Fibrous Xanthoma Research Articles

Offbeat phenomenon in antrum of highmore – Inflammatory myofibroblastic tumor

The Inflammatory Myofibroblastic Tumor (IMT) is a rare benign pathology of the head and neck region that tends to mimic a malignancy on account of its aggressive clinical and radiographic features. There are various descriptive terms for this lesion due to the uncertainty of the biologic nature of the lesion, namely: Fibrous Xanthoma, Plasma Cell Granuloma, Pesudosarcoma, Lymphoid Hamartoma, Myoxid Hamartoma, Inflammatory Pseudotumor and Benign Myofibroblastoma. Inflammatory Myofibroblastic Pseudotumor occurs throughout the body, most frequently in the peritoneal cavity, retroperitoneal space and lungs, among extra pulmonary lesions 15% accounts for the head and neck region. Most commonly, they are located in the region of the orbit. Inflammatory Myofibroblastic tumor presentation in maxillary sinus is rare and has an invasive potential. We have presented a case report of Inflammatory Myofibroblastic tumor of maxillary sinus, correlating Clinical, radiographic, and histopathologic along with immunohistochemical profile to arrive at a diagnosis, with special emphasis on the disputed nosology of this lesion and the latest therapeutic update.

RARE FORM OF PRESENTATION IN A HISTIOCYTOMA CASE

Malignant fibrous histiocytoma is also known as malignant fibrous xanthoma and is a form of neoplasm with uncertain histological origin. This tumour is rich in histiocytes and fibroblasts. MFH represent one on the most common type of soft tissue sarcoma but it has a low incidence in general population. We report the case of a 60 years old male patient, presented at the University Emergency Hospital Bucharest for a tumoral mass on the left hemithorax, in evolution for about 1 year, with an accelerated grow in the last 6 months. This case is typically because the patient is a male and he is 60 years old (range 50-70 years). In the same time, there are some particularities that make the case special and determine us to draw some conclusions about the management of this pathology. Histiocytoma represent a rare tumoral pathology and it is difficult to do a differential diagnostic preoperatively. Surgery is the cornerstone of treatment for all soft tissues. The goal of surgery is to eradicate disease in the affected area. All excisions of tumoral mass must be with resection margins for oncological security to avoid the possible complications.

Pyogenic Granuloma of Gingiva Masquerading Inflammatory myofibroblastic tumor: Case Report of a Misleading Case

Inflammatory myofibroblastic tumor (IMT) is a specific entity composed of myofibroblastic cells with a variable admixture of inflammatory cells, including mature lymphocytes, histiocytes, plasma cells and eosinophils, with infiltrates of collagen fibrils. It occurs predominantly in the soft tissues and also, sometimes, in the head and neck region. Various synonyms used for it include inflammatory myofibrohistiocytic tumor, inflammatory pseudotumor, xanthomatous pseudotumor, plasma cell granuloma, myxoid hamartoma, lymphoid hamartoma, fibrous xanthoma, benign myofibroblastoma, pseudosarcoma and most recently, inflammatory myofibroblastic tumor (IMT). The recent nomenclature describes it as being a reactive lesion. Herewith, we are reporting a case of inflammatory myofibroblastic tumor (IMT) with no bone involvement with a brief review on its clinical and biological behavior.

A rare giant cell tumor of the tendon sheath of the dorsal hand in a child.

A rare giant cell tumor of the tendon sheath of the dorsal hand in a child.

Primary Malignant Fibrous Histiocytoma of the Breast: A Case Report and Review of 38 Chinese Cases

Introduction First proposed by O’Brien and Stout in 1964, malignant fibrous histiocytoma (MFH) is a soft tissue neoplasm with poor prognosis. MFH is also known as pleomorphic fibrous xanthoma, malignant fibrous xanthoma, and pleomorphic fibrous histiocytoma. A possible origin of MFH is the tissue histiocyte and fibroblastic cell. MFH is most frequently found in the lower and upper extremities and retroperitoneum and it very rarely occurs in breasts. Here, we report a case of primary MFH of the breast in a 37-year-old Chinese female and review 38 other similar cases from a total of 23 Chinese reports in the past 22 years.

