a cortical osseous lesion on the humeral shaft with a satellite lesion. A 21-year-old man... (Review)
Review
We report an extremely rare case of malignant myoepithelioma presenting as a cortical osseous lesion on the humeral shaft with a satellite lesion. A 21-year-old man presented with persistent pain of the right upper arm after local trauma that had occurred 2 months earlier. Radiological examination revealed an expansile osseous tumour based on the cortex of the humeral shaft as well as a satellite lesion. En bloc resection was performed. Microscopic examination with immunohistochemical staining was used to establish a diagnosis of malignant myoepithelioma. Osseous malignant myoepithelioma occurring in long tubular bones rather than in bony structures with salivary tissue is extremely rare. Here, we demonstrate radiological and pathological features of a malignant myoepithelioma that developed in the cortex of the humeral shaft and review previously reported cases.
Topics: Bone Neoplasms; Diagnosis, Differential; Humans; Humerus; Magnetic Resonance Imaging; Male; Myoepithelioma; Positron-Emission Tomography; Young Adult
PubMed: 20603404
DOI: 10.1259/bjr/64670838 -
Head and Neck Pathology Jul 2013Fat-containing tumors of the salivary glands are uncommon. Their wide histological spectrum varies from pure lipomatous neoplasms similar to their cutaneous and soft... (Review)
Review
Fat-containing tumors of the salivary glands are uncommon. Their wide histological spectrum varies from pure lipomatous neoplasms similar to their cutaneous and soft tissue counterparts to mixed lipoepithelial lesions specific to the salivary glands. With few exceptions, these uncommon lesions affect mainly the elderly, with a mean age at presentation of ≥ 50 years and show a predilection for males. A few cases occur in childhood; some of them represent congenital lesions. In decreasing order of frequency, ordinary (soft-tissue type) lipoma, oncocytic lipoadenoma, non-oncocytic sialolipoma, and pleomorphic adenoma/myoepithelioma with extensive lipometaplasia are the main variants of fat containing tumors encountered in the salivary glands. While pleomorphic adenoma/myoepithelioma with lipometaplasia behave in the same way as their non-fat-containing counterparts, other lipomatous salivary gland tumors listed above are cured with simple excision and do not carry a risk of recurrence. Other lipoma variants (spindle cell lipoma, osteolipoma, fibrolipoma, angiolipoma, pleomorphic lipoma, lipoblastoma and hibernoma) are exceptionally rare in the salivary gland. Atypical lipomatous tumors/liposarcoma have been only rarely reported in the salivary gland and they behave in a similar fashion to their soft-tissue counterparts. Diffuse lipomatosis and lobular fatty atrophy are the two tumor-like lesions that might closely mimic sialolipoma, particularly in limited biopsy material without knowledge of the gross findings. This review summarizes the clinicopathological features of the main types of salivary fat-containing lesions and discusses their differential diagnoses.
Topics: Aged; Female; Humans; Male; Middle Aged; Neoplasms, Adipose Tissue; Salivary Gland Neoplasms
PubMed: 23821211
DOI: 10.1007/s12105-013-0459-7 -
Head and Neck Pathology Jul 2013Myoepithelial neoplasms are tumors composed almost exclusively of cells with myoepithelial differentiation. They frequently contain spindle, plasmacytoid or epithelioid... (Review)
Review
Myoepithelial neoplasms are tumors composed almost exclusively of cells with myoepithelial differentiation. They frequently contain spindle, plasmacytoid or epithelioid shaped cells and may have oncocytic or clear cytoplasmic features. They are uncommon, accounting for 1.5 % of all salivary gland tumors and for 2.2-5.7 % of major and minor salivary gland tumors, respectively. Recently this author, together with several colleagues, have described three unusual myoepithelial tumors, two benign and one malignant that contained abundant intracellular mucin material, which they termed the mucinous variant of myoepithelioma. This represents a unique, previously undescribed subtype that does not fit in the current classification system. A literature review revealed several similar cases reported as "signet ring-cell" adenocarcinomas of salivary gland, which stained for myoepithelial markers, in addition to containing intracellular mucin material, that are more accurately classified as mucinous myoepithelioma. To date, there are 17 reported mucinous myoepitheliomas; four were classified as benign and 13 as malignant. Thirteen arose in minor salivary glands and four in the parotid gland. One patient presented with a lymph node metastasis. With minimal follow-up currently available, this appears to be a benign to low-grade malignancy.
Topics: Biomarkers, Tumor; Cystadenocarcinoma, Mucinous; Humans; Immunohistochemistry; Myoepithelioma; Salivary Gland Neoplasms
PubMed: 23821216
DOI: 10.1007/s12105-013-0464-x -
CytoJournal Jan 2007Myoepithelioma of the breast is a rare tumor and the cytologic features have only been described in one previous report.
BACKGROUND
Myoepithelioma of the breast is a rare tumor and the cytologic features have only been described in one previous report.
