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Oral Oncology Aug 2020Myopericytoma is a rare mesenchymal tumor characterized by a hemangiopericytoma-like vascular pattern with perivascular myoid differentiation. To date, only 11 cases of... (Review)
Review
Myopericytoma is a rare mesenchymal tumor characterized by a hemangiopericytoma-like vascular pattern with perivascular myoid differentiation. To date, only 11 cases of oral myopericytoma have been reported. To the best of our knowledge, myopericytoma with gingival involvement and associated with calcifications has not been reported, expanding their clinicopathological spectrum. Herein, we report a 12-year-old girl female patient who presented a gingival nodule diagnosed as ossifying myopericytoma, which should be considered in the differential diagnosis when assessing oral soft tissue lesions, especially in pediatric patients.
Topics: Child; Female; Humans; Immunohistochemistry; Myopericytoma
PubMed: 32505552
DOI: 10.1016/j.oraloncology.2020.104826 -
Asian Journal of Surgery Oct 2023
Topics: Humans; Myopericytoma; Magnetic Resonance Imaging
PubMed: 37286461
DOI: 10.1016/j.asjsur.2023.05.067 -
Histopathology Oct 2022In 2017, a subset of cellular variants of myofibroma and myopericytoma with a smooth muscle-like immunophenotype and harbouring recurrent SRF::RELA gene fusions was...
AIMS
In 2017, a subset of cellular variants of myofibroma and myopericytoma with a smooth muscle-like immunophenotype and harbouring recurrent SRF::RELA gene fusions was reported. Although the anatomical distribution was found to be quite broad, no tumours with these gene fusions in the female reproductive system have been illustrated to date.
METHODS AND RESULTS
Herein, we report the histological and immunophenotypical features of three uterine tumours with SRF::RELA gene fusions. Microscopically, all three tumours were composed of cellular oval to spindle cells arranged in intersecting fascicles with variable amounts of collagen and a rich capillary network. Mitotic figures were scant. Regarding immunohistochemistry, diffuse staining for desmin, oestrogen receptor and progesterone receptor was observed in all three cases. The first case exhibited focal staining for h-caldesmon, whereas the latter two cases had diffuse staining. Furthermore, SRF::RELA rearrangement was observed in all three cases by using next-generation sequencing (NGS). Follow-up, ranging from 11 to 15 months, was available for these three patients, all of whom were well without evidence of disease.
CONCLUSIONS
In conclusion, we reported a special group of uterine neoplasms with myogenic differentiation harbouring SRF::RELA rearrangement. Although the follow-up time was limited, morphological characteristics and other studies with follow-up data supported that this type of uterine neoplasm appeared to behave in a benign manner. Further studies with longer follow-up are needed to clarify the biological nature of this particular type of uterine tumour.
Topics: Biomarkers, Tumor; Female; Gene Fusion; Humans; Immunohistochemistry; Myofibroma; Oncogene Proteins, Fusion; Serum Response Factor; Transcription Factor RelA; Uterine Neoplasms
PubMed: 35852178
DOI: 10.1111/his.14724 -
Journal of Cutaneous Pathology Aug 2022Epithelioid angioleiomyoma (EALM) is rare in the skin and subcutaneous tissues. To the best of our knowledge, only two previous cases of this tumor have been reported....
Epithelioid angioleiomyoma (EALM) is rare in the skin and subcutaneous tissues. To the best of our knowledge, only two previous cases of this tumor have been reported. We document here the case of an 83-year-old woman who underwent complete removal of a squamous cell carcinoma of the retromolar trigone and lymph node dissection of the neck. An incidental EALM was observed in the adipose tissue. The tumor formed a unilocular, poorly demarcated neoplasm measuring 0.3 cm, and showed cavernous angiomatous spaces with villiform growth of large epithelioid cells arranged in clusters. Besides the epithelioid cells of muscular origin, bundles of well-differentiated smooth muscle cells were observed. Epithelioid cells accounted for 70% of the total. The neoplasm originated in the wall of a medium-sized vein. Epithelioid and spindle cells were positive for alpha-smooth muscle actin, calponin, h-caldesmon, and muscle-specific actin. The endothelial cells lining the vascular spaces showed intense and diffuse positivity for CD31 and ERG. The main differential diagnosis includes metastatic carcinoma, melanoma, perivascular epithelioid cell neoplasm, myopericytoma, glomangiomyoma, epithelioid glomus tumor, and epithelioid leiomyosarcoma. This report expands the morphological spectrum of the EALM. Awareness of this uncommon morphologic variant of angioleiomyoma and the use of adequate techniques can avoid misdiagnosis.
