Juvenile Xanthogranuloma of the Sellar Region with a 5-Year Medical History: Case Report and Literature Review

2021 ◽  
pp. 1-8
Author(s):  
Dekui Cheng ◽  
Fengyu Yang ◽  
Ziji Li ◽  
Fan Qv ◽  
Wei Liu
Keyword(s):  
Medical History ◽  
Pediatric Population ◽  
Benign Lesion ◽  
Giant Cells ◽  
Juvenile Xanthogranuloma ◽  
Epithelial Tissue ◽  
Sellar Region ◽  
Optic Chiasma ◽  
Suprasellar Region ◽  
Field Disturbance

<b><i>Introduction:</i></b> Xanthogranuloma of the sellar region is a rare benign lesion, and there are few cases reported in children. Its histogenesis is controversial, and it is difficult to strictly differentiate it from craniopharyngioma (CP), Rathke’s cleft cyst, or pituitary adenoma. <b><i>Case Presentation:</i></b> A 16-year-old boy presented with a rare xanthogranuloma of the sellar region after complaining of retardation of growth 5 years previously. The ophthalmologic evaluation revealed no visual field disturbance. Endocrinological examination revealed hypopituitarism. Magnetic resonance imaging showed an intrasellar mass extending into the suprasellar region and compressing the optic chiasma, which appeared mixed signals on T1-weighted images. Endonasal transsphenoidal resection of the tumor was performed. Histological analysis of the tumor sections demonstrated granulomatous tissue with cholesterol clefts, hemosiderin deposits, fibrous tissues, multinucleated giant cells, and lymphocyte. Thus, the tumor was pathologically diagnosed as xanthogranuloma of the sellar region, which is different from adamantinomatous CP. There was no epithelial tissue in any part of the tumor including tumor capsule but have focal necrosis and calcification. His endocrinological dysfunction did not recover, so a hormonal replacement was continuously required. <b><i>Conclusion:</i></b> Xanthogranuloma of the sellar region is a rare entity but must be considered in the differential diagnosis of lesions of the sellar region, even in pediatric population. We should think about this disease when dealing with children with stunted growth accompanied by a long medical history. Our case demonstrates the natural progression of the disease, suggesting that xanthogranuloma of the sellar region without epithelial components may be an independent disease.

Intramuscular Juvenile Xanthogranuloma in an Adult

2005 ◽  
Vol 129 (2) ◽  
pp. e31-e34 ◽  
Author(s):  
Fang-Ying Kuo ◽  
Hock-Liew Eng ◽  
Shih-Hao Chen ◽  
Hsuan-Ying Huang
Keyword(s):  
Pediatric Population ◽  
Cutaneous Lesion ◽  
Giant Cells ◽  
Adult Patients ◽  
Factor Xiiia ◽  
Juvenile Xanthogranuloma ◽  
Age Related ◽  
Histologic Pattern ◽  
Morphologic Variation ◽  
The Right

Abstract Juvenile xanthogranuloma (JXG) is a self-limited cutaneous lesion that most often occurs in infancy. Approximately 10% to 30% of JXGs occur in adult patients, where most cases present as cutaneous papulonodular lesions, with only rare cases involving extracutaneous sites. Intramuscular JXG is extremely rare and has received little attention. On review of the literature, all of the 6 previously reported intramuscular JXGs were noted in the pediatric population. The authors hereby describe a case of adult intramuscular JXG that occurred in a woman who initially had a dermal JXG in the nasal skin at the age of 48 years and then developed a slow-growing painless intramuscular JXG in the right forearm 4 years later. Both the dermal and intramuscular lesions revealed similar histologic features and consisted of diffuse infiltrates of histiocytes with eosinophilic and foamy cytoplasm, lymphocytes, eosinophils, and Touton giant cells in varying proportions. However, central fibrosis and a focal storiform arrangement of spindled fibroblast-like cells in the intramuscular lesion resulted in a histologic pattern reminiscent of a fibrous histiocytoma. Immunohistochemically, the intramuscular JXG was positive for CD68, factor XIIIa, CD31, and vimentin. This case underscores the fact that intramuscular JXG can also involve adult patients and its morphologic variation is more likely to be time dependent rather than site specific or age related.

