Juvenile xanthogranuloma: Forms of systemic disease and their clinical implications

Freyer, David R.; Kennedy, Rosemarie; Bostrom, Bruce; Kohut, Gloria; Dehner, Louis P.

doi:10.1016/s0022-3476(96)70247-0

Cited by 269 publications

(257 citation statements)

References 38 publications

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“…[9][10][11][12] Cases of juvenile xanthogranulomas (JXG), which are derived from dermal dendrocytes and benign fibrous histiocytomas (BFH), tumors composed of fibroblastic and histiocytic cells, have been described in the central nervous system. 13,14 A careful review of the literature revealed no similar cases of supratentorial dural-based dendritic cell histiocytoma other than 12 such lesions reported briefly by Jaffe and Favara in abstract form. 15 In 4 of those patients, the lesion involved dura and/or adjacent bone.…”

Section: Discussionmentioning

confidence: 95%

Radiologic Findings in a Patient with Frontal Parafalcine Dendritic Cell Histiocytoma

Abi‐Ghanem¹,

Uzuner

Askin³

et al. 2007

American Journal of Neuroradiology

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SUMMARY:We report the case history and radiologic findings of a patient with a biopsy-proven dendritic cell histiocytoma presenting as a single intracranial extra-axial mass and no systemic disease. Even though this entity is relatively rare, it should nevertheless be considered in the differential diagnosis of dural-based space-occupying central nervous system lesions.A 10-year-old African American girl presented with a 1-year history of headache worsening over the last 6 months and associated with vomiting for 2 weeks. She denied any constitutional symptoms, and her neurologic examination was unremarkable. A brain MR imaging study showed a 3.3 ϫ 2.9 ϫ 2.9 cm, minimally lobulated, enhancing extra-axial mass centered in the right parafalcine location with minimal extension across the falx associated with leftward midline shift ( Fig 1B). There was significant edema of the frontal lobes with T2/fluid-attenuated inversion recovery hyperintensity of the white matter bilaterally (Fig 1A). 2D timeof-flight intracranial MR venogram showed focal obstruction of the anterior aspect of the superior sagittal sinus and collateral drainage through a left parasagittal vein. She subsequently underwent a subtotal surgical resection, because the danger of bleeding from cortical veins made total tumor removal unacceptably risky. Frozen sections were inconclusive, but final pathology revealed a dendritic cell histiocytoma. The tumor cells (Fig 2) were large, with a glassy, pink cytoplasm and prominent single nucleoli. The mitotic activity was low, and necrosis was absent. Numerous markers for epithelial, central nervous system, or lymphoid lineage and for melanoma were negative. Immunostains for CD68, factor XIIIa (macrophage and histiocyte markers), were positive, but CD1a and S100 immunostains were negative. The tumor was identified as a "non-Langerhans cell" dendritic cell histiocytoma.The patient's headaches subsided postoperatively. Neurologic examination remained normal after her operation. Her postsurgical imaging revealed some residual left parafalcine tumor adjacent to the superior sagittal sinus with surrounding vasogenic edema. The midline shift had resolved (Fig 1C). The patient was placed on steroid therapy and pain medication for headaches due to postoperative edema. She completed 14 sessions of radiation therapy. Since that time, she has been on steroid therapy with pain medication with no improvement of her headaches. Follow-up MR studies have demonstrated a slight decrease in the size of tumor with decreasing surrounding edema.

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Section: Discussionmentioning

confidence: 95%

Radiologic Findings in a Patient with Frontal Parafalcine Dendritic Cell Histiocytoma

Abi‐Ghanem¹,

Uzuner

Askin³

et al. 2007

American Journal of Neuroradiology

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“…It most commonly presents as a solitary cutaneous mass, but rarely may be systemic, affecting the central nervous system, liver, spleen, deep soft tissues and/or the hematopoietic system, sometimes with a lethal outcome (Freyer et al 1996). JXG in the larynx is quite rare, and our search of the English literature revealed only 5 such cases (Table 1), in which JXG was localized at the aryepiglottic fold (Benjamin et al 1995;Wang et al 2010) or the subglottis (Thevasagayam et al 2001;Sahhar et al 2003;Somorai et al 2007).…”