Topics: Actins; Aged, 80 and over; Angiomyoma; Biomarkers, Tumor; Endothelial Cells; Female; Glomus Tumor; Humans; Soft Tissue Neoplasms
PubMed: 35357042
DOI: 10.1111/cup.14233 -
International Journal of Surgery Case... Aug 2021Myopericytoma (MPC) is a rare benign soft tissue neoplasm that arises from perivascular smooth muscle-like myoid cells that share features of both glomus and smooth...
INTRODUCTION AND IMPORTANCE
Myopericytoma (MPC) is a rare benign soft tissue neoplasm that arises from perivascular smooth muscle-like myoid cells that share features of both glomus and smooth muscle cells. It usually slow growing solitary tumor that might mimic aggressive sarcoma.
CASE PRESENTATION
45 years old male, with unremarkable medical history, presented with fast growing mass on the left 1st webspace that was noticed 6 months prior to presentation. Investigations with US and MRI showed highly vascular solid mass that was worrisome for malignant lesion. Surgical excision was done and histopathology confirmed the diagnosis of myopericytoma.
CLINICAL DISCUSSION
MPC is a rare perivascular neoplasm that's reported mainly in the extremities with lower limbs are most affected sites. Usually it presents as solid painless slowly growing mass. However, Multiple papers reported different rare presentations like multiple, painful and some with malignant transformation.
CONCLUSION
MPC is a benign tumor that might mimic aggressive sarcoma. Such cases should be approached with high suspicion and proper investigation and management should be followed promptly.
PubMed: 34298419
DOI: 10.1016/j.ijscr.2021.106220 -
Neurosurgery Aug 2019Myopericytoma is an emerging class of neoplasm originating from the perivascular myoid cellular environment, previously classified as a variant of hemangiopericytoma.... (Review)
Review
BACKGROUND AND IMPORTANCE
Myopericytoma is an emerging class of neoplasm originating from the perivascular myoid cellular environment, previously classified as a variant of hemangiopericytoma. Most reported myopericytomas are found in soft tissues of the extremities; however, infrequent cases are described involving the central nervous system. Intracranial myopericytoma remains rare. Here, we describe the first report of myopericytoma occurring at the cervicomedullary junction in close proximity to the vertebral artery, mimicking a vascular lesion.
CLINICAL PRESENTATION
A 64-yr-old woman presented with radiating neck pain. Magnetic resonance imaging revealed a well-circumscribed enhancing lesion adjacent to the vertebral artery-accessory nerve complex. She underwent a far lateral craniotomy and cervical laminectomy to obtain proximal vertebral artery control and adequate exposure of the lesion, which appeared most consistent with neoplasm at surgery. Histopathology revealed a grade I myopericytoma. A gross total resection was achieved, and the patient has no evidence of recurrence 3 yr after surgery.
CONCLUSION
Tumors of perivascular origin include hemangiopericytoma, glomus tumor, myofibroma, and myopericytoma and are uncommon lesions intracranially. Consideration of and distinction among these perivascular tumors is critically important, as they each have distinct clinical behaviors and management. Myopericytoma can mimic other neoplastic and cerebrovascular pathologies, but it most commonly has a benign course and can be surgically cured if a gross total resection can be achieved. Rarer myopericytoma variants that adopt a more malignant course have been described, and ongoing molecular studies may identify mutations or activated signaling pathways that can be targeted to offer chemotherapeutic options in the future.