scholarly journals Kaposiform Hemangioendothelioma: clinicopathological characteristics of 8 cases of a rare vascular tumor and review of literature

2021 ◽  
Vol 16 (1) ◽  
Author(s):  
Qurratulain Chundriger ◽  
Muhammad Usman Tariq ◽  
Jamshid Abdul-Ghafar ◽  
Arsalan Ahmed ◽  
Nasir Ud Din
Keyword(s):  
Pediatric Population ◽  
Adult Population ◽  
Correct Diagnosis ◽  
Kaposi Sarcoma ◽  
Minor Component ◽  
Vascular Tumor ◽  
Giant Cells ◽  
Clinicopathological Characteristics ◽  
Multinucleated Giant Cells ◽  
Kaposiform Hemangioendothelioma

Abstract Background Kaposiform Hemangioendothelioma (KHE) is a rare vascular tumor of intermediate malignant potential which shows locally aggressive growth but only rarely metastasizes. It is mostly considered to be a tumor of pediatric population but its occurrence in the adults is not uncommon as once considered. Histologically, KHE can mimic other soft tissue neoplasms of different behaviors (e.g. Kaposi Sarcoma, hemangioma) and establishing the correct diagnosis is important for appropriate treatment. Herein, we describe the clinicopathological features of 8 cases of KHE which will be helpful in making their diagnosis. Methods We reviewed pathology reports, microscopy glass slides and obtained follow up information about 8 cases of KHE which were diagnosed at our institution from January 2008 till June 2020. Immunohistochemical stain for HHV8 was also performed. Results Age ranged from 7 months to 25 years. Seven patients were less than 20 years of age and one patient was 25 years old. Equal gender distribution was observed. Extremities were the most common sites of involvement, followed by head and neck, pancreas and ischiorectal region. 2 cases were resection specimen and all others were incisional biopsies. The largest tumor size was 5.5 cm in one of the resections. The incisional/fragmented tissues were all less than 5 cm in aggregate. Most cases showed predominance of nodular growth and a minor component of spindle cell population along with lymphangiomatosis like vascular channels, with evidence of microthrombi in 2 cases. Few multinucleated giant cells were observed in 2 cases. None of the cases exhibited significant nuclear atypia or mitotic activity. One of the cases arising in dermis showed underlying bone involvement. HHV8 was negative in 7/7 cases. Conclusions KHE can also involve adult population and it should always be considered in the differential diagnoses of a vascular lesion. Presence of multinucleated giant cells is a rare finding. Knowledge about histological features and potential mimics is helpful in avoiding misdiagnosis.

Juvenile xanthogranuloma of supra-sellar region: a rare presentation

2015 ◽  
Vol 34 (11) ◽  
pp. 368-370 ◽  
Author(s):  
Katelyn O. Stepan ◽  
Aseem Sharma ◽  
Michael R. Chicoine ◽  
Ravindra Uppaluri ◽  
Sonika Dahiya
Keyword(s):  
Juvenile Xanthogranuloma ◽  
Sellar Region ◽  
Rare Presentation

scholarly journals Extensions of the Sphenoid Sinus

Neurosurgery ◽  
2010 ◽  
Vol 66 (4) ◽  
pp. 797-816 ◽  
Author(s):  
Jian Wang ◽  
Sharatchandra Bidari ◽  
Kohei Inoue ◽  
Hong Yang ◽  
Albert Rhoton
Keyword(s):  
Computed Tomography ◽  
Sphenoid Sinus ◽  
Middle Cranial Fossa ◽  
Cranial Base ◽  
Sellar Region ◽  
Computed Tomography Images ◽  
Suprasellar Region ◽  
Cranial Fossa ◽  
Minimally Invasive Access ◽  
Selection Of

