Xanthoma Cells Research Articles

Esophageal verruciform xanthoma following radiotherapy

Verruciform xanthoma (VX) is an uncommon benign lesion of unclear etiology which has only been reported twice before in the esophagus. We describe a 70-year-old male who presented an exophytic esophageal lesion incidentally found upon endoscopy 2.7 years following radiation therapy for unresectable squamous cell carcinoma of the tracheal carina. Histologically, the lesion showed a papillary surface change with numerous foamy histiocytes within the lamina propia papillae. Xanthoma cells were strongly positive for vimentin and CD68 (KP1). Polymerase chain reaction did not demonstrate human papillomavirus (HPV) infection. Our results indicate that esophageal VX is not an HPV-induced lesion and suggest a causal relationship between VX and radiotherapy, as previously noted. Histological differential diagnosis is discussed and emphasis is placed on obtaining adequate biopsy material for accurate diagnosis.

Xanthogranuloma in Adolescence

Xanthogranuloma in Adolescence

Verruciform Xanthoma in an Adolescent

Verruciform xanthoma is a benign mucocutaneous, verrucous, papillary lesion characterized by large foam cells in the parakeratotic layer, lipid-laden macrophages (xanthoma cells), epidermal hyperplasia, and hyperkeratosis. Verruciform xanthoma is thought to be a reactive rather than a neoplastic process secondary to epithelial damage and the presence of foamy histiocytes. The human papillomavirus has not been proven to be a causative factor. Differential diagnoses include verrucous carcinoma, condyloma acuminatum, seborrheic keratosis, verruca simplex, and vulvar intraepithelial neoplasia. We describe the clinical and pathologic findings of a 16-year-old girl with verruciform xanthoma of the vulva, the third such reported case in an adolescent girl. It is important to recognize this rare entity because it can mimic many other conditions, and the usual treatment modalities for wartlike growths on the vulva (i.e., imiquimod, podophyllin, and trichloroacetic acid) are not effective. Wide local excision seems to be the only effective and curative treatment modality for verruciform xanthoma, as has been reported in the literature and is such with our case.

Verruciform xanthoma of buccal mucosa: A case report with review of literature

Verruciformxanthoma (VX) is an uncommon benign mucocutaneous lesion of unknown etiology. It appears as a papule or single plaque with verrucous or papillomatous surface and variable color from reddish pink to gray. It occurs primarily in the masticatory mucosa. Histologically, VX is characterized by the presence of parakeratinized epithelium with thin rete ridges and connective tissue papillae extending up to the surface. The papillae characteristically consist of foam cells, also called xanthoma cells. We report a case of VX in the buccal mucosa and discuss their clinical and histopathological findings.

Vulvar Verruciform Xanthoma

Verruciform xanthoma (VX) is a rare benign tumor that usually involves the oral cavity. Since the first report of this tumor in 1971, only 9 cases have been reported on the vulva, and 3 of these were associated with another vulvar condition. We describe the clinicopathologic features of 10 patients with vulvar VX and focus on their associated conditions. The mean age of the patients was 68 years (range, 51-80 years). The VX lesions were asymptomatic, yellowish-orange verrucous plaques. The diagnosis was clinically suspected in 2 cases; other suggested diagnoses were condyloma or squamous cell carcinoma. All of the patients had an associated vulvar condition: lichen sclerosus (6 patients), lichen planus (2 patients), Paget disease, or radiodermatitis. Under microscopy, the VX lesions displayed parakeratosis, acanthosis without atypia, and elongated rete ridges. Xanthomatous cells were aggregated in the papillary dermis. Vulvar VX is a benign tumor with misleading clinical features. All 10 cases were associated with a vulvar condition, mainly a lichen sclerosus. Therefore, VX might represent a reaction pattern induced by different conditions, mainly characterized by damage to the dermoepidermal junction. When confronted with the diagnosis of vulvar VX, clinicians may look for an associated vulvar condition.

