Myopericytoma is a benign tumor that typically occurs within subcutaneous tissue and most often involves the distal extremities, followed by the proximal extremities, neck, thoracic vertebrae and oral cavity. Complete resection is often curative. Malignant myopericytoma is extremely rare and has a poor prognosis. Here, we report for the first time a case of malignant myopericytoma originating from the colon.

A 69-year-old male was admitted to our hospital with right upper quadrant pain for five days. Imaging suggested a liver mass with hemorrhage. A malignant hepatic tumor was the initial diagnosis. Surgical resection was performed after a complete preoperative work up. Initial postoperative pathology suggested that the mass was a malignant myoblastoma unrelated to the liver. Four months after the first surgery, an enhanced computed tomography (CT) scan revealed a recurrence of the tumor. The diagnosis of malignant myopericytoma derived from the colon was confirmed on histopathological examination of the specimen from the second surgery. The patient did not return to the hospital regularly for surveillance. The first postoperative abdominal CT examination six months after the second surgery demonstrated multiple liver metastases. Survival time between the diagnosis of the tumor to death was approximately one year.

Malignant myopericytoma is a rare cancer. Preoperative diagnosis may be difficult. Due to a lack of treatment options, prognosis is poor.

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.

Myopericytomas are benign soft tissue tumors which are rarely found as intracranial masses.

A review of SCOPUS and PubMed databases for case reports and case series was done for patients with intracranial myopericytomas. Data on demographics, clinical features, imaging, surgical management employed, and outcomes were collected.

We found a total of 9 cases in the literature and we describe an additional case from our own experience. The mean age at presentation was 50.1 years (32-64 years), with a female predominance. Most tumors were in the posterior fossa and were multifocal in AIDS patients. The most common clinical manifestations were visual disturbances, headaches, and vomiting. An association with EBV was reported in two cases and was also found in our own case. Gross total excision and even subtotal excision were definitive therapies with no recurrences reported with a mean follow-up of 22.7 months. Our case is the first EBV-associated intracranial myopericytoma in a child.

Intracranial myopericytomas are rare; some have an association with EBV and immunodeficient states. Surgery is the only necessary treatment and outcomes are generally favorable for these benign neoplasms.

A 71-year-old male with a 4-month history of bulging, tearing, and redness in the right eye presented with vision loss, proptosis, conjunctival hyperemia, and chemosis. Magnetic resonance imaging showed a right intraconal solid mass with extraconal extension, hyper-intense in T2 sequences with heterogeneous contrast enhancement. Complete excision of the mass was performed through a lateral orbitotomy. Histological analysis revealed a neoplasm with high vessel density, solid growth of oval cells, a concentric proliferation of the wall of small vessels, and a weak and patchy positivity for smooth muscle actin. These findings were consistent with the diagnosis of myopericytoma. After surgery, visual acuity improved in the affected eye and after 18 months of follow-up there have been no signs of recurrence.

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.

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.

A 33-year-old woman presented with a six-month history of spontaneous radial nerve palsy and no identified lesion on imaging. She underwent operative exploration where an hourglass deformity was seen and resected. Pathology returned as a rare tumor, a myofibroma. The patient regained full radial nerve function.

A trial of observation is often indicated in the cases of isolated nerve palsy where anatomic lesions have been eliminated. This case highlights that imaging studies can miss a tumor involving nerve and that painless, spontaneous nerve palsy may be a time where early surgical intervention offers a better chance of recovery.

A 49-year-old female with history of headache, nausea and vomiting with some weeks of evolution, without neurological symptoms. Radiology revealed an expansive lesion near the inferior vermix and cerebellar tonsils, with heterogeneous gadolinium uptake and mass effect on the fourth ventricle, representing a probable extraventricular origin for the lesion. Pathological examination showed a proliferation of oval/spindle cell proliferation with eosinophil cytoplasm and small and monotonous nuclei, without mitoses. The cells had a concentric growth, surrounding thin-walled blood vessels with foci of stromal myxoid degeneration and whorled pattern. The vessels had a haemangiopericytoma pattern and were lined by non-atypical endothelial cells. The tumorous cells expressed vimentin, alpha-smooth actin and heavy-chain caldesmon and were negative for epithelial membrane antigen, protein S100, HMB45, CD34, calponin and desmin, thus providing the final diagnosis of intracranial myopericytoma.