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Sarangarajan Ranganathan
The study of Histiocytic lesions has been a passion of Pepper Dehner over the years. He has contributed several case series and reviews on various categories of these diseases for over 4 decades, with his earliest articles in the 1970s. He has written on all aspects of the disease including seminal articles on Langerhans cell histiocytosis (LCH) and their prognostic features, his experiences with regressing atypical histiocytosis, his encounters with malignant histiocytosis, and classic articles on juvenile xanthogranuloma...
September 1, 2016: Seminars in Diagnostic Pathology
Alpana Singh, Garima Vats, A G Radhika, Pragati Meena, Gita Radhakrisnan
Xanthogranuloma is a non-neoplastic presentation of chronic inflammation commonly seen in gallbladder, kidney and rarely seen in genital organs. Only one case has been reported in cervix. Here, we report a case of 60-year-old postmenopausal lady who presented with history of fever and purulent discharge per-vaginum. On speculum examination, cervix had an ulcer extending from 3 to 5 o'clock position. Uterus was bulky. On probing the ulcer, a 1-cm deep sinus was identified. Ultrasound showed enlarged uterus and fluid collection suggestive of pyometra...
September 2016: Obstetrics & Gynecology Science
Daniel Vargas, J Caleb Richards, Daniel Ocazionez, Arlene Sirajuddin, Lorna Browne, Carlos S Restrepo
OBJECTIVE: The objectives of this article are: (1) to review common and rare manifestations of systemic and pulmonary Langerhans cell histiocytosis, Rosai-Dorfman disease, Erdheim-Chester disease and juvenile xanthogranuloma; (2) to provide the reader with important pathologic, epidemiologic and clinical features of these diseases. CONCLUSION: The histiocytoses are a diverse group of diseases which typically manifest with multiorgan involvement. Understanding the pathologic, epidemiologic, and clinical features of these entities can help the radiologist suggest an accurate diagnosis of histiocytosis when typical imaging features are encountered...
September 7, 2016: British Journal of Radiology
Ioannis Petrakakis, Ariyan Pirayesh, Joachim K Krauss, Peter Raab, Christian Hartmann, Makoto Nakamura
OBJECTIVE: Apart from the "common" lesions (e.g. pituitary adenomas, Rathke's cleft cysts, meningiomas and craniopharyngiomas), there is a plethora of rare tumors or tumor-mimicking lesions in the sellar and suprasellar region (SSR). Due to a lack of characteristic imaging features, there is a dilemma in distinguishing these rare lesions from the more "common" ones preoperatively. Consequently, both diagnosis and definition of surgical goals, as well as further treatment strategies continue to be challenging for all attending physicians...
October 2016: Clinical Neurology and Neurosurgery
Anne Boyd, Jesse Newman, Lucie Turcotte, Ingrid C Polcari
No abstract text is available yet for this article.
October 2016: Journal of Pediatrics
Jonathan Nadjiri, Klaus Woertler, Katja Specht, Norbert Harrasser, Andreas Toepfer
Erdheim-Chester disease (ECD) is a rare non-Langerhans cell histiocytosis with a broad spectrum of organ manifestations, presenting with characteristic radiological and histological findings. Soft tissue manifestations (xanthogranulomas) have been reported to be most commonly found in the region of the orbits. We report bilateral Achilles tendon xanthogramlomas in a 36-year-old male with biopsy-proven and B-RAF V600E-positive ECD. Although rare, ECD should be considered in the differential diagnosis of intratendinous masses...
October 2016: Skeletal Radiology
D Miguel, J Lukacs, T Illing, P Elsner
Necrobiotic xanthogranuloma (NXG) is an uncommon non-Langerhans cell histiocytosis involving skin and extracutaneous tissues. The lesions are usually asymptomatic and commonly appear in the periorbital area. Paraproteinemia is closely associated with NXG and its pathogenesis remains unclear. NXG prognosis is poor with several treatments showing variable results. Treatment of monoclonal gammopathy with alkylating agents does not necessarily influence the activity of the skin disease and vice versa. The aim of this systematic review is to summarize all reported treatments of necrobiotic xanthogranuloma of the skin, with or without underlying malignant condition and based on articles from the PubMed database using the query 'necrobiotic xanthogranuloma treatment', both in English and German, about 'human' subjects and published between 1980 and 2014, documenting adequate treatment for NXG...
