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Sellar xanthogranulomas are extremely rare intracranial lesions, particularly in pediatric patients, and their diagnostic and therapeutic challenges prompt thorough investigation. We describe a case of a two-year-old toddler diagnosed with sellar xanthogranuloma, highlighting the challenges encountered in its diagnosis and management. The child presented with symptoms, including headache, ptosis of the left eye, and neurological deficits. Brain computed tomography (CT) and magnetic resonance imaging (MRI) revealed a hypodense sellar lesion. The patient underwent a left pterional craniotomy for resection of the mass. Histopathological examination suggested the diagnosis of sellar xanthogranuloma, characterized by foamy macrophages, giant cells, lymphocytic infiltrates, fibrous proliferation, necrotic detritus, and hemosiderin deposits. Further diagnostic precision was achieved through immunohistochemical staining, including CD1a and langerin, which successfully ruled out the possibility of Langerhans cell histiocytosis (LCH), reinforcing the diagnosis of sellar xanthogranuloma. The successful surgical resection of the lesion led to a favorable outcome, evidenced by the significant alleviation of symptoms as well as the restoration of normal neurological function. Post-operative assessments demonstrated a marked improvement in the patient\'s quality of life, and there were no observed complications or recurrence of the lesion during the follow-up period. In summary, our case report not only highlights the rarity and diagnostic challenges of sellar xanthogranulomas but also emphasizes the importance of collaborative medical expertise in achieving accurate diagnosis and successful therapeutic outcomes in pediatric patients. The successful management of this case offers valuable insights into the clinical presentation, diagnostic complexities, and treatment strategy of sellar xanthogranulomas, further enriching our understanding of this uncommon intracranial pathology.

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Xanthogranulomatous inflammatory reactions are benign inflammatory processes characterized by aggregating lipid-laden foamy macrophages. Although cases have been reported in different organ systems, these rare reactions predominantly occur in the kidney and gallbladder. We present a 92-year-old female who noticed a palpable, tender mass in the lower inner quadrant of her right breast with no skin changes. She was referred to surgery by her primary care physician on suspicion of malignancy and further evaluation. Ultrasound-guided biopsy, ordered by the primary care provider, revealed a suspicious high-grade malignant neoplasm of uncertain origin. Pathological findings include the presence of an unusual population of malignant epithelioid cells with a striking xanthogranulomatous reaction, along with numerous Touton-like histiocytes. These findings are comparable in morphology to a recently reported xanthogranulomatous epithelial tumor. Given the lack of history of breast carcinoma in this patient as well as the lack of immunohistochemical studies suggesting breast carcinoma, treatment involved continuing standard of care for an unusual high-grade sarcoma via lumpectomy. A positron emission tomography (PET) scan was ordered to ensure there was no spread or alternate origins of the cancer tissue. This case report brings to light the findings of a probable xanthogranulomatous tumor in breast tissue, an exceptionally rare phenomenon in breast cancer, especially in the elderly population. Due to the rarity of xanthogranulomatous tumors in the breast, prognosis and standardized treatment have yet to be established.

Juvenile xanthogranuloma (JXG) is the most common type of non-Langerhans cell histiocytosis. JXG is a rare benign tumor, which may be present at birth or develop later. The classical form of JXG is characterized by a red-yellowish benign papule or nodule with predilection sites on the head, neck, and trunk, although lesions can appear on extremities or extracutaneous sites. In most cases there is only one lesion, whereas numerous papules or nodules may occur. Special forms of JXG such as mixed, giant, subcutaneous, eruptive, clustered, and plaque-like have been reported and associations between JXG and systemic diseases have been made. Diagnosis mainly relies on the clinical appearance, and histology usually can confirm the disease. Here we present a very rare case of symmetrical giant facial plaque-type juvenile xanthogranuloma (SGFP-JXG) and compare it with classical JXG, variations of JXG, and discuss the differential diagnosis. A 4-year-old Caucasian female presented with plaque-like lesions composed of yellowish confluent papules on both the cheeks. The histological evaluation revealed a histiocytic lesion with a formation of Touton giant cells and immunohistochemistry results confirmed the diagnosis of the SGFP-JXG. In comparison to classical JXG, the onset of SGFP-JXG sometimes occurs later and the spontaneous resolution period may be prolonged. No associated diseases and no systemic involvements were observed. Histopathology is required to differentiate this form of JXG from other histiocytosis. To the best of our knowledge, only four cases of SGFP-JXG have been reported in the literature so far.

