Paraneoplastic syndrome due to angiomatoid fibrous histiocytoma: a known presentation of an uncommon diagnosis in a rare site and age.

A woman in her 70s presented with a small subcutaneous retrosacrococcygeal mass and a history of elevated erythrocyte sedimentation rate present for several years. It was misdiagnosed as an inflammatory process of unclear origin. She underwent further investigation with the appearance of weight loss and weakness. A sacrococcygeal mass was noted on CT scan. A core needle biopsy was inconclusive for chordoma versus myoepithelioma. Wide surgical resection of the tumour including the coccygeal bone was performed. Following surgery, all the systemic symptoms resolved with normalisation of inflammatory markers. The pathological examination showed a relatively circumscribed multinodular myxoid tumour with lymphatic tissue cuff. Pan-sarcoma fusion analysis detected an EWSR1 (Exon7)-CREB1 (Exon7) fusion gene. The lesion was diagnosed as angiomatoid fibrous histiocytoma with paraneoplastic syndrome presentation of several years' duration.

Primary Renal Malignant Fibrous Histiocytoma in an End-Stage Renal Disease Patient.

Besides renal cell carcinoma with increased risk reported in the dialysis population, other unusual types of renal tumors should also be considered. However, to the best of our knowledge, renal sarcomas have never been reported among end-stage renal disease patients undergoing dialysis in the literature. In this study, we present the first case of a primary renal malignant fibrous histiocytoma (MFH, also called undifferentiated pleomorphic sarcoma) in a 41-year-old woman with end-stage renal disease.

Surgical Treatment of Oral Cavity Nodular Fasciitis.

BACKGROUND: Nodular fascitiis is a myofibroblastic neoplasm of the soft tissue that rarely affects oral cavity. With a broad pattern of presentation, sometimes Nodular Fascitiis can have a rapid growth and appear highly cellular with local aggressiveness on biopsies, thus simulating a sarcoma. The aim of this paper is to present a case of troublesome diagnosis of nodular fascitiis mimicking a Malignant Fibrous Histiocytoma, with the purpose of alert clinicians and pathologists on the difficulties that can be met in the differential diagnosis between these 2 lesions. A 42-year-old male presented an exophytic lesion on the cheek. After the excisional biopsy, histological and immunohistochemical evaluations revealed a picture of doubtful significance. With a careful analysis, the diagnosis of nodular fasciitis was made and the patient was not further treated. At a 3-year follow-up, no recurrence was found. Differential diagnosis within myofibroblastic neoplasm can be a real challenge for both Clinicians and Pathologist. A coordinated team-work is mandatory to avoid clinical malpractice and unnecessarily aggressive treatment.

Intracranial angiomatoid fibrous histiocytoma with Hodgkin lymphoma.

Angiomatoid fibrous histiocytoma (AFH) is a rare soft tissue tumour of uncertain differentiation and low metastatic potential, which occurs predominantly in children and young adults. It occurs mostly within the extremities, trunk, head and neck. We report the case of a 32-year-old female that was operated in our hospital in 2016 and twice in 2017. The patient had headaches and neck pain initially in 2016. We discuss the radiographic and histologic features initially found and the findings that ultimately led to the diagnosis of AFH. The patient had a past history of Hodgkin lymphoma.

Treatment of spinal malignant fibrous histiocytoma, a case report and literature review. / Tratamiento del histiocitoma fibroso maligno vertebral, reporte de caso y revisión de la literatura.

OBJECTIVE: To present a case of spinal malignant fibrous histiocytoma in the fourth lumbar vertebra that received treatment by an L4 spondylectomy and placement of intervertebral expander and posterior fixation. CASE REPORT: A 47-year-old male patient with no relevant history presented with lumbar pain of 2 months' evolution. Treated conservatively, with slight improvement in pain, the patient persisted with low back pain irradiation to pelvic members, predominantly left-sided, accompanied by weakness and claudication. Clinically, he presented with paresthesias 3/5, hypoaesthesia L4, L5 and S1, predominantly left-sided. Lumbosacral computerized axial tomography evidence of an osteolytic lesion in the L4 body, predominantly left-sided, with invasion of the lumbar canal with poorly delimited margins; lumbosacral spine MRI showed hyperintense lesion in T2, heterogeneous, with irregular borders involving more than 60% of the vertebral body of L4 with invasion of the spinal canal causing compression to the roots. He was treated with an L4 spondylectomy and placement of intervertebral expander and posterior fixation. CONCLUSION: Spondylectomy is an effective option for the treatment of spinal malignant fibrous histiocytoma that involves combined approaches. However the challenge is greater since it requires a knowledge of the great abdominal vessels and multidisciplinary intervention.

