Isolated infantile myofibroma of the calvarium: Report of a case with a literature review.

OBJECTIVE: Infantile myofibromatosis is a rare entity of childhood characterized by benign myofibroblastic tumors in the soft tissues, the bones, and occasionally the viscera. Solitary skeletal lesions are relatively uncommon. Calvarial involvement should be distinguished from more aggressive tumors for appropriate treatment. METHODS: We reviewed solitary infantile myofibroma of the calvarium and discussed the relevant computed tomography and magnetic resonance imaging findings along with differential diagnosis. A case study of the frontal bone in a 5-month-old girl was also presented. RESULTS: Fourteen cases were reviewed, including the current case. Of the 13 cases with known sex, eight were male and five female. The mean age was 3.03 with an age range of 0.41-9 years. Nine of the 14 tumors were in the frontal bone. The lesions were intradiploic with tabula interna and/or externa of the calvaria involvement. The mean largest diameter was 22.3 mm. Upon computed tomography, all the lesions were expansile and lytic, and hypoattenuated, isoattenuated or occasionally hyperatenuated. Calcification was not seen. On magnetic resonance imaging, most neoplasms were hypointense on T1-weighted and T2-weighted images. Neoplasms showed hypointense signal on diffusion-weighted imaging and hyperintense on apparent diffusion coefficient, without restricted diffusion in three cases. All lesions were intensely enhanced after gadolinium administration. Treatment was total surgical resection and recurrence was not observed during follow-up. CONCLUSIONS: Infantile myofibromas are rare, typically intradiploic expansile lytic lesions with tabula interna and/or externa involvement. Distinctive imaging features include the presence of hipointense signals on T2-weighted magnetic resonance images without restricted diffusion on diffusion-weighted imaging. A slow-growing, firm, painless, and nontender mass with supportive imaging findings should raise suspicion of the disease.

Intraosseous myofibroma mimicking an odontogenic lesion: case report, literature review, and differential diagnosis.

BACKGROUND: Intraosseous myofibroma of the jaw is a rare neoplasm of mesenchymal origin with limited comprehensive understanding. It typically affects patients in the first two decades of life with a male predilection. CASE PRESENTATION: This study presents a rare case of myofibroma mimicking an odontogenic lesion in a 2-year-old boy. The patient presented with an incidental finding of a painless swelling of the right mandibular ramus of unknown etiology. Imaging analysis revealed a solid, expansile lesion adjacent to the germinal zone of the right mandibular first molar. Histopathologic analysis and immunohistochemistry after incisional biopsy suggested a possible central odontogenic fibroma, and the patient underwent total enucleation, leading to the final diagnosis of intraosseous myofibroma. Follow-up examinations showed no evidence of recurrence. CONCLUSIONS: This report contributes to the understanding of myofibroma in pediatric patients and underscores the critical role of meticulous histopathologic examination for effective surgical planning and optimal patient outcomes.

Solitary intraosseous myofibroma: a rare case diagnosed from mandibular fracture.

Intraosseous myofibroma is a rare tumor of benign nature, slow growth, and low morbidity. The aim of this article is to report a case of pathologic fracture associated with the incidental diagnosis of myofibroma in the mandible of an adolescent. A 15-year-old girl reported that she experienced a physical assault resulting in facial injuries 1 month previously and had since experienced severe pain, malocclusion, and chewing difficulty. The cone beam computed tomographic examination revealed multiple features suggestive of pathologic fracture associated with a hypodense lesion with lobulated limits, as well as expansion and thinning of the cortical bone in the left mandible. The histopathologic diagnosis of the lesion indicated myofibroma. Treatment consisted of enucleation and curettage of the lesion with reduction and internal fixation of the fracture. After 18 months, the osteosynthesis plates and an impacted mandibular third molar were removed. Curettage of the lesion in association with treatment of the mandibular fracture proved to be effective for both bone consolidation and absence of recurrence while restoring mandibular functionality.

Epibulbar solitary myofibroma in an elderly patient: a case report.

A 93-year-old male patient presented with abrupt expansion of an old epibulbar mass at the temporal area of the left eye. He had a medical history of previously treated laryngeal cancer with surgery and radiotherapy. The tumor, despite being firmly attached to the underlying sclera, was excised completely and histopathological examinations revealed a solitary myofibroma. The patient had a 4-month uneventful follow-up with excellent wound healing. Solitary myofibroma may be a differential diagnosis for epibulbar masses in elderly patients.

Pneumonectomy for Pediatric Tumors-a Pediatric Surgical Oncology Research Collaborative Study.

