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Paper 2
Published in Amanda Rabone, Benedict Thomson, Nicky Dineen, Vincent Helyar, Aidan Shaw, The Final FRCR, 2020
Amanda Rabone, Benedict Thomson, Nicky Dineen, Vincent Helyar, Aidan Shaw
Xanthogranulomatous cholecystitis, although uncommon, can look very similar to gallbladder cancer. It is an inflammatory condition characterised by multiple intramural nodules within a thickened gallbladder wall. All the other options in the question can be seen in both xanthogranulomatous cholecystitis and gallbladder cancer. The presence of hypoattenuating mural nodules is more suggestive of xanthogranulomatous cholecystitis. Given the similarities in the radiological features, a cholecystectomy is usually performed.
Endometrial inflammation
Published in T. Yee Khong, Annie N. Y. Cheung, Wenxin Zheng, Richard Wing-Cheuk Wong, Hao Chen, Diagnostic Endometrial Pathology, 2019
T. Yee Khong, Annie N. Y. Cheung, Wenxin Zheng
Also referred to as histiocytic endometritis, this is a rare variant of endometritis with some 21 cases documented in the literature. Most women are postmenopausal. It may mimic malignancy clinically and radiologically.15 On macroscopic examination, the endometrium is thickened and may have a friable yellow appearance. Histologically, there is a prominent stromal infiltrate of macrophages with regular small nuclei and abundant foamy cytoplasm (Figure 4.15). The foamy macrophages are admixed with chronic inflammatory cells and occasional multinucleate giant cells. In addition, there may be cholesterol clefts, endometrial necrosis and focal calcification. It is sometimes confused with metastatic adenocarcinoma, but the lack of immunoreactivity for epithelial cytokeratins and strong CD68 immunoreactivity of the foamy macrophages will rule out this diagnostic error. The commonest reported association is with endometrial adenocarcinoma that has been irradiated. It has been postulated that the xanthogranulomatous inflammation represents a response to necrotic tumor.
Non-Neoplastic Salivary Gland Diseases
Published in John C Watkinson, Raymond W Clarke, Terry M Jones, Vinidh Paleri, Nicholas White, Tim Woolford, Head & Neck Surgery Plastic Surgery, 2018
Stephen R. Porter, Stefano Fedele, Valeria Mercadante
Xanthogranulomatous sialadenitis is a very rare disorder manifesting itself as a solitary painless swelling within a major salivary gland.88 It is histopathologically characterized by the presence of lipid-laden macrophages (xanthoma cells or foamy macrophages) with chronic and acute inflammation as well as non-caseating granulomas. The investigation of this disorder is similar to that of a salivary gland malignancy. It has been suggested that xanthogranulomatous sialadenitis may arise as a consequence of FNAB, but there are few supporting data. Excision would seem to be the logical treatment, particularly in view of the presence of Warthin’s tumour in association xanthogranulomatous sialadenitis of one patient.89, 90
IgG4-related ophthalmic disease in association with adult-onset asthma and periocular xanthogranuloma: a case report
Published in Orbit, 2023
Brigitte M. Papa, H. Miles Prince, Alan A. McNab, Penny McKelvie
Adult xanthogranulomatous disease (XG) of the orbit and ocular adnexa comprises a heterogeneous group of rare fibroinflammatory disorders presenting with orbital masses and often xanthelasma-like lesions of the eyelids.1 There are four distinct subtypes: 1) adult onset xanthogranulomatous disease of the orbit (AOX), 2) adult-onset asthma with peri-ocular xanthogranuloma (AAPOX), 3) necrobiotic xanthogranuloma (NXG), and 4) Erdheim–Chester disease (ECD).1 AOX is a localised disorder of the peri-ocular soft tissues. AAPOX comprises AOX with additional features of adult-onset asthma, often nasal and paranasal sinus disease and reactive lymphadenopathy.1 NXG characteristically presents with periorbital subcutaneous skin lesions, which tend to ulcerate, and histological features of necrobiosis with palisading necrosis.1 ECD can affect the orbit, typically posteriorly, and often has systemic manifestations.1 It has been found to have activating mutations within the MAPK pathway, in particular, BRAF V600E mutations in more than 60% of the cases.2 Compared to the other subtypes of adult XG, ECD has a worse prognosis, but this has been improved with treatment with vemurafenib, a BRAF inhibitor.3 The stereotypical pathological features of XG include an infiltration of foamy xanthomatous macrophages and Touton giant cells.1
Adult xanthogranulomatous disease of the orbit: case report of spontaneous regression and review of treatment modalities
Published in Orbit, 2020
Michelle M. Maeng, Kyle J. Godfrey, Sanjai Jalaj, Michael Kazim
Adult xanthogranulomatous disease involving the orbit and periorbita is a rare disease without clear standards for surgical or medical management. The authors report a case of adult orbital xanthogranuloma, without systemic disease, who, after 10 years of observation, demonstrated sustained radiographic regression of the lesions. This represents one of the first reports of spontaneous regression of untreated orbital xanthogranuloma, to the best of the authors’ knowledge. We present our case in the context of a major review of various treatment strategies described in the literature, including surgical resection, radiotherapy, plasmapheresis, corticosteroids, multiple immunomodulatory medications, and observation.
A case of xanthogranulomatous inflammation of the urethra: treatment with a steroid-based non-surgical approach
Published in Scandinavian Journal of Urology, 2019
André Jacobsen, Ulla Nordström Joensen, Pernille Hammershøj Jensen
We provide a report of a steroid-based therapeutical approach that could be considered as an alternative to surgery for some patients with localized xanthogranulomatous inflammation, a rare disease where a high level of evidence for any treatment will likely never be obtained.