Burkitt lymphoma in a scalp region: a case report of it's recurrence in a 13-year-old child.

Introduction: Burkitt lymphoma (BL) is an aggressive non-Hodgkin lymphoma characterized by chromosome 8 MYC gene translocation. It manifests in three clinical types: immunodeficiency-related, sporadic (nonendemic), and endemic (African), each differing in epidemiology and clinical behavior. Treatment typically involves enrollment in clinical trials or intensive chemotherapy regimens like R-CODOX-M/IVAC. The authors present a case of recurrent BL following treatment. Case report: A 13-year-old female presented with a gradually progressive swelling in the left parieto-occipital region. Examination revealed normal vital signs and a Glasgow coma scale, with seronegative findings on investigations. An excision of a subganglion soft tissue tumor was performed, revealing histopathological features suggestive of a small round blue cell tumor. After chemotherapy, the patient experienced a recurrence in the scalp region, diagnosed as BL. Discussion: While scarce reports exist on BL in the scalp region, cases have been documented in various body locations. Treatment strategies, including chemotherapy and surgery, have shown promising results in managing the disease and improving symptoms. Conclusion: The recurrence of BL is rare, highlighting the importance of vigilance in monitoring patients post-treatment. The authors report a case of recurrent BL in a 13-year-old female, emphasizing the need for continued research and surveillance in managing this aggressive malignancy.

Unmasking Sturge-Weber syndrome in adulthood: a case with extrafacial port-wine stain and delayed neurological symptoms.

Background: Sturge-Weber syndrome (SWS) is a rare neurocutaneous disorder characterized by a facial port-wine birthmark, leptomeningeal angiomatosis, and glaucoma. This case report highlights the challenges of diagnosing SWS when presenting with atypical features. Here, the authors present a 55-year-old man with an extrafacial port-wine stain and delayed-onset seizures, deviating from the classic triad. Case presentation: A 55-year-old man presented with a recent seizure and a characteristic port-wine birthmark extending beyond the typical facial region. Neurological examination revealed no weakness, speech difficulties, or coordination problems. Ophthalmological examination didn't reveal glaucoma. Limited resources restricted access to advanced imaging like MRI scans. However, based on the constellation of clinical findings, including the facial birthmark with angiomatosis and the new-onset seizure, the patient received a diagnosis of SWS. Treatment with Levetiracetam was initiated to prevent future seizures, and patient education on managing diabetes and hypertension was provided. Clinical discussion: This case underscores the importance of considering SWS in diagnosing adult-onset seizures, especially with a characteristic facial birthmark. The delayed presentation and isolated seizure suggest potentially less severe brain involvement. Resource limitations necessitated a clinical diagnosis and treatment with readily available medications. Conclusion: This case highlights the challenges of diagnosing atypical SWS presentations. Early diagnosis is crucial for prompt management and improved patient outcomes. Future research should focus on developing robust diagnostic tools and exploring novel treatment options for atypical SWS presentations.

Epidermal cyst protruding from the occipital region of the scalp: a case report.

Introduction and importance: Epidermal cysts are the most common subcutaneous tumor typically observed on the scalp, face, neck, back, or trunk. Epidermal cysts conventionally range in size from 1 to 5 cm, with sizes greater than 5 cm rarely reported. Case presentation: Here, we present a case of a 58-year-old female housewife who presented to our surgery outpatient department (OPD) with a history of a mass in the left occipital region. The mass was first noticed 3 years back and was small and associated with mild itching. The mass progressively increased in size over the course of 3 years. Her present complaint was a painful mass associated with itching, headache, and pus discharge when compressed. The pain was relieved by taking medication. Clinical discussion: The epidermal cyst was confirmed on biopsy, and subsequent excision of the cyst was done under general anesthesia; the occipital bone was eroded by the inferior part of the cyst - reconstruction of scalp defect done by rotational scalp flap. Conclusion: Epidermal cysts, being a slow-growing benign tumor, can pose diagnostic difficulties, especially when located in the scalp area. In addition to that, when cranial bones and intracranial structures are affected by the cyst, they can even lead to complications and interventional difficulties.

