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Abstract Objective: The aim of this study was to report 6 cases of sinonasal/basicranial myxofibrosarcoma and review demographic data, clinical symptoms, diagnostic methods, therapeutic methods, and prognosis on this subject in the literature. Methods: We conducted a retrospective analysis of six patients referred to our hospital during a 10-year period from 2010 to 2020. Results: In this series, there were four males and two females. The tumours originated from the maxillary sinus in all six cases. The symptoms were nonspecific, and all patients underwent endoscopic resection alone or combined with open resection. The postoperative period was uneventful. All patients were diagnosed with myxofibrosarcoma. After a median follow-up period of 36 (6-52) months, the recurrence rate of MFS was 66.7% (4/6), and the mortality rate was 50% (3/6). Conclusion: Sinonasal/basicranial MFS is a rare neoplasm, and the most common primary site is the maxillary sinus. When diagnosing MFS, osteolysis may help rule out benign lesions. So far surgery is the mainstay of treatment for sinonasal/basicranial MFS. Our surgical strategy using endoscopic resection or endoscopic resection combined with open resection to achieve a negative surgical margin and using re-excision operations to treat relapsed cases may be recommended to rhinologists who treat MFS. Prospective randomised trials are needed to study the role of chemotherapy, radiotherapy, and our surgical strategy for sinonasal/basicranial MFS. Diagnosing patients at an earlier stage and better patient compliance with follow-up plans may improve the prognosis of patients.
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Myxofibrosarcoma is an unusual type of malignant fibrous histiocytoma that arises in subcutaneous tissue in the limbs of elderly patients and uncommonly involves the breast.We report a rare case of myxofibrosarcoma that occurred in a male breast. A 57-year-old man was referred to our department for a right breast mass. Physical examination revealed an irregular lump without skin involvement. Ultrasonography and mammography showed an oval and hypoechoic opacity with increased internal vascularity. The patient had a core needle biopsy that showed a sarcoma of the breast. The patient underwent a simple mastectomy. The pathology report confirmed the diagnosis of high-grade myxofibrosarcoma of the breast and the patient had local radiotherapy. After a follow-up of 18 months, the patient has no evidence of local recurrence or distant metastases. Myxofibrosrcoma of the breast is extremely rare but should be diagnosed as soon as possible because of the rapid growth of the tumor and the better prognosis in the early stages.
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Se describe el caso clínico de un paciente de 61 años de edad, con antecedente de buena salud, atendido en el St. Paul's Milennium Medical Hospital de Etiopía por presentar, desde hacía 2 años, una lesión en la encía marginal, con abombamiento de ambas corticales, que se extendía del primer premolar inferior derecho al izquierdo, por lo cual se decidió ingresarlo en el Servicio de Cirugía Maxilofacial para su estudio y tratamiento. Se trataba de un tumor poco frecuente y de gran tamaño, con amplia diseminación ganglionar hacia los niveles IV y V de ambas partes del cuello, de manera que se realizó el procedimiento quirúrgico. La evolución fue favorable en el posoperatorio inmediato y egresó de la institución 7 días después, pero continuó con radioterapia. Y seguimiento por consulta externa durante 24 meses después.
The clinical case of a 61-year-old patient, with a history of good health, treated at St. Paul's Milennium Medical Hospital in Ethiopia for presenting, for 2 years, a lesion in the marginal gum, with bulging of the gums, is described. both cortices, which extended from the lower right to the left first premolar, for which it was decided to admit him to the Maxillofacial Surgery Service for study and treatment. It was a rare and large tumor, with extensive lymph node dissemination to levels IV and V in both parts of the neck, so the surgical procedure was performed. The evolution was favorable in the immediate postoperative period and he was discharged from the institution 7 days later, but continued with radiotherapy. And follow-up by outpatient service for 24 months afterwards.