Unclassified glioneuronal tumor with advanced lipidization

Lipidization is observed only occasionally in primary neuroectodermal tumors of the central nervous system. It may reflect lipomatous transformation of tumor cells into xanthomatous and/or adipocyte-like cells. We report a unique case of mixed glioneuronal tumor with marked lipomatous changes in a young patient with intractable epilepsy. MRI revealed a well-circumscribed lesion in the right temporal lobe. Histopathological findings showed the pleomorphic tumor with numerous cells containing large lipid droplets, resembling mature adipocytes, that were arranged in clusters or scattered within the neoplastic tissue. The tumor was composed of both glial and neuronal elements. Some tumor cells displayed features intermediate between glial and neuronal cells. The reticulin fibers were limited to blood vessels. Mitotic figures, vascular proliferation, and necrosis were absent, and MIB-1 labeling index was less than 1%. Diffuse immunoreactivity for GFAP and S100-protein was observed. In some heavily lipidized cells, the lipid droplets were surrounded by a cytoplasmic rim of GFAP immunoreactivity. Numerous cells exhibited immunostaining for NSE and synaptophysin. This is the first documented case of glioneuronal tumor with extensive lipomatous transformation, which might be considered as a heavily lipidized unclassified pleomorphic glioneuronal tumor or a variant of lipoganglioglioma with marked pleomorphism and severe lipidization.

Pigmented pleomorphic xanthoastrocytoma: A rare variant and literature review

A 16-year-old male teenager presented with seizure and loss of consciousness for 20 min. Magnetic resonance imaging demonstrated a mass occupying the right medial temporal lobe. Histological examination revealed a non-pigmented area with spindle-shaped and large xanthomatous pleomorphic cells and a pigmented region with pigmented neoplastic cells with fascicular arrangement. Immunohistochemical studies showed the tumor was positive for GFAP and low index of Ki-67. Considering the patient's history, clinical data and pathological findings, we rendered a rare variant named pigmented pleomorphic xanthoastrocytoma.

Pyélonéphrite xanthogranulomateuse diffuse du nourrisson

We report a case of a renal mass in a 4-month boy, which occured during the assessment of a pelvi-calyceal dilatation diagnosed at 23 weeks of gestational age. There was no history of urinary infection, fever or weight loss. Physical examination revealed a mass of the left flank with significant flank tenderness. Laboratory test showed a biological inflammatory syndrome and urine culture was negative. Investigations including ultrasound and computed tomography scan were suggestive of diffuse xanthogranulomatous pyelonephritis with a non-functioning left kidney. Left total nephrectomy was performed through a lumbar incision with an extraperitoneal approach. The kidney was enlarged with a dilated pelvis containing pus upstream of a proximal ureteral atretic segment. Pathological examination of the kidney confirmed the diagnosis of diffuse xanthogranulomatous pyelonephritis. The boy remains well at 1 year follow-up. Xanthogranulomatous pyelonephritis is very rare in infants. It is an uncommon severe progressive renal infection resulting in destruction of renal parenchyma, histologically replaced by xanthomatous cells and granulomatous reaction. Pathogenesis of xanthogranulomatous pyelonephritis remains unclear. But it is well known that urinary tract obstruction and renal lithiasis are determining factors. It can occur in variant clinical forms but its symptoms remain non-specific. Curative treatment consists in nephrectomy and definitive diagnosis is made on histological examination of the kidney. This diagnosis should be discussed when a renal mass occurs in a context of malformative uropathy and xanthogranulomatous pyelonephritis have to be included in the differential diagnosis of renal mass in infants and children.

Primary xanthofibroma in the calcaneus: a case report

Xanthoma or xanthofibroma is a lesion, characterized by foamy histiocytes (xanthoma cell) and is mostly seen in soft tissue. Xanthoma may also occur in in the skeletal system of patients with an abnormal lipid metabolism. We present a 22-year-old man with primary xanthofibroma in the calcaneus, who was treated by curettage and grafting of the lesion.

Benign Fibrous Histiocytoma of the Lower Lip

Benign fibrous histiocytoma (BFH) is one of the most common tumors of the superficial and deep soft tissues; it is commonly localized on the skin of the extremities and presents as a slow growing solitary nodule, made up of a mixture of fibroblastic and histiocytic cells. To our knowledge, involvement of the soft tissue of the oral cavity is rare. We present a case of BFH of the lower lip in a 61-year-old white woman who presented with a painless, slow-growing lump inside the lower lip. The lesion was surgically removed. Histologically, it was characterized by uniform spindle-shaped cells arranged in a prominent whorled or storiform pattern. Scattered xanthomatous cells, multinucleated giants cells, lymphocytes, and deposits of hemosiderin were noted throughout the lesion. This rare tumor should be considered in the differential diagnosis of the oral soft-tissue neoplasms.