Section: Discussionmentioning

confidence: 95%

“…In contrast to LCH, the prognosis of solitary JXG is favorable and spontaneous regression may occur, despite rare cases of relapse (Freyer et al 1996;Somorai et al 2007). LCH in children over the age of 5 years tends to appear as an isolated lesion and most of such cases are benign with spontaneous regression.…”

Section: Discussionmentioning

confidence: 97%

“…Among histiocytic disorders, making a distinction between JXG and Langerhans cell histiocytosis (LCH) is often important clinically because the prognosis of LCH is worse than that of JXG, and this can usually be accomplished reliably on the basis of histology especially by immunohistochemistry using histiocytic cell markers (Somorai et al 2007). JXG appears in children under 2 years old and shows a tendency to enlarge locally in the early period of the disease (Freyer et al 1996). Among tumors affecting the larynx, JXG is quite rare, and only five cases have been reported in the English literature (Benjamin et al 1995;Thevasagayam et al 2001;Sahhar et al 2003;Somorai et al 2007;Wang et al 2010).…”

Section: Introductionmentioning

confidence: 99%

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Isolated Juvenile Xanthogranuloma in the Larynx of a Three-Year-Old Child

Kawamoto

Katori

Honkura

et al. 2013

Tohoku J. Exp. Med.

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Juvenile xanthogranuloma (JXG) is a benign manifestation of non-Langerhans cell histiocytosis characterized by yellowish cutaneous nodules. Its occurrence in the larynx is very rare, but laryngeal JXG may cause severe respiratory distress. We report a patient with isolated laryngeal JXG treated by laryngomicrosurgery, and this is the first report of JXG extending to vocal fold. A 3-year-old girl presented with hoarseness and inspiration stridor. A bulky tumor was found in right glottic to subglottic region. Subtotal resection of the tumor was carried out by laryngomicrosurgery, and airway distress was diminished after the operation. In pathological examination, the resected specimen showed proliferation of histiocytic cells and spindle cells with Touton giant cells that are characterized by polynuclei or wreath nuclei and are known to appear in JXG but not in LCH. Immunohistochemistry of histiocytic cell markers demonstrated positivity for CD68, lysozyme, alpha1-anti-chymotrypsin, factor XIIIa and vimentin, and negativity for CD1a and S-100, leading to diagnosis of JXG, but not LCH. The patient was thus expected with benign prognosis, and additional resection of the tumor including vocal fold was not indicated in the initial treatment. Six weeks later, the JXG recurred and a second procedure using CO 2 laser was needed. The tumor did not re-grow thereafter, and there was no residual voice handicap. Because of its favorable prognosis and tendency for spontaneous regression, JXG in the larynx needs to be considered carefully with regard to whether reduction surgery and/or tracheotomy are necessary, and thus precise diagnosis is required.

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“…Etiopathogenesis is poorly understood. The rarity of the disease has led to the paucity of literature regarding the etio-pathogenesis of the disease [10]. Juvenile xanthogranuloma (JXG) presents as single skin lesion or multiple skin lesions [10].…”

Section: Discussionmentioning

confidence: 99%

Intra-abdominal Xanthogranulomatoses Secondary to Postoperative Bile Leak

Gohel¹

2015

J Cytol Histol

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Xanthogranuloma is one of the 2 disorders of non-Langerhan cell histocytoses. It is a benign tumor histologically characterized by foamy histiocytes, numerous Touton giant cells and rare eosinophils. The histology differs from Langerhan cell histocytosis disorders in which convoluted histocytes, numerous eosinophils and rare Touton cells are seen. Grossly, the lesion appears as golden yellow papules or nodules. Electron microscope characteristics include presence of cytoplasmic lipid. The etio-pathogenesis is not fully understood, however granulomatous reaction of histiocytes to an unknown stimulus is postulated. Here, we discuss a rare presentation of peritoneal xanthogranulomatoses in a patient caused due to postoperative bile leak.

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Juvenile xanthogranuloma: Forms of systemic disease and their clinical implications

Cited by 269 publications

References 38 publications

Radiologic Findings in a Patient with Frontal Parafalcine Dendritic Cell Histiocytoma

Radiologic Findings in a Patient with Frontal Parafalcine Dendritic Cell Histiocytoma

Isolated Juvenile Xanthogranuloma in the Larynx of a Three-Year-Old Child

Intra-abdominal Xanthogranulomatoses Secondary to Postoperative Bile Leak

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