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[Acquired benign myofibroma of the skin (adult myofibroma)].

Adult myofibroma is an acquired skin tumour that is histologically identical to infantile myofibroma. Histologically, four tumour variants can be distinguished: leiomyoma type, cellular spindle cell type, haemangiopericytoma or glomus type, and biphasic type. Practical help in diagnosis is supplied by the hamartomatous composition of the leiomyoma-type, hyalinized spindle cell whorls, endothelium-lined haemangiopericytoma-like vascular channels, and desmin-negative, muscle-actin-positive myofibroblasts, which may show spindle cell or undifferentiated mesenchymal cell differentiation. The tumours clinically present as firm, occasionally bluish cutaneous or subcutaneous nodules. Adult myofibroma should be recognized as a distinct benign neoplasm, most probably of vascular origin. Clinicians and dermatopathologists must be aware that lesions identical to those of infantile myofibroma can occur in adults.

Adolescent↗

[A case of giant skull myofibroma occupying left anterior cranial fossa].

We report a rare case of giant skull myofibroma occupying left anterior cranial fossa. A 53-year-old woman presented with left exophthalmos for 2 years. Neurological examination showed left exophthalmos, disturbance of bilateral visual acuity, and bitemporal hemianopsia. A CT scan revealed an ossifing mass at left anterior cranial fossa. On magnetic resonance images, the tumor showed iso-intensity on T 1-weighted image, heterogeneous high intensity on T 2-weighted image, and was heterogeneously well-enhanced after administration of Gd-DTPA. The tumor was fed mainly by middle meningeal artery. The patient underwent surgery and the tumor was removed totally. Histological diagnosis of the tumor was myofibroma. The patient has been followed every other month by MRI without any adjuvant therapy. There has been no tumor recurrence for 19 months. There is no other myofibroma in her body, therefore the patient was diagnosed as solitary myofibroma of the skull. Our case is the first report of solitary myofibroma of the skull because we could not find any reports on solitary myofibroma of the skull in the past literature.

Female↗

[Facial myofibroma of the newborn simulating sarcoma].

INTRODUCTION: Myofibromatosis is a rare but probably under estimated tumor. OBSERVATION: We report a case of a newborn presenting with a facial tumor suggestive of sarcoma. Discrepancy between clinical features and histologic pattern of angioma suggested the diagnosis of myofibroma. The diagnostic of myofibromatosis was made later by specific labelling (actin-smooth muscle) and was confirmed by major spontaneous involution. DISCUSSION: The histology of myofibroma associates fibrous and angiomatous patterns that may be confusing when examining a small biopsy. Due to confusing semiology and histology, subcutaneous myofibromas are certainly misdiagnosed and specific actin labelling (actin, HHF 35) may be of interest. Myofibromas are the most frequent tumor among fibromatoses. The diagnosis should be suspected on the stony consistence of the tumor uncompatible with angiomatous histology. Contrary to multicentric myofibromas of which the prognosis depends on visceral involvement and compressions (72 p. 100 of fatal outcome), solitary subcutaneous myofibroma has a very good prognosis with spontaneous involution.

Diagnosis, Differential↗

Cutaneous adult myofibroma: a vascular neoplasm.

Infantile myofibromatosis is a distinctive type of fibromatosis that usually develops during the immediate perinatal period. There are variants with solitary and multiple tumors. Lesions confined to the skin, soft tissue, and bone carry a good prognosis, showing spontaneous regression. The prognosis, however, is much less favorable when visceral lesions are present and the outcome may be fatal. Only recently it became obvious that there is an adult counterpart of infantile myofibromatosis, characterized by solitary lesions that have a predilection for involve the dermis and show no tendency to regression, although they have an entirely benign biological behavior. These lesions have been named cutaneous myofibroma or solitary myofibroma of adults. We have studied the clinical, histopathological and immunohistochemical characteristics of 53 examples of cutaneous adult myofibroma. In addition, 2 cases were examined ultrastructurally. The patients were mostly adults with ages ranging from 6-83 years. The lesions presented as solitary, usually painless nodules of variable duration on the skin, usually located on the extremities. Histopathologically, four patterns were identified: nodular or cellular type, multinodular or biphasic type, leiomyoma-like or fascicular type, and vascular type. A correlation between the histopathologic pattern and the lesional age was observed: vascular type of cutaneous adult myofibroma in early lesions, nodular and multinodular lesions in fully developed lesions, and leiomyoma-like or fascicular type in late lesions. Immunohistochemically, the spindle cells were desmin negative, but expressed immunoreactivity for vimentin, pan-smooth muscle actin, and alpha-smooth muscle actin. Ultrastructurally, neoplastic cells showed characteristics of undifferentiated mesenchymal cells with features of fibroblasts, myofibroblasts and pericytes. Primitive vascular formations were seen in the form of irregular clefts between adjoining cells. We conclude that cutaneous adult myofibroma is a little-known benign vascular neoplasm probably derived from myopericytes.

