Low-Grade Myofibroblastic Sarcoma in the Mandibular Canal: A Case Report.

Low-grade myofibroblastic sarcoma (LGMS) represents an atypical myofibroblastic tumor characterized by a diffusely infiltrating pattern of spindle-shaped tumor cells. It was classified as a distinct soft tissue tumor by the World Health Organization in 2002. LGMS occurs mostly in adult patients and has a predilection for the head and neck region. So far, only a few cases of LGMS located in the mandible have been reported. Aggressive surgical resection with clear margins is the primary treatment for LGMS. Because of its rarity, reports of radiation therapy are limited, and the therapeutic effect is still controversial. We present the case of an 8-year-old girl with LGMS of the mandibular canal to highlight the clinical features and rarity and to improve the understanding of the therapeutic effect of radiotherapy on LGMS.

Long-term follow-up and pregnancy after complete sacrectomy with lumbopelvic reconstruction: case report and literature review.

BACKGROUND: Sacrectomy remains a technically complex procedure for resection of malignant pelvic neoplasia. Commonly, postoperative complications include permanent neurological deficits. Only a few studies have reported the long-term functional outcomes of patients who had undergone sacrectomy. CASE PRESENTATION: We previously reported on the utilization of complete sacrectomy and lumbopelvic reconstruction for the management of primary myofibroblastic sarcoma of the sacrum and ilium in a 15-year-old female patient. In this report, we update her postoperative course with an additional 5 years of follow-up and Health-Related Quality of Life (HRQoL) outcomes. During this time period, she gave birth to two healthy full-term babies. CONCLUSION: To the best of our knowledge, this is the first report of pregnancy after total sacrectomy and lumbopelvic reconstruction. We outline some of the challenges in the obstetrical management of this patient.

[Clinical analysis of low grade myofibroblastic sarcoma in head and neck].

OBJECTIVE: To explore the clinical characteristic, the CT, MRI features, diagnosis and treatment of low grade myofibroblastic sarcoma in head and neck. METHOD: Six cases of low grade myofibroblastic sarcoma in head and neck were diagnosis and reviewed retrospectively. Routine preoperative CT and MRI examination were performed. RESULT: All cases were operated one case with radiotherapy before operation was given with a total dose of 60 Gy. The patients were follow-up for 6 months to 5 year and no recurrence was found. No complications occurred in 6 cases. CONCLUSION: It has been confirmed in this research that LGMS is a low-grade malignangt tumor. It was difficult to make early diagnosis through frozen section. The final diagnosis depend on paraffin section and immunohistochemisty. Extended local excision with tumor-free margin is the treatment of choice.

Ependymosarcoma with eosinophilic granular cells.

Ependymosarcoma is a new entity of malignant gliomas composed of ependymal and sarcomatous components. Were port a rare case of ependymosarcoma with eosinophlic cells which occurred to the right trigon of the lateral ventricle.A 62-year-old man complained of headaches over a 2-month period. A hard, gray mass was found in the right trigon of the lateral ventricle during the operation.Although he received radiation and chemotherapy, the patient died due to tumor disseminating through the whole brain within 7 months after the operation. The histological examination revealed that the anaplastic glial components intermingled with the sarcomatous components. Immunohistochemically, sarcomatous cells were positive for α smooth muscle actin and desmin. However, anaplasticglial cells were not positive for these markers. In addition, Masson trichrome stain showed a plethora of collagen fibers between sarcomatous cells, but no collagen fibers were produced by the glial tumor cells. Solid focal papillary lesions of the glial tumor showed dot-like epithelial membrane antigen and diffuse cytoplasmic D2-40 immunoreactivity. Based on the above findings, these anaplastic glial tumor cells should show focal ependymal differentiation, and sarcomatous cells show myofibroblastic differentiation. In addition, almost 10%of the tumor cells in the neoplasm showed bright eosinophilic granules in the cytoplasm. These cytoplasmic eosinophilic granules and bundles were negative on PAS staining. Intracytoplasmic eosinophilic granules of tumor cells were strongly positive for αB-crystallin, HSP 27 and GFAP, respectively. These findings suggest that the clinicopathological characteristics of the present case should be consistent with the criterion of ependymosarcoma by Rodriguez et al.

