Indeterminate Cell Histiocytosis of the Spine: A Case Report

2021 ◽  
pp. 1-6
Author(s):  
Dallas Louis Sheinberg ◽  
Jean-Paul Bryant ◽  
Roberto J. Perez-Roman ◽  
Toba N. Niazi
Keyword(s):  
Dendritic Cells ◽  
Pediatric Population ◽  
Surgical Excision ◽  
Mass Effect ◽  
Giant Cells ◽  
Neoplastic Disease ◽  
Case Presentation ◽  
Systemic Involvement ◽  
Lower Back ◽  
Ct And Mri

<b><i>Introduction:</i></b> Indeterminate cell histiocytosis is a rare neoplastic disease characterized by proliferation of dendritic cells that share morphologic and immunophenotypic features of Langerhans cells and non-Langerhans histiocytes. ICH lesions are typically restricted to the skin; however, there have been rare reports of extracutaneous and systemic involvement. <b><i>Case Presentation:</i></b> We describe a case of a 13-year-old female presenting with complaints of worsening lower back pain for 1 year. CT and MRI of the lumbar spine demonstrated a well-defined bony, lytic, expansile lesion of the posterior and mid-endplate of the L4 vertebra with mass effect on the thecal sac. The patient underwent L3–L5 decompression and fusion with surgical excision of the vertebral body tumor. Microscopic examination of the lesion showed benign fibrohistiocytic proliferation with giant cells, and immunohistochemical staining revealed a phenotype consistent with indeterminate cell histiocytosis (S100+ CD1a+ langerin−). <b><i>Discussion/Conclusion:</i></b> ICH is an extraordinarily rare neoplastic disease of dendritic cells that has a poorly understood pathogenesis. This case expands the spectrum of potential ICH extracutaneous involvement to now include the spine, a location previously undocumented in the literature in the pediatric population.

scholarly journals Congenital giant Bednar tumor in a child: surgical challenges in a young infant: a case report

2020 ◽  
Vol 16 (1) ◽  
Author(s):  
Vidhya Gunasekaran ◽  
Nitin J. Peters ◽  
Ram Samujh
Keyword(s):  
Case Report ◽  
Wide Local Excision ◽  
Rare Case ◽  
Pediatric Population ◽  
Young Infant ◽  
Dermatofibrosarcoma Protuberans ◽  
Surgical Excision ◽  
Case Presentation ◽  
Lower Back

Abstract Background Bednar tumor is a pigmented variant of the uncommon dermatofibrosarcoma protuberans (DFSP). It is a disease of the middle aged and elderly and has been rarely described in the pediatric population. Case presentation We report a rare case of a giant Bednar tumor present since birth, in a 2-year-old male child. It was present over the lower back and managed by surgical excision. Conclusions Bednar tumor is a rarity in children and is managed with aggressive wide local excision with margin of at least 1 cm. A close clinical follow-up is mandatory.

scholarly journals Recurrent schwannoma of the tongue in a pediatric patient—report of a rare case with an updated review of literature

2021 ◽  
Vol 37 (1) ◽  
Author(s):  
Anup Singh ◽  
Vaisakh Kuzhikkali ◽  
Arvind Kumar Kairo
Keyword(s):  
Pediatric Population ◽  
Surgical Excision ◽  
Review Of Literature ◽  
Case Presentation ◽  
Nerve Sheath Tumors ◽  
Peripheral Nerve Sheath Tumors ◽  
Nerve Sheath ◽  
Cervical Sympathetic ◽  
Mobile Tongue

Abstract Background Head and neck is a relatively common site of occurrence for the peripheral nerve sheath tumors, and majority of these tumors are seen involving neck, involving vagus nerve, and cervical sympathetic chain. Schwannomas involving mobile tongue are rarely encountered, especially in the pediatric population Case presentation We present a case of recurrent tongue schwannoma in a 13-year-old female successfully managed with transoral excision. At a follow-up of 3 years, no recurrence is observed. Conclusion Surgical excision is the recommended modality of treatment for lingual schwannomas, and when excised adequately, recurrences are not expected. A clear margin of surrounding normal tissue should be aimed for to avoid possible recurrence.

scholarly journals Nasolabial cyst: case report and review of management options

BMC Surgery ◽  
2020 ◽  
Vol 20 (1) ◽  
Author(s):  
Abdulhakeem Almutairi ◽  
Abeer Alaglan ◽  
Mazyad Alenezi ◽  
Sultan Alanazy ◽  
Osama Al-Wutayd
Keyword(s):  
Soft Tissue ◽  
Nasal Obstruction ◽  
Surgical Excision ◽  
Mass Effect ◽  
Treatment Modalities ◽  
Open Approach ◽  
Management Options ◽  
Case Presentation ◽  
Complete Surgical Excision