Adolescent↗

Solitary central myofibroma presenting in the gnathic region.

Solitary myofibroma of adults is an uncommon neoplasm that typically arises in soft tissue and subcutaneous sites in the head and neck, but rarely within bone. When encountered in the jaws, the lesions exhibit clinical and radiographic features suggestive of an odontogenic tumor or cyst as well as several other neoplastic conditions. Tooth mobility, displacement of teeth, and dramatic jaw expansion may be observed. Analogous to other sites of involvement, gnathic myofibromas are biologically indolent and show little or no recurrence following excision. In rare instances, however, the ability to obtain adequate surgical margins by conservative measures may be limited; thus, issues of local control may supercede the importance of biologic potential. We present the radiologic and histopathologic findings in a case of central myofibroma presenting as a large lytic lesion of the mandible. Myofibroma involving the jaw bones represents a unique diagnostic and therapeutic challenge, and accurate diagnosis and management is predicated on careful correlation of radiographic and pathologic findings.

Adult↗

Central (intraosseous) myofibroma of the mandible: clinical, radiologic, and histopathologic features of a rare lesion.

OBJECTIVE: Myofibroma frequently manifests as a solitary soft tissue tumor and less as simultaneous multiple tumors in both soft tissues and bones. The present study evaluated clinical, radiological, and histopathologic features of myofibroma of the jaws. STUDY DESIGN: Nineteen cases from the literature and 4 new cases were analyzed. RESULTS: At initial diagnosis, age ranged from birth to 34 years (mean 7.2 years, median 6 years). Male/female ratio was 2.3:1. Myofibromas were radiolucent solitary lesions located solely in the mandible: 70% unilocular, 30% multilocular; 67% with well-defined borders. Conservative treatment was performed on 75% of the patients; 25% underwent partial jaw resection. Where information was provided, no lesion recurred during 6-month to 17-year follow-up. CONCLUSION: Although rare, myofibroma of the mandible should be considered in the differential diagnosis of radiolucent lesions (particularly unilocular), especially in children. Treatment of choice is conservative surgery to minimize potential functional and/or esthetic damage.

Age Distribution↗

Solitary infantile myofibroma of the orbital bone.

Infantile myofibroma of the orbit is an extremely rare condition. Only a few instances of this condition have ever been reported. A 3-year-old boy visited our clinic with lateral lower eyelid swelling and a palpable mass in the left eye, which had apparently persisted for 2 months. A computed tomography scan revealed a well-circumscribed mass in the inferolateral orbital portion of the zygomatic bone, coupled with erosion of bone and orbital extension with reactive hyperostosis. Immunohistochemical stains proved positive for smooth muscle actin, supporting the diagnosis of solitary infantile myofibroma of the orbital bone (zygoma). Although rarely found in the orbit, solitary infantile myofibroma can display more aggressive or malignant neoplasm. Immunohistochemistry is integral to the differential diagnosis and a systemic evaluation for multicentric myofibroma is essential.

Child, Preschool↗

Adult acral cutaneous myofibromas in a patient with generalized morphea.

Myofibroma is the term for a group of solitary or generalized soft tissue tumors that may be located in the skin or within skeletal muscle, bone, and viscera. These tumors occur most commonly in children. However, examples of myofibroma have been reported in adults, in whom they usually present as solitary lesions of the head and neck, trunk, and extremities. "Cutaneous nodules" have been reported in both localized and systemic scleroderma, but to our knowledge, there are no specific reports of myofibromas developing in this patient population. We report a case of acral myofibromas in a patient with generalized cutaneous morphea. This occurrence is of interest in view of the possible role of myofibroblasts in the pathogenesis of scleroderma.