Abstract OBJECTIVE The transsphenoidal approach has been extended in recent years from tumors of the sellar region to lesions involving other areas bordering the sphenoid sinus including the cavernous sinus, Meckel's cave, middle cranial fossa, planum sphenoidal, suprasellar region, and clivus. The goal of this study was to examine various pneumatized extensions of the sphenoid sinus that may facilitate extended approaches directed through the sinus. METHODS The sphenoid sinus and its surrounding structures were examined in 18 cadaver heads, and the results were correlated with the findings from 100 computed tomography images of the sinus. The sellar type of the sphenoid sinus in which the pneumatization extended beyond the anterior sellar wall was further classified according to the various extensions of the sinus. METHODS The sphenoid sinus and its surrounding structures were examined in 18 cadaver heads, and the results were correlated with the findings from 100 computed tomography images of the sinus. The sellar type of the sphenoid sinus in which the pneumatization extended beyond the anterior sellar wall was further classified according to the various extensions of the sinus. RESULTS The sellar type of the sphenoid sinus was classified into the following 6 basic types based on the direction of pneumatization: sphenoid body, lateral, clival, lesser wing, anterior, and combined. The recesses and prominences, formed by pneumatization of the sinus, act as “windows” opening from the sinus in different areas of the cranial base and may facilitate minimally invasive access to lesions in the corresponding areas. CONCLUSION The variations in the extensions of pneumatization of the sphenoid sinus may facilitate entry into areas bordering the sphenoid sinus and play a role in the selection of a surgical approach to lesions bordering the sinus.

scholarly journals A Case of Pituitary Germinoma With Complete Recovery of Visual Field

2021 ◽  
Vol 5 (Supplement_1) ◽  
pp. A561-A562
Author(s):  
Majid Alameri ◽  
Hessa Boharoon ◽  
Abdulla Mohamed Alnuaimi ◽  
Florian Wernig ◽  
Michael Seckl ◽  
...  
Keyword(s):  
Cerebrospinal Fluid ◽  
Visual Field ◽  
Diabetes Insipidus ◽  
Visual Loss ◽  
Hormone Replacement ◽  
Tumour Marker ◽  
Tumour Markers ◽  
Optic Chiasma ◽  
Excellent Outcome ◽  
Suprasellar Region

Abstract Central nervous system germ cell tumours (GCTs) are rare, accounting for 0.1-3.4% of all primary brain malignancies. Intracranial GCTs (ICGCTs) usually arise within the pineal recess (50-65%), sellar-suprasellar region (25-35%) and rarely in the basal ganglia-thalamus (5-10%). A peakonset occurs during the second decade of life with a median age at diagnosis between10-12 years and a male predominance. Histologically, ICGCTs are classified into germinomas accounting for two thirds of cases and non-germinomatous GCTs. ICGCTs present with clinical features dependent on their location and tumour size. Symptoms most often arise from compressed optic structures, increased intracranial pressure and central endocrine abnormalities, particularly diabetes insipidus (DI). Clinical findings combined with elevated tumour marker levels within the serum and/or cerebrospinal fluid (CSF) can be diagnostic. Case: A 30-year-old lady who survived a childhood leukaemia presented as an emergency with a two-month history of headaches, dizziness and near-complete visual loss. Cranial MRI identified a large sellar mass with suprasellar extension compressing the optic chiasma and hypothalamus. Biochemical and endocrine profiles confirmed diabetes insipidus, hypocortisolaemia and hypothyroidism all requiring hormone replacement. Visual field examination revealed total left visual loss and a right temporal hemianopia. Cerebrospinal fluid analysis identified an elevated human chorionic gonadotropin (HCG) level at 16 IU/L (normal &lt;2), with a normal alpha-fetoprotein (AFP) level. Both tumour markers were normal in the serum. Emergency Etoposide-Cisplatin (Em-EP) chemotherapy was initiated for a suprasellar GCT followed by the EPOMB-ITMTX regimen [Etoposide-Cisplatinwith intrathecal Methotrexate (ITMTX) alternating with Vincristine-Methotrexate-Bleomycin]. After four cycles EPOMB-ITMTX, the vision was fully restored, brain MRI demonstrated an excellent radiological response and the CSF HCG normalised. Our patient received volumetric-modulated arc cranial radiotherapy (VMAT) with a total 54 Gy in 16 fractions. She remains well without disease recurrence for 14 months. Tumour markers are normal with stable MRI brain and whole-spine. Endocrine follow-up confirms recovery in all pituitary axes except the gonadotrophins for which she remains on hormone replacement therapy. Conclusion: This case demonstrates an excellent outcome for a young adult diagnosed with a suprasellar GCT treated with chemotherapy followed by stereotactic radiotherapy. It highlights the importance in keeping a high clinical index of suspicion in young adults presenting with a midline intracranial tumour, visual dysfunction and an endocrinopathy. CSF analysis identified an elevated tumour marker level, which bypasses the need for a histological diagnosis.

