Fibrous Xanthoma Research Articles

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.

Pleomorphic xanthoastrocytoma: A distinctive meningocerebral glioma of young subjects with relatively favorable prognosisA study of 12 cases

Twelve cases of a distinctive form of supratentorial astrocytoma occurring in young subjects (ages 7 to 25) are reported. The tumors were superficial and involved the leptomeninges extensively. The tumor cells display marked pleomorphism, including bizarre giant cells and a number of mitotic figures, but no necrosis. Many contain large amounts of lipid in their cytoplasm and are surrounded by reticulin fibers, thus simulating a mesenchymal tumor. For these reasons, some examples of this tumor have been previously interpreted to represent meningocerebral fibrous xanthomas. Immunoperoxidase technique performed in nine of the twelve cases has, however, established the presence of glial fibrillary acidic protein in the tumor cells, which are therefore considered to be astrocytic. By electron microscopy many tumor cells are surrounded by basal laminae, accounting for the abundant reticulin network demonstrable in silver preparations. Since subpial astrocytes are known to be partly covered by a basal lamina, it is likely that they are the cells of origin for this neoplasm. In contrast to its pleomorphic cytology, the biological behavior of this tumor appears to be relatively favorable, and long survival times (up to 25 years) have been recorded in some cases. (These tumors are distinct from intracranial fibrous xanthomas of mesenchymal derivation. Cells of the latter are negative on GFAP stain.)

Atypical fibrous histiocytoma with myxoid stroma.A rare lesion arising from dura mater of the brain

Intracranial fibrohistiocytic tumors are rare. This report is concerned with a 24-year-old white male who had an 8 month history of intermittent visual disturbance. Craniotomy revealed a large, completely intradural lesion on the floor of the left anterior cranial fossa without involvement of the leptomeninges or brain parenchyma. Gross and microscopic examination revealed that the fibrogenic portion of the tumor was composed of spindle shaped neoplastic cells arranged in storiform pattern, a hallmark of fibrohistiocytic tumor. In contrast to three previously reported cases of "fibrous xanthomas" involving the leptomeninges and superficial cortex of the brain, the present lesion is clearly originated from mesenchymal stem cell of the dura. The other distinct gross and microscopic feature was the presence of myxoid component which constituted about 40% of the entire lesion. The myxoid component had not been observed in previous 5 reported cases of intracranial fibrohistiocytic lesions. Since there was cellular pleomorphism with extremely rare mitosis, it was felt that the lesion be best designated as atypical fibrous histiocytoma with myxoid stroma.

Benign fibrous xanthoma of the parotid gland:A case report

A fibrous xanthoma of the parotid gland is reported, the literature is reviewed with a discussion of the classification of such tumours. The present case is possibly the first report of a typical benign fibrous xanthoma of the parotid gland.

Multiple fibrous xanthomas of tendon sheath

Fibrous xanthomas of the digital tendon sheath system are a common benign tumour. The varying recurrence rates cited in the literature have usually been attributed to inadequate surgical excision. However, Boyes (1964) has suggested "seeding" as a possible mechanism to explain persistence of the tumour. Our case of multiple fibrous xanthomas at different levels in a single digital sheath system is presented as an example of "seeding" along the tendon sheath.

Retroperitoneal fibrohistiocytic tumors in children.Report of five cases

Five rare retroperitoneal fibrohistiocytic tumors of children are described mainly in reference to the pathological aspects. A more detailed description is given of a retroperitoneal xanthogranuloma, including an ultrastructural study. This case is particularly interesting for its rapid transformation into a fibroxanthosarcoma. The other cases include two fibroxanthosarcomas, a storiform fibrous xanthoma, and a malignant histiocytoma. The most difficult points in the recognition and in the differential diagnosis of these tumors are discussed, and emphasis is placed on some interesting pathological features. Prognosis of such tumors appears difficult to assess from our series since two patients died in the immediate postoperative period, one died one and a half years after the discovery of the tumor, and two are alive and well, but with a short follow-up.

A Case of Fibrous Xanthoma Developed in Metaphysis of Long Bone

Fibrous Xanthoma- a rare primary bone tumor developed in the femoral metaphysis of a twenty-six years old male is described. Histologically, stromal storiform pattern, spindle shaped cells, foamy cells and giant cells are found. This feature is similar to fibrous xanthoma classified to soft tissue tumor, but it is not clear whether fibrous xanthoma of bone is different from non-ossifying fibroma or not. And it is difficut to distinguish histologically this case from malignancy, too.

Primary malignant fibrous histiocytoma (fibrous xanthoma) of bone

The probable histiocytic origin of a group of benign and malignant tumors known as histiocytomas or fibrous xanthomas appears to be well established and is based on a combination of conventional light microscopy, tissue culture and ultrastructural studies. Our experience with 23 cases of primary malignant fibrous histiocytoma of bone is presented, together with a review of additional cases documented in the literature. Particular emphasis has been placed on the radiologic aspects of primary malignant fibrous histiocytoma of bone, together with a brief review of its clinical and histologic features. The fact that the skeleton can be involved de novo rather than by direct extension from neighboring soft tissue tumors or by metastatic spread is a relatively new concept.