Aged↗

Solitary myofibroma of the sclera.

PURPOSE: To report a novel case of solitary myofibroma involving the sclera. METHODS: Case report and review of the literature. RESULTS: A 19-year-old woman had an expanding and painful epibulbar mass clinically diagnosed as nodular scleritis that did not respond to anti-inflammatory therapy. A biopsy showed a proliferation of spindle-shaped cells that reacted strongly positively with immunoperoxidase stains for vimentin and smooth-muscle actin. The diagnosis of solitary myofibroma was made. Six months postoperatively, the patient was well, and the lesion had not recurred. CONCLUSIONS: Myofibroma should be considered in the differential diagnosis of an epibulbar mass, especially in the setting that would mimic nodular scleritis.

Adult↗

Solitary intraosseous infantile myofibroma of the orbital roof.

BACKGROUND: There are several rare tumors that can cause proptosis in an infant, including infantile myofibroma. METHODS: A 3-month-old infant developed a painless, bone-destructive superomedial orbital mass, raising concern for orbital malignant neoplasms. Computed tomography disclosed a bone-destructive mass of the sphenoid wing. On magnetic resonance imaging, the intraosseous mass was well-circumscribed, surrounded by cortical bone, and showed prominent enhancement. RESULTS: Superomedial orbitotomy and biopsy revealed a lesion composed of spindled to stellate cells, without mitotic activity, set in a fibromyxoid stroma. Immunohistochemical stains were positive for vimentin and actin. Ultrastructurally, there were actinlike thin filaments, mitochondria, and rough endoplasmic reticulum confirming a myofibroblastic proliferation and supporting the diagnosis of congenital infantile myofibroma. CONCLUSION: Infantile myofibroma is a benign tumor that occurs rarely in the ocular region but can cause prominent bone destruction, misleading the clinician to suspect a malignant neoplasm.

Female↗

Myofibroma of the upper arm in a 52-year-old woman.

Myofibromas are mesenchymal tumors that most often present in infancy. There are sporadic cases in adults throughout the literature. Myofibroma will demonstrate a variable appearance on CT and ultrasound, but tends to have a fairly consistent appearance on MR imaging. This report describes the MR and sonographic findings as well as the pathologic correlation of a myofibroma in the arm of a middle-aged woman. The patient was well, without evidence of recurrence 6 months following resection.

Arm↗

Myofibromas presenting in the oral cavity: a series of 9 cases.

OBJECTIVES: Solitary myofibromas are well described in the head and neck, but oral examples are less well known, and jaw lesions are rare. We studied the clinicopathologic features of a series of such lesions. DESIGN: Nine cases of oral myofibromas were retrieved from archives and studied. RESULTS: Two cases involved the mandible (intraosseous), 3 involved the gingiva, 2 involved the tongue, and 2, the hard palate. There were 4 men and 5 women, aged 9 months to 50 years (mean, 24 years; median, 27 years). Deep lesions showed typical histology, with paucicellular lobules and intervening hemangiopericytoma-like zones. In ulcerated submucosal lesions, these features blended superficially with cellular fascicles. The tumors expressed smooth muscle actin but lacked desmin and S100 protein. None of the tumors recurred or metastasized. CONCLUSION: Myofibromas appear in osseous, intramuscular, and submucosal aspects of the oral cavity. Ulceration imparts a fascicular appearance that makes superficial biopsy specimens difficult to interpret.

Actins↗

[Cutaneous myofibroma--late manifestations].

Infantile myofibromatosis is a rare, but nevertheless well-known, entity that was first described in 1954 in Stout's classic article on juvenile fibromatosis. Usually the tumour mass is noted at birth or during the first weeks of life. Solitary lesions are at least twice as common as multiple lesions. A case of a 17-year-old woman with a tumour macroscopically resembling a basal cell epithelioma is presented. This tumour did not develop until the patient was 17 years old and cannot be distinguished histologically from an infantile myofibroma. Because of the very late appearance of this tumour the term "cutaneous myofibroma" is proposed for it. In young patients with a basal cell epithelioma-like lesion the diagnosis of cutaneous myofibroma has to be considered.