Juvenile xanthogranuloma (JXG) is an idiopathic granulomatous inflammatory condition that usually affects children. Intraocular involvement, especially bilateral, is rare in JXG. Most patients with ocular lesions are typically infants and usually present with hyphema, iridocyclitis, and secondary glaucoma. We report a case of a 3-month-old baby girl who presented to our emergency department with bilateral hyphema that started 3 weeks ago. She was medically free with no history of any recent trauma or preceding febrile illness. General physical examination showed a quiet baby with multiple hyperpigmented macules over the inner thigh and right upper arm, with one pinkish nodule over the occiput. She also had high intraocular pressures. A detailed ophthalmic assessment was done under general anesthesia. The nodular lesion was excised and sent for histopathological evaluation, which confirmed the diagnosis of JXG. Treatment of JXG cases present a challenge to ophthalmologist due to rebleeding and refractory glaucoma. Our case was admitted multiple times for rebleeding and refractory glaucoma and was treated with full antiglaucoma drops, steroid drops and peribulbar injection of steroid.

Juvenile xanthogranuloma (JXG) is a rare and benign tumor in infants. A solitary lesion on the eyelid has been reported in patients with JXG. We report a 15-year-old boy with multiple involvement of JXG on both eyelids. A mass on the left inner canthus was resected because of disturbance of the visual field and a risk of malignancy in terms of central ulceration in the lesion. The mass was examined by light microscopy. The mass had Touton giant cells with a wreath of nuclei surrounded by foamy histiocytes. No malignancy was observed. The mass showed no recurrence after resection.

Xanthogranuloma is a benign, non-Langerhans cell histiocytosis primarily diagnosed in infants and children, although a subset occurs in adults. Multifocal eruptive presentation of xanthogranuloma is very rare with only 4 previous cases reported in the literature to our knowledge. We describe a case of eruptive xanthogranuloma in a 49-year-old man who presented with sudden onset of numerous asymptomatic, red-yellow to orange papules on the face, scalp, axilla, flank and scrotum. Histologic features were consistent with xanthogranuloma with diffuse mixed infiltrate of foamy histiocytes, Touton giant cells and lymphocytes. Other than temporarily elevated non-fasting triglycerides, lab values have been unremarkable including serum plasma electrophoresis; however, the patient will continue to be monitored for ocular and other extracutaneous involvement and hematologic malignancies.

Xanthogranulomas (XG) are benign proliferative disorder of histiocytes, a non-Langerhans cell histiocytosis. Whose etiology is unknown. The nature of these lesions is controversial and could be either reactive or neoplastic; the presence of monoclonal cells does, however, favor the second hypothesis. Xanthogranuloma is frequently found in young adults and children (under 20 years old), mainly in the skin. In about 5%-10% of all Juvenile XG (JXG) cases xanthogranuloma are extracutaneous. Within this group, the site most frequently involved is the eye. Other involved organs are heart, liver, adrenals, oropharynx, lung, spleen, central nervous system and subcutaneous tissue, although involvement of the spine is uncommon. Isolated lesions involving the sacral region are extremely rare. To date, this is the first reported case of a giant JXG arising from S1 with extension into the pelvic region in an adult spine.

Necrobiotic xanthogranuloma (NXG) is a very rare, progressive variant of non-Langerhans cell histiocytosis. It is known to be associated with multisystem involvement and paraproteinemias. A 65-year-old female presented with chronic, slowly growing, asymptomatic periorbital nodules. The lesions had recurred after local excision elsewhere. No systemic involvement or paraproteinemias were detected. A provisional diagnosis of isolated cutaneous NXG was made which was confirmed by histopathology and immunohistochemistry staining. The lesions were surgically excised with excellent cosmetic and functional results. There was no recurrence over a period of 9 months. To our knowledge, this is the second case of NXG reported from India and the first without any systemic manifestations.