Inflammatory Myofibroblastic Tumor Presenting as Gross Hematuria in a Pediatric Patient With VACTERL Syndrome Following Bladder Augmentation.

Inflammatory myofibroblastic tumors (IMT) are rare and poorly understood inflammatory neoplasms. Most commonly occurring in the liver and gastrointestinal tract, cases of bladder involvement have been rarely reported. Bladder IMT generally presents with gross hematuria and can be differentiated from other bladder tumors by expression of anaplastic lymphoma kinase. We report the occurrence of an Bladder IMT detected following lower urinary tract reconstruction with bladder augmentation.

Acute Complete Oculomotor Nerve Palsy in a Young Male Due to a Skull Base Myofibroma.

ABSTRACT: A 25-year-old male patient visited the ophthalmology clinic because of upper eye lid ptosis in the right eye, binocular double vision, and light sensitivity. He was diagnosed with a complete third nerve palsy caused by a skull base myofibroma, a rare clinical entity that has not been described before in oculomotor nerve palsy.

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.

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.

Intradermal Proliferative Fasciitis Occurring With Chondrodermatitis Nodularis Helicis.

Nodular fasciitis is a benign myofibroblastic tumor. Its uncommon variant, proliferative fasciitis (PF), can present in an even less common intradermal form. We report a case of intradermal PF of the ear in a 45-year-old man who presented with recurrent episodes of pain and swelling of the lesion. Histologic examination showed a dermal, nodular proliferation of ganglion-like basophilic fibroblasts with prominent nuclei and nucleoli, admixed with foamy histiocytes and areas of spindle cells arranged in intersecting fascicles in a fibromyxoid background. Lesional cells stained positive for smooth muscle actin and were negative for AE1/AE3, p63, and Melan-A. CD68 highlighted intervening histiocytes. We postulate that the underlying chondrodermatitis nodularis helicis was a triggering etiology, consistent with the current speculation that intradermal PF results from trauma.

Ruptured Infantile Myofibroma of the Head Presenting in a Neonate: Case Report and Review of the Literature.

BACKGROUND: Infantile myofibroma/myofibromatosis (IM/M) is a myofibroblastic proliferative disorder often seen in infants and children. IM/M can result in congenital tumors of the head and neck and may occasionally present to the neurosurgeon. CASE DESCRIPTION: We report a case of a solitary ruptured myofibroma of the head in a newborn patient. The lesion was initially suggestive of encephalocele. We describe the presentation and management of this patient, including relevant imaging, histopathologic evaluation, and surgical technique. We subsequently review the literature of IM/M of the head and neck, highlighting the 3 forms of the condition, each requiring a distinct management strategy. CONCLUSIONS: Although this tumor rarely presents to the neurosurgeon, it may do so in the process of ruling out other more dangerous conditions. It is therefore important to consider this diagnosis in masses that occur in the head and neck of newborns.

Dysuria and fever in a young woman diagnosed as having inflammatory myofibroblastic tumour of the urinary bladder.

A 38-year-old woman presented with dysuria and fever. Her medical and family histories were unremarkable. CT scan of the abdomen revealed a polypoid mass of 4×2.6×2.2 cm. Her cystoscopy showed a 4×2 cm solid broad-based growth at trigone of the urinary bladder. She underwent transurethral resection of the urinary bladder tumour (TURBT). Histopathology revealed a poorly circumscribed proliferation of spindle cells arranged in a haphazard and fascicular manner along with many traversing blood vessels in a myxoid and hyalinised stroma. Immunohistochemistry was positive for anaplastic lymphoma kinase-1, smooth muscle actin, CD10, cytokeratin and desmin; and negative for CD34 and S-100 protein. Ki-67 proliferative index in the tumour was <1%. The patient was diagnosed as having inflammatory myofibroblastic tumour of the urinary bladder. After TURBT, her fever and urinary symptoms resolved. Her 1-month postoperative period was uneventful. She has been advised regular follow-up.

Transcatheter management of mid-aortic syndrome secondary to myofibroma presenting in infancy with severe left ventricular dysfunction: a case report.

In this study, we summarise a case of a myofibroma causing mid-aortic syndrome due to obstruction of the distal thoracic and abdominal aorta leading to severe left ventricular dysfunction. The patient was managed with percutaneous intervention via balloon dilation and stent placement. On follow-up, the patient has normalisation of ventricular function, is off anti-hypertensives, and is being monitored for re-stenosis.

Laryngeal inflammatory myofibroblastic tumor.

Inflammatory myofibroblastic tumor seldom involves the larynx, as only about 50 to 60 cases have been described in the literature. Even though these tumors are often not aggressive, they have the potential for invasion and local recurrence. We describe the case of a 27-year-old man who was admitted to an emergency department with signs of upper airway obstruction secondary to an obstructive mass. Histology identified the mass as an inflammatory myofibroblastic tumor of the subglottis. The patient underwent an emergency tracheotomy followed by a partial laryngectomy. During 14 months of follow-up, he remained free of active disease.

Myofibroma of the cervical spine presenting as brachialgia.

Myofibromas are rare, benign tumors of myofibroblasts. Their occurrence in adults, involving bone outside of the head and neck, is especially uncommon. The authors report the case of a 34-year-old woman who presented with left-sided brachialgia. Magnetic resonance imaging identified an expansile soft-tissue lesion of the C6-7 facet joint. En bloc resection via a left posterior midline approach was undertaken. Histopathological analysis confirmed the lesion to be a myofibroma. Brachialgia resolved following surgery and there is no evidence of recurrence at 20 months follow-up. Myofibroma is a rare cause of primary soft-tissue tumor of the spine. Surgical excision remains the mainstay of treatment.