Letter to the Editor: Fibroxanthoma: A Complication of a Biodegradable Screw

To the Editor: A recent case report in Clinical Orthopaedics and Related Research used the misleading term “fibroxanthoma” to describe the histologic findings of a biopsy obtained near the site of a previously inserted resorbable polymer screw [7]. Among the polymers currently used in degradable orthopaedic devices are polyglycolic acid (PGA), polylactic acid (PLA), poly-DL-lactide (PDLA), and poly-DL-lactide-co-glycolide (PLGA). The main degradative mechanism for these polymers is hydrolysis [9], a process that eventually releases the base monomers, such as lactic acid or glycolic acid that ultimately are metabolized into carbon dioxide and water. The rate of dissolution of a degradable polymer can be influenced by many factors, including the polymer composition and crystallinity, size of the device, additives, method of sterilization, local pH, and local mechanical factors [8]. The dissolution process leads to reduced polymer chain length, and loss of mechanical strength. If the device breaks into pieces, the larger fragments are relatively inert, but smaller fragments are phagocytosed by macrophages and giant cells. The highest tissue concentration of macrophages and giant cells correlates with the dissolution kinetics of the polymer [4, 6]. The process of biologic resorption is asymptomatic in most patients, but if the degrading polymer is in a subcutaneous location, then the edema and inflammatory reaction can create a mass that will contain macrophages and giant cells [2]; special techniques may be needed to observe fragments of residual polymer. Some cases of tunnel widening and bone resorption also have been associated with degrading polymer screws [3], although this is nonspecific as it also has been reported associated with metal screws [1]. The term “fibroxanthoma” is rarely used nowadays, but usually is considered to be either a synonym for a benign fibrous histiocytoma, or to represent a subset of benign fibrous histiocytomas that contain prominent xanthomatous cells (“foamy” histiocytes) [5]. A fibroxanthoma (ie, benign fibrous histiocytoma) is a mass that, by definition, is not associated with a foreign-body reaction. In the case report, Sadat-Ali and coauthors [7] describe a patient who had a soft tissue swelling develop at the site of the tibial tunnel, 3 years after a biodegradable screw had been used for ACL reconstruction. A biopsy “was sent for histopathologic examination, which resulted in a diagnosis of fibroxanthoma” [7]. Not surprisingly, the cells showed positive staining with CD-68, a marker for macrophages. Correct diagnosis of bone and soft tissue lesions often requires correlation between clinical and histologic findings, and in the context of this patient, the biopsy should not be interpreted as a fibroxanthoma any more than the similar macrophage reaction to a resorbable suture would be considered a fibroxanthoma. Whether this process is viewed as an inflammatory complication, or an expected, transient foreign-body reaction depends on the presence or absence of symptoms [2]. Although the process is asymptomatic and resolves without treatment in most patients, a symptomatic lesion could be associated with screw degradation, and debridement of the screw fragments is expected to be curative.

Symptomatic intraventricular xanthogranulomas

Abstract This article reviews symptomatic intraventricular xanthogranulomas, based on a case presentation. Bilateral xanthogranulomas of the choroid plexus were removed surgically from the lateral ventricles of a 12-year-old boy. At 9 years of age, he had evidence of increased intracranial pressure and was hospitalized. Dense enhancing masses were detected in computerized tomogram (CT) brain scan. The lesions were in the region of trigones with extension into the temporal horns and into the right occipital horn. The masses were brightly yellow and greasy. They measured 8.5 x 5.5 x 3.5 cm and 10 x 6.5 x 4.5 cm, respectively, and proved to be xanthogranulomas. Review of 35 reported symptomatic intraventricular xanthogranulomas revealed 11 lesions in the lateral ventricles in which six of them were bilateral. Twenty-two lesions were in the third ventricle, and two lesions were in the fourth ventricle. The lesion shows no significant sexual predilection. The patients’ average age is 37.6 years for males, 32.4 years for females, and 34.3 years for both sexes. The size of symptomatic lesions ranged from 1 to 3 cm in diameter but a few were large, up to 8 to 10 cm. The origin of foamy (xanthoma) cells in the xanthogranulomas arising in the choroid plexus is thought to be multicentric including the choroidal epithelium and stromal arachnoidal cells that have undergone xanthomatous changes. Increased intracranial pressure is the significant clinical feature of the intraventricular xanthogranulomas as in other mass lesions within the skull. Surgical extirpation is the treatment of choice if the lesion is accessible and the patient’s general condition is suitable.

