1/12. Urachal inflammatory myofibroblastic tumor with ALK gene rearrangement: a study of urachal remnants. OBJECTIVES: Abnormalities of the urachus are rare among children and include a patent tract and cyst formation. These structures can also be affected by infection and abscess development. They are usually diagnosed during infancy and treated by surgical resection. Involvement of this remnant by either benign or malignant tumors is very infrequent. A few cases of mesenchymal tumors, such as desmoid tumor and leiomyoma, involving the urachus have been described in published reports. methods: We studied an inflammatory myofibroblastic tumor arising from the urachus in a 10-year-old boy. In addition, we reviewed 101 cases of urachal remnants retrieved from the surgical pathology and autopsy files in the Department of pathology at the Children's Hospital boston diagnosed in the past 82 years. RESULTS: The urachal inflammatory myofibroblastic tumor showed anaplastic lymphoma kinase (ALK) rearrangement by immunohistochemistry and fluorescence in situ hybridization techniques. No other neoplasms were diagnosed in the analyzed population. CONCLUSIONS: We describe an example of inflammatory myofibroblastic tumor involving the urachus. Involvement of the urachus by tumors is rare, but these should be considered in the differential diagnosis of urachal lesions. ( info) |
2/12. Clinico-pathological conference 2002. INTRODUCTION: Six cases are reported, each presented at the 11th Biennial Congress of the International association of Oral Pathologists as an instructive case for differential diagnosis on the basis of clinical, imaging or histological features. CLINICAL PICTURE: Case diagnoses included a large, possibly intraosseous, myofibroma presenting with an oral mass; Langerhans cell histiocytosis with facial skin lesions; an intraosseous vascular hamartoma of the maxilla with worrying radiological features; an unusual mixed radiolucency of the jaw caused by cemento-ossifying fibroma; an osteosarcoma of the posterior mandible causing a well-defined radiolucency and an intraoral squamous cell carcinoma in a child. ( info) |
3/12. 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. ( info) |
4/12. 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. ( info) |
5/12. Solitary infantile gastrointestinal myofibroma: case report. We report a case of a 7-year-old child who required emergency surgery for acute abdomen and suspected acute appendicitis. During surgery a tumor located in the small bowel that caused intestinal occlusion was found. Histopathologic analysis showed a solitary gastrointestinal myofibroma. This is a very rare tumor, especially as a single lesion, because in world literature, there are less than 10 cases reported. ( info) |
6/12. 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. ( info) |
7/12. Intramandibular inflammatory myofibroblastic tumor--a true neoplasm or reactive lesion? OBJECTIVE: Inflammatory myofibroblastic tumor (IMT) is an uncommon lesion reported to arise in various organs and by convention believed to be a nonneoplastic reactive inflammatory condition. Because of its radiographic and histologic similarities to malignancy, IMT can pose a considerable diagnostic dilemma. The purpose of this study is to report the first intramandibular IMT and to discuss the possible nature of this lesion. STUDY DESIGN: Detailed documentation of clinical, radiographic, and histologic features; literature review. RESULTS: This lesion showed distinct radiographic, histologic, and immunohistochemical features. The lesion recurred 14 months after initial removal with almost identical histology. CONCLUSION: This is the first documented case of an intramandibular IMT. Due to the rarity of these lesions, their clinical sequelae and prognosis are undetermined. As a result of specific immunohistochemical phenotyping and clinical behavior of this lesion, we favored a diagnosis of inflammatory myofibroblastic tumor. Wide excision with a long-term clinical follow-up is required. ( info) |
8/12. 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. ( info) |
9/12. Inflammatory myofibroblastic tumor associated with renal cell carcinoma. A 46-year-old man was referred for evaluation of a 13-cm renal mass. Right radical nephrectomy was performed. Pathologic evaluation revealed inflammatory myofibroblastic tumor (IMT) and renal cell carcinoma. To our knowledge, only 28 cases of IMT associated with the kidney have been reported in published studies. This is the first reported case of IMT associated with malignancy in the urogenital tract. Some investigators have suggested that biopsy-proven IMT can be managed conservatively, but the findings of this report have demonstrated the need for radical nephrectomy in this patient population to rule out associated malignancy. ( info) |
10/12. 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. ( info) |