Subject(s)
Histiocytoma, Malignant Fibrous , MyxosarcomaABSTRACT
Objective To investigate the features of soft tissue myxofibrosarcoma (MFS)on MRI.Methods The MRI data of 15 cases with MFS confirmed by surgery and pathology were analyzed retrospectively.Results Of all the 1 5 cases,7 were located in the thigh,3 were located in the lower leg,2 were located in the forearm,1 was located in the shoulders,1 was located in the back and 1 was located in the buttocks.The average maximum diameter of all lesions was (9.43±3.1)cm.Fifteen cases showed isointensity with slightly hypointensed fiber septa on T1WI.The signal intensity of MFS on T2WI was mixed.Five cases of low-grade MFS with myxoid area showed slightly hyperintensity,with sporadic small nodular and striped fibrous septa which showed hypointensity on T2WI and inhomogeneous enhancement.In 5 cases of high-grade MFS,the solid part and the septa showed hypointensity on T2WI;the myxoid matrix area and the necrotic area showed hyperintensity on T2WI.The myxoid matrix part showed mild inhomogeneous enhancement with peritumoral enhancement of"tail sign".Among the 5 cases,the solid part of 4 cases showed middle to severe annular heterogeneous enhancement,while the solid part of the remaining 1 case showed nodular enhancement.Five cases of intermediate-grade MFS showed slightly hyperintensity or isointensity on T2WI,and nodules and fibrous septa showed hypointensity.The enhanced solid part and myxoid matrix area showed mild to moderate inhomogeneous enhancement,and there were 2 cases with"tail sign".Conclusion MRI features of soft tissue myxofibrosarcoma have some characteristics including isointensity on T1WI,mixed signal intensity on T2WI,heterogeneous enhancement and peritumoral enhancement of"tail sign".
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Myxofibrosarcoma is the most common soft tissue sarcoma that occurs in late adult life, mainly occurring in the lower extremities and trunk. However, head and neck myxofibrosarcoma is extremely rare. The most reliable treatment of adult soft tissue sarcoma is surgical resection with negative margin. A 79-year-old man presented with a left cheek mass first detected six months ago. The pathologic report of the mass showed that it was myxofibrosarcoma and consequently postoperative radiotherapy was done. However, distant and locoregional metastasis occurred postoperatively. We report this case with a brief review of literature.
Subject(s)
Adult , Aged , Humans , Cheek , Head , Histiocytoma, Malignant Fibrous , Lower Extremity , Myxosarcoma , Neck , Neoplasm Metastasis , Radiotherapy , SarcomaABSTRACT
Objective To investigate the MRI characteristics of primary myxofibrosarcoma (MFS) in the extremity soft-tissues.Methods The MRI of 14 cases of MFS confirmed by operation and pathology were analyzed retrospectively.Results In the 14 cases,9 were in the thigh,3 in the lower leg,and 2 in the upper arm.The volumes of the tumors were relatively big and all located in the subcutaneous fat or superficial inter-muscular space.The tumor showed expanding growth and had well-defined boundary.On T1 WI,one case showed isosignal intensity,one showed equal signal intensity mostly,and 12 cases showed patch slightly low signal intensity;On fat suppression T2 WI and STIR,tumors showed high-low mixed signal intensity,which mainly showed high signal intensity;On MRI enhanced study,inhomogeneous enhancement was observed in most of the tumors,and "tail sign" was showed in 11 cases.Conclusion MFS is of characteristics on MRI,the position of tumor is shallow,the tumor is relatively big and has clear boundary.On T1 WI,most tumors show iso-low mixed signal intensity.On fat suppression T2WI and STIR,tumors show high-low mixed signal intensity;On enhanced MRI,inhomogeneous enhancement and "tail sign" are its characteristic signs.
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El mixofibrosarcoma es una neoplasia maligna infrecuente, que puede originarse en los tejidos blandos, se lo ha estadificado como de alto o bajo grado y la localización más frecuente son los miembros inferiores. El reconocimiento clínico del mismo es dificultoso, en primer lugar porque la clínica de otros sarcomas de partes blandas es similar y además semejan lipomas o aún quistes. La extirpación quirúrgica amplia es el tratamiento de elección, por la propensión de este sarcoma a la recidiva local. Los catalogados como de alto grado en un 30 % de los casos, pueden originar metástasis a distancia, especialmente a hueso, pulmón y ganglios linfáticos. La histopatología asegura el diagnóstico en la mayoría de los casos. Comunicamos el caso de un mixofibrosarcoma de bajo grado, en un hombre de 43 años, que a los tres años de control evolutivo, luego de la resección quirúrgica, no presenta recaída local ni distante.
Myxofibrosarcoma is an uncommon soft tissue sarcoma that is grading as low or high malignancy. The principal sites of involvement are the lower limbs. Clinical recognition is difficult since it resembles a cyst, a lipoma, other soft tissue sarcoma or even benign conditions as panniculitis. The wide surgical excision is the main therapeutic approach because local recurrence is frequent. The high grade variant is associated in 30 % of the cases with distant metastasis, especially to the lung, bone and lymph nodes. The histopathological features allow an accurate diagnosis in most cases. A 43 year-old man with a myxofibrosarcoma of the chest wall is reported with a follow-up of two years without recurrence.
ABSTRACT
El mixofibrosarcoma es un subtipo de sarcoma que comúnmente se presenta en las extremidades de personas ancianas. La presentación clínica no es característica y el aspecto histológico es altamente heterogéneo, lo que frecuentemente retrasa el diagnóstico o conduce a uno equivocado. Técnicas de histoquímica e inmunohistoquímica son mandatorias para establecer el diagnóstico de MFS. Presentamos el caso de un hombre de 57 años para ilustrar lo poco sugerente de este diagnóstico dada la presentación clínica. El manejo de este tumor es con cirugía, eventualmente radioterapia y seguimiento estricto.
Myxofibrosarcoma is a subtype of sarcoma commonly found in the extremities of elderly people. The clinical presentation is not characteristic and the histological aspect is highly heterogeneous, which often delays the diagnosis or leads to the wrong one. Histochemistry and immunohistochemistry techniques are required to establish the diagnosis of SFM. We present the case of a 57-year-old man to illustrate the unimpressive nature of this diagnosis given the clinical presentation. The management of this tumor is with surgery, eventually radiotherapy and strict follow-up.
Subject(s)
Humans , Male , Middle Aged , Skin Neoplasms/pathology , Fibrosarcoma/pathology , Myxosarcoma/pathology , Physical Examination , Immunohistochemistry , Upper Extremity , Fibrosarcoma/surgery , Fibrosarcoma/diagnosis , Myxosarcoma/diagnosisABSTRACT
Primary cardiac myxofibrosarcoma is an extremely rare malignant tumor of the heart, which can be difficult to diagnose. We report such a case, which recurred 7 months after the initial heart surgery. A 63-year-old woman with progressive dyspnea was seen by a physician. A transthoracic echocardiography revealed a large mobile mass in the left atrium, causing severe mitral stenosis. The patient was referred to our hospital for an operation. The tumor, which arose from the anterior mitral annulus, was resected, and mitral and tricuspid annuloplasty were performed. The histopathological diagnosis was myxoma. The postoperative course was uneventful. Seven months later, the patient reported suffering from dyspnea and leg edema. The echocardiography showed multiple recurrences of tumor in the left atrium. A second operation was performed to reduce the mass volume as a palliative treatment because some large fragile tumors occupied the left atrium. The patient died 5 months after the second operation due to metastasis. The final diagnosis by histopathology was myxofibrosarcoma.
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Background: De-differentiated liposarcomas (DDLSs) are uncommon tumors with a wide histopathological spectrum. Materials and Methods: Over an 8-year period (2003-2011), 25 DDLSs, after review, were critically analyzed for histopathological features. Results: Twenty-five tumors, in 14 men and 11 women (M: F = 1.2:1), with age range of 22-88 years (mean, 53.9), occurred in retroperitoneum (14) (56%), thigh (3) (12%), paratesticular region (3) (12%), chest wall (2) (8%), leg (1) (4%), shoulder (1) (4%), and groin (1) (4%). Tumor size (21 tumors) varied from 7.5 to 25 cm (mean, 17.5). Histopathologically, DD component was high grade in 19 (76%) and low grade in 6 (24%) tumors. Whereas the most common WD component was adipocytic type; the most common DD component was pleomorphic sarcomatous (13) (52%), followed by myxofibrosarcomatous (MFS)-type (6) (24%). Low-grade DD components included MFS (2), fibrosarcoma (2), myogenic/myofibroblastic type (1), and IMFT-type (1). Three tumors displayed meningothelial-like whorls and metaplastic bone formation. Heterologous elements, noted in 11 (44%) tumors, included bone (8) and rhabdomyoblastic differentiation (2). Two tumors displayed homologous differentiation, reinforced with MDM2 staining. S100-P was diffusely positive in WD components (5/7) and focally in DD components (2/9). All patients were treated with surgery, including 10, who underwent adjuvant radiotherapy. Outcomes (16 patients, 64%), over 1-48 months included 10 patients free of disease, 4 died of disease, and 2 patients alive with disease. Conclusions: This study forms the largest documentation of DDLSs, including its wide histopathological spectrum, from our country. Rare cases overlap with pleomorphic liposarcoma. S100-P and MDM2 are useful in substantiating adipocytic differentiation, especially in selected cases. Analysis of adequate tumor sections is vital for correct identification of a DDLS. Surgical excision with adjuvant RT forms optimal treatment.
Subject(s)
Adult , Aged , Aged, 80 and over , Diagnosis, Differential , Female , Histocytochemistry , Humans , Immunohistochemistry , India , Liposarcoma/diagnosis , Liposarcoma/pathology , Male , Middle Aged , Proto-Oncogene Proteins c-mdm2/analysis , S100 Proteins/analysis , Tertiary Care CentersABSTRACT
Myxofibrosarcoma, also known as a myxoid variant of a malignant fibrous histiocytoma (MFH), is one of the most common sarcomas in the extremities of elderly people. The lesion is characterized by a high frequency of local recurrence but is uncommon in the head and neck regions. Low-grade myxofibrosarcoma, which is commonly misinterpreted as being benign, has a tendency for histological and biological progression in local recurrences, highlighting the importance of an accurate diagnosis and wide surgical excision of the primary lesion. We report a rare case of low-grade myxofibrosarcoma of the mandible located in the left mandibular body and angle area. The tumor was first diagnosed as a myxofibroma and was resected initially. After the final biopsy the patient underwent combined chemo-radiotherapy. The progress of the patent was uneventful until the one year follow up.
Subject(s)
Aged , Humans , Biopsy , Extremities , Fibroma , Follow-Up Studies , Head , Histiocytoma, Malignant Fibrous , Mandible , Neck , Recurrence , SarcomaABSTRACT
Myxofibrosarcoma is one of the most common soft tissue tumors in elderly patients, mostly arising in the extremities, and rarely arising in the chest wall. A 53-year-old women presented with a painful chest wall mass in the manubrium. We excised the mass. The mass was located subdermally, but had infiltrated the underlying muscle layer, and was histologically diagnosed as an intermediate grade myxofibrosarcoma showing myxoid changes and hypercellularity. Here we report a rare case of chest wall myxofibrosarcoma and present a review of the literature.
Subject(s)
Aged , Female , Humans , Middle Aged , Extremities , Manubrium , Muscles , Thoracic Wall , ThoraxABSTRACT
Myxofibrosarcoma is also known as a myxoid variant of malignant fibrous histiocytoma, and this is one of the most common sarcomas in the extremities of elderly people; it is characterized by a high frequency of local recurrence. We herein report on a case of myxofibrosarcoma of the bladder. A 58-year-old man was referred to our hospital because of microscopic hematuria. Computed tomography(CT) and intravenous pyelography(IVP) showed a 12x7cm sized bladder mass in the pelvic cavity. The cystoscopic finding shows a protruding mass at the dome and posterior wall of the bladder. After exploratory laparotomy and tissue frozen biopsy were done, radical cystectomy and uretero-ileo-cutaneous anastomosis were then carried out. The diagnosis of the tumor was confirmed by histopathological examination. The myxofibrosarcoma of the bladder was completely excised, and there was no evidence of recurrence after 10 months of follow-up.