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.

Post-surgical thyroid bed myofibroma simulating a recurrent papillary thyroid carcinoma: A case report and review of the literature.

RATIONALE: Myofibromas are rare benign spindle cell tumors of the soft tissue, bone, or internal organs that occur at any age. Here, we report a post-surgical thyroid bed myofibroma that mimicked a papillary thyroid carcinoma. PATIENT CONCERNS: A 56-year-old male presented with a mass in the thyroid surgical bed, detected 3 years post thyroidectomy following papillary carcinoma. DIAGNOSIS: Thyroid ultrasonography revealed a well-defined, lobulated, hypoechoic, solid nodule, with large rod-like echogenic foci in the thyroid surgical bed. The development of a postoperative suture granuloma was considered. However, ultrasonography performed 12 months later showed a marked increase in the lesion size. Two fine needle aspiration cytology yielded nondiagnostic results. INTERVENTION: Considering the possibility of local tumor recurrence, surgical resection was performed. OUTCOME: The diagnosis of a myofibroma was confirmed, and no additional treatment was administered. LESSONS: It is challenging to differentiate lesions occurring on the thyroid surgical bed after surgery, from recurrent thyroid cancer. A lesion measuring 6 mm, with a degree of punctate echogenicity, suggests tumor recurrence. Moreover, myofibromas are extremely rare. This case highlights that it is advisable to perform a core needle biopsy in cases of nondiagnostic fine needle aspiration results.

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.

Myofibroma mimicking peripheral nerve sheath tumour with ulnar nerve compression symptoms.

We report a case of myofibroma encasing the ulnar nerve on the medial aspect of the left arm with motor and sensory deficit secondary to compression. Initially, the tumour appeared to be a benign peripheral nerve sheath tumour based on preoperative imaging, with clinical examination positive for left hand clawing and a positive Wartenberg's and Froment's sign. However, intraoperative dissection demonstrated that the mass did not originate from the ulnar nerve proper, lowering suspicion for a peripheral nerve sheath tumour. Histopathological analysis showed spindle cell neoplasm, consistent with myofibroma. The patient underwent hand occupational therapy subsequently, with improvement of grip strength from 5 lb to 12 lb by 4 months postoperatively and resolution of clawing of the hand postoperatively. We discuss differentiating features for this rare occurrence of solitary adult myofibroma, where the final diagnosis was only made after formal histopathological analysis.

A rare case of solitary palatal myofibroma in a young pediatric patient.

Myofibroma (MF) is a benign spindle cell neoplasm rarely found in the oral cavity. It is common in males than females (2:1) and mostly seen to develop before 2 years of age with few cases reported in adults. This article reports a rare case of solitary MF of the hard palate in an 8-year-old female child; highlighting the clinical features, histopathology, differential diagnosis while emphasizing the importance of immunohistochemistry in establishing an accurate diagnosis and management of the same. The objective should be to differentiate benign versus malignant spindle cell lesions of smooth muscle, nerve tissue, fibrocytic, and histiocytic origin. Rapid growth of the lesion often raises suspicion of malignancy and may lead to misdiagnosis and inappropriate management.

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.

[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.

Intraosseous solitary myofibroma of the orbit in an adolescent woman.

A 16-year-old woman presented with a painless, progressive, hard swelling in the left inferolateral orbital wall for the past 1 year. There was no diminution of vision or limitation of ocular motility. Imaging revealed an intraosseous, well-defined, expansile, soft tissue lesion in the inferolateral wall of the left orbit. A left anterior orbitotomy with complete surgical excision was performed. Histopathological evaluation of the specimen revealed fascicular pattern of spindle cells with a rich network of slit-like, branching blood vessels. Tumour cells exhibited smooth muscle actin and vimentin positivity but were negative for CD-34 and STAT-6. In absence of any systemic manifestation, a diagnosis of intraosseous solitary orbital myofibroma was made. The case highlights the importance of integrating clinical, radiological and histopathological features in overcoming the diagnostic challenge of differentiating myofibroma from other mesenchymal neoplasms. It also brings forth the importance of complete resection and curettage to prevent recurrence.

Oral myofibroma presenting as an aggressive gingival lesion.

Myofibromas are benign neoplasms of myofibroblastic origin and rarely encountered in the oral cavity. Myofibroma may frequently grow rapidly leading to suspicion of malignancy. This may lead to a tendency for aggressive management. The histopathology of this tumour has similarity with other spindle cell tumours and often requires immunohistochemical staining for diagnosis. Here, we present a case of myofibroma in a 15-year-old female patient who reported with an aggressive gingival swelling and discuss the various histopathological differential diagnosis.

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.

Infantile Myofibroma: Case Report and Review of the Literature.

We report a case of solitary infantile myofibroma (IM) with partially CD34-positive neoplastic cells on the back of a newborn boy. Ultrasonography showed a multilocular mass with a hypoechoic center surrounded by an isoechoic rim. Histopathological analysis revealed that the lesion was composed of small, round cells that were tightly packed and uniform. The cells had oval nuclei and were pale, CD34-positive, and richly cellular. They had interlacing fascicles of spindle cells with features of myofibroblasts with α-smooth muscle actin positivity. We speculate that neoplastic cells in most IMs differentiate towards myofibroblasts. However, in rare cases, their differentiation is more primitive and they express CD34, with or without α-smooth muscle actin expression.

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.

Extended Endonasal Endoscopic Complete Resection of a Solitary Intraorbital Myofibroma: A Case Report and Literature Review.

BACKGROUND: Infantile myofibromatosis is a rare benign disease of mesenchymal origin. It occurs mostly in infants but can occur in children and adults. It presents in 2 forms: solitary and multicentric. The presence of an orbital component, whether as a solitary lesion or as part of the multicentric disease, is even rarer. Surgery is required when these tumors behave aggressively and grow rapidly or when they are large enough to cause compression symptoms. Several surgical approaches have been described to resect such lesions. CASE DESCRIPTION: We present a case of a solitary intraorbital myofibroma extending into the optic canal in a 6-year-old girl that was completely resected via an extended endonasal endoscopic approach. CONCLUSIONS: This case report highlights the advantages of the extended endonasal endoscopic approach in terms of intraoperative and postoperative factors.

Spontaneous Radial Nerve Palsy due to an Unrecognized Myofibroma: A Case Report.

CASE: 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. CONCLUSIONS: 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.

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.

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.