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Experimental and Therapeutic Medicine Aug 2024Mixed epithelial and stromal tumors (MESTs) of the kidney are rare renal neoplasms, primarily affecting middle-aged women. These tumors are characterized by a mix of...
Mixed epithelial and stromal tumors (MESTs) of the kidney are rare renal neoplasms, primarily affecting middle-aged women. These tumors are characterized by a mix of epithelial and stromal components. While generally benign, MESTs require accurate diagnosis and appropriate management due to the potential for malignant transformation. The present study reports the case of a 75-year-old male patient who underwent a partial nephrectomy following the incidental discovery of a kidney tumor. Histopathological examination revealed a partially cystic tumor with solid areas, measuring 26 mm in diameter. The tumor had cysts lined with cuboidal cells and an ovarian-like stroma. The solid component consisted of elongated cells with eosinophilic cytoplasm and oval nuclei, showing angiocentric growth around small blood vessels without nuclear atypia or mitoses. Since the morphology of the solid component could not reveal the differentiation of those cells, immunohistochemical staining was performed and a myopericytoma/myofibroma component was established, mostly based on the positivity of smooth muscle actin, muscle-specific actin, h-caldesmon, estrogen receptor, progesterone receptor, solute carrier family 2 facilitated glucose transporter member 1 and collagen IV, along with a lack of staining for desmin, CD34, CD31 and CD99. Thus, to the best of our knowledge, for the first time in the literature, MEST with myopericytoma/myofibroma stromal component in a male patient was reported.
PubMed: 38939172
DOI: 10.3892/etm.2024.12610 -
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 -
The American Journal of Case Reports May 2024BACKGROUND Soft tissue tumors have various subtypes, among which sarcomas exhibit high malignant potential and poor prognosis. Malignant epithelioid tumor with GLI1... (Review)
Review
BACKGROUND Soft tissue tumors have various subtypes, among which sarcomas exhibit high malignant potential and poor prognosis. Malignant epithelioid tumor with GLI1 alterations was originally found in myopericytoma with t(7;12) translocation. However, recent studies indicated that it is a distinct tumor type characterized by multiple nodular distributions of oval or round epithelioid cells with a rich capillary network and a lack of specific immunophenotype. There are only a few cases reported worldwide and the optimal treatment is still being explored. CASE REPORT We report the case of a 31-year-old patient who presented with severe anemia and a large soft tissue mass in the duodenum. The patient underwent surgical resection with a negative margin, and none of the 15 lymph nodes tested positive for the tumor. Postoperative pathology and FISH testing further confirmed the presence of GLI1 disruption and S-100 and SMA negativity. Genetic testing revealed the ACTB-GLI1 fusion. No specific medication was offered after the surgery. No tumor recurrence was found during the 23-month follow-up period. The patient's quality of life is currently satisfactory. CONCLUSIONS Soft tissue sarcomas characterized by GLI1 gene rearrangement have a relatively less aggressive and metastatic nature, with the solid mass spreading minimally even as it grows. Patients can benefit from surgical resection, resulting in a relatively long period of tumor-free survival.
Topics: Humans; Adult; Zinc Finger Protein GLI1; Sarcoma; Duodenal Neoplasms; Gene Rearrangement; Male
PubMed: 38778503
DOI: 10.12659/AJCR.943271 -
Journal of Cutaneous Pathology May 2024A neurofibroma with focal glomus-like body differentiation is an unusual phenomenon recently encountered in an excision specimen from the right lateral distal forearm of...
A neurofibroma with focal glomus-like body differentiation is an unusual phenomenon recently encountered in an excision specimen from the right lateral distal forearm of a 26-year-old man. Glomus cells are modified smooth muscle cells normally present in glomus-like bodies but can also be found in glomus tumors (GT) or lesions considered in the spectrum of GT, including myopericytoma, myofibroma, and angiolipoma. Neurofibromas are peripheral nerve sheath tumors derived from the neural crest cells. While both GT and its variants and neurofibroma are thought to be derived from different cell types, there is growing evidence that glomus cells have a neural crest origin. This is based on multiple theories, with some overlapping pathways, including neural crest cell differentiation, Schwann cell reprogramming, VEGF expression, and NF1 gene biallelic inactivation. This report adds to the growing evidence of possible neural crest origin for glomus cells and would help explain finding glomus-like bodies scattered through a neurofibroma.
PubMed: 38698729
DOI: 10.1111/cup.14637 -
International Journal of Surgical... Apr 2024Myopericytoma, a perivascular myoid neoplasm, is commonly found in the dermis or subcutaneous tissues; however, its occurrence in visceral organs is unusual. Here, we...