[Resection and reconstruction in cases of musculosceletal soft tissue sarcomas]. / Sebészi radikalitás és rekonstrukciós lehetõségek végtagi lágyrészsarcomáknál.

Soft tissue sarcomas are rare, reaching some 1.5% of all malignant tumors. While formerly the surgical management of sarcomas almost exclusively consisted of amputation, in the recent years limb saving surgery has become the first choice of therapy. Negative factors affecting the survival rate are: histologically high-grade tumor, size and localization of the tumor, vascular invasion, extensive tumor necrosis, certain subgroups, local recurrence and oncologically positive surgical margin at the resection. Many modern reconstruction possibilities are essential for the safe limb saving surgery with wide surgical margins, such as bone allograft implantation, tumor endoprostheses reconstruction, vascular grafting and plastic surgery. There should always be an attempt to perform limb saving surgery, however life quality, life expectancy and survival are more important considerations influencing essentially the surgical method of choice. In our follow-up study no significant difference in recurrence rate was found between the group of patients with sarcomas requiring a complex reconstruction procedure and the group of those treated by only resection methods (32% versus 47%).

LOW-GRADE MYOFIBROBLASTIC SARCOMA OF THE LARYNX: CASE REPORT AND REVIEW OF LITERATURE.

Low-grade myofibroblastic sarcoma (LGMS) is a very rare, atypical myofibroblastic tumor with fibromatosis-like features with predilection mostly in head and neck region. LGMS occurs primarily in adult patients with a slight male predominance. Only few cases of LGMS affecting the larynx have been reported in literature to this date. We describe a case of low-grade myofibroblastic sarcoma of the larynx in a 40-year-old male patient. The clinicopathological characteristics, immunohistochemical findings and treatment are discussed.

Radomiosarcoma paratesticular: a propósito de un caso / Paratesticular phabdomyosarcoma: a case report

OBJETIVO: Presentar un caso de radomiosarcoma paratesticular y revisión de la literatura. MÉTODOS: Describimos el caso de un varón de 16 años remitido a nuestro servicio por masa paratesticular izquierda de crecimiento progresivo, con imagen ecográfica de tumoración paratesticular heterogénea con flujo Doppler en su interior. RESULTADOS: Se realizó orquiectomía izquierda, con diagnóstico de rabdomiosarcoma. El paciente rechazó el tratamiento quimioterápico adyuvante. Tras 13 meses libre de enfermedad, reingresó por dolor cólico izquierdo detectándose en ecografía y TC una masa retroperitoneal paraaórtica izquierda que condicionaba uréterohidronefrosis grado III, y metástasis pulmonares. A pesar de instaurarse quimioterapia de rescate, no respondió presentando rápida progresión de la masa abdominal con importante deterioro general que no permitió el abordaje quirúrgico, siendo éxitus a las pocas semanas. CONCLUSIÓN: Los sarcomas paratesticulares son tumores infrecuentes y de mal pronóstico(AU)

OBJECTIVE: To report a case of paratesticular rhadomyosarcoma and to perform a bibliographic review. METHODS: We report the case of a 16-year-old male referred to our Department because of a left paratesticular hard tumor with progressive growth. Ultrasound examination showed a paratesticular heterogeneous mass with Internal flow on Doppler. RESULTS: The patient underwent left inguinal orchiectomy, with pathological diagnosis of rhabdomyosarcoma. He refused adjuvant chemotherapy. After being disease-free for 13 months, he presented with left colic pain. Ultrasound and CT examinations showed a left paraaortic retroperitoneal mass causing grade III ureterohydronephrosis, and lung metastases. Despite rescue chemotherapy treatment, there was no response and the abdominal mass progressed. A surgical approach was not possible since patient showed a rapid clinical worsening leading to his death a few weeks later. CONCLUSIONS: Paratesticular sarcomas are very uncommon tumors with poor prognosis(AU)

Differential diagnosis of inflammatory myofibroblastic tumour and low-grade myofibroblastic sarcoma: two case reports with a literature review.

