Rare Presentation of Giant Cell Tumor in the Internal Auditory Canal: Case Report and Review of the Literature.
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ABSTRACT:
SUBMITTER: Jada AS
PROVIDER: S-EPMC4520973 | biostudies-other | 2015 Jul
REPOSITORIES: biostudies-other
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Project description:Background:Exostoses of the internal auditory canal is a rare finding that may present with disabling symptoms of dizziness, hearing loss, and vestibular dysfunction based on the extent of cranial nerve compression. The purpose of this case report is to discuss the presentation and outcomes in a patient who presented with this disorder. Case Description:A 19-year-old female presented to the neurotologist with left ear discomfort, pain with left lateral gaze, and dizziness. She underwent extensive evaluation including audiometric testing, videonystagmography, and neuroimaging, which confirmed left auditory and vestibular hypofunction and compression of the contents of the internal auditory canal from the exostosis. After extensive counseling, the patient elected to undergo a suboccipital craniectomy to remove the internal auditory canal exostosis. She experienced complete resolution of symptoms. Conclusions:Exostoses of the internal auditory canal, although rare, can present with severe symptoms of dizziness, hearing loss, and vestibular hypofunction based on the extent of cranial nerve compression. Imaging, particularly with thin-cut computed tomography, is invaluable in making the correct diagnosis. Severe cases can be treated successfully with surgery with minimal or no complications and excellent outcome.
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Project description:IntroductionMucocele is a slow growing, benign but locally aggressive cystic structure lined by true epithelium. It often results due to obstructed sinus outflow or obstruction of gland-like mucous retention cyst. It can cause bony destruction and might result in orbital symptoms like diplopia, orbital displacement, visual disturbances. Other clinical features are facial numbness, dental problems, etc. Radiological evaluation is the preferred diagnostic modality. Surgical removal is the treatment of choice both endoscopic and open (could well luc) approach or combined approach are preferred. Here we report a very typical case of maxillary mucocele who presented with subtle symptoms of nasal obstruction. The study was done in compliance with SCARE guidelines.[1].Case presentationWe present a very unique case of 24 years man with complaints of nasal obstruction and swelling over the right cheek for 2 years. He had a history of facial trauma two years back. Diagnosis was made on the basis of radiological examination CT (Computed Tomography) scan. He underwent enucleation via Cold well Luc's approach with good postoperative results.ConclusionMaxillary mucoceles are slow growing benign lesions. However, they are locally aggressive and cause bony destruction resulting into orbital and dental symptoms. Thus early recognistion with regular folllowr up and planning for surgical intervention can help avoid complications.
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Project description:Uterine leiomyosarcoma (uLMS) is a rare and aggressive malignancy with poor clinical outcomes. Even when localized, uLMS is associated with high rates of local and distant recurrences that are usually fatal. Common sites of recurrence are lung, liver, pelvic lymph nodes, and vertebral and long bones, though atypical patterns of recurrence have been described. Among them, intracranial recurrence appears as a rare finding, almost exceptional in skull and dura. We describe the case of a solitary skull metastasis from uLMS in a 39-year-old woman, which represents the third reported case of skull recurrence in literature. After multidisciplinary discussion, the patient underwent surgery and received adjuvant radiotherapy. After 4 months, she is currently alive, without evidence of extracranial disease. This case highlights the importance of suspecting and recognizing atypical and extremely rare metastasis to this region. We encourage the need for large case series in order to provide further information about cranial recurrences of uLMS taking into account the paucity of data currently available in literature and the frequently unpredictable behavior of this rare and highly lethal disease.
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Project description:Introduction and importanceRetroperitoneal liposarcomas (RPLS) are usually grow large with frequent recurrences. Complete surgical excision remains the gold standard treatment for primary and even recurrent tumours. Their prognosis depends on their histological type and grade. We report a recurrent giant de-differentiated RPLS weighing 18.55 kg which was completely excised. To the best of our knowledge, this is one of the largest liposarcoma reported in the literature.Case presentationA 40 year old female presented with a gradually progressing large abdominal lump for 1year. She had had a similar large lump twice in the past and undergone excision of the tumour elsewhere. Firm non-tender mass felt all over abdomen with edema noted over abdominal wall and bilateral lower limbs. PET CT showed large heterogeneously enhancing mass occupying almost the entire abdominopelvic cavity. 50 × 40 × 40cm tumour was completely excised and biopsy showed grade 2 dedifferentiated liposarcoma (DDLS). She is under close follow up with no recurrence at 12months.Clinical discussionDDLS have lower risk of distant metastases but have a high risk of local recurrence. The most important favourable prognostic factor in these tumours is complete resection with negative margins. Because of the ineffectiveness of current chemotherapy and the requirement of intolerably high radiation doses, surgical excision remains the most effective treatment even for the localized recurrences of RPLS.ConclusionThe dedifferentiated subtype should be suspected in locally aggressive RPLS. Close follow up with early detection of recurrences and prompt excision with negative margins lowers the risk of recurrences and improves survival.