OBJECTIVE: To describe utilization and long-term outcomes of pneumonectomy in children and adolescents with cancer. SUMMARY BACKGROUND DATA: Pneumonectomy in adults is associated with significant morbidity and mortality. Little is known about the indications and outcomes of pneumonectomy for pediatric tumors. METHODS: The Pediatric Surgical Oncology Research Collaborative (PSORC) identified pediatric patients <21 years of age who underwent pneumonectomy from 1990 to 2017 for primary or metastatic tumors at 12 institutions. Clinical information was collected; outcomes included operative complications, long-term function, recurrence, and survival. Univariate log rank, and multivariable Cox analyses determined factors associated with survival. RESULTS: Thirty-eight patients (mean 12 ±â€Š6 yrs) were identified; median (IQR) follow-up was 19 (5-38) months. Twenty-six patients (68%) underwent pneumonectomy for primary tumors and 12 (32%) for metastases. The most frequent histologies were osteosarcoma (n = 6), inflammatory myofibroblastic tumors (IMT; n = 6), and pleuropulmonary blastoma (n = 5). Median postoperative ventilator days were 0 (0-1), intensive care 2 (1-3), and hospital 8 (5-16). Early postoperative complications occurred in 10 patients including 1 death. Of 25 (66%) patients alive at 1 year, 15 reported return to preoperative pulmonary status. All IMT patients survived while all osteosarcoma patients died during follow-up. On multivariable analysis, metastatic indications were associated with nonsurvival (HR = 3.37, P = 0.045). CONCLUSION: This is the largest review of children who underwent pneumonectomy for cancer. There is decreased procedure-related morbidity and mortality than reported for adults. Survival is worse with preoperative metastatic disease, especially osteosarcoma.

An unusual case of a solitary cardiac myofibroma causing severe right ventricular outflow tract obstruction in an infant.

Cardiac tumours are relatively uncommon, particularly in children. Myofibroma is an extremely rare variety of cardiac tumour, which nearly always arises in the context of infantile myofibromatosis. Herein, we present a case of a solitary cardiac myofibroma causing right ventricular outflow tract obstruction in a 2-month-old male infant.

[Adult myofibroma: a clinicopathological analysis of 15 cases].

Objective: To investigate the clinicopathological features and immunophenotype of adult myofibroma with emphasis on its differential diagnosis. Methods: The clinical, pathologic data and immunohistochemical profiles were analyzed in 15 cases of adult myofibroma diagnosed between 2014 and 2020 in Department of Pathology, Fudan University Shanghai Cancer Center. The literature was reviewed. Results: There were seven males and eight females, with age at presentation ranging from 22 to 74 years (mean 54 years; median 57 years). Tumor occurred principally in the extremities (n=9), less frequently involved the head and neck region (n=3) and trunk (n=2); one case was located in the vertebral canal of C6-7. Fourteen cases were solitary; one case was multifocal. Most patients presented with a slowly growing painless subcutaneous nodule, about 1 to 2 cm in size. One patient with multifocal disease and the patient with spinal lesion complained of intermittent pain. The duration of symptoms ranged from 2 months to 10 years. Microscopically, they were well circumscribed. All cases showed biphasic growth pattern, consisting of relatively well-differentiated eosinophilic nodules alternating with dark-staining primitive-appearing areas. The light-staining hypocellular nodules were composed of myofibroblast-like plump spindle cells within an eosinophilic stroma, which assumed pale blue myxochondroid appearance and hyalinization of varying degree. The dark-staining areas were composed of compact short spindled to ovoid cells with hyperchromatic nuclei and low mitotic activity, frequently showing a distinctive hemangiopericytoma-like architecture. By immunohistochemistry, the myoid spindled cells and the primitive cells stained variably for α-SMA, MSA and calponin, but were consistently negative for desmin and ß-catenin. Conclusions: Adult myofibroma tends to occur in the middle to old aged patients with a predilection for the dermis or subcutis of the extremities. It is a benign neoplasm which can be cured by excision in most cases. Familiarity with its distinctive clinicopathological features helps in the distinction from other myofibroblastic neoplasms.

Solitary intraosseous myofibroma of the ilium in an adult patient: A case report and literature review.

BACKGROUND: Myofibroma is a rare benign tumor typically occurring in the skin and subcutaneous tissues of the head and neck in infants and young children. The incidence of solitary intraosseous and adult myofibroma is extremely low. Although there have been a few reported cases of solitary intraosseous myofibroma in adult patients, most of these cases involved the craniofacial bones. METHODS: We present the case of a 64-years-old woman presenting with minimal pain of the right buttock and groin. RESULT: Radiographs and computed tomography showed a huge tumor spreading around the ilium, and non-uniform calcifications were noted inside the tumor. Iliac tumor biopsy was conducted, and intraosseous myofibroma was suspected. We performed surgical resection of the mass, and a final diagnosis of myofibroma with metaplastic bone production was made. CONCLUSION: We have presented an extremely rare case of solitary intraosseous myofibroma in an adult patient.