Fibrous xanthomas of the brain: Report of two cases

Summary Two patients are presented with respectively a fibrous xanthoma and a xanthosarcoma of the brain. Four cases with these rare neoplasms of the brain were described before by Kepes and coworkers in 1973. The histological characteristics and differential diagnosis are discussed.

Fibrous Histiocytomas of the Orbit

Fibrous histiocytomas are a complex group of tumors, that feature cells resem­ bling fibroblasts and histiocytes and that ex­ hibit a distinctive but inconstant cellular ar­ rangement referred to as a (matted) or cartwheel pattern. This particu­ lar subgroup of soft tissue tumors was first identified and characterized by Stout and coworkers. Considerable confusion has en­ veloped these tumors as a result of their ar­ cane terminology—fibrous histiocytoma, xanthofibroma, fibrous xanthoma, xanthogranuloma, dermatofibroma, atypical fibrous xanthoma, atypical fibrous histiocytoma, and storiform fibrous xanthoma. Since they are essentially localized tumors, they should not be regarded as relatives of the systemic reticuloendothelioses and other more widespread histiocytic disorders.' The purpose of this communication is to report on the experience at the Edward S. Harkness Eye Institute with a group of relatively homogeneous or­ bital tumors that satisfy the criteria for fibrous histiocytoma.

Malignant fibrous xanthoma of retroperitoneum

Fibrous xanthomas of the retroperitoneum are rare. A case is reported. The most valuable diagnostic procedure is tissue-culture technique where the neoplastic histiocytic origins of these tumors can be confirmed.

Retroperitoneal xanthogranuloma and xanthosarcoma (malignant fibrous xanthoma).

A case of retroperitoneal xanthosarcoma (malignant fibrous xanthoma, malignant variant of xanthogranuloma) is described. In addition to the typical histologic features of retroperitoneal xanthogranuloma, this tumor showed obvious pleomorphism and mitotic activity of the proliferating histiocytes. The patient developed visceral metastases and died. The ultrastructural studies confirmed the presence of histiocytic-type cells. A review of cases diagnosed as retroperitoneal xanthogranuloma, including cases of malignant fibrous xanthoma, indicates an extremely poor prognosis for the histologically malignant variant. The usual histologic variant has an unpredictable behavior, some patients remaining well following local excision or even totally inadequate therapy while others have died following complete removal of the tumor.

Fibrous xanthomas and xanthosarcomas of the meninges and the brain.

Three cases of intracranial fibrous xanthomas and a case of multicentric cerebral xanthosarcoma are reported. All three fibrous xanthomas developed in the temporal area of boys in their early teens, one was within the leptomeninges (without dural attachment), the other two involved meninges and the superficial portions of the temporal lobe itself. These tumors were characterized by mono- and multinucleated cells with morphological features of histiocytes, Touton type giant cells and a “storiform pattern” in areas of spindleshaped tumor cells. Because of cellular atypism, giant cells and mitotic figures such tumors may suggest the diagnosis of glioblastoma multiforme but the absence of glial fibers, negative Cajal impregnation, presence of reticulin fibers in close proximity to tumor cells and the morphological similarity to the bizarre cells found in atypical xanthofibromas of the skin and soft tissues help to establish the diagnosis. Since the menigeal forms are probably derived from local meningeal mesenchyme, occasional abortive whorls and pseudopsammoma bodies may be encountered, the overall picture, however, is very different from meningiomas. Two patients had a 2.5 and a 12 year long symptomfree survival, respectively. The third boy had a local recurrence 14 months after initial removal which was excised and the patient is presently doing well. The xanthosarcoma first developed in the right frontal lobe of a 26 year old woman. This tumor was almost exclusively made up of various sized anaplastic cells filled with birefringent lipids. It is suggested that this tumor which had a diffuse network of reticulin, had originated from primitive adventitial cells. It was histologically more malignant than the first three and the patient died within a year after removal of the frontal lobe tumor, from a second mass in the cerebellum. The relationship of this tumor to glioblastomas and to other types of giant cell sarcomas is discussed.

Intra- and extraosseous malignant histiocytoma (malignant fibrous xanthoma).

Twenty cases of malignant histiocytoma with skeletal involvement were analyzed, and the clinical, radiological, and pathological findings in this entity were investigated, with emphasis on lesions which primarily or secondarily affect bone. These studies showed that malignant histiocytoma or fibrous xanthoma, although known by a confusing variety of labels, is a neoplastic entity of histiocytic origin distinct from the histiocytosis “X” family of lesions and the classic xanthomatoses.

Fibrous xanthoma of the larynx.

The fibrous xanthoma is a somewhat unusual tumor which has never before been reported in the larynx. A fibrous xanthoma involving the laryngeal surface of the epiglottis was excised with a supraglottic laryngectomy and neck dissection, and the patient has done well. Although only approximately 1% of these tumors prove to be malignant by metastasizing, there are no reliable histologic criteria for determining whether this tumor will behave in a malignant manner. Since these tumors recur locally if not adequately excised, the treatment of choice is adequate excision, including a margin of normal tissue.