Adolescent↗

Solitary infantile myofibroma compromising the airway.

Infantile myofibromatosis is an uncommon and benign condition presenting in the neonatal period. It is self-limiting disease that may present as a localised or generalised process. Various examples of this entity have been reported in the literature. This report describes a neonate with a rapidly growing oropharyngeal lesion obstructing the airway that had the typical histological features of an infantile myofibroma. This case report highlights that a solitary myofibroma may be incredibly extensive making complete excision impossible and can be particularly challenging to manage in terms of airway stabilisation.

Airway Obstruction↗

Solitary calvarial myofibroma presenting in adolescence.

Myofibroma is a benign fibrous tumour that may be found in the soft tissues, skeleton, and internal organs. It is a rare lesion but is the commonest fibrous tumour in infancy. Involvement of the skull has been reported in some 21 cases in the English-language literature. None of these, however, has arisen outside infancy. Here, we report a myofibroma arising from the temporal bone in a 17-year-old girl.

Adolescent↗

Solitary intraosseous orbital myofibroma in four cases.

PURPOSE: To report four pediatric cases of a bone-destructive orbital tumor that proved to be a benign solitary intraosseous myofibroma. METHODS: Four children, aged 1, 7, 11, and 72 months, developed eyelid fullness or slight proptosis. A solitary intraosseous mass was detected in the inferior (n = 2), superotemporal (n = 1), and supranasal (n = 1) orbital walls. On CT, the mass showed bone destruction. On MRI, the mass appeared circumscribed within a shell of normal surrounding bone and with features of isointensity on T1-weighted images, intense homogeneous gadolinium enhancement, and midrange signal on T2-weighted images. Complete surgical excision of the mass in each case was performed. RESULTS: On histopathologic examination, the tumors were characterized by whorls and bands of plump myoid cells with eosinophilic cytoplasm consistent with myofibroblasts, sometimes associated with a hemangiopericytoma-like vascular pattern. The diagnosis was also supported by immunohistochemical studies showing positive immunoreactivity for vimentin and smooth muscle actin. None of the patients have shown recurrence in follow-up ranging from 6 months to 3 years. CONCLUSIONS: Myofibroma is a benign tumor that can occur in the orbital bony wall of children. It can manifest as a bone destructive mass and can simulate a malignant process.

Child↗

Solitary testicular myofibroma: a case report and review of the literature.

Myofibromas are benign mesenchymal neoplasms of myofibroblastic origin. Most present as solitary lesions at any age, but the presentation of multiple lesions in newborns and infants is known as infantile myofibromatosis. Multicentric lesions commonly involve soft tissues and bone and may involve internal organs, where they are associated with an unfavorable prognosis. Solitary lesions involving the viscera are rare. We report a case of a 3-month-old male infant with a left testicular mass detected during an evaluation for suspected torsion. The patient underwent orchiectomy, revealing a nodular mass with grossly evident foci of necrosis. Histologically, the lesion exhibited small fascicles of plump eosinophilic, smooth muscle actin-positive spindle cells, alternating with larger areas of primitive cells with vesicular nuclei and scant cytoplasm arranged around a hemangiopericytoma-like vasculature. To our knowledge, this is the first report of a myofibroma localized within the testis.

Actins↗

Solitary myofibroma of the lumbar vertebra: adult case.

We present the first known adult case of solitary myofibroma of bone, which affected a lumbar vertebra in a 33-year-old male. Radiography identified a purely lytic lesion with a sclerotic rim in the right pedicle of L1. CT showed an expansile lytic lesion with a sclerotic rim. MRI of the lesion revealed an isointense signal on T1-weighted images, an inhomogeneously hyperintense signal on T2-weighted images, and marked enhancement with gadolinium. Pathological study showed a mixed picture of nodular proliferation of spindle-shaped myoid cells and hemangiopericytomatous proliferation of short spindle/small round cells. The tumor cells were immunoreactive for smooth muscle actin and immunonegative for desmin. This case of solitary myofibroma of bone is exceptionally rare because of its occurrence in an adult older than 20 years of age and its location at an extra-craniofacial site.

Adult↗