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Indian Journal of Dermatology 2020
PubMed: 32180615
DOI: 10.4103/ijd.IJD_310_18 -
International Journal of Surgery Case... 2020A pericytic tumor is a group of mesenchymal neoplasm found in superficial tissues and only rarely described in viscera. The family of pericytic tumors includes glomus...
INTRODUCTION
A pericytic tumor is a group of mesenchymal neoplasm found in superficial tissues and only rarely described in viscera. The family of pericytic tumors includes glomus tumors and variants, myopericytoma including myofibroma, and angioleiomyoma etc. The renal pericytic tumor is extremely rare, and only few comprehensive discussions about this entity have been done.
PRESENTATION OF CASE
A 58-year-old man was transferred to our institute with suspicions of renal cell carcinoma. The kidney dynamic computed tomography scan showed a 3 cm sized solid mass in the upper pole of the right kidney. Laparoscopic radical nephrectomy was performed due to the deep-seated mass. Pathological result confirmed that the kidney mass was renal pericytic tumor.
DISCUSSION
Although general biological behavior of published renal pericytic tumors is likely to be benign, the clinicopathologic experiences are very limited. Therefore, we should evaluate the malignant potential of the entity according to the parameters proposed for soft tissue tumors, including tumor location, tumor size, growth pattern, cellularity, cytological atypia, and mitotic figures with atypical forms. The current case shows several worrisome features, including an extremely rare tumor location, partially infiltrative growth, and a mildly increased proliferating index, which resulted in it being classified as an uncertain malignant potential.
CONCLUSION
We described the first case of renal pericytic tumor, addressing uncertain malignant potential, in a Korean male, which would be a distinct mesenchymal neoplasm differentiating from other groups of perivascular tumor families based on histological and immunohistochemical features.
PubMed: 31855704
DOI: 10.1016/j.ijscr.2019.11.009 -
Medicine and Pharmacy Reports Oct 2019Myopericytoma is a benign tumor that shows a pericytic line of differentiation. The lesion is generally located in the dermis and subcutaneous tissue and has a...
Myopericytoma is a benign tumor that shows a pericytic line of differentiation. The lesion is generally located in the dermis and subcutaneous tissue and has a predilection for distal extremities, but may be found in any part of the body. We present a case of 48-year old male who came with painful swelling in right middle finger for the past 2 years. Histopathology showed proliferation of spindle-shaped cells exhibiting a hemangio-pericytomatous pattern with various sized vessels lined by flattened endothelium and surrounded by a concentric arrangement of spindle-shaped cells. Tumor cells showed membranous positivity for smooth muscle actin but negative for desmin and CD34. Hence diagnosis of intravenous myopericytoma was established.
PubMed: 31750447
DOI: 10.15386/mpr-1377 -
Fetal and Pediatric Pathology Apr 2021Myofibromatosis is a distinctive mesenchymal disorder occurring predominantly in childhood, which on microscopy shows peripheral light areas of spindle cells and central...
INTRODUCTION
Myofibromatosis is a distinctive mesenchymal disorder occurring predominantly in childhood, which on microscopy shows peripheral light areas of spindle cells and central cellular areas of primitive oval to spindle cells arranged around hemagiopercytomatous vessels. PDFGRB mutations in the familial and multifocal sporadic forms and fusions in the cellular variants have been identified. The index case is being presented to discuss the clinico-pathological features, differential diagnosis, and management of the lesion.
CASE PRESENTATION
An 11-year-old male presented with an infraorbital mass of 3 months duration. The mass was excised and microscopy revealed the morphological features of myofibroma with tram-track SMA immunopositivity. Nodular fasciitis and fibromatosis were the differentials considered.
CONCLUSION
The gene fusion may represent a subset that in the future may be used to differentiate these myofibromas/myopericytomas from the fusion myopericytomas, and may be used to perhaps separate out familial myofibromas from other myofibromas.
Topics: Child; Diagnosis, Differential; Humans; Male; Mutation; Myofibroma; Myofibromatosis; Receptor, Platelet-Derived Growth Factor beta
PubMed: 31738635
DOI: 10.1080/15513815.2019.1686785 -
Cutis Sep 2019
Topics: Adult; Histiocytoma, Benign Fibrous; Histiocytosis, Non-Langerhans-Cell; Humans; Leg; Male; Myopericytoma; Nevus, Epithelioid and Spindle Cell; Skin Neoplasms; Solitary Fibrous Tumors
PubMed: 31675399
DOI: No ID Found -
Actas Dermo-sifiliograficas May 2020
Topics: Diagnostic Tests, Routine; Humans; Myopericytoma
PubMed: 31627853
DOI: 10.1016/j.ad.2018.09.015 -
The American Journal of Surgical... Dec 2019The entity "pericytoma with t(7;12)" was described as a rare, distinct perivascular myoid neoplasm provisionally classified within the family of myopericytic tumors that...