Cerebellar malignant fibrous histiocytoma

Malignant fibrous histiocytoma (MFH) is uncommon within the central nervous system. MFH is a malignant tumor composed of a mixture of fibroblastic and histiocytic cells, and is also known as pleomorphic fibrous xanthoma, malignant fibrous xanthoma, and pleomorphic fibrous histiocytoma. It is a pleomorphic sarcoma originally found in soft tissue and there are few reported cases within the central nervous system, in particular the cerebellum. To the best of my knowledge, this is third case arising from the cerebellum. This tumor is difficult to diagnose and may be difficult to treat.

Fibrohistiocytic Tumors and Related Neoplasms in Children and Adolescents

Fibrohistiocytic tumors (FHTs) in children and adolescents range from the benign fibrous histiocytoma, or dermatofibroma, to a variety of intermediate and malignant neoplasms, such as dermatofibrosarcoma protruberans and high-grade undifferentiated pleomorphic sarcoma (malignant fibrous histiocytoma). Those tumors as a group are comprised of fibroblasts, myofibroblasts, and histiocytes-dendritic cells with a variably prominent inflammatory infiltrate consisting of lymphocytes and eosinophils. Dendritic cells are also a major constituent of another group of neoplasms that include Langerhans cell histiocytosis, follicular and interdigitating cell sarcomas, and juvenile xanthogranuloma. These latter tumors are considered in this discussion for the sake of differential diagnosis and their possible histogenetic relationship to FHTs. Recent studies have suggested that the relationship between the fibroblast and histiocyte in the FHTs may reflect the intrinsic capacity to transdifferentiate from one to the other morphologic and functional state. The so-called "facultative fibroblast," as a cell with fibroblastic and histiocytic properties, was discussed in the context of the fibrous xanthoma 50 years ago. Possibly the entire histogenetic concept of FHTs should be reconsidered in light of current studies.

Inflammatory myofibroblastic tumor

Inflammatory myofibroblastic tumor is an uncommon lesion of unknown cause. It encompasses a spectrum of myofibroblastic proliferation along with varying amount of inflammatory infiltrate. A number of terms have been applied to the lesion, namely, inflammatory pseudotumor, fibrous xanthoma, plasma cell granuloma, pseudosarcoma, lymphoid hamartoma, myxoid hamartoma, inflammatory myofibrohistiocytic proliferation, benign myofibroblatoma, and most recently, inflammatory myofibroblastic tumor. The diverse nomenclature is mostly descriptive and reflects the uncertainty regarding true biologic nature of these lesions. Recently, the concept of this lesion being reactive has been challenged based on the clinical demonstration of recurrences and metastasis and cytogenetic evidence of acquired clonal chromosomal abnormalities. We hereby report a case of inflammatory pseudotumor and review its inflammatory versus neoplastic behavior.

Clinicopathologic analysis of primary atypical fibrous xanthoma of the esophagus

Clinicopathologic analysis of primary atypical fibrous xanthoma of the esophagus

Clinicopathologic analysis of primary atypical fibrous xanthoma of the esophagus

Clinicopathologic analysis of primary atypical fibrous xanthoma of the esophagus

A case of malignant fibrous histiocytoma of the head after trauma and radiation therapy

Malignant fibrous histiocytoma (MFH) firstly described as “malignant fibrous xanthoma” by O’ Brien and Stout in 1964, is the most common soft tissue sarcoma of late adult life. Uncertain histogenesis and numerous subtypes make MFH a rather controversial entity. MFH only rare arises from structures of the head and neck. When it does, it most often originates in facial structures, particularly the maxilla. This report details a case of a patient with malignant fibrous histiocytoma presenting clinically as a right-sided large indurated frontoparietal mass, three months after head trauma and eight years after radiation therapy for brain lymphoma located in the right frontal and parietal lobes. Radical excision was a surgical challenge because of the extensiveness of the lesion.