CASE PRESENTATION
The present case comprises a 70 year old woman with a mammographic equivocal and ultrasonographic suspicious lesion. The aspirates were cellular and consisted mainly of single spindle or polymorphic, polygonal cells. The nuclei were generally large, ranging from 2 - > 5 x RBC. Most nuclei had a distinct medium-sized nucleolus. The nuclear outlines were irregular with buds and folds. The chromatin was granular. In the background there was abundant granular metachromatic ground substance and some metachromatic stromal fragments. A few mitotic figures were found. The cytologic diagnosis was suspicious for malignancy and a metaplastic carcinoma where only the non-epithelial component had been aspirated, or a non-epithelial lesion, was suggested.Macroscopically the tumor was round, seemingly well circumscribed, firm and with a white cut surface. The lesion consisted of spindled and polygonal cells with distinct pleomorphism. There were 6-9 mitoses per high power field (HPF). The tumor infiltrated in the surrounding fatty tissue. On immunohistochemistry, tumor cells were positive for smooth muscle actin, keratin MNF 116 and vimentin. Desmin and S-100 were negative.Ultrastructurally, there were abundant tonofilaments, including globular filamentous bodies and granulated endocytoplasmic reticulum with many dilated cisterns. The histologic diagnosis was malignant myoepithelioma.
CONCLUSION
The case mirrors completely the WHO definition and the previous cytological and histological descriptions of malignant myoepitheliomas in the literature which describe a spindle cell population with unequivocal nuclear atypia, metachromatic background substance and mitoses.
PubMed: 17250774
DOI: 10.1186/1742-6413-4-3 -
Head and Neck Pathology Mar 2023Myoepithelial neoplasms of the salivary gland are benign or malignant neoplasms composed exclusively of neoplastic myoepithelial cells. These tumors, including the... (Review)
Review
BACKGROUND
Myoepithelial neoplasms of the salivary gland are benign or malignant neoplasms composed exclusively of neoplastic myoepithelial cells. These tumors, including the benign myoepithelioma and the malignant counterpart myoepithelial carcinoma, exhibit a wide range of cytomorphologic features and architectural patterns.
METHODS
Review.
RESULTS
Myoepithelial cells can be epithelial, plasmacytoid, clear cell, spindle cell, and/or oncocytic cell, arranging as trabeculae, solid sheets, nests, cords, and/or single cells. A stromal component is commonly but not universally present, Therefore, their differential diagnoses are quite broad, including salivary gland neoplasms especially those with a myoepithelial component, plasmacytoma, melanoma, and various mesenchymal tumors.
CONCLUSION
In this review, we summarize the characteristic histologic features, useful immunohistochemical panel, and common molecular alterations of myoepithelial tumors and their top differential diagnoses. A logical stepwise algorithmic approach and an immunohistochemical panel to include multiple myoepithelial markers are essential to establish the correct diagnosis.
Topics: Humans; Biomarkers, Tumor; Immunohistochemistry; Salivary Gland Neoplasms; Salivary Glands; Myoepithelioma; Carcinoma
PubMed: 36928733
DOI: 10.1007/s12105-022-01502-0 -
The International Journal of... 2011Over the last few years, the discovery of basal-type mammary carcinomas and the association of the regenerative potential of the mammary epithelium with the basal... (Review)
Review
Over the last few years, the discovery of basal-type mammary carcinomas and the association of the regenerative potential of the mammary epithelium with the basal myoepithelial cell population have attracted considerable attention to this second major mammary lineage. However, many questions concerning the role of basal myoepithelial cells in mammary morphogenesis, functional differentiation and disease remain unanswered. Here, we discuss the mechanisms that control the myoepithelial cell differentiation essential for their contractile function, summarize new data concerning the roles played by cell-extracellular matrix (ECM), intercellular and paracrine interactions in the regulation of various aspects of the mammary basal myoepithelial cell functional activity. Finally, we analyze the contribution of the basal myoepithelial cells to the regenerative potential of the mammary epithelium and tumorigenesis.
Topics: Animals; Breast; Breast Neoplasms; Cell Communication; Cell Differentiation; Epithelial Cells; Extracellular Matrix; Female; Humans; Mammary Glands, Animal; Mice; Myoepithelioma; Paracrine Communication; Signal Transduction; Stem Cells
PubMed: 21948739
DOI: 10.1387/ijdb.113385mm -
Advanced Biomedical Research 2012Myoepitheliomas are benign neoplasms of salivary glands derived from myoepithelial cells. These tumors can occur at any age but are most common in young adults. This...
Myoepitheliomas are benign neoplasms of salivary glands derived from myoepithelial cells. These tumors can occur at any age but are most common in young adults. This tumor is usually located in the parotid gland and the minor salivary glands of the soft palate and represents less than 1% of all salivary gland tumors. The myoepithelioma is classified in the follow cells types: spindle, plasmacytoid, reticular, epitheliod, and clear, additionally, mixed histological forms are described. The plasmacytoid myoepithelioma from palate salivary glands is considered as a rare entity. A 45-year-old lady presented with an asymptomatic, well-circumscribed, solid mass located on the hard palate, which was gradually increasing in size. A clinical impression of Pleomorphic Adenoma was made which on histopathological examination revealed cords, clusters, and sheets of homogenous, large cells with plasmacytoid characteristics and a prominent eosinophilic cytoplasm. Ductal and acinar differentiation were absent thus ruling out the pleomorphic adenoma, whereas, features consistent with plasmacytoid myoepithelioma were evident.
PubMed: 23326808
DOI: 10.4103/2277-9175.102985