scholarly journals Giant Cell Tumor of Rib Arising Anteriorly as a Large Inframammary Mass: A Case Report and Review of the Literature

2012 ◽  
Vol 2012 ◽  
pp. 1-4 ◽  
Author(s):  
Amit Sharma ◽  
Amy E. Armstrong
Keyword(s):  
Giant Cell Tumor ◽  
Giant Cell ◽  
Surgical Resection ◽  
Benign Lesion ◽  
Giant Cells ◽  
Cell Tumor ◽  
Tissue Mass ◽  
Surgical Specimen ◽  
Female Patients ◽  
Cortical Destruction

Introduction. Giant cell tumor of the bone is a rare benign lesion that infrequently affects the ribs, and if present, is usually located posteriorly. The rarity of this tumor poses diagnostic and therapeutic problems for physicians, especially when it is located in the anterior arc of the rib in close proximity to the breasts in female patients.Case Presentation. We report the case of a 32-year-old Asian female with a giant cell tumor of her anterior rib, presenting as a large inframammary mass. Computed tomography showed a tumor arising from the 7th rib anteriorly with marginal sclerosis, cortical destruction, and a soft tissue mass. She was treated with surgical resection, and the defect was reconstructed primarily. The surgical specimen measured 28.0 × 24.0 cm. The microscopic examination showed a large number of multinucleate giant cells scattered over the parenchyma. Patient recovered uneventfully and continues to be recurrence-free six years after surgical resection.Conclusion. We report the largest known case of giant cell tumor arising from the anterior aspect of a rib. We recommend including giant cell tumor in the differential diagnosis of chest wall masses especially in female patients, regardless of the size on clinical examination.

JUVENILE XANTHOGRANULOMA WITH INCONSPICUOUS FOAM CELLS AND GIANT CELLS

1995 ◽  
Vol 34 (9) ◽  
pp. 653-655 ◽  
Author(s):  
WARREN S. TANZ ◽  
Y. ALYSSA KIM ◽  
ROBERT A. SCHWARTZ ◽  
THOMAS WALTERS ◽  
CAMILA K. JANNIGER ◽  
...  
Keyword(s):  
Foam Cells ◽  
Giant Cells ◽  
Juvenile Xanthogranuloma

scholarly journals Central Odontogenic Fibroma Accompanied by a Central Giant Cell Granuloma-Like Lesion: Report of a Case and Review of Literature

2022 ◽  
Author(s):  
Monir Moradzadeh Khiavi ◽  
Abbas Karimi ◽  
Hassan Mirmohammad Sadeghi ◽  
Samira Derakhshan ◽  
Seyed Mobin Tafreshi ◽  
...  
Keyword(s):  
Giant Cell ◽  
Benign Lesion ◽  
Excisional Biopsy ◽  
Giant Cells ◽  
Histopathological Analysis ◽  
Giant Cell Granuloma ◽  
Multinucleated Giant Cells ◽  
Central Giant Cell Granuloma ◽  
Odontogenic Fibroma ◽  
Central Odontogenic Fibroma

Central giant cell granuloma (CGCG) is a benign non-neoplastic intraosseous lesion mainly found in the anterior mandible. It is characterized by multinucleated giant cells, representing osteoclasts or macrophages. Central odontogenic fibroma (COF) is an uncommon benign lesion of the jaws. It originates from the odontogenic ectomesenchyme. In rare cases, COF may accompany a CGCG. To date, 49 cases of COF accompanied by CGCG-like lesions have been reported in the literature. In this paper, we present another case of COF-CGCG in a 46-year-old female. The lesion was located in the posterior mandible. Excisional biopsy was carried out, and histopathological analysis revealed multinucleated giant cells with numerous strands of odontogenic epithelium. A literature review of previously reported cases was also performed.  

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