Post-traumatic chylous knee effusion

Chylous joint effusion is a rare condition in which synovial fluids containing large amounts of lipids take on a milky appearance as a result. We report on a 19-year-old male patient with posttraumatic chylous knee effusion. Several days after striking his knee against the ground because of a traffic accident, his left knee showed obvious swelling. Aspiration of his knee was performed, yielding 70 ml of purulent-appearing fluid. To distinguish this condition from purulent or tuberculosis arthritis, arthroscopic biopsy and debridement were performed. Arthroscopic examination visualized distinctive yellow-white soft lesions covering much of the joint capsule, resembling a cobweb. Tissue cultures for bacteria were negative. Pathologically, we identified clusters of xanthoma cells with fibrin exudation due to disruption of the synovium and intra-articular fat pad necrosis. Centrifuging the aspiration fluid yielded a thick creamy lipid layer as the supernatant. A fresh drop preparation showed that the specimen contained innumerable fat globules, which stained red with oil red O stain. The patient was able to walk without difficulty or further swelling of his knee at the end of the second postoperative week. Posttraumatic chylous effusion is self-limited. Purulent arthritis or tuberculosis arthritis, however, should still be the presumptive diagnosis in such cases. Arthroscopic irrigation and debridement should be considered for these traumatic cases to confirm diagnosis and to speed up recovery.

Diagnostic Difficulties in Diffuse Xanthogranulomatous Pyelonephritis: A Case Report and Review of the Literature

Xanthogranulomatous pyelonephritis is a rare, severe, and chronic infection of the kidney characterized by the replacement of renal parenchyma by the xanthoma cells which is difficult to diagnose radiologically. We report a 40-year-old male who had vague abdominal mass and fever for more than 2 months. Weight loss and fatigue were the associated symptoms. Ultrasonography revealed a grossly enlarged right kidney with replacement of renal parenchyma by multiple hypoechoic areas with perinephric thickening and a large staghorn calculus. An abdominal computed tomogram demonstrated an irregular, enlarged right kidney with multiple low-density round areas and calculi. Diminished excretion of contrast media and a severe perinephric inflammatory reaction were present. A presurgical diagnosis of xanthogranulomatous pyelonephritis was made. First, drainage of the extrarenal abscess under proper antibiotic coverage was done and later, a two stage nephrectomy was done considering the debilitating condition of the patient.

Clinicopathological study of diffuse panbronchiolitis--with special reference to relationship between the clinical duration and respiratory bronchiolar lesions

Fifteen autopsy cases of diffuse panbronchiolitis, with clinical disease duration ranging from 2 months to 56 years since appearance of cough and sputum, were analyzed by reconstruction study using serial sections, with particular regard to the respiratory bronchiolar lesions and clinical duration. In addition, proximal bronchi and bronchioles were examined focusing on dilatation, round cell infiltration and goblet cell metaplasia, and peribronchiolar emphysematous lesions were graded according to the degree of alveolar destruction. Morphological changes of the respiratory bronchioles were classified into five types. Active lesions, with lymphoplasmocytic and xanthoma cell infiltration were classified into three types according to the presence and the size of intraluminal granulation tissue. Scar lesions showing marked fibrohyalinous change were classified into two types, those with and without respiratory bronchiolar stenosis. Active lesions decreased and scar lesions increased with clinical duration. Respiratory bronchiolar lesions were distributed almost equally from the upper lobe to the lower lobe. In these fifteen autopsy cases, the total number of respiratory bronchiolar lesions did not vary with disease duration. Emphysematous lesions increased with clinical duration, and showed a relationship to the presence of scar lesions with stenosis. Bronchiolar dilatation was prominent in cases with a long clinical course. Segmental and subsegmental bronchial dilatation were recognized mainly in the middle, lingula and lower lobes, but there was no relationship between clinical duration and bronchiolar dilatation.

Xanthomatous Hypophysitis Mimicking a Pituitary Adenoma: Case Report and Review of the Literature

Background. Hypophysitis is an inflammatory disease of the pituitary gland that may mimic pituitary tumors clinically and radiologically. Case Description. We report a case of a xanthomatous hypophysitis initially diagnosed as pituitary adenoma. A 31-year-old woman presented with headache, diabetes insipidus, and amenorrhea. A head CT scan showed no intrasellar changes, while an MRI scan showed a sellar cystic mass. An endocrinological work up revealed mild hypocortisolism and diabetes insipidus (DI). Transsphenoidal surgery was performed. The intraoperative histological examination suggested a pituitary adenoma. The removed tissue showed central necrosis surrounded by accumulation of foamy cells and xanthomatous epithelioid cells. The patient made an uneventful postoperative recovery, Nevertheless, DI persisted and the adenohypophysis hypofunction did not recover. Conclusion. We describe an unusual inflammatory lesion of the pituitary gland mimicking an adenoma. A high level of clinical suspicion of inflammatory disorders is necessary for correct diagnosis and optimal management.