Median nerve schwannoma with a diagnostic delay of 8 years: a case report.

Introduction: Schwannomas are benign tumors of the peripheral nerve sheath, and the median nerve is the most commonly involved nerve. These benign tumors of the peripheral nerve sheath are very rare; they are clinically and radiologically similar to most other benign swellings of the hand; thus, they are often misdiagnosed. Case Presentation: A 41-year-old lady presented with an 8-year-long history of swelling over the distal forearm. The tumor measured 3.5×3.5×3.5 mm and was located over the flexor aspect of the distal part of her right forearm. Schwannoma was suspected from the clinical presentation and imaging, but the final diagnosis was established only after the surgery and histopathological analysis. At the follow-up after 1 year, the patient is doing well and her symptoms have not recurred. Conclusion: Imaging characteristics of schwannoma can be misinterpreted as some other condition, making the preoperative diagnosis very difficult and important. Thus, clinicians should be aware of such swellings, especially those that have been unnoticed or misdiagnosed, and provide optimal diagnoses to confer good outcomes.

Rare case of multiple neurofibromas of the scalp and trunk in association with intradural extramedullary spinal tumor: a case report.

Neurofibromas are tumors of neural connective tissue composed of Schwann cells and fibroblasts. They can occur anywhere in the body, primarily as a solitary mass or as a component of neurofibromatosis. Only 1/4 of the intradural extramedullary spinal tumors are neurofibromas. While most of the neurofibromas are asymptomatic, the authors report a patient with symptomatic multiple neurofibromas of the scalp and trunk that lacked the salient features of neurofibromatosis-1. Case presentation: A 63-year-old male from Saptari, Nepal, with multiple insidious swellings in the scalp and trunk region, presented with complaints of weakness of the bilateral lower limb for 6-7 years resulting in difficulties in walking and bleeding from the most significant swelling that was in the occipital area. Clinical findings and investigations: Masses were present all over the scalp, averaging about 4×4 cm, with active bleeding from the two swellings. The authors performed the neurological assessment and histopathological and radiological investigations. Interventions and outcomes: A confirmed diagnosis of neurofibroma was made with myelography revealing an intradural extramedullary spinal tumor. A laminectomy with total excision of the tumor was performed. Relevance and impact: Meningiomas and nerve sheath tumors (schwannomas and neurofibromas) can both develop in the intradural extramedullary spinal compartment. Contrast-enhanced MRI is the most sensitive and specific imaging modality to evaluate possible spinal column lesions. Surgical excision, partial or complete, is the hallmark treatment of neurofibroma.

Large posterior mediastinal ganglioneuroma with intradural cervical spine extension: A rare case report and review of literature.

Introduction: Ganglioneuroma (GN) is a rare benign tumor of ganglion cell origin and can occur anywhere along the sympathetic chain. These tumors can grow to a significant size without any symptoms unless they exert a mass effect on the region they grow and start showing symptoms. Spinal extensions are rare and they may produce neurological symptoms warranting further investigation. Case presentation: We described a case of posterior mediastinal ganglioneuroma in a 4-year-old boy with cervical extension who presented with quadriparesis. The radiological scan revealed large ganglioneuroma having an intradural extramedullary extension with a large posterior mediastinal component compressing and displacing the surrounding structures. He underwent consecutive surgeries for complete excision of the tumor following which he regained his power in his upper and lower limbs over the period of 2 months. Conclusion: Para spinal mass with consistent radiological features suggests ganglioneuroma but confirmation should be done with biopsy. Complete excision of the tumor is the treatment of choice with close follow-up for clinical improvement and recurrence.

Invasive cutaneous squamous cell carcinoma of scalp: A case report.