Primary malignant fibrous histiocytoma of the mandible: review of literature and report of a case

Malignant fibrous histiocytoma (MFH), the most frequent soft tissue sarcoma of adulthood, was first described by O’Brien and Stout in the 1960s. 1 O’Brien J.E. Stout A.P. Malignant fibrous xanthoma. Cancer. 1964; 17: 1445 Crossref PubMed Scopus (732) Google Scholar The concept of MFH is now widely recognized as a distinct clinicopathologic entity. 2 Weiss S.W. Enzinger F.M. Malignant fibrous histiocytoma. An analysis of 200 cases. Cancer. 1978; 41: 2250 Crossref PubMed Scopus (1224) Google Scholar However, regarding its histogenesis, arguments have been proposed supporting either fibroblastic or primitive mesenchymal cell differentiation. 3 Hollywood K. Fletcher C. Malignant fibrous histiocytoma Morphologic pattern or pathologic entity. Semin Diagn Pathol. 1995; 12: 210 PubMed Google Scholar

Malignant Fibrous Histiocytoma: A Method to Control Intraoperative Hemorrhage by Clamping the Feeding Arteries

Malignant fibrous histiocytoma (MFH), also referred to in the past as malignant fibrous xanthoma and fibroxanthosarcoma, is a tumor of mesenchymal tissue origin. A case of retroperitoneal MFH was reported. In this paper, we describe a method of hemostasis and intraoperative control of hemorrhage during resection of retroperitoneal MFH by snaring the feeding arteries. The patient was successfully operated on using this technique.

Multicentric Intracranial Fibrous Xanthoma

Intracranial fibrous xanthoma is extremely rare; only 11 cases have been reported so far. The authors report a case of multicentric intracranial fibrous xanthoma. Precontrast computed tomographic (CT) scans revealed a left frontal subdural mass, which had previously been diagnosed as a chronic subdural hematoma at another hospital. However, operation disclosed no hematoma but a granulomatous tumor. The biopsied specimen was histologically diagnosed as fibrous xanthoma. Postoperative postcontrast CT scans showed intense homogeneous enhancement at the clival and the left frontal regions, both of which appeared as iso- to low-intensity areas on T1-weighted magnetic resonance (MR) images and as low-intensity areas on T2-weighted MR images. She gradually recovered by conservative treatment but suddenly died of cerebral infarction. Autopsy revealed fibrous xanthomas in the left frontal and the clival regions. This is the first report of the use of MR imaging for intracranial fibrous xanthoma, and its features differ from those of common intracranial parenchymal tumors, MR imaging can be helpful in the diagnosis of this tumor.

Treatment of Malignant Fibrous Histiocytoma and Atypical Fibrous Xanthomas with Micrographic Surgery

Fibrous tumors of the soft tissue are usually benign, but some fibrous neoplasms such as dermatofibrosarcoma protuberans (DFSP), atypical fibroxanthoma (AFX), and malignant fibrohistiocytoma (MFH) can be very destructive locally with a high recurrence rate after local excision. On occasion, they can metastasize. Previous reports have confirmed the high success rate of Mohs micrographic surgery for the treatment of DFSP, but data have been lacking on the potential benefit of this surgical approach for MFH and AFX tumors. Over the past 6 years, we have treated 17 patients with MFH (20 tumors) and 5 patients with AFX with Mohs micrographic surgery. A retrospective analysis of the surgical results is presented. To date (average 3-year follow-up), all patients contacted are tumor free with only one recurrence; no patient has developed metastatic disease. Our results to date are very encouraging; they lend support to Mohs micrographic surgery as a desired surgical approach for these difficult-to-cure neoplasms.

Xanthomatous lesions of the mandible: Osseous expression of non-X histiocytosis and benign fibrous histiocytoma

Seven cases of xanthomatous lesions of the manidble, followed from 1 to 18 years, are documented. All lesions were benign and painless; radiographic appearances varied from single and well-demarcated to diffuse and multilocular. Microscopic examination showed sheets of histiocytes with xanthoma or foam cells, fibroblasts and fibrous tissue, and inflammatory cells as well as cells in transition, with characteristics of both histiocytes and fibroblasts. Immunohistochemical staining and electron microscopic observation provided further evidence of the biphasic nature of this cell population. Twelve similar cases were found in the literature under a variety of diagnostic or descriptive labels. Our findings show that these lesions are histiocytic in nature and that they range from small, innocuous lesions, which we regard as the osseous counterpart of non-X histiocytosis, to larger, more destructive lesions diagnosed as benign fibrous histiocytoma (fibrous xanthoma) of bone.