Gene fusion involving the insulin-like growth factor 1 receptor in an ALK-negative inflammatory myofibroblastic tumour.

AIMS: Inflammatory myofibroblastic tumour (IMT) is a soft tissue tumour primarily affecting children and young adults. Approximately 50% of IMTs have gene fusions involving the receptor tyrosine kinase (RTK)-encoding ALK gene, providing a molecular rationale for treating IMT patients with unresectable tumours with tyrosine kinase inhibitors (TKI). However, a subset of IMT instead displays fusions affecting other RTKencoding genes, so far including NTRK3, PDGFRB and ROS1. Also, IMTs with variant RTK fusions may respond well to TKI treatment, but can be dif?cult to identify as they are negative for ALK staining at immunohistochemistry, the standard method for detection of ALK rearrangements. MATERIALS AND METHODS: We used RNA-sequencing to search for alternate fusion events in an ALK-negative IMT. RESULTS AND CONCLUSIONS: We found a novel fusion gene - FN1-IGF1R. The FN1 gene, encoding ?bronectin, is thought to provide a strong promoter activity for the kinase domain of the RTK insulin-like growth factor 1 receptor, a mechanism similar to previously described RTK fusions in IMT.

Myopericytoma in an unusual location.

Myopericytoma is a soft-tissue tumor of perivascular cells (pericytes). It is slow-growing, usually asymptomatic, and generally benign, although a malignant variant has been described. The etiology is unknown, but it has been associated with local trauma. The most common location is on the distal extremities. Histologically, it is characterized by a well-circumscribed, non-encapsulated proliferation of spindle shaped cells similar to myofibroblasts with oval nuclei and eosinophilic cytoplasm, arranged in perivascular concentric rings. There are few mitoses and no necrosis is reported. The immunohistochemical analysis is positive for smooth muscle actin and negative or weakly positive for desmin. A low Ki-67 proliferation index is typical. Treatment is surgical excision with free margins. Recurrences after adequate excision are uncommon. We describe a 48-year-old woman with a myopericytoma in an unusual location (next to the inner corner of her left eye) who was treated with surgical excision; there has been no recurrence after 5 years of follow up.

[Inflammatory Pulmonary Myofibroblast Tumor Suspected of Pulmonary Metastasis of the Lung Cancer;Report of a Case].

Inflammatory myofibroblastic tumor (IMT) of the lung is a rare disease. The patient underwent the chemoradiotherapy for primary left lung cancer which showed complete responce (CR) by the treatment. A different nodule of 10 mm in diameter was found in the left lung by computed tomography (CT). Positron emission tomography(PET) showed positive detection correspond to the nodule ( SUVmax 4.82). A partial lung resection was carried out, and the tumor was diagnosed as IMT pathologically.

A rare case report for dermatomyofibroma in nasion.

Dermatomyofibroma is a benign and rare proliferation of myofibroblasts and fibroblasts of the skin. Dermatomyofibroma commonly locates at the shoulder and neck of young adults and adolescents. Other frequently affected anatomic sites are upper arms, thigh, chest wall, back, axillary region and abdomen. Herein, we reported a case of dermatomyofibroma occurred in the nasion. The asymptomatic firm nodule and histopathological features were consistent with dermatomyofibroma. Immunohistochemically, the tumor cells expressed vimentin, HHF35 and α-smooth muscle actin (α-SMA). The patient was followed up for 2 years after excision of the tumors and recurrences were not observed.

Myofibroma in Infancy and Childhood.

Myofibromas are rare fibroblastic-myofibroblastic tumors in children. Their biological behavior is unpredictable and spontaneous regressions have been described. This is a retrospective review of clinical characteristics, treatment, and outcome of children diagnosed with myofibroma between 1999 and 2013 at Texas Children's Hospital. The median age at diagnosis of 42 patients was 37 months. Approximately two thirds of the patients were male. The median length of follow-up was 50.5 months (range, 0 to 165 mo). Thirty-eight patients (90%) had solitary lesions; 19 (50%) in the head and neck, 10 (26%) in the limbs, and 9 (24%) in the trunk. Twelve patients underwent a complete surgical resection. Of the 30 patients with positive margins, only 1 had tumor progression. Two patients had multicentric involvement, and 2 patients had generalized disease with visceral involvement. One patient with generalized disease and a progressive maxillary sinus mass was treated with vinblastine and methotrexate chemotherapy followed by complete surgical resection. All patients were alive at last follow-up. Myofibromas of childhood demonstrate clinical variability, and may spontaneously regress. Positive surgical margins do not adversely affect outcome. The rare patient with progressive unresectable disease may benefit from chemotherapy.