Primary undifferentiated pleomorphic cardiac sarcoma with MDM2 amplification presenting as acute left-sided heart failure.

Primary cardiac tumours are a rare clinical entity that can present with myriad of non-specific cardiopulmonary symptoms. We describe a case of a 61-year-old previously healthy woman who presented with progressive dyspnoea and lower extremity swelling, suggestive of acute left-sided heart failure. Transthoracic echocardiogram revealed a large, 3.7×3.2 cm intracardiac mass resulting in severe mitral valvular dysfunction. The patient underwent surgical resection of the mass, however, negative margins were not obtained, and the tumour quickly returned. Histological and molecular analysis was consistent with the diagnosis of undifferentiated pleomorphic sarcoma with murine double minute 2 (MDM2) amplification. Given the overall grim prognosis, the patient chose to pursue comfort-based care. She died at home 9 months after the initial diagnosis. Here, we provide an updated review of the literature for the classification of undifferentiated pleomorphic cardiac sarcoma and potential treatment modalities.

Splenic malignant fibrous histiocytoma with concurrent hypertension and epistaxis in an Alaskan malamute dog.

BACKGROUND: Malignant fibrous histiocytoma has been uncommonly described in dogs. Several extranasal neoplasias have been reported to result hypertensive epistaxis. There are, however, no published case reports of extranasal malignant fibrous histiocytoma with concurrent hypertension and epistaxis in dogs. CASE PRESENTATION: A 10-year-old dog presented with a spontaneous massive epistaxis persisting for 5 days. The dog exhibited unstable hypertension, which was considered as a cause of epistaxis. The complete blood count, prothrombin time, and activated partial thromboplastin time were within the reference limits, and other systemic examination showed no abnormalities except for a splenic mass occupying more than one third of the abdomen. Histologic examination of the resected spleen revealed the characteristic features of a malignant fibrous histiocytoma. One week after splenectomy, the hypertension and epistaxis resolved clinically and did not recur on the 5-month follow-up. CONCLUSIONS: The dog's blood pressure and epistaxis normalized after malignant fibrous histiocytoma resection suggesting that hypertensive epistaxis may be a rare manifestation of canine malignant fibrous histiocytoma.

Undifferentiated sarcoma presenting as a slowly enlarging facial mass.

Head and neck sarcomas are rare and consist of a variety of histologic subtypes. We present a case of undifferentiated/unclassified sarcoma (UUS) of the maxillary sinus, a tumor subtype historically known as malignant fibrous histiocytoma (MFH) or undifferentiated pleomorphic sarcoma (UPS). A 50-year-old female patient presented with worsening facial pain and dysphagia. Physical examination demonstrated a large, ulcerated mass protruding from the oral cavity. Computed tomography demonstrated a large, enhancing mass centered in the right maxillary sinus with local invasion. The initial biopsy was read as "central giant cell granuloma." Conservative management yielded no improvement, and the tumor grew steadily. The patient underwent a total maxillectomy with resection of the orbital floor and an anterior ethmoidectomy, followed by radiation and chemotherapy. In addition to treatment of this patient, we discuss a review of the literature and the clinical presentation, radiologic, and histologic findings of this disease.

Therapeutic response of metastatic angiomatoid fibrous histiocytoma carrying EWSR1-CREB1 fusion to the interleukin-6 receptor antibody tocilizumab.

Angiomatoid fibrous histiocytoma (AFH) is a rare soft tissue tumor that has been associated with EWSR1-CREB1 gene fusion. Outcome in patients with unresectable distant metastases is generally fatal. Interleukin-6 (IL-6) secretion has been described in tumors with EWSR1-CREB1 fusion, and may promote tumor growth due to autocrine stimulation. Tocilizumab is an IL-6 receptor antibody that has potential benefit as a targeted therapy in refractory neoplasms with IL-6 secretion. We describe a child with metastatic AFH with EWSR1-CREB1 fusion and elevated IL-6 whose disease progressed during treatment with traditional chemotherapeutic agents, but improved after targeted therapy with tocilizumab.