Bilateral intracranial fibrous xanthoma

We present a case of fibrous xanthoma in a 6-year-old boy who showed disturbance of consciousness and signs of increased intracranial pressure. A computed tomographic (CT) scan showed bitemporal enhanced masses and hydrocephalus. The patient was operated on in two stages. The tumors were located intradurally and extracerebrally and were firmly attached to the dura mater at the base of the middle fossa. Glial fibrillary acidic protein stain was negative, and histologic examination suggested a diagnosis of bilateral fibrous xanthoma. Postoperative CT scan showed residual tumors and an enlarged choroid plexus adjacent to it. A CT scan at 12 years of age showed no enhanced tumor, but disclosed bitemporal high-density areas and diffuse brain atrophy; the choroid plexus reverted to normal size. The patient showed mental and physical retardation, perhaps owing to the compression of the hypothalamus during early childhood. This would appear to be the first known case of bilateral intracranial fibrous xanthoma.

Multicentric reticulohistiocytosis, another lipid disorder with normolipidemic xanthomatosis?

This report describes a 72-year-old female patient with 2 types of severely disfiguring xanthomas, particularly on the facial area and the upper arms. Biopsy revealed xanthoma-type cells, histiocytes, and multiple giant cells. The soft yellowish facial xanthomata were rich in esterified cholesterol, whereas the cholesterol content of the colorless, firm, fibrous xanthoma of the arms was relatively low. The clinical course of insidiously starting polyarthritis, the formation of skin and tendon xanthomas (at the age of 63) and, finally, the histological findings verified the diagnosis of multicentric reticulohistiocytosis. The family history and findings were noncontributory. The patient had symptoms of moderate coronary heart disease and died from an acute myocardial infarction. Her total cholesterol and triglyceride levels were 182 and 89 mg/dl, and the HDL cholesterol value was 43 mg/dl. Plasma levels of apolipoprotein A-I, A-II and B were normal, and her apoprotein E pattern was E 3/E 3. The triglyceride/cholesterol ratio of VLDL, the concentration of apo-LDL (LDL protein) and the LDL-protein/cholesterol ratio were normal. The apo-LDL production, the total clearance rate of apo-LDL, and the receptor-mediated and receptor-independent catabolism of LDL were also normal. Fecal output of neutral sterols and bile acids and the total body cholesterol synthesis were normal, but cholesterol absorption tended to be increased. The response to cholestyramine treatment showed a 44% decrease in the serum cholesterol level and a normal 5-fold increase in cholesterol synthesis. The patient's xanthoma formation could not be associated with any of the causes known to promote the development of normolipidemic xantomatosis. It is suggested that multicentric reticulohistiocytosis should be considered in the differential diagnosis of patients with normolipidemic xanthomatosis.

Myxofibroxanthoma of the Fourth Ventricle

Intracranial fibrous xanthomas are extremely rare. Only 10 cases have so far been reported. A fourth ventricular tumor in an elderly female, identified histopathologically as a myxofibroxanthoma, is described here. After total excision of the tumor, she recovered uneventfully. The histological features and diagnosis of intracranial fibrous xanthomas are discussed.

Kidney Fibroxanthoma (Malignant Fibrous Xanthoma): A Rare Tumor and an Unusual Cause of Retroperitoneal Hemorrhage

A case of kidney fibroxanthoma (malignant fibrous xanthoma, malignant variant of xanthogranuloma), a rare malignant neoplasm of kidney, is described. In addition to the typical histologic features of retroperitoneal xanthogranuloma, this tumor showed obvious pleomorphism and mitotic activity of the histiocytes. We present this case in view of the rarity of this neoplasm and the unusual presentation as massive retroperitoneal hemorrhage.