The entity "pericytoma with t(7;12)" was described as a rare, distinct perivascular myoid neoplasm provisionally classified within the family of myopericytic tumors that demonstrates t(7;12)(p22;q13) translocation with resultant ACTB-GLI1 fusion and biologically was felt to behave in an indolent fashion. However, a recent study showed that tumors with this and similar translocations may have variable morphology and immunohistochemical phenotype with inconsistent myopericytic characteristics and a propensity for metastasis, raising questions regarding the most appropriate classification of these neoplasms. Herein, we report 3 additional patients with tumors harboring t(7;12) and ACTB-GLI1 fusion. The tumors arose in adults and involved the proximal tibia and adjacent soft tissues, scapula and adjacent soft tissues, and ovary. All tumors were composed of round-to-ovoid cells with a richly vascularized stroma with many small, delicate, branching blood vessels, where the neoplastic cells were frequently arranged in a perivascular distribution. Both tumors involving bone showed histologic features of malignancy. By immunohistochemistry, all tested tumors were at least focally positive for smooth muscle actin (3/3) and CD99 (patchy) (2/2), with variable staining for muscle-specific actin (2/3), S100 protein (1/3), epithelial membrane antigen (2/3), and pan-keratin (1/3); all were negative for desmin and WT1 (0/3). The 2 patients with bone tumors developed metastases (27 and 84 mo after diagnosis). Whether these tumors are best classified as malignant myopericytoma variants or an emerging translocation-associated sarcoma of uncertain differentiation remains to be fully clarified; however, our study further documents the potential for these tumors to behave in an aggressive fashion, sometimes over a prolonged clinical course.
Topics: Actins; Adult; Biomarkers, Tumor; Bone Neoplasms; Cell Differentiation; Chromosomes, Human, Pair 12; Chromosomes, Human, Pair 7; Female; Gene Fusion; Genetic Predisposition to Disease; Humans; Male; Middle Aged; Myopericytoma; Ovarian Neoplasms; Phenotype; Sarcoma; Translocation, Genetic; Treatment Outcome; Zinc Finger Protein GLI1
PubMed: 31567194
DOI: 10.1097/PAS.0000000000001360 -
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 -
Virchows Archiv : An International... Dec 2019Myopericytomas (MPC) are rare mesenchymal tumors, originating from the perivascular myoid cells. They predominantly occur in the skin and superficial soft tissues of the...
Myopericytomas (MPC) are rare mesenchymal tumors, originating from the perivascular myoid cells. They predominantly occur in the skin and superficial soft tissues of the extremities, while visceral involvement is rare. Histological features and clinical course are usually benign. To the best of our knowledge, MPC is still an uncharacterized tumor entity of the female internal genital tract. We describe three MPC cases involving the female internal genital tract: (1) a uterine wall MPC arising in a 49-year-old woman with progressive pelvic/abdominal pain; (2) a cervix MPC of a 49-year-old woman who presented with metrorrhagia, and (3) a MPC presenting as a simple ovarian cyst in a 26-year-old woman with pain located in the left iliac fossa. All patients were surgically treated, and recurrence occurred in two cases. The histological and immunohistochemical findings, supporting the diagnosis of MPC, are presented; in particular, one case showed characteristics pointing towards an uncertain biological behavior/low-grade malignancy. A literature search was conducted to identify previous reports of gynecological MPC and for possible alternative diagnoses. Leiomyoma, epithelioid leiomyoma, angioleiomyoma, perivascular epithelioid cell tumor, solitary fibrous tumor, and low-grade endometrial stromal sarcoma should be considered in the differential diagnosis. Awareness of possible occurrence of this rare neoplasm in the female genital tract is important to reach a correct diagnosis in the spectrum of mesenchymal tumors. Considering the risk of recurrence, we recommend careful evaluation of surgical margins and complete surgical removal whenever possible.
Topics: Adult; Biomarkers, Tumor; Diagnosis, Differential; Female; Humans; Leiomyoma; Middle Aged; Myopericytoma; Neoplasm Recurrence, Local; Perivascular Epithelioid Cell Neoplasms; Soft Tissue Neoplasms; Solitary Fibrous Tumors
PubMed: 31410559
DOI: 10.1007/s00428-019-02645-2 -
Oral Surgery, Oral Medicine, Oral... Oct 2019The aim of this study was to analyze myopericytoma in the oral and maxillofacial region in terms of clinical appearance, diagnosis, treatment, and outcomes.
OBJECTIVE
The aim of this study was to analyze myopericytoma in the oral and maxillofacial region in terms of clinical appearance, diagnosis, treatment, and outcomes.
STUDY DESIGN
Data on 5 new patients with myopericytoma in the oral and maxillofacial region treated at our department were collected and analyzed.
RESULTS
There were 2 males and 3 females (age range 10-62 years; mean age 43.8 years). All of the 5 patients presented with masses showing benign biologic behavior. Imaging examinations with use of computed tomography or magnetic resonance imaging showed heterogeneous regions with internal contrast-enhancement or cystic change in 3 cases. All of the patients underwent surgery. Histologic examination showed a broad morphologic spectrum characterized by concentric and perivascular growth of ovoid, plump spindled, and/or round myoid tumor cells. Immunohistochemical examination showed positive staining for vimentin and smooth muscle actin, and negative for CD34 and desmin. During the follow-up period (8-56 months), there was no tumor recurrence.
CONCLUSIONS
Myopericytoma in the oral and maxillofacial region always exhibits benign biologic behavior and a heterogeneous region with internal contrast-enhancement or cystic change on imaging examinations. Surgery is the first choice of treatment and results in good clinical outcomes.
Topics: Adolescent; Adult; Child; Desmin; Female; Humans; Immunohistochemistry; Male; Middle Aged; Mouth Neoplasms; Myopericytoma; Neoplasm Recurrence, Local; Young Adult
PubMed: 31350225
DOI: 10.1016/j.oooo.2019.06.004