Abstract Background Nasolabial cysts are rare, non-odontogenic, soft-tissue cysts that develop between the upper lip and nasal vestibule with an overall incidence of 0.7% out of all maxillofacial cysts. The predominant presentation of a nasolabial cyst is a painless localized swelling with varying degrees of nasal obstruction. Several treatment modalities have described in the management of the nasolabial cyst. In this paper, we present a case of a nasolabial cyst in a 44 years old man with discussions of the treatment modalities in the lights of the literature. Case presentation We present a case of a nasolabial cyst in a 44-year-old man that slowly increased in size through a period of 3 years, with associated mild pain and nasal obstruction. It had caused a mass effect upon the maxilla, resulting in scalloping. The cyst was excised entirely with no evidence of recurrence at the two months follow up. Conclusions The nasolabial cyst is a rare soft-tissue cyst. Complete surgical excision using an open approach performed to our case, which considered with the complete endoscopic removal of the best treatment for the nasolabial cysts with a rare recurrence rate.

scholarly journals Orbital migration of schistosome eggs: a case report

2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Nouhoum Guirou ◽  
Serge Resnikoff ◽  
Abba Kaka Hadja Yakoura ◽  
Michel Gouda ◽  
Seydou Bakayoko ◽  
...  
Keyword(s):  
Lacrimal Gland ◽  
Surgical Excision ◽  
Giant Cells ◽  
Epithelioid Cell ◽  
Pathological Examination ◽  
Case Presentation ◽  
Urine Examination ◽  
The Right ◽  
Orbital Migration

Abstract Background Ocular damage, including damage to the conjunctiva, lacrimal gland, eyelids, and orbit, caused by Schistosoma haematobium is sporadic. We report a clinical case of orbital migration of schistosome eggs. Case presentation A 14-year-old boy of Malian nationality presented with a painless swelling of the upper right eyelid, which had been gradually increasing for approximately 3 months. Visual acuity was logMAR 0.10 and 0.00 in the right and left eye, respectively. External examination revealed a right palpebral mass, pushing the globe slightly downward and inward. Computed tomography revealed a mass of the right lacrimal gland. Total excision of the mass was performed by transpalpebral orbitotomy. Pathological examination revealed an inflammatory granulomatous infiltrate of the lacrimal gland consisting of lymphocytes, eosinophils, giant cells, epithelioid cell, histiocytes and calcified Schistosoma eggs with terminal spine. Urine examination revealed eggs of S. haematobium. Praziquantel 40 mg/kg was administered to the patient. The hematuria stopped after 1 week. After 3 years of follow-up, no recurrence was noted. Conclusions The bilharzian granuloma of the lacrimal gland is an ectopic site of the parasite. In this case, the granuloma was cured by surgical excision followed by a course of Praziquantel.

scholarly journals Sarcoidosis presenting as bilateral lacrimal gland swelling: a pediatric case report

2021 ◽  
Vol 19 (1) ◽  
Author(s):  
Margaret S. Powell ◽  
Ashley W. Cross ◽  
Jared Tallo ◽  
Edward W. Cheeseman ◽  
Mileka R. Gilbert
Keyword(s):  
Lacrimal Gland ◽  
Pediatric Population ◽  
Pediatric Ophthalmology ◽  
Pediatric Case ◽  
Case Presentation ◽  
Systemic Involvement ◽  
Eyelid Swelling ◽  
Initial Improvement ◽  
Disease Entities ◽  
Noncaseating Granulomas

Abstract Background To describe a case of pediatric sarcoidosis which initially presented as papillary conjunctivitis before manifesting as bilateral lacrimal gland swelling without other known systemic involvement. Case presentation A 10-year-old female presented to the pediatric ophthalmology clinic with complaints of bilateral eyelid swelling, tearing and itching for approximately 1 month. Her history and exam were most consistent with allergic conjunctivitis, for which she was started on a standard topical regimen. Despite initial improvement, she re-presented with significantly worsened eyelid swelling and minimal allergic symptoms. Enlargement of the lacrimal glands were palpable at this time. Lacrimal gland biopsy was obtained which demonstrated noncaseating granulomas. Systemic workup did not reveal evidence of disease involvement elsewhere. Conclusions Sarcoidosis in the pediatric population may present in a myriad of ways and is well-known to mimic other disease entities. We present a case of pediatric sarcoidosis which presented initially as papillary conjunctivitis before manifesting as bilateral lacrimal gland swelling without systemic involvement.

scholarly journals Giant pedunculated hepatic hemangioma necessitating surgical excision in a neonate: A case report

2020 ◽  
Vol 9 ◽  
pp. 15
Author(s):  
Abdulrahman Almuawi ◽  
Mohammed Daboos ◽  
Ramakrishna Prasad
Keyword(s):  
Case Report ◽  
Abdominal Mass ◽  
Surgical Excision ◽  
Bowel Function ◽  
Mass Effect ◽  
Postoperative Recovery ◽  
Hepatic Hemangioma ◽  
Respiratory Compromise ◽  
Case Presentation ◽  
Giant Hepatic Hemangioma