Angiomatoid Fibrous Histiocytoma With Paraneoplastic Platelet Storage Pool Deficiency.

Angiomatoid fibrous histiocytoma is a rare soft tissue tumor usually discovered in young individuals. This tumor is often mistaken for a hematoma and typically misdiagnosed. It is commonly found in the extremities and may be associated with a site of recent or previous trauma. Characteristic histology includes nodules of histiocytoid spindle cells with pseudoangiomatoid spaces, fibrous pseudocapsules, and lymphoid cuffing. We describe the case of an 8-year-old girl who presented after incision and drainage of a superficial thigh lesion and experienced subsequent chronic bleeding of her wound. Her initial presentation was concerning for an underlying bleeding disorder, and laboratory analysis uncovered a paraneoplastic platelet function disorder that resolved with therapy of the primary tumor.

Retrovesical malignant fibrous histiocytoma: a rare tumor.

Malignant fibrous histiocytoma (MFH) originating from the retrovesical space is a very rare tumour. A 61-year-old man presented to our hospital with complaints of retention of urine and burning sensation during micturition since 6 months. CT scan abdomen showed a large retrovesical mass between the urinary bladder and rectum, measuring 11×9×12 cm, displacing the urinary bladder. Serum PSA (Prostate Specific Antigen) value was within normal range. Biopsy from retrovesical mass and immunohistochemistry was suggestive of MFH. Wide excision of the retrovesical mass was done. Histopathology confirmed the diagnosis of MFH. He received adjuvant radiotherapy. He is on regular follow-up since the last 2 years after radiotherapy with no signs and symptoms of disease recurrence.

[Malignant fibrous histiocytoma as a rare cause of acute limb ischemia]. / Malígny fibrózny histiocytóm ako raritná prícina akútnej koncatinovej ischémie.

Malignant fibrous histiocytoma (MFH) represents a rare malignant affection of heart and aorta. Its clinical presentation depends on the localisation, size, degree of invasion and metastasis. Previously, relatively few cases of acute tumour mass embolisation into the visceral and limb arterial system were described in the literature. In the present case study we describe a case of acute ischemia of both lower extremities caused by thromboembolic mass of MFH cells. According to literary sources this tumour type is characterized by poor prognosis as it was in the case of our patient.

Primary hepatic malignant fibrous histiocytoma combined with invasion of inferior vena cava: A case report and literature review.

RATIONALE: Malignant fibrous histiocytoma (MFH), primary presented in liver, was very rare and displayed a poor prognosis because of high aggression. As a few of cases had been reported merely, we shared the case of primary hepatic MFH combined with invasion of inferior vena cava (IVC). PATIENTS CONCERNS: A 69-year-old women presented with abdominal pain. DIAGNOSES: Abdominal computed tomography and magnetic resonance imaging indicated a soft mass about 5.4 × 4.2 cm in the caudate lobe, accompanied with IVC invaded. INTERVENTIONS: After the multidisciplinary consultation, laparotomy was performed, followed by chemotherapy and radiotherapy. Primary hepatic MFH was demonstrated pathologically. Till now, the patient was alive for >22 months after surgery and no evidence of recurrence or distant metastasis was suspected. OUTCOMES: We discussed the integrated procedure of diagnosis and treatment, combined with data from literature review. LESSONS: To our knowledge, the primary hepatic MFH combined with invasion of IVC was hardly reported. Despite the poor prognosis, the comprehensive treatment integrating the surgery, chemotherapy, and radiotherapy showed the satisfactory disease-free and overall survival. However, further investigations are definitely warranted.

Unusual localization of a primary pleomorphic malignant fibrous histiocytoma on the mitral valve: a case report and review of the literature.