July 19, 2016: Journal of the European Academy of Dermatology and Venereology: JEADV
Ken Matsubara, Harushi Mori, Nozomi Hirai, Kumi Yasukawa, Takafumi Honda, Jun-Ichi Takanashi
MRI in a 2-year-old female presenting afebrile seizures and left blepharoptosis revealed multiple well-marginated round-shaped lesions, isointensity to gray matter on T1- and T2-weighted images with homogenously reduced diffusion and diffuse contrast enhancement. MRS revealed elevation of taurine, choline and glutamate, and reduction of N-acetylaspartate. A brain biopsy confirmed a diagnosis of juvenile xanthogranuloma (JXG). JXG should be considered when MR spectroscopy shows elevated taurine and glutamate, which has only previously been reported in medulloblastomas...
November 2016: Brain & Development
M Klingner, G Hansel, J Schönlebe, U Wollina
Necrobiotic xanthogranuloma is a rare non-Langerhans-cell histiocytosis. A 62-year-old woman presented with yellowish erythematous plaques, nodules, and papules in the periorbital region and the extremities. She had a nodular tumor grown on the left upper lid that clinically resembled a keratoacanthoma. Histologically it was a xanthogranulomatous lesion. She suffered from monoclonal gammopathy of unknown significance of κ‑type. Treatment was realized with a combination of systemic dapsone/prednisolone and topical corticosteroids...
July 5, 2016: Der Hautarzt; Zeitschrift Für Dermatologie, Venerologie, und Verwandte Gebiete
Suvradeep Mitra, Saumya Gupta, Prema Menon, K L N Rao, Amanjit Bal
Juvenile xanthogranuloma (JXG) is a histiocytic disorder of childhood that mostly affects the skin although extracutaneous and visceral manifestations are well documented in English literature. The renal involvement is less commonly documented as a manifestation of xanthogranuloma and the reported cases occur in conjunction with cutaneous xanthogranuloma, especially in association with multiple skin lesions. We present a case of isolated renal JXG presenting as a renal mass with perinephric soft tissue and muscle involvement but without any cutaneous manifestation...
July 1, 2016: Fetal and Pediatric Pathology
Brian P Hibler, Kristin L Eastman, Daniel D Bennett, Andrew M Swanson, B Jack Longley, Gary S Wood
A 50-year-old woman presented to our clinic for evaluation of numerous recurrent, pruritic papules on her upper extremities. She reported a 2- to 3-year history of up to eight unique lesions on the bilateral upper arms that would initially appear as firm papules before gradually softening and flattening out, leaving residual pink macules (Figure 1A). Her medical history was notable for mild hyperlipidemia. On presentation, she had several erythematous papules with overlying telangiectasias scattered throughout her bilateral upper arms...
2016: Skinmed
Austen N Knapp, Wasim A Samara, Carol L Shields, Jerry A Shields, Ralph C Eagle
An 8-year-old healthy boy underwent surgery for excision of a painless, enlarging vascularized conjunctival tumor. Histopathology disclosed a mass comprised of interweaving spindle cells and scattered histiocytes in a fibrous matrix, consistent with benign fibrous histiocytoma. This rare tumor can resemble several conditions, including scleritis/episcleritis, inflamed pterygium, juvenile xanthogranuloma, foreign body granuloma, solitary fibrous tumor, amelanotic melanoma, and squamous cell carcinoma.
August 2016: Journal of AAPOS: the Official Publication of the American Association for Pediatric Ophthalmology and Strabismus
Larissa S Higgins, Ronald S Go, David Dingli, Shaji K Kumar, S Vincent Rajkumar, Angela Dispenzieri, Francis K Buadi, Martha Q Lacy, John A Lust, Prashant Kapoor, Nelson Leung, Yi Lin, Taxiarchis V Kourelis, Morie A Gertz, Robert A Kyle, Wilson I Gonsalves
INTRODUCTION: Necrobiotic xanthogranuloma (NXG) is a rare chronic granulomatous disorder of the skin associated with a monoclonal gammopathy. PATIENTS AND METHODS: The present report describes the findings from a single tertiary medical center retrospective study, including the clinical features of 35 patients with NXG and monoclonal gammopathy from 2000 to 2015 and their subsequent disease course and treatment response. The median age at diagnosis was 56 years (range, 26-88 years)...
August 2016: Clinical Lymphoma, Myeloma & Leukemia
Lorena Martínez-Leboráns, Altea Esteve-Martínez, Anna Agustí-Mejías, Isabel Febrer Bosch
No abstract text is available yet for this article.
May 13, 2016: Anales de Pediatría: Publicación Oficial de la Asociación Española de Pediatría (A.E.P.)
Baocheng Wang, Huiming Jin, Yang Zhao, Jie Ma
BACKGROUND: Juvenile xanthogranulomas (JXGs) are uncommon non-Langerhans cell histiocytic proliferations which occur most often in children. Rare cases of intracranial JXGs in children have been reported. The precise treatment strategy for intracranial JXG with high fatality is still unclear. METHOD: We present four cases of intracranial JXG with 2-6 years of follow-up. Review of the previous literature since 1980 revealed another 39 pediatric intracranial JXGs...
July 2016: Acta Neurochirurgica
Emily H Smith, Lori Lowe, Paul W Harms, Douglas R Fullen, May P Chan
BACKGROUND: Expression of p16 is frequently evaluated in melanocytic lesions. Expression of p16 in cutaneous histiocytic, fibrohistiocytic and undifferentiated lesions has not been well characterized. METHODS: We evaluated p16 expression in a cohort of histiocytic (reticulohistiocytoma, Langerhans cell histiocytosis, xanthogranuloma, Rosai Dorfman disease and xanthoma), fibrohistiocytic (dermatofibroma, epithelioid fibrous histiocytoma and dermatofibrosarcoma protuberans) and undifferentiated (atypical fibroxanthoma and pleomorphic undifferentiated sarcoma) lesions...
August 2016: Journal of Cutaneous Pathology
Christopher K H Burris, Maria E Rodriguez, Meisha L Raven, Cat N Burkat, Daniel M Albert
PURPOSE: The aim of this study was to report a case of Adult-Onset Asthma with Periocular Xanthogranulomas (AAPOX) associated with systemic IgG4-related disease (IgG4-RD). OBSERVATIONS: A 57-year-old man presented with bilateral periorbital swelling for 1 year. Histopathology of a left orbital biopsy showed fibro vascular connective tissue inundated with foamy, lipid-laden histiocytes and touton giant cells with lymphocytic inflammation. Additional stains revealed CD68 positivity, and S100 negativity...
April 2016: American Journal of Ophthalmology Case Reports
Hae-Eul Lee, Sue-Jeong Kim, Myung Im, Young Lee, Chang-Deok Kim, Jeung-Hoon Lee, Young-Joon Seo
No abstract text is available yet for this article.
April 2016: Annals of Dermatology
Bipasha Mukherjee, Neha Shrirao
No abstract text is available yet for this article.
May 2016: Annals of Allergy, Asthma & Immunology
Ujjawal Khurana, Uma Handa, Harsh Mohan
Granular cell tumors (GCTs) are uncommon neoplasms with recently postulated origin from Schwann cell or neural crest. It can appear in different parts of the body and is most commonly found in the tongue. The cutaneous presentation is not that uncommon. Fine needle aspiration cytology (FNAC) has been suggested to be the diagnostic modality of choice. It will help to differentiate benign tumors from malignant ones and to differentiate GCT from frequent misdiagnoses such as granular histiocytic reaction, xanthogranuloma, rhabdomyoma, oncocyte rich lesions, alveolar soft part sarcoma (ASPS), epithelioid sarcoma, and carcinoma...
January 2016: Journal of Cytology
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