Adult orbital xanthogranulomatous disease: review of the literature.

This article provides an overview of the pathologic features of adult orbital xanthogranulomatous disease, a rare heterogeneous group of disorders that includes 4 clinical syndromes: adult-onset xanthogranuloma, necrobiotic xanthogranuloma, adult-onset asthma and periocular xanthogranuloma, and Erdheim-Chester disease. The diagnosis is made by biopsy of the lesion, demonstrating tissue infiltration by the hallmarks of xanthoma cells and Touton giant cells. The differential diagnosis is broad, including syndromes within the adult xanthogranulomatous disease category as well as other entities involving the eyelid and the orbital tissues. Because of its rarity and sometimes close similarity to other disease entities, it is often misdiagnosed initially. This article focuses on the morphology and immunohistochemical patterns in diagnosis of adult orbital xanthogranulomatous disease with emphasis on adult-onset asthma and periocular xanthogranuloma in particular, its clinical features and associated systemic manifestations in differential diagnosis, as well as the current management strategy.

Adult Orbital Xanthogranulomatous Disease: Review of the Literature

This article provides an overview of the pathologic features of adult orbital xanthogranulomatous disease, a rare heterogeneous group of disorders that includes 4 clinical syndromes: adult-onset xanthogranuloma, necrobiotic xanthogranuloma, adult-onset asthma and periocular xanthogranuloma, and Erdheim-Chester disease. The diagnosis is made by biopsy of the lesion, demonstrating tissue infiltration by the hallmarks of xanthoma cells and Touton giant cells. The differential diagnosis is broad, including syndromes within the adult xanthogranulomatous disease category as well as other entities involving the eyelid and the orbital tissues. Because of its rarity and sometimes close similarity to other disease entities, it is often misdiagnosed initially. This article focuses on the morphology and immunohistochemical patterns in diagnosis of adult orbital xanthogranulomatous disease with emphasis on adult-onset asthma and periocular xanthogranuloma in particular, its clinical features and associated systemic manifestations in differential diagnosis, as well as the current management strategy.

Flat type verruciform xanthoma of the tongue and its differential diagnosis

We present herein a case of oral verruciform xanthoma (VX) in order to discuss this relatively rare entity in light of current information. A 38-year-old woman, non-smoker, presented with a lesion at the left ventral surface of the tongue. The lesion is characterized by a mild epithelial proliferation and sub-epithelial accumulation of foam cells, which were positive for CD68, and negative for CD1a and S-100 protein. Human papillomavirus (HPV) DNA typing for low, intermediate, and high-risk groups was also performed and no etiological link between HPV and this lesion was found. The past medical history of the patient was unremarkable and further investigations did not reveal any biochemical abnormalities or systemic disease. Verruciform xanthoma is an uncommon benign lesion of undetermined etiology. It is a superficial normolipemic xanthoma, probably reflecting a multifactorial reactive and dystrophic process unrelated to HPV. It is noteworthy that in a small and superficial biopsy, xanthoma cells may be scanty and can be missed, especially if the pathologist is unfamiliar with the existence of this uncommon lesion. Its clinical and pathological recognition and correct diagnosis is critical because VX can occur in conjunction with other systemic and cutaneous inflammatory diseases; therefore, it necessitates further clinical assessment.

Surgically Discovered Xanthogranulomatous Pyelonephritis Invading Inferior Vena Cava with Coexisting Renal Cell Carcinoma

Xanthogranulomatous pyelonephritis (XGP) is a chronic inflammatory process that results in replacement of renal and/or perirenal tissue with a diffuse infiltrate of inflammatory cells referred to as xanthoma cells. We present a case of a 49-year-old man with an incidentally discovered renal mass with inferior vena cava (IVC) thrombus, who was found intraoperatively to have a significant inflammatory process involving the posterior wall of his IVC and right renal vein consistent with XGP surrounding a focus of clear cell renal cell carcinoma in the midportion of his right kidney.