Six Cases of Myofibroma--The Adult Counterpart of Infantile Myofibromatosis: Case Report.

The adult counterpart of infantile myofibromatosis is rare and is known as myofibroma. Cases are rare, and previous ones have been reported as isolated case reports; hence, there has been no consensus regarding the clinical presentation, surgical reconstruction, histological features, and recurrence of hand myofibromas. Over a 10-year period, the senior author treated 6 patients. We review our cases as well as 6 previously reported cases. The presentation is usually a single hand mass in a young adult. The tumor may arise from the lower dermis or from deeper fibrous structures of the hand including the palmar fascia. Tumors that arise from the dermis are best treated by skin excision to ensure complete excision. Histologically, the tumor is composed of highly cellular myofibroblast proliferation and is strongly positive to smooth muscle actin immune stain. The recurrence rate after excision is low.

Surgical Management of Myofibroma of the Gengiva.

The purpose of this paper is to report a rare patient of oral myofibroma in a 12-year old patient and to describe its clinical, histopathologic, and immunohistochemical features to establish the correct diagnosis and surgical management.Pathological and immunohistochemical examination is a mandatory method for establishing a definitive diagnosis of this lesion avoiding unnecessary treatment. Surgical excision and careful postoperative observation should be a treatment option.

Prenatal Diagnosis of Infantile Myofibroma with Postnatal Imaging Correlation.

Infantile myofibroma is the most common fibrous tumor in infancy. A majority of these lesions are solitary and occur in the head and neck region. Unless visceral sites are involved, the clinical course is typically benign. However, the difficulty in these cases is the differentiation of a benign myofibroma from a solitary malignant neoplasm and determination of visceral involvement. Prenatal diagnosis of this condition is rarely described in the literature, but it does indeed have a role in perinatal management. Our patient was initially identified by prenatal ultrasound with fetal MRI for further characterization of the mass. We present the case of a prenatally diagnosed solitary infantile myofibroma of the lower extremity and highlight the role of prenatal imaging in the diagnosis and treatment of this condition.

Pure endobronchial inflammatory myofibroblastic tumor in children.

Isolated endobronchial inflammatory myofibroblastic tumor is an unusual diagnosis among endobronchial masses in childhood. The presenting signs and symptoms may mimic asthma. Rigid bronchoscopy is effective for the diagnosis and treatment. Follow-up is mandatory to check for recurrent disease. Here in, the authors report on a 9-year-old girl with endobronchial inflammatory myofibroblastic tumor to emphasize the possibility of endobronchial lesion in children with longstanding obstructive symptoms.

Solitary myofibroma of the mandible in an adult with magnetic resonance imaging and positron emission tomography findings: a case report.

Myofibroma is a benign tumor composed of myoid spindle cells. The prevalence of myofibroma in the oral cavity is very low, with the mandible being the most common site. This report describes an adult case of myofibroma that arose on the mandible and includes magnetic resonance imaging (MRI) and positron emission tomography (PET) findings. On the MRI T1-weighted images, the tumor appeared with signal iso-intensity and was highly and heterogeneously enhanced with contrast material. On the T2-weighted images, it appeared with increased signal intensity. 18 F-fluorodeoxyglucose (FDG)-PET imaging showed abnormal strong accumulation of FDG in the left mandibular region. The tumor was removed by marginal resection of the left mandible under general anesthesia. Histopathological findings revealed that the tumor stroma contained abundant thin-walled vessels. The postoperative course was uneventful, and we found no evidence of recurrence at the postoperative 34-month follow-up.

An inflammatory myofibroblastic tumor exhibiting immunoreactivity to KIT: a case report focusing on a diagnostic pitfall.

Inflammatory myofibroblastic tumors (IMTs) and gastrointestinal stromal tumors (GISTs) are both spindle cell tumors, and occur rarely in the wall of the urinary bladder. In general, immunostaining allows differentiation of IMTs and GISTs. Most IMTs are positive for anaplastic lymphoma kinase (ALK) and negative for KIT, whereas most GISTs are ALK-negative and KIT-positive. Here, we describe a case of a spindle cell tumor in the wall of the urinary bladder. The spindle cells were positive for both ALK and KIT, and it was thus difficult to determine whether the tumor was an IMT or a GIST. We eventually diagnosed an IMT, because ALK gene rearrangement was confirmed by fluorescent in-situ hybridization. Cytoplasmic staining for KIT and the absence of other GIST markers, including DOG1 and platelet-derived growth factor α, indicated that the tumor was not a GIST. Therefore, IMTs should be included in the differential diagnosis of spindle cell tumors, even those that are KIT-positive.