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Project description:IntroductionHidradenoma papilliferum, also known as papillary hidradenoma, is a rare tumor of the sudoral glands, that occurs almost exclusively in women, on the vulvar and perineal region. Non-genital papillary hydradenoma is an even more rare occurrence, though some cases have been described in literature.This is a report of a 56 years old patient who presented with unilateral hearing loss secondary to a mass of the external auditory canal (EAC). A biopsy allowed us to make the diagnosis of a papillary hidradenoma of the EAC. The treatment was based on surgery; the tumor was removed while respecting the adjacent structures. Follow-up of the patient shows no actual recurrence.ConclusionEctopic Hidradenoma papilliferum located in the EAC is a rare occurrence, and should be considered as a possible diagnosis when investigating a mass of the EAC.
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Project description:The estimated incidence of pediatric testis tumor is 0.5-2.0 per 100,000 children, accounting for 1-2% of all pediatric tumors. Mixed germ cell tumors (MGCT) in prepubertal males are exceedingly rare, with only one previous case report found in the literature. We report a case of a MGCT in an infant. For prepubertal males, GCTs typically present with a painless scrotal mass, though trauma, testis torsion and hydrocele are also common presentations. Similar to such tumors in postpubertal males, ultrasonography, computed tomography, and tumor markers are integral to determine the best treatment. The patient described in this report presented with a painless scrotal mass. Following orchiectomy, the patient was found to have MGCT that was limited to the testis. With prudent management, these patients tend to have favorable prognoses.
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Project description:Intracardiac masses are unusual findings in infants, and most of them are benign. Nevertheless, they may be associated with a significant degree of hemodynamic instability and/or arrhythmias. Malignant tumors of the heart rarely occur in children. Rhabdoid tumors are aggressive tumors with a dismal prognosis even when diagnosed early. Although rhabdomyomas are common cardiac tumors in infants, they are mostly benign. The most common sites of involvement are the kidneys and central nervous system, but soft tissues, lungs, and ovaries may also be affected. The diagnosis can be challenging, particularly in sites where they do not usually occur. In the present paper, we report the case of a 2-year-old boy diagnosed with cardiac rhabdoid tumor highlighting the importance of molecular studies and recent genetic discoveries with the purpose of improving the management of such cases. The aim of this educational case report and literature review is to raise awareness of cardiac masses in children and to point out diagnostic hints toward a cardiac tumor on various imaging modalities. Given the rarity of all tumors involving the heart and the lack of symptom specificity, a high degree of suspicion is needed to arrive at the correct diagnosis.
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Project description:Cherubism is characterized by progressive, painless, bilateral enlargement of the mandible and/or maxilla resulting from the replacement of bone with multilocular cysts composed of fibrotic stromal cells and osteoclast-like cells. Here we report one Chinese cherubism case that has been misdiagnosed for more than forty years. The patient displayed no typical clinical or radiographical signs of cherubism due to multi-surgical treatments. Her histopathologic examination revealed the proliferating fibrous connective tissue with few multinucleated giant cells. The family history suggested us to perform sequence analysis of the SH3BP2 gene, a candidate marker for cherubism, in the family, and it was found that both the proband and the son had a missense mutation in SH3BP2 in exon 9 (p. Arg415Gln). Here we emphasize the importance of gene testing in the diagnosis of suspected cherubism, especially for those cases with non-typical clinical, radiographic and histological presentations.
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Project description:Introduction and importanceBrown tumor (BT) is defined as osteolytic lesion of an underlying state of hyperparathyroidism. Hyperparathyroidism will activate osteoclasts which initiate active bone resorption foci of lytic-cysts with hemosiderin depositions that pigment it with its characteristic brown pathologic gross appearance. Devastating fractures and injuries can occur to affected bones and surrounding tissue that require emergent intervention and correction.Case presentationWe present a case of a medically free 31-year-old female patient, who presented complaining of unsteadiness and progressive lower limbs weakness over 40 days of duration. Subsequent lab tests showed elevated PTH levels, along with 3.5 × 1.8 cm heterogeneous soft tissue mass involving the right pedicle on T7 level compressing the corresponding level of the spinal cord. Surgical management aimed to decompress the spinal cord and to obtain a biopsy for histopathologic examination which revealed a brown tumor. Neck ultrasound and Sestamibi scan indicated the presence of hyperactive and hyperplastic parathyroid tissue most suggestive of parathyroid adenoma.Clinical discussionVarious presentations of Brown Tumor depend on the bone affected, despite the rarity of spinal involvement, yet expanding tumors can manifest either with back pain, radicular pain, paresthesia, weakness, paralysis, or incontinence. The highest incidence rates of spinal brown tumors affect adults over the age of 40. Management goals are to decompress the neuronal tissue emergently and to prevent further bony lytic deterioration.ConclusionThe objective of this study is to provide an overview of primary hyperparathyroidism-related spinal brown tumors, presentation, and summary of previously reported similar cases in the literature.
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Project description:There have been relatively few reports of bilateral internal auditