Myxoid Malignant Fibrous Histiocytoma Research Articles

A huge left buttock mass in an old female

IntroductionMyxofibrosarcoma also known as myxoid malignant fibrous histiocytoma is a rare soft tissue sarcoma that has a predilection for the elderly (beyond the 6th decade) Clinical observationWe report a case of a 64 years old patient that presented gradual appearance of huge tumor on her left buttock. Radiologic examination (MRI) concluded to MFS. There was no evidence of systemic involvement. The patient was directed in an oncology center, where the diagnostis of MFS was confirmed through surgical biopsy. DiscussionMFS is one of the most frequent sarcoma of soft tissue that occurs in elderly patients. This is an infiltrant tumor that is extending through muscular fascia. MRI is the gold standard in characterisation; it makes the positive diagnosis of MFS thanks to the myxoid signal, triple signal and the tail sign that is corresponding to the extension of the tumor into the muscular fascia and peri nervous spaces. ConclusionCross-sectionnal imaging in particular MRI plays a key role in the positive diagnosis of soft tissue tumors. It makes possible to approach with great precision the exact nature of the tumor thanks to specific radiological semiology without however claiming to replace histology.

FDG PET/CT in Dendritic Fibromyxolipoma of the Thigh.

Dendritic fibromyxolipoma is a rare recently described benign soft tissue tumor, which may be misinterpreted histologically as myxoid liposarcoma or myxoid malignant fibrous histiocytoma. We describe FDG PET/CT and MRI findings of a dendritic fibromyxolipoma incidentally detected in a case with rectal adenocarcinoma. The dendritic fibromyxolipoma showed inhomogeneous FDG uptake with SUVmax of 4.0 mimicking malignancy.

Positive outcome in a high-grade myxofibrosarcoma: a case report

Abstract Myxofibrosarcoma or myxoid malignant fibrous histiocytoma is one of the most common sarcomas of the limb. It is usually treated multimodally. Most frequent sites of metastasis are the bone, lung and lymph nodes. The present paper is a case report of a 65-year-old male with myxofibrosarcoma of the fibularis longus muscle, for which he first underwent surgery - tumor resection with appropriate margins. The tumor was staged pT2b cN0 cM0. Postoperative PET-CT revealed metabolically inactive pulmonary nodules. Two months after surgery, he underwent adjuvant radiotherapy, a total dose of 60 Gy and 6 courses of chemotherapy (doxorubicin and ifosfamide). Pulmonary nodules have been stationary on all subsequent imagistic studies. He is free of recurrence on long-term follow-up.

