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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 -
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 -
Cureus Jan 2022Myopericytoma (MPC) is an uncommon benign neoplasm of the skin and soft tissues belonging to a spectrum of tumors that are histologically recognized by their distinctive...
Myopericytoma (MPC) is an uncommon benign neoplasm of the skin and soft tissues belonging to a spectrum of tumors that are histologically recognized by their distinctive perivascular myoid cell differentiation. These distinct tumors are more prevalent among middle-aged males, and they arise more frequently in the subcutaneous tissue of the four extremities. In this paper, myopericytoma is reported in a 59-year-old Saudi male, presented with a painless small cyst involving the left ankle suspected clinically to be a ganglion cyst. Following surgical excision of the cyst, the diagnosis of myopericytoma was made based on the histopathological pattern of the disease. This paper focuses on the clinical and histopathological findings of myopericytoma and emphasizes the importance of immunohistochemistry as well as molecular testing in reaching the final diagnosis.
PubMed: 35186568
DOI: 10.7759/cureus.21307 -
Modern Pathology : An Official Journal... Mar 2023Pericytic tumors are subclassified as myopericytomas, myofibromas, angioleiomyomas, and glomus tumors according to the current World Health Organization classification....
Pericytic tumors are subclassified as myopericytomas, myofibromas, angioleiomyomas, and glomus tumors according to the current World Health Organization classification. These pericytic tumors form a continuous morphologic spectrum, including those with combined morphology. However, to our knowledge, no widely accepted criteria for classifying tumors with combined morphology are available. Recent studies have identified platelet-derived growth factor receptor-beta (PDGFRB) gene mutations in a subset of myofibromas, myopericytomas, and myopericytomatoses but not in angioleiomyomas. NOTCH receptor 3 (NOTCH3) mutations have been reported in a subset of infantile myofibromatosis. To assess their potential role in classifying pericytic tumors, we investigated PDGFRB and NOTCH3 mutations in 41 pericytic tumors of variable morphology, including some combined forms. Our results show these mutations to be present in a variety of pericytic tumors, such as myopericytomas (PDGFRB, 3/11; NOTCH3, 4/11), myopericytomatoses (1/2; 1/2), myofibromas (3/6; 0/6), angioleiomyomas (2/13; 3/13), and glomus tumors (5/9; 1/9). Point mutations were identified in 3 tumors in PDGFRB exon 12 (Y562C, S574F, and G576S), 12 tumors in PDGFRB exon 14 (M655I, H657L, and N666K), and 9 tumors in NOTCH3 exon 25 (A1480S/T, D1481N, G1482S, T1490A, E1491K, G1494S, and V1512A). All PDGFRB mutations and NOTCH3 G1482S, T1490A, and G1494S mutations were classified as "deleterious/damaging" by ≥4 of 6 pathogenicity prediction tools in silico. Five-mutation-positive tumors, including 1 myopericytoma-angioleiomyoma, 2 myopericytomatoses-myofibroma, 1 myofibroma-myopericytoma and 1 angioleiomyoma-myopericytoma, were of combined morphology. Therefore, we found PDGFRB and NOTCH3 mutations to be detectable in a much wider variety of pericytic tumors than previously reported and confirmed myopericytomas, myofibromas, angioleiomyomas, and glomus tumors as members harboring PDGFRB or NOTCH3 mutations. Our results thus suggest that PDGFRB or NOTCH3 mutations are not useful for subclassifying members of the pericytic tumor family.
Topics: Humans; Myopericytoma; Angiomyoma; Glomus Tumor; Myofibroma; Receptor, Platelet-Derived Growth Factor beta; Mutation; Receptor, Notch3
PubMed: 36788105
DOI: 10.1016/j.modpat.2022.100070 -
Diagnostics (Basel, Switzerland) Oct 2022Myopericytoma is a rare vessel wall tumor, a subtype of hemangiopericytoma that usually develops subcutaneously. Intravascular myopericytoma is a rarer subtype, with...
Myopericytoma is a rare vessel wall tumor, a subtype of hemangiopericytoma that usually develops subcutaneously. Intravascular myopericytoma is a rarer subtype, with only few cases reported in the literature and even fewer with imaging modalities included. We report the case of a 36-year-old man who was referred to our institution with a painless, palpable mass in the right arm and was evaluated with MRI, grey-scale and Doppler-mode ultrasound. Tumor histopathology and imaging characteristics are presented together with the role that each imaging modality played in the management of the patient.
PubMed: 36292162
DOI: 10.3390/diagnostics12102473 -
Ear, Nose, & Throat Journal Jan 2023A myopericytoma in the auricle is rare. If an auricle contains a large, firm, red-brown mass, excision should be considered because the mass may be a myopericytoma....