Inflammatory myofibroblastic tumour (IMT) and low-grade myofibroblastic sarcoma (LGMS) have similar morpho logical and immunophenotypic features, but LGMS is more malignant than IMT and the treatment requires a wider surgical margin plus post-operative chemotherapy or radiotherapy. To date, only 28 cases of IMT and two cases of LGMS have been reported in the laryngopharynx. Recent studies have suggested that anaplastic lymphoma kinase (ALK) and cytokeratin are important markers for differentiating between the two tumours. Here, two cases involving different myofibroblastic tumours of the larynx are reported. Based on the histological and immunohistochemical results, case 1 was diagnosed as IMT involving the right arytenoepiglottic fold, while case 2 was diagnosed as LGMS involving the epiglottic-glossal surface. There was no recurrence or metastasis in either case after post-operative follow-up (12 and 14 months, respectively). It is difficult to distinguish IMT from LGMS; both morphological and immunohistological analyses are required.

Low-grade myofibroblastic sarcoma of the sacrum.

Myofibroblastic tumors are soft-tissue neoplasms arising from myofibroblasts, ubiquitous cells sharing ultrastructural features of muscular and fibroblastic cells. Vasudev and Harris described a malignant counterpart of these benign tumors in 1978. Most reported cases of myofibroblastic sarcoma have arisen in the head and neck region and the soft tissues of the extremities. To the best of the authors' knowledge, there have been only 8 previous reports on primary myofibroblastic sarcoma of the bone. The authors report a new case of this rare tumor affecting the sacrum and ilium of a 15-year-old girl and discuss the role of total sacrectomy and lumbopelvic reconstruction for treatment of this disease.

[Myofibroblastic sarcoma of the larynx : a case report and review]. / Myofibroblastische Sarkome des Larynx : Fallbericht und Ubersicht.

BACKGROUND: Myofibroblastic sarcomas or myofibrosarcoma, are extremely rare malignant neoplasms of myofibroblasts. They are characterized by the pattern of cells and special immunohistochemical markers such as vimentin, desmin and alpha-smooth-muscle actin. PATIENT AND METHOD: The case of a patient with a history of frequently relapsing papillomas of the larynx is reported. Chronic laryngitis with focal low-grade dysplasia of the squamous epithelium was diagnosed approximately 1 year after the first treatment of the papillomas. After approximately 2 years the pathologist diagnosed the rare myofibroblastic sarcoma of the larynx. The patient underwent laryngectomy due to the spread of the tumor with a bilateral selective neck dissection. The patient is at present still free of recurrence and metastases. RESULTS AND CONCLUSIONS: There is a great danger of misjudging a myofibroblastic sarcoma as an inflammatory myofibroblastic tumor and consequently to delay the urgently needed treatment. Therefore, an overview of the present state of knowledge about diagnosis and treatment of myofibroblastic sarcomas will be given based on this case report.

Low-grade myofibroblastic sarcoma of the palm.

We report here a case of an unusual spindle cell tumour of the palm with myofibroblastic differentiation, which was surgically excised. Histologically and immunohistochemically, it was a low-grade myofibroblastic sarcoma. After 25 months follow-up the patient is well and free of recurrence.

[Non-epithelial tumors of the large intestine]. / Nienablonkowe nowotwory jelita grubego.

UNLABELLED: Colorectal cancer may arise from any kind of tissue constituting normal wall of the large intestine, however, in majority of cases, it is of epithelial origin. Endocrine, mesenchymal neoplasms and non-granulomatic lymphomas belong to rarely occurring cancers of the large intestine. Mesenchymal neoplasms may derive from muscles, nervous system, fibrous connective tissue, fat tissue and from blood and lymphatic vessels. The paper presents 3 cases of non-epithelial neoplasms of the large intestine (myosarcoma and 2 myomas). All cancers concerned women older than 50 years (mean age 64.6 +/- 11.4 years) and were localized in the right hemicolon. In all cases episodes of hemorrhage from the lower part of the digestive tract triggered the diagnostic procedures. Surgical treatment varied from hemicolectomy with lymphadenectomy to endoscopic excision of a small lipoma. Histopathological verification and immunohistochemical staining were obtained after their removal. Clinical course and intraoperative situation were decisive as to type of surgery. Although mesenchymal tumors are very rare among large intestine neoplasms, one should consider their occurrence while carrying preoperative diagnosis. CONCLUSION: Occurrence of other tumors than neoplasms should be taken into consideration while carrying preoperative diagnosis.