Fibroxanthosarcoma of the soft tissues. A type of malignant fibrous histiocytoma.

The clinical and pathologic features of 30 soft tissue tumors which have a storiform fibrous stroma and bizarre histiocytes and giant cells are reviewed. Because of the resemblance of these lesions to the fibrous histiocytomas and because three of the tumors metastasized, they have been designated as fibroxanthosarcomas. Local recurrence was also common. Fibroxanthosarcoma occurs in many areas of the soft tissues but most commonly in the extremities. Histologically, it resembles storiform fibrous xanthoma (dermatofibrosarcoma protuberans) but can be separated from this entity by the presence of anaplastic bizarre giant cells and numerous mitoses. A proposed nomenclature for the fibrous histiocytomas is also presented. It is concluded that fibroxanthosarcoma is a distinctive malignant fibrous histiocytoma with a bizarre histologic pattern which can be distinguished from other soft tissue sarcomas and from the other fibrous histiocytomas.

Conjunctival fibrous xanthoma.

OPHTHALMOLOGISTS generally are aware of the variety of ocular conditions associated with disturbances of fat metabolism. The ocular structures may be affected by fat storage reactions (following trauma or infection), the lipidoses, or may be involved in association with elevated serum lipids (or with normal serum lipids). Tumors composed of fibrous tissue and histiocytes are variously referred to as histiocytomas, xanthomas, or xanthogranulomas. When histiocytes are associated with more abundant amounts of fibrous tissue, the term fibrous xanthoma may be applied. This paper describes a patient with a solitary, apparently benign fibrous xanthoma of the bulbar conjunctiva. To the best of our knowledge, this represents the third case of fibrous xanthoma of the conjunctiva to be reported in the literature. 1 Report of a Case The patient was a 67-year-old white man who was admitted to the Vanderbilt University Medical Center on March 21, 1967, for excision of a conjunctival

The histiocyte in radiology; with case reports of retroperitoneal xanthogranuloma and malignant fibrous xanthoma.

The histiocyte is derived from the reticulum cell and is a member of the reticuloendothelial or reticulohistiocytic system. It is capable of enzymatic production, phagocytosis of bacteria, lipids, erythrocytes, cellular and foreign debris. It is also a faculative fibroblast.It participates in systemic diseases such as metabolic histiocytosis, malignant histiocytosis, and histiocytosis-X.It is also the principal cell in many granulomatous diseases including endogenous lipid pneumonia of lung and xanthogranulomatous pyelonephritis of kidney. Malacoplakia also appears to be a type of histiocytic granuloma.Localized neoplastic or reactive tumor of skin, subcutaneous tissue, lung, mediastinum, retroperitoneum, synovium, bursa and tendon sheath have been variously termed histiocytoma, fibrous xanthoma, xanthogranuloma, xanthofibroma, giant cell tumor, pigmented villonodular synovitis, bursitis and tenosynovitis. A very small percentage of these apparently benign lesions are capable of metastasizing.

Fibrous xanthoma of synovium (gaint-cell tumor of tendon sheath, pigmented nodular synovitis. A study of one hundred and eighteen cases

Fibrous xanthoma of synovium (gaint-cell tumor of tendon sheath, pigmented nodular synovitis. A study of one hundred and eighteen cases

Soft-tissue tumors, with special reference to those o hisiocytic origin

Tumors of the soft tissues are very various; they reproduce only partly the characteristics of the tissues of origin and present many modulations due principally to the stage of differentiation. The definition of these lesions must be precise as the different groups have quite unlike behavior and require an appropriate treatment. They are usually classified as tumor-like lesions, benign neoplasms, malignant neoplasms. This classification, however, is not sufficient for a precise prognostic and theraphy as there are behaviour anomalies, such as tumors which grow by infiltration and do not metastasize and encapsulated tumors which develop metastases. For instance, every mesenchymal tumor grows into the skin by infiltration but only few of them produce metastasis; some fibromatosis are true neoplasms which may display insidious infiltrative growth and frequently recurrence appears after the removal but there are no metastases. Sarcomas of varying histological types may have a different behavior requiring a different form of treatment. New knowledge on tumors of the soft tissues is due essentially to the combination of research by «in vitro» culture and clinical-pathological studies. The demonstration of the histogenesis of some tumors allowed the reclassification of fibrous growth deriving from facultative fibroblastic cells. These tumors are composed of fibroblasts with a xanthomatous component, giant cells and phagocytic foam cells: histogenetically they are histiocytic fibroblasts. Istiocytes may behave as fibroblasts or fagocytes and sometimes they assume the two appearances in a same tumor. Among these tumors the so called protuberans fibrosarcomas, the sclerosing hemangiomas, many giant cell tumors, and the fibrous xanthomas must be considered as histiocytic tumors or fibrous histiocytomas. The malignant counterpart of fibrohistiocytomas, that is the malignant xanthofibroma, the malignant histiocytoma and the rare malignant protuberans fibrosarcoma, should be recognized.