Introduction and importance: Cutaneous Squamous cell carcinoma(cSCC) represents approximately 16% of scalp tumors. Overall, cSCC is significantly more common in the oropharyngeal mucosa than the skin. Smoking is a risk factor for mucosal lesions, risk factors for developing cutaneous SCC include age, ultraviolet light exposure, chronic scarring, history of ionizing radiation, androgenetic alopecia (in men), and immunosuppression. The rates of bone invasion in cutaneous SCC have not been well noted in the literature. Case report: We report a case of 57-year-old man with invasive squamous cell carcinoma of scalp with extension into bone, cortex and dura mater. Gross total removal of the tumor with extension of bony defect followed by repair of dura and repair of skin defect by VY advancement flap was done under general anaesthesia. At a 3-month follow-up, his wound is well healed. No evidence of metastasis is noted. Discussion: Cutaneous squamous cell carcinoma (cSCC) is the second most common non-melanoma skin cancer, and its incidence is steadily increasing. Although the majority of cSCCs are successfully eradicated by surgical excision, advanced cSCC poses a significant risk in terms of morbidity, impact on quality of life, and risk of death. Conclusions: Invasive cutaneous squamous cell carcinoma of scalp is a rare entity. Invasion to bone, cortex, and dura mater is furthermore rare. Therefore, proper management of advanced cSCC is of the utmost importance since local invasion, delayed diagnosis, and metastasis contribute to increased costs and morbidity. A multi-disciplinary team approach is recommended.

Giant occipital encephalocele complicated with obstructive hydrocephalus: A case report.

Introduction: and importance: An encephalocele is a type of congenital neural tube defect defined by herniation of intracranial contents via a cranial defect. When an encephalocele is greater than the size of the head, it is referred to as a "giant encephalocele." The occurrence of encephalocele has been documented to be 1-4 instances per 10,000 live births. Surgery is challenging. Case presentation: A 1-month-old baby boy sustained a huge swelling on the back of his head since birth, and it increased gradually over time. On examination, he had a huge occipital swelling measuring about 20 × 15 × 17 cm in size. A diagnosis of giant occipital encephalocele was established. Surgical excision and repair was done. After 1 month, he developed obstructive hydrocephalus and a ventriculo-periotoneal shunting was performed. On regular follow-up, he is in a good state of health. Discussion: Surgery imposes challenges for the anaesthesiologists and neurosurgeons due to its complex site, enormous size, intraoperative blood loss, and prolonged anaesthesia. A team approach is necessary for its successful treatment. Conclusions: Based on our experience, we would like to deliver following recommendations in the surgical management of giant encephalocele. Surgery should be done quickly to ensure good prognosis. Proper positioning, efficient intubation, infections and sepsis control should be emphasized. Also, fortification of food with folic acid, as well as increased education and awareness of women on the need for antenatal care may also decrease the risk of this disease.

Von Hippel-Lindau disease: A case report.

INTRODUCTION AND IMPORTANCE: Von Hippel-Lindau (VHL) disease is a rare autosomal dominantly inherited genetic condition. Von Hippel characterized the illness independently in 1911, and Lindau in 1926. Its prevalence is estimated to be about 1 in every 36,000 live births. VHL is characterized by the production of several benign and malignant tumors, as well as cysts in other organs. For proper prognosis, good clinical judgement and timely diagnosis is warranted. CASE PRESENTATION: Herein, we report a case of a 50-year-old man with several central nervous system (CNS) lesions, retinal lesions, and renal cortical cysts with a diagnosis of VHL disease who was surgically treated. At a 3-month follow-up, he improved drastically with a marked alleviation of his signs and symptoms. DISCUSSION: VHL is characterized by the creation of various benign and malignant tumors, as well as cysts in multiple organs, and is passed down through generations in an autosomal dominant pattern with near-complete penetrance. CNS lesions are surgically treated. Regular follow-up should be ensured. CONCLUSIONS: VHL disease is an extremely complicated disease with the need for diagnosis and genetic tests in the patient and family members, as well as intensive supervision of carriers of the mutated gene, thereby improving early diagnosis and successful treatment of the malignancies. The high cost of diagnostics and surgical therapies is a severe issue. Government care and financial assistance are critical considerations.