Topics: Cervical Vertebrae; Female; Humans; Magnetic Resonance Imaging; Middle Aged; Myopericytoma; Neck Pain
PubMed: 29889275
DOI: 10.1093/neuros/nyy262 -
British Journal of Neurosurgery Jun 2024Malignant myopericytoma is an exceedingly rare soft tissue tumour with only 14 documented cases in the scientific literature. Myopericytomas can occur in association... (Review)
Review
Malignant myopericytoma is an exceedingly rare soft tissue tumour with only 14 documented cases in the scientific literature. Myopericytomas can occur in association with HIV-AIDS and have a predilection for internal organs such as the bronchus, larynx, liver and brain. Ebstein-Barr virus encoded small RNA(EBER) was positive in three out of 14 cases. In this case report we present the second documented case of a malignant myopericytoma, with a positive EBER found intracranially - specifically in the supratentorial compartment - invading the superior sagittal sinus. Gross total excision of the tumour was achieved, and the patient responded well with no recurrence with only highly active antiretroviral therapy (HAART). No chemotherapy or radiotherapy has been necessary.
Topics: Humans; Myopericytoma; Male; Brain Neoplasms; Middle Aged; Antiretroviral Therapy, Highly Active; Magnetic Resonance Imaging; HIV Infections; Female; Adult
PubMed: 34259108
DOI: 10.1080/02688697.2021.1950630 -
Advances in Experimental Medicine and... 2019Tumors of mesenchymal origin are a diverse group, with >130 distinct entities currently recognized by the World Health Organization. A subset of mesenchymal tumors grow...
Tumors of mesenchymal origin are a diverse group, with >130 distinct entities currently recognized by the World Health Organization. A subset of mesenchymal tumors grow or invade in a perivascular fashion, and their potential relationship to pericytes is a matter of ongoing interest. In fact, multiple intersections exist between pericytes and tumors of mesenchymal origin. First, pericytes are the likely cell of origin for a group of mesenchymal tumors with a common perivascular growth pattern. These primarily benign tumors grow in a perivascular fashion and diffusely express canonical pericyte markers such as CD146, smooth muscle actin (SMA), platelet-derived growth factor receptor beta (PDGFR-β), and RGS5. These benign tumors include glomus tumor, myopericytoma, angioleiomyoma, and myofibroma. Second and as suggested by animal models, pericytes may give rise to malignant sarcomas. This is not a suggestion that all sarcomas within a certain subtype arise from pericytes, but that genetic modifications within a pericyte cell type may give rise to sarcomas. Third, mesenchymal tumors that are likely not a pericyte derivative co-opt pericyte markers in certain contexts. These include the PEComa family of tumors and liposarcoma. Fourth and finally, as "guardians" that enwrap the microvasculature, nonneoplastic pericytes may be important in sarcoma disease progression.
Topics: Adult; Animals; Glomus Tumor; Humans; Pericytes; Receptor, Platelet-Derived Growth Factor beta; Sarcoma; Soft Tissue Neoplasms
PubMed: 31147874
DOI: 10.1007/978-3-030-16908-4_4 -
Journal of Radiology Case Reports May 2023This case study presents a 66-year-old man referred to the Otolaryngology and Head and Neck Surgery department due to a one-history of persistent pain in the left...
This case study presents a 66-year-old man referred to the Otolaryngology and Head and Neck Surgery department due to a one-history of persistent pain in the left posterior cervical region. No abnormalities were detected in the oral and pharyngeal regions during clinical and endoscopic examinations. Subsequently, a magnetic resonance imaging revealed a lesion (14 × 12 × 14 mm) into the left parapharyngeal space, with high signal intensity on T2-weighted images, enhancement after contrast medium, restricted signal on diffusion weighted imaging and high vascularization on perfusion MRI. The histological examination of the lesion led to a diagnosis of myopericitoma. Post-surgery, no adjuvant therapy was administered. Myopericytomas are rare soft-tissue benign neoplasms, predominantly reported in extremities, with a limited number of cases in the head and neck region and almost never described in the literature with elective localization in the parapharyngeal space.
Topics: Humans; Male; Aged; Magnetic Resonance Imaging; Pharyngeal Neoplasms; Parapharyngeal Space; Myopericytoma; Contrast Media; Diagnosis, Differential
PubMed: 38828029
DOI: 10.3941/jrcr.v17i12.5186 -
Annals of Dermatology Dec 2022
PubMed: 36478436
DOI: 10.5021/ad.20.326