Solitary myofibroma of the oropharynx causing airway obstruction in an adult.

Myofibromas are benign neoplasms believed to be the most common fibrous proliferation of childhood. We present an unusual case of a 44-year-old woman who developed acute airway obstruction from a myofibroma in the oropharynx and accordingly required emergent tracheotomy tube placement. Serial laser excisions to adequately remove the entire lesion while maintaining pharyngeal structure and function were performed, and the patient was successfully decannulated. To date she has remained free of signs and symptoms of recurrence. Although rare in adults, solitary myofibromas should be considered in the differential diagnosis of any subcutaneous or submucosal head and neck lesion. Moreover, clinicians treating adult and pediatric patients with known solitary or multicentric forms of myofibroma should be aware of its potential for airway obstruction. Patients found to have a pharyngeal myofibroma should be managed with airway stabilization, surgical excision with preservation of speech and swallow function, and close postoperative monitoring for recurrence.

Mandibular swelling in a 5-year-old child--mandibular myofibroma.

A 5-year-old girl of African descent presented with a history of progressive painless swelling on the right side of the jaw since the past 2-3 months. Orthopantomogram showed a radiolucent lesion near the angle of the mandible. Subsequent CT scan revealed a 2 cm×2 cm radiolucent lesion with intense periosteal reaction surrounding the lesion and destruction of the overlying cortex. Radiological perplexity aroused regarding the possibility of eosinophilic granuloma or some other malignant lesion. Incisional biopsy performed and microscopy showed spindle cell tumor. Immunohistochemistry confirmed it as myofibroma. Myofibroma is a rare benign tumour involving mesenchyme. Involvement of the mandible is rare. Radiological presentation with strong periosteal reaction is a rarity and has rarely been reported in the medical literature. We conclude that intraosseous myofibroma can sometimes have strong periosteal reaction and careful radiological evaluation is a prerequisite for accurate diagnosis and to avoid unnecessary aggressive therapy.

An unusual timing for symptomatic chest pain in an adult chest wall myofibroma: a case report.

INTRODUCTION: Myofibromas are benign mesenchymal neoplasms that can present as solitary and multicentric lesions. They can occur in several locations and can occur at any age from neonates to elderly patients. However, most of the lesions are found in neonates and babies. It rarely occurs in adults. CASE PRESENTATION: A 29-year-old Taiwanese man presented with persistent dull chest pain in his right lateral chest wall for 2 weeks. A chest X-ray showed a faint patchy opacity over the periphery of his right upper lung zone. Computed tomography and magnetic resonance imaging showed a lobulated mass at the intercostal space between his right fifth and sixth ribs with contrast enhancement and bone invasion. Malignancy could not be excluded. A percutaneous needle aspiration biopsy failed due to technique issues, so he underwent a thoracotomy and the tumor was excised with Marlex mesh repairs for the thoracic defect. Pathology confirmed a myofibroma without malignancy. He recovered uneventfully and no local recurrence was detected at the 1-year follow-up examination. CONCLUSIONS: Chest wall myofibroma presenting with chest pain has never been reported in adults. It is a challenge to differentiate myofibroma from malignancy in chest wall preoperatively, such as seen in our patient. To the best of our knowledge, this has not been previously reported in the scientific literature. Although myofibroma rarely occurs in the chest wall and adults, it must be suspected in any chest wall tumor presenting with chest pain.

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 intestinal myofibroma an unusual cause of neonatal intestinal obstruction.

Congenital solitary myofibroma is an exceptional tumor of newborn period and presents as solitary or multiple lesions usually confined to soft tissues. It induces intestinal obstruction or perforation, which most frequently involves the jejunum and ileum. However, jejunoileal atresia is the most frequently encountered cause of small bowel obstruction in the neonatal period. We report a new case of solitary myofibroma located in the wall of the ileum, measuring 2.3 cm2 in size, about 25 cm from the ileocecal junction, in a 17-day-old baby girl who presented with abdominal distention and bilious vomiting. Laparotomy was performed on the patient and the tumor was removed; the patient did well after surgery. Despite all the common causes of intestinal obstruction-intestinal atresia, Hirschsprung disease, anorectal anomaly, malrotation, and meconium passage problem in the neonatal period-myofibroma of the small bowel has to be considered because treatment is fairly easy and prognosis is excellent.

Infantile myofibromas obstructing opposite ends of the gastrointestinal tract.

Myofibromas are benign congenital tumors of soft tissue that can present at birth or during infancy in solitary or multicentric forms. Visceral myofibromas are rarely reported, but are typically symptomatic due to involvement of vital structures. We present two cases of congenital myofibromas, one obstructing the proximal esophagus and the other obstructing the distal rectum. Lessons learned from the treatment of these two patients are shared and the pertinent literature is reviewed.

Upper airway distress due to an oropharyngeal infantile myofibroma.

Infantile myofibroma is an uncommon fibrous tumour of infancy, usually present at birth. It is a rare cause of child airway obstruction, with only one neonatal case reported. We report an eight months age case which was referred to our Unit for increasing respiratory distress. CT scan showed a well defined margins mass measuring 7×5×5cm arising from the left parapharyngeal space and occupying the oropharynx. We performed a complete surgical resection through a transoral approach with no events during the surgery. The patient has been followed-up by the first author for five years with no signs of recurrence or sequelaes.