Pulmonary Myxoma: A Rare Benign Tumor of the Lung

SESSION TITLE: Student/Resident Case Report Poster - Lung Cancer II SESSION TYPE: Student/Resident Case Report Poster PRESENTED ON: Tuesday, October 25, 2016 at 01:30 PM - 02:30 PM INTRODUCTION: Pulmonary myxoma is a rare benign tumor of the lung. Identification of this entity is important due to unknown incidence of the lesion and to avoid unnecessary invasive procedures. Pulmonary myxomas have been reported as sporadic case reports and can been seen arising in the lungs, pleura and trachea. As the use of computed tomography (CT) scan is increasing we are likely to see more of these benign lesions. CASE PRESENTATION: Patient is a 62 year-old active smoker who was recently diagnosed with cholangiocarcinoma. She has a past medical history of adenomatous goiter, hypertension and asthma. During the work up she was found to have a 1 cm nodule in the right upper lobe. Positron emission tomography scan showed minimal uptake in the lesion which was then biopsied and showed lobular myxo-mucinous blobs and benign slightly inflamed bronchio-alveolar tissue, negative for malignancy. She underwent cholecystectomy and right liver lobectomy. Following this she was referred to pulmonary clinic for follow up of the pulmonary nodule and had a repeat CT scan done which showed increased volume of the mass and hence it was re-biopsied and showed similar pathology suggestive of pulmonary myxoma. DISCUSSION: The earliest case of pleuro pulmonary myxoma was reported by Barkley and Cardozo1 in 1957 where they described a 54 year-old female active smoker who had presented with a consolidation and after treatment was found to have a left sided rounded opacity. She underwent thoracotomy due to increasing size on the radiograph and was found to have multiple pleural based lesions and a larger 3 cm mass in the lingula which was resected. This was shown to be made up of benign myxomatous tissue on histology. In addition to pleuropulmonary myxomas, there are case reports describing myxomas arising in the pulmonary artery, trachea2 and endobronchially. The differential diagnosis for a myxoma can be myxoid liposarcoma, myxoid chondrosarcoma, myxoid malignant fibrous histiocytoma, pulmonary hamartoma, angiomyolipoma and pulmonary lipoma. From these case reports it is observed that the myxomas tend to increase in size with time and are prone to recur after resection. Cases where complete resection was done had no recurrence during the documented follow up period. CONCLUSIONS: 1. Pulmonary myxoma is a rare benign tumor of the lung. 2. Myxomas tend to increase in size with time and are prone to recur after resection. 3. Myxomas can be seen independently and may co-exist with other malignancies3 making their identification important. Reference #1: Barkley H, Cardozo RH. A myxomatous tumour of pleuro-pulmonary origin. Thorax 1957;12:264-7. Reference #2: Nadrous HF, Krowka MJ, Myers JL, Allen MS, Sabri AN. Tracheal myxoma: a rare benign tracheal tumor. Mayo Clinic proceedings 2004;79:931-3. Reference #3: Hasleton PS, Langdale-Brown B, Rahman A, Smyrniou N, Barber PV. Pleural myxoma associated with a pulmonary squamous cell carcinoma. Respiratory medicine 1989;83:443-4. DISCLOSURE: The following authors have nothing to disclose: Sophie Korzan, Rahul Mutneja, Omar Ibrahim No Product/Research Disclosure Information

Cytodiagnosis of Sacral Chordoma

We report the cytological findings of a sacro-coccygeal chordoma in a 53 year male diagnosed preoperatively by guided fine needle aspiration cytology. The smears shows characteristic Physalliphorous cells in a metachromatic background of myxoid material entrapping cords of cuboidal cells. Differential diagnosis in cytology include conventional and myxoid chondrosarcoma, myxoid liposarcoma, myxoid malignant fibrous histiocytoma(MFH), metastatic mucinous carcinoma and myxo-papillary ependymoma. The distinguishing features between these neoplasms are discussed. Preoperative diagnosis of chordoma permits optimum planned surgery.

An adolescent presenting with malignant fibrous histiocytoma of the testis: a case report

IntroductionMalignant fibrous histiocytoma is a very common subtype of soft-tissue sarcoma in middle and late adulthood. However, malignant fibrous histiocytoma of the testis is very rare in adolescents.Case presentationWe report here the case of a 14-year-old Han Chinese boy, who presented with left scrotal mass lasting for 20 days along with distending pain for 5 days. A physical examination revealed a chicken egg-sized, firm, well-defined mass and unclear epididymis. A B-scan ultrasonography of the left scrotum displayed a 9.0×5.2×4.5cm medium- or low-echoic lobulated mass, which suggested a left testicular neoplasm. A fine needle aspiration cytology examination revealed that the cells obtained from the patient’s testicular neoplasm were composed of myxoid spindle, and ovoid cells with nuclear atypia and mitotic activity, and arranged in a whirlpool or storiform pattern. Under histological examination, the tumor cells were arranged in a storiform pattern, which displayed mucoid matrix degeneration, and grew invasively. Consequently, a histopathological diagnosis suggested myxofibrosarcoma (or myxoid malignant fibrous histiocytoma).ConclusionsAn ultrasonic examination combined with fine needle aspiration cytology should be helpful for the initial differential diagnosis of testicular malignant fibrous histiocytoma. However, the final confirmation relies on histopathological examination. To the best of our knowledge, this is the first reported case of malignant fibrous histiocytoma of the testis in an adolescent.