Myofibroblastic sarcoma in meningioma: a new variant of "metaplastic" meningioma.

The authors describe a mixed malignant dural tumor composed of meningioma and myofibroblastic sarcoma (MFS). The meningioma component displayed epithelial membrane immunoreactivity and interdigitating cellular processes with desmosomal junctions on electron microscopy. MFS cells were immunoreactive for smooth muscle actin and vimentin, and focally for factor XIIIa, CD31, CD34, and Ulex europeus lectin receptors. Electron microscopy showed collections of intermediate filaments, stress fibers, subsarcolemmal densities of microfilaments, occasional fibronexus fibrils, few pinocytic vesicles, and discontinuous external lamina. After gross total removal, the tumor recurred 1 year later as aggressive MFS only. Development of MFS in continuity with meningioma suggests induction of MFS by meningioma or a divergent differentiation of precursor of the neoplastic arachnoid cell.

Stent fabrication for graft immobilization following wide surgical excision of myofibroblastic sarcoma of the buccal mucosa: a clinical report.

Sarcomas of the head and neck are rare tumors derived from cells of mesenchymal origin. This article briefly discusses the epidemiology, etiology, and classification of head and neck sarcomas. Emphasis is placed on myofibrosarcoma, a malignant tumor of the myofibroblasts. Histologic criteria, prognostic factors, and the multidisciplinary management of these tumors are reviewed. A situation of a surgically excised myofibrosarcoma of the buccal mucosa is reported. In this patient, a stent was fabricated to stabilize a split-thickness skin graft used for the buccal mucosa reconstruction.

Myofibrosarcoma of the nasal bone.

Myofibrosarcoma is a recently recognized rare tumor that mainly occurs in adults. These tumors are composed of a collagenous stroma and pleomorphic stellate to spindle-shaped cells that resemble smooth muscle cells with eosinophilic cytoplasm and tapered nuclei. We present a case of myofibrosarcoma of the nasal bones in a 4-year-old girl who showed rapid enlargement of a painless glabellar swelling. Computed tomography and magnetic resonance imaging revealed an expanding solid mass with erosion of the surrounding nasal bones. After excision and histopathological examination, this tumor was identified as a myofibrosarcoma. This is the first report of such a tumor localized to the glabellar region. This case report contributes to better awareness of an extremely rare type of glabellar lesion in children.

Myofibrosarcoma of the adrenal gland.

Adrenal masses have varying presentations. Most commonly, adrenal masses are discovered incidentally on CT or MRI during an evaluation for an unrelated complaint. Although the majority of these are nonfunctional cortical adenomas, hormonally active tumors and adrenocortical carcinoma must also be considered in the differential diagnosis. Rarely, retroperitoneal tumors may mimic an adrenal mass. We report a case of a 49-year-old man with anemia and weight loss who was found to have a large retroperitoneal mass arising from the adrenal gland. Surgical treatment involved en bloc resection of the right kidney, adrenal gland, segments 7 and 8 of the liver, and a portion of the right hemidiaphragm. Final pathology revealed a low-grade myofibrosarcoma. We believe that this is the first case report of a myofibrosarcoma of the adrenal gland. Myofibrosarcomas are rare malignant tumors composed of myofibroblasts that arise from the deep soft tissues. These tumors have a predilection for the head and neck, trunk, or extremities. Myofibrosarcomas can be differentiated from other sarcomas by immunohistochemical staining and pathologic features. We will briefly discuss the workup of an adrenal mass and focus on the diagnosis of myofibrosarcoma.