Background: Hepatic hemangioma (HH) is the most common hepatic tumor in small infants. It rarely presents during the neonatal period. Fetal giant hepatic hemangiomas tend to result in hydrops fetalis and cardiac failure due to cardiovascular overload. The purpose of this case report is to demonstrate the importance of pre and postnatal diagnosis of liver hemangioma with unusual presentation in neonatal life with huge mass causing respiratory compromise, and bowel compression. Case Presentation: We present a rare case of giant pedunculated hepatic hemangioma, diagnosed prenatally as a huge intra-abdominal mass. Patient developed respiratory distress due to large abdominal mass, thrombocytopenia, anemia and bowel compression thus necessitated surgical excision. Postoperative recovery was uneventful. Conclusion: Giant hepatic hemangioma may become fatal due to a huge mass effect on respiration and bowel function, along with associated thrombocytopenia and anemia. Adequate monitoring and support are mandatory in such cases and surgical excision at times becomes lifesaving.

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.

Efficacy and safety of denosumab treatment in a prepubertal patient with cherubism

2020 ◽  
Vol 33 (7) ◽  
pp. 963-966
Author(s):  
Haruka Kawamura ◽  
Satoshi Watanabe ◽  
Takashi I ◽  
Izumi Asahina ◽  
Hiroyuki Moriuchi ◽  
...  
Keyword(s):  
Autosomal Dominant ◽  
Therapeutic Potential ◽  
Giant Cells ◽  
Efficacy And Safety ◽  
Autosomal Dominant Disorder ◽  
Osteolytic Lesions ◽  
Case Presentation ◽  
Childhood Growth ◽  
Receptor Activator ◽  
Denosumab Treatment

AbstractBackgroundDenosumab is an inhibitor of receptor activator of nuclear factor kappa-B ligand, which strongly suppresses osteoclasts. Cherubism is a rare autosomal dominant disorder characterized by symmetrical swelling of the jaws, in which the bone is replaced by a fibrous granuloma containing osteoclast-like giant cells.Case presentationWe report the efficacy and safety of denosumab treatment in a prepubertal boy with progressive cherubism. The treatment consisting of eight subcutaneous denosumab injections (120 mg/dose) in 6 months not only suppressed the expansion of the osteolytic lesions but also dramatically ossified them. However, a transiently decreased growth rate and rebounded asymptomatic hypercalcemia were associated with the treatment.ConclusionsThe present case demonstrated the therapeutic potential of denosumab for treatment of cherubism, although adverse effects, especially those on childhood growth, remain obscure. Further studies are needed to establish a safe and effective protocol for denosumab treatment of children.

scholarly journals Intravenous leiomyomatosis with intracardiac extension managed by one-stage surgical excision: a case presentation

2020 ◽  
Vol 51 (1) ◽  
Author(s):  
Rohit Bhoil ◽  
Vijay Thakur ◽  
Yashwant Singh Verma ◽  
Sabina Bhoil ◽  
Ashwani Tomar ◽  
...  
Keyword(s):  
Surgical Excision ◽  
Intravenous Leiomyomatosis ◽  
Case Presentation ◽  
One Stage ◽  
Intracardiac Extension

scholarly journals Osteochondroma of the pubic symphysis causing hematuria: a case report and literature review

BMC Urology ◽  
2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Li-cheng Song ◽  
Qian Xu ◽  
Hui Li ◽  
Zhi-jun Li ◽  
Ya Li ◽  
...  
Keyword(s):  
Bladder Tumor ◽  
Multidisciplinary Approach ◽  
Bladder Tumour ◽  
Surgical Excision ◽  
Pubic Symphysis ◽  
Careful Analysis ◽  
Bone Neoplasm ◽  
Imaging Data ◽  
Case Presentation

Abstract Background Osteochondroma is the most common benign bone neoplasm and is sometimes referred to as osteocartilaginous exostosis. The symptoms caused by osteochondroma are rare, especially the urogenital complications. Therefore, this tumour is sometimes misdiagnosed. Case presentation This report described a 70-year-old woman with hematuria who was initially misdiagnosed with a bladder tumour in the outpatient department by a urologist. However, during cystoscopy, we found that the mass did not resemble a bladder tumor. Multidisciplinary approach with careful analysis of the imaging data suggested the diagnosis of osteochondroma. Open surgical excision of the mass was done and histology confirmed the diagnosis of benign osteochondroma. After 6 months of follow-up, the patient was still asymptomatic. Conclusions This case illustrates that hematuria is caused by not only urogenital disease but also osteochondroma. We present this case to draw the attention of clinicians to osteochondroma of the pubic symphysis.

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