Primary malignant fibrous histiocytoma involving the left pulmonary vein presenting as a left atrial tumor

A 35-year-old woman presented with 4 months history of progressively increasing intermittent dyspnea and hemoptysis. Transthoracic echocardiography revealed a loculated mass in the left atrium (LA). A provisional diagnosis of LA myxoma was made. Intraoperatively the tumor was found extending into and closely adherent to the left pulmonary vein and could not be completely cleared off from the pulmonary venous wall. The histopathological examination of the tumor revealed it to be a myxoid malignant fibrous histiocytoma.

Recurrent intracranial myxofibrosarcoma presenting as an extensive fronto-parieto-occipital SOL: An unusual sarcoma of meningeal origin

The histogenesis of malignant fibrous histiocytoma (MFH) has een a matter of considerable debate through decades, and recent oncept does not favor a histiocytic origin as proposed initially. he storiform-pleomorphic variant is now rather a morphologic attern, encountered in many undifferentiated or dedifferentiated umors of mesenchymal or non-mesenchymal origin. The diagosis of MFH is hence reserved for tumors in which no specific ine of differentiation can be ascertained on generous sampling, upplemented by immunohistochemical panel and/or electron icroscopy [1,2]. Myxoid MFH, having a relatively better prognosis, as been designated as ‘myxofibrosarcoma’ with inclusion under he ‘myofibroblastic’ category [1,2]. We herein describe a case of ecurrent myxofibrosarcoma in a 21 year old female, presenting s an extensive fronto-parieto-occipital lesion of meningeal oriin, which to the best of our knowledge, is the second intracranial yxofibrosarcoma to be documented in literature.

Massas mediastínicas: análise de uma casuística.

Mediastinal masses are tumoral lesions arising from intra-thoracic organs. They are an infrequent diagnostic challenge, requiring a structured clinical and radiological approach. The study of the clinical, radiological and pathological characteristics, treatment and prognosis of patients, with mediastinal masses, admitted to an Internal Medicine ward in an University Hospital. A retrospective analysis of the medical records of patients with this diagnosis made between years 2000 and 2008. Twenty-eight patients were included (15F/13M), with a mean age of 55.5 years (17-88). Twenty-six patients presented with symptoms, most frequently, dyspnoea, fatigue, dry cough, chest pain and anorexia. At examination, six exhibited superior vena cava syndrome. Chest X-ray showed pathological changes in 26. Chest CT scan added relevant information in all cases whenever it was performed. Histology was most frequently obtained by mediastinoscopy, open surgical biopsy, percutaneous thoracic biopsy and at necropsy. Histological diagnosis was not possible in ten patients. Final diagnoses included: lymphoma in four patients; sarcoidosis, thymic hyperplasia and undifferentiated squamous carcinoma of unknown origin in two patients each; other diagnosis in single cases were: thyroid teratoma, thymoma, atrial myxoid malignant fibrous histiocytoma, ganglioneuroblastoma, neuroendocrine thymic carcinoma, squamous cell lung cancer and germinative cell tumour. Thirteen patients were submitted to surgery, chemo and/or radiotherapy. Fifteen patients died during admission or when in follow-up. At our center, mediastinal masses are frequently of a malignant origin, affecting relatively young people; a late diagnosis and an associated poor prognosis was the rule, prompting for early intervention to improve outcome.

Myxoid Malignant Fibrous Histiocytoma Presenting as a Midline Nasal Mass

Myxoid malignant fibrous histiocytoma is a rare type of pediatric non-rhabdomyosarcoma soft-tissue sarcoma. The case of a 5-year-old girl is presented, highlighting the potential for multiple pitfalls and aberrant differential diagnoses that need to be identified for successful treatment of pediatric myxofibrosarcomas. An awareness of these tumors and a call for standardized postsurgical treatment protocols is necessary in order to successfully treat children with this disease.

Myxofibrosarcoma Arising in the Maxillary Sinus: A Case Report with a Review of the Ultrastructural Findings and Differential Diagnoses

This is a case report of a patient presenting with a destructive lesion with histologic features of a low grade malignancy in a predominantly myxoid matrix. Various low grade myxoid malignancies were considered in the differential diagnosis of which an overview is presented. A literature review of the ultrastructural findings and possible histogenesis is discussed along with the diagnostic criteria and recent change in the terminology regarding the malignancies previously diagnosed as myxoid malignant fibrous histiocytomas. A final diagnosis of a myxofibrosarcoma was only possible after assessing the immuno-histochemical profile, results of histochemical stains and ultrastructural features of this lesion.

Myxofibrosarcoma: Prognostic Factors and Survival in a Series of Patients Treated at a Single Institution

First described in 1977, myxofibrosarcoma is one of the most common sarcoma subtypes of the elderly. Until some years ago, myxofibrosarcoma was diagnosed as "myxoid malignant fibrous histiocytoma." The aim of this retrospective case series analysis was to investigate prognostic factors and the clinical outcome of a cohort of patients with myxofibrosarcoma treated at a single institution. We reviewed 158 patients with localized myxofibrosarcoma who underwent surgery at the Istituto Nazionale Tumori of Milan, Italy, over 15years. Local recurrence, distant metastases, and survival were analyzed. One hundred twenty patients had primary tumors, while 38 patients had locally recurrent tumors. Five-year overall survival was 77%. Tumor size, grade, and margins were statistically significant predictors of survival. Five-year local recurrence and distant metastases rate were 18% and 15%, respectively. Surgical margins were the only statistically significant prognosticator of local relapses. Patients treated with radiotherapy had the same prognosis as nontreated patients, but likely they had worse local presentations. The histological grade correlated with distant recurrences but not with local relapses. The value of adjuvant chemotherapy could not be determined. Patients with myxofibrosarcoma have a better disease-specific survival than other sarcoma subtypes, but also a higher local relapse rate. This is likely related to the peculiar local growth pattern of these tumors. Adequate surgery should be pursued, while the role of adjuvant therapies need to be investigated.

Giant Malignant Myxoid Fibrous Histiocytoma of the Chest Wall

A 79-year-old woman was admitted with a giant chest wall mass (Figures 1 and 2) and symptoms of dyspnoea that were progressively aggravated during the last 3 months. The mass was initially detected by the patient 1 year earlier, but she did not ask for medical consultation because of its initial small dimensions and the lack of symptoms. The patient underwent uncomplicated resection of the giant mass (measuring 34 cm in its maximal dimension and weighting 6.9 kg) en bloc with the muscles in contact. The tumor destroyed the third through seventh ribs, and these were also resected; the chest wall defect was reconstructed with a 2-mm e-polytetrafluoroethylene mesh. Re-establishment of the chest wall shape and re-expansion of the left lung had as result relief of dyspnoea symptoms and improvement of the quality of life. Histologic and immunohistochemical examination of the tumor established the diagnosis of high-grade myxoid malignant fibrous histiocytoma (MFH)/myxofibrosarcoma. The tumor was predominantly composed of myxoid areas ( 50%) with spindle cells and intermediate atypia and nonmyxoid areas with high cellularity and atypia. The immunohistochemical stainings for vimentin, myoglobulin, CD68, and CD34 (focally) were positive, whereas the stainings for smooth muscle actin and desmin were negative. Despite MFHs are the most common soft tissue sarcomas in adults, primary MFHs of the chest wall are relatively rare tumors.1,2 MFHs tend to reach large dimensions if left untreated within short periods of time. Radical surgery is the optimal treatment for MFHs of the chest wall including all the surrounding structures in contact with the tumor, even if the tumor has large dimensions. Local recurrence is observed in 30% of cases, probably as the result of nonradical surgery or spread of tumor cells within the operative field during resection or extirpation of large tumors.2

Myxofibrosarcoma in the skin

Myxofibrosarcoma, also known as myxoid malignant fibrous histiocytoma, is increasingly recognized as a distinct malignant neoplasm of fibroblastic origin with variable clinical and histopathologic features. Myxofibrosarcomas are among the most common malignant mesenchymal neoplasms of older adults, and approximately two thirds develop within the dermis or subcutis. Herein, we describe the clinicopathologic features of four cases of myxofibrosarcoma involving the skin. Three of these cases were initially misdiagnosed as benign cutaneous neoplasms, two as myxoid neurofibroma. These cases illustrate the clinicopathologic spectrum encompassed by myxofibrosarcoma in the skin and highlight the diagnostic difficulties it may present.

Malignant Fibrous Histiocytoma of the Lateral Conjunctiva and Anterior Orbit

A 25-year-old woman presented for evaluation of a rapidly enlarging mass of the left bulbar conjunctiva. Debulking biopsy was performed, which led to the diagnosis of myxoid malignant fibrous histiocytoma. The patient underwent left orbital exenteration with clear margins. Histopathology showed involvement of the conjunctiva substantia propria, superior conjunctival fornix, and episclera, as well as extension from the lateral rectus muscle sheath. Immunohistochemical staining was strongly positive for CD68, alpha1-antitrypsin, CD34, and vimentin.

Myxofibrosarcome dans une forme anétodermique

Association of malignant cutaneous tumor and secondary anetoderma is rare. Secondary anetoderma in myxofibrosarcoma has not been described to date. We report a case below. A 80-year-old woman presented with a 40 x 40 mm, round, flesh-colored lesion on her left buttock. Physical examination showed a soft, protuberant lesion, without firm underlying subcutaneous mass. Pathologic examination of the surgical specimen revealed a myxofibrosarcoma, with focal loss of elastic fibers in the overlying dermis. There was no evidence of systemic involvement. One year later, she developed a recurrent tumor at the same site, with similar clinical presentation, which was treated by broad excision. Secondary anetoderma is usually seen in association with cutaneous infections and benign skin tumors. An anetodermic presentation of myxofibrosarcoma has not been reported to our knowledge. Myxofibrosarcoma (formerly referred to as myxoid malignant fibrous histiocytoma) is characterized by an abundant myxoid background in at least one half of the tumor. The tumor recurs in almost two-thirds of cases and metastasizes in one-fourth. Our case confirms that a unique, acquired anetodermic lesion can reveal a malignant tumor. A large deep biopsy should be performed systematically when this variety of anetoderma is observed.

Myxofibrosarcoma (myxoid malignant fibrous histiocytoma) showing cutaneous presentation: report of two cases

Myxofibrosarcoma or myxoid malignant fibrous histiocytoma is one of the most common fibroblastic sarcomas in older patients. It is characterized by a tendency for predominantly subcutaneous, multinodular, diffusely infiltrative growth, which may extend to the overlying dermis and present as a cutaneous lesion. Histologically, it comprises a spectrum ranging from hypocellular low-grade myxoid to high-grade pleomorphic sarcoma. Because the dermal presentation usually appears relatively banal, accurate diagnosis is sometimes challenging. In this report, we present two cases of myxofibrosarcoma with dermal involvement.

Malignant fibrous histiocytoma of the spermatic cord: A case report

4804 Background: Malignant fibrous histiocytoma (MFH) is a common sarcoma of the soft tissue but it rarely occurs in the spermatic cord. Methods: We present a case of 54-year-old male who presented with a right painless scrotal mass for 1 year, which had progressively enlarged. Past medical history was not significant. Results: Ultrasound and a CT scan of the abdomen and pelvis showed a 7.4x7.1cm solid scrotal mass that appeared to involve the inguinal canal, possibly the spermatic cord on the right side. Metastatic work up was negative. He underwent right radical orchiectomy. Grossly, he was found to have an enormous right hemiscrotal mass, about the size of softball. The left testicle was pushed over from midline, but felt normal. The histologic diagnosis was myxoid malignant fibrous histiocytoma, high grade, focally involving the inked margin. Patient received adjuvent radiation therapy and so far there was no evidence of local recurrence. Conclusions: Malignant fibrous histiocytoma (MFH) is a common sarcoma of the soft tissue in late adult life and is usually found in the region of the extremities and retroperitoneum. It rarely occurs in the genitourinary organs and even rarely in the spermatic cord and so rarely been reported. The pre-operative clinical diagnosis of spermatic cord tumor is difficult and is usually confused with inguinal hernia, spermatocele, epididymo-orchitis and testicular tumor. The definitive diagnosis requires pathologic examination of the surgically resected specimen. It is known to spread rapidly and aggressively with a 2-year survival rates between 34 and 43%. The follow up in the reported cases have been relatively short and the characteristics of this tumor remain poorly understood. No significant financial relationships to disclose.

Myxoid malignant fibrous histiocytoma and pleomorphic liposarcoma share very similar genomic imbalances

Malignant fibrous histiocytoma (MFH) is the most common soft tissue sarcoma. Nevertheless, the validity of this heterogeneous pathological entity has been recurrently questioned by pathologists. Recently, analyses by comparative genomic hybridization (CGH) of a large series of MFHs suggested that MFHs consist of morphologic modulation of other poorly differentiated sarcomas like leiomyosarcomas (LMS) or dedifferentiated liposarcomas (DLPS). We report here an analysis by CGH of 22 myxoid MFHs (mMFH), one of the five histological subtypes of MFH, and of nine pleomorphic liposarcomas (pLPS), a rare poorly differentiated LPS. The chromosome imbalances encountered in the series of mMFH were very similar to those observed in the series of pLPS studied in the laboratory and in the series of 14 pLPS published in the literature. The most frequent gains involved chromosome subregions: pericentromeric regions of 1, 5p, 19p, 19q and 20q. Losses found in the chromosomal arms 1q, 2q, 3p, 4q, 10q, 11q and 13q were also recurrent. The use of a clustering software did not separate the two pathological groups (mMFH and pLPS) on the basis of genomic data. Moreover, pLPS–mMFH represented, according to the clustering software results, an entity clearly distinguished from other soft tissue sarcomas, LMS in particular, with which they share common genetic aberrations. Additional studies are needed to identify genes targeted by these genomic aberrations, and implicated in the oncogenesis of these tumor subtypes. The characterization of common gene alterations in both tumor groups would suggest a closer relationship between these two types of soft tissue sarcomas.

Expression of DOL54 is not restricted to myxoid liposarcomas with the FUS-DDIT3 chimera but is found in various sarcomas

The DOL54 gene [also known as megakaryocyte stimulating factor, articular superficial zone protein (SZP) or proteoglycan 4 (PRG4)], was cloned as a downstream target gene of the FUS-DDIT3 chimera, which is the fusion gene that characterizes myxoid liposarcoma (MLS). Activation of DOL54 was found to require an intact DNA binding domain of the DDIT3 protein and to be dependent on the presence of the N-terminal part of the FUS protein. Although originally suggested to be of oncogenic significance, expression analysis of DOL54 in tumors has so far been limited to a few cases of liposarcoma and malignant fibrous histiocytoma (MFH). In the present study we were interested to evaluate whether DOL54 expression can be associated with other fusion genes in which FUS is the 5'-partner. Thus, we investigated the expression of DOL54 in low grade fibromyxoid sarcoma (LGFMS) carrying the FUS-BBF2H7 chimeric transcript. We also included synovial sarcomas (SS), Ewing tumors (ET), extraskeletal myxoid chondrosarcomas (EMC) and MFH. The first 3 of these tumor types are characterized by chromosomal translocations that give rise to fusion genes not involving FUS, while no specific chimeric genes have been reported in MFH. DOL54 expression was found in 8/12 LGFMS carrying the FUS-BBF2H7 chimera but also in 8/10 of the examined MFH, 5/7 SS, 2/5 ET and 7/7 examined EMC. The results of our study clearly show that expression of DOL54 is not only a characteristic feature of MLS with the FUS-DDIT3 chimera but that this is a frequent finding also in various other sarcomas.