INTRODUCTION: It has been reported that cardiac malignant fibrous histiocytomas occur more frequently in the left side of the heart, especially in the left atrium, but rarely invade the mitral valve. We present a case with a giant malignant fibrous histiocytoma with an unusual localization involving almost the entire left atrium, mitral valve, and left superior pulmonary vein. CASE PRESENTATION: We describe the case of a 54-year-old woman from Kosovo admitted to our emergency department with dyspnea. A transthoracic echocardiography demonstrated a giant mass localized on her left atrium. Our patient underwent emergent total surgical removal of the mass. The mass extended between her left superior pulmonary vein, and extended to her left atrium and the posterior mitral valve leaflet. We formulated a surgical plan for total separation of the mass from the endocardium. Total removal was performed and her left side pulmonary veins were entirely freed from the mass. We then performed a mitral valve replacement. The differential diagnosis included other masses of the left atrium, including thrombi, vegetations, and cardiac tumors. Postsurgical histopathologic results showed a pleomorphic malignant fibrous histiocytoma. Six monthly follow-up cardiac and abdominal sonographic examinations revealed no tumor recidivism. CONCLUSION: We reviewed 90 cases with malignant fibrous histiocytoma reported in the literature. Our case was especially unusual because of the primary location in the mitral valve, the pleomorphic variant, and the dimensions and extension. Complete surgical resection is mandatory to ameliorate symptoms and to obtain histologic information.

Peripheral and Neuraxial Chemical Neurolysis for the Management of Intractable Lower Extremity Pain in a Patient with Terminal Cancer.

We present the case of a 74-year-old man with Stage IV metastatic, multifocal, malignant fibrous histiocytoma (T2b, N1, M1, G4) invading the proximal area of the left lower extremity and resulting in intractable neuropathic pain along the distribution of the femoral nerve. He described the pain as being so severe to cause inability to ambulate without assistance or to sleep in a supine or prone position. After a spinal cord stimulation trial and a trial of intrathecal (IT) hydromorphone, both performed at an outside institution, had failed to achieve adequate pain relief, we decided to perform a femoral nerve chemical neurolysis with phenol under ultrasound (US) guidance. The intervention provided 6 months of almost complete pain relief. With the tumor spreading in girth distally and proximally to the scrotal and pelvic areas as well as to the lungs, and pain returning back to baseline, we proceeded with a second femoral nerve chemical neurolysis. Unfortunately we were not able to achieve adequate pain relief. Therefore we opted to proceed with a diagnostic injection of local anesthetic under fluoroscopic guidance at the left L2, L3, and L4 nerve roots level. This intervention provided 100% pain relief and was followed, a few days later, by chemical neurolysis with phenol 3%. The patient reported complete pain relief with the procedure and no sensory-motor related side effects or complications. He was able to enjoy the last 6 weeks of life with his wife and family, pain-free. With this report we add to the limited literature available regarding the management of intractable cancer pain with chemical neurolysis in and around the epidural space.

Paraneoplastic Syndrome of Angiomatoid Fibrous Histiocytoma May Be Caused by EWSR1-CREB1 Fusion-induced Excessive Interleukin-6 Production.

We describe a 7-year-old girl with angiomatoid fibrous histiocytoma (AFH) presenting severe inflammatory symptoms. The cytokine/chemokine profile of serum samples before and after surgery demonstrated that interleukin (IL)-6 had decreased by the greatest percentage. The AFH cells were immunopathologically positive for IL-6 and Tyr705-phosphorylation of signal transducer and activator of transcription 3. The EWSR1-CREB1 fusion gene detected in the tumor leads to continuous activation of CREB1 and IL-6 production, because the promoter region of IL-6 has a CREB binding site. Thus, IL-6 plays pivotal roles in both paraneoplastic syndrome and the oncogenesis of AFH.

Primary intracranial angiomatoid fibrous histiocytoma presenting with anaemia and migraine-like headaches and aura as early clinical features.

A 17-year-old female with migraine with aura complained of fatigue and was diagnosed with anemia. Three years later, changes in her headache pattern prompted hospital referral. Brain MRI showed a bi-lobed extra-axial intracerebral tumor encroaching both parieto-occipital regions. The resection specimen yielded a rare diagnosis of primary intracranial angiomatoid fibrous histiocytoma (AFH). Tumor removal resulted in cessation of her migraine and anemia. AFH may cause systemic symptoms--in this case fatigue and anemia--long before focal neurological symptoms are present. This is the first report of an intracranial AFH presenting as migraine with visual aura and anemia.