A myopericytoma in the auricle is rare. If an auricle contains a large, firm, red-brown mass, excision should be considered because the mass may be a myopericytoma. After excision, histopathological and immunohistochemical diagnoses are essential to rule out malignancy. Long-term follow-up is required because the tumor is slow-growing.
Topics: Humans; Neoplasms
PubMed: 33356520
DOI: 10.1177/0145561320984576 -
Respiratory Medicine Case Reports 2021Myopericytoma is a benign neoplasm presenting cells with different shapes, from oval to spindle, and myoid showing with perivascular growth, which frequently originates...
Myopericytoma is a benign neoplasm presenting cells with different shapes, from oval to spindle, and myoid showing with perivascular growth, which frequently originates from the skin and soft tissues of distal extremities, trunk, head, and neck regions. These tumors rarely have been reported to occur in visceral sites. There is only one case of myopericytoma showing pulmonary involvement with multiple nodules. Although most myopericytomas behave in a benign manner, some cases of malignant myopericytoma arising in both superficial soft tissue and visceral locations have been described. We describe two cases of pulmonary tumors with myopericytoma-like features.
PubMed: 33614407
DOI: 10.1016/j.rmcr.2021.101355 -
The American Journal of Surgical... Jan 2020Pericytic tumors comprise a histologic continuum of neoplasms with perivascular myoid differentiation, which includes glomus tumors, myopericytoma, myofibroma, and...
Pericytic tumors comprise a histologic continuum of neoplasms with perivascular myoid differentiation, which includes glomus tumors, myopericytoma, myofibroma, and angioleiomyoma. Despite their morphologic overlap, recent data suggest a dichotomy in their genetic signatures, including recurrent NOTCH gene fusions in glomus tumors and PDGFRB mutations in myofibromas and myopericytomas. Moreover, SRF-RELA fusions have been described in a subset of cellular variants of myofibroma and myopericytoma showing myogenic differentiation. Triggered by an index case of an unclassified cellular myoid tumor showing a novel SRF-ICA1L fusion we have investigated our files for cases showing similar histology and screened them using a combined approach of targeted RNA sequencing and fluorescence in situ hybridization. A fusion between SRF exon 4 and ICA1L exon 10 or 11 was identified in a total of 4 spindle cell tumors with similar clinicopathologic features. Clinically, the tumors were deep-seated and originated in the trunk or proximal lower extremity of adult patients (age range: 23 to 55 y). Histologically, the tumors were composed of cellular fascicles of monomorphic eosinophilic spindle cells showing increased mitotic activity, harboring densely hyalinized stroma, often with focal areas of necrosis. All 4 tumors had similar immunoprofiles with positivity for smooth muscle actin, calponin, and smooth muscle myosin heavy chain. Tumors were negative for desmin and caldesmon, markers often seen in SRF-RELA-positive tumors with similar morphology. Follow-up information was available in 3 patients. Two patients had no evidence of disease, 2 and 5 years after surgical resection. One patient, a 35-year-old male patient with a 19 cm deep-seated tumor with brisk mitotic activity (>20 mitoses in 10 HPF), developed lung metastases 7 years after initial diagnosis. In summary, we report a series of 4 cellular myoid tumors with novel SRF-ICA1L gene fusions, characterized by bland spindle cell fascicular growth, expression of specific smooth muscle markers, elevated mitotic activity, marked stromal hyalinization, focal coagulative necrosis, and potential for malignant behavior. Given the morphologic overlap with related cellular myopericytic tumors with SRF-RELA fusions, it is likely that SRF-ICA1L fusions define a similar subset of neoplasms composed of immature smooth muscle cells.
Topics: Adult; Angiomyoma; Autoantigens; Cell Transformation, Neoplastic; Cohort Studies; Female; Gene Fusion; Glomus Tumor; Humans; Male; Middle Aged; Myofibroma; Myopericytoma; Serum Response Factor; Young Adult
PubMed: 31478943
DOI: 10.1097/PAS.0000000000001336 -
Radiology Case Reports Jan 2021Myopericytoma is a rare perivascular tumor commonly arising in the superficial soft tissue and subcutaneous tissue of the distal extremities. We report the first case of...
Myopericytoma is a rare perivascular tumor commonly arising in the superficial soft tissue and subcutaneous tissue of the distal extremities. We report the first case of myopericytoma occurring in the breast, focusing on the imaging and histopathological characteristics of the tumor. From an imaging perspective, myopericytoma presents a well-circumscribed, marked hypervascularity, and intense enhancement after injection of contrast material. Imaging examinations, such as ultrasonography and magnetic resonance imaging, can contribute to the detection of tumor invasion to adjacent structures or distant metastases, and provide evidence for a treatment plan.
PubMed: 33204380
DOI: 10.1016/j.radcr.2020.10.051