Myopericytoma, a perivascular myoid neoplasm, is commonly found in the dermis or subcutaneous tissues; however, its occurrence in visceral organs is unusual. Here, we present an extremely rare tumor of intravascular myopericytoma of the right renal vein. A 44-year-old man was incidentally diagnosed with a mass in the right kidney during a routine checkup. A nephrectomy was performed because the urologist suspected renal cancer. A gross examination of the resected specimen revealed a well-circumscribed brown mass in the renal hilum. Histologically, the tumor showed a concentric multilayered proliferation of spindle cells surrounding blood vessels. Immunohistochemical staining showed that the spindle-shaped tumor cells were negative for desmin but positive for α-smooth muscle actin and h-caldesmon, indicating their myoid nature. We confirmed that the tumor was located in the right renal vein because it was encased within a thick wall that was desmin-positive and contained elastic fibers, as shown by Elastica van Gieson staining. The patient was diagnosed with an intravascular myopericytoma of the right renal vein. There are several differential diagnoses for renal mesenchymal tumors, including angiomyolipoma. This emphasizes the importance of considering these uncommon tumors when examining nephrectomy specimens.
PubMed: 38689469
DOI: 10.1177/10668969241246490 -
BMC Oral Health Apr 2024Myopericytoma is a rare spindle cell tumor of mesenchymal origin, typically benign, characterized by concentric proliferation of tumor cells around blood vessels within... (Review)
Review
BACKGROUND
Myopericytoma is a rare spindle cell tumor of mesenchymal origin, typically benign, characterized by concentric proliferation of tumor cells around blood vessels within subcutaneous tissue. It primarily occurs in middle-aged adults and is often located in distal extremities, although cases have been reported in proximal extremities and head-neck regions. However, occurrences within the oral cavity are exceedingly rare. To date, literature reviews have identified only two cases in children under 10 years old and reported only five cases of myopericytoma occurring in the lip region. We provide a comprehensive review and analysis of all documented cases to better understand this condition.
CASE PRESENTATION
A 7-year-old girl presented to oral and maxillofacial surgery with the discovery of a painless mass on the inner aspect of the upper lip. The diagnosis of myopericytoma was confirmed by histological examination (HE staining), alcian blue staining, and immunohistochemistry.
CONCLUSIONS
Following surgical excision, there were no signs of recurrence at a 3-month follow-up. The pathological diagnosis of myopericytoma is quite challenging, and immunohistochemical testing is necessary.
Topics: Adult; Middle Aged; Female; Humans; Child; Myopericytoma; Hemangiopericytoma; Lip; Immunohistochemistry
PubMed: 38643070
DOI: 10.1186/s12903-024-04106-y -
Journal of Ultrasound Apr 2024
PubMed: 38637472
DOI: 10.1007/s40477-024-00884-x -
Indian Journal of Pathology &... Nov 2023Angioleiomyoma is a benign neoplasm that arises from vascular smooth muscle cells. Angioleiomyoma of the endometrium is very uncommon. The differential diagnoses of this...
Angioleiomyoma is a benign neoplasm that arises from vascular smooth muscle cells. Angioleiomyoma of the endometrium is very uncommon. The differential diagnoses of this entity are myopericytoma, angiomyofibroblastoma, endometrial stromal tumor, and perivascular epithelioid cell tumor. 31-year-old and 45-year-old patients presented with heavy menstrual bleeding, lower abdomen pain, and dysmenorrhea. Perspeculum and radiological investigations showed an endometrial polyp. They underwent diagnostic hysteroscopy, polypectomy, and endometrial biopsy. Polypectomy specimens of both cases revealed polypoidal lesions lined by the endometrium. The core of the polyp was arranged in long intersecting bundles of spindle cells and interconnecting anastomotic patterns with many intervening thick-walled blood vessels. These spindle cells have oval and cigar-shaped nuclei, fine chromatin, and a moderate amount of eosinophilic cytoplasm, resembling smooth muscle cells. These smooth muscle cells of the vessel wall were merging with the adjacent walls of the blood vessel. There was no nuclear atypia or necrosis. The mitotic rate was 0-1/10 HPF. Focal areas of hyalinization and adipocytic components were noted in one case. The endometrial glands did not show intraepithelial or invasive neoplasia. On immunohistochemistry (IHC), these spindle cells were diffuse and strongly immunopositive for SMA and Desmin. CD34 highlighted the endothelial lining of the prominent thick-walled blood vessels. By correlating with histomorphology and IHC positivity, a diagnosis of angioleiomyomatous polyp of endometrium was rendered. We report two uncommon cases of angioleiomyoma of the endometrium and discuss the differential diagnosis and literature review.
PubMed: 38394403
DOI: 10.4103/ijpm.ijpm_108_23 -
Journal of the Belgian Society of... 2024: Myopericytoma is a rare soft tissue tumor but should be considered in the differential diagnosis of infants with a fast-growing perivascular tumor.
: Myopericytoma is a rare soft tissue tumor but should be considered in the differential diagnosis of infants with a fast-growing perivascular tumor.
PubMed: 38312148
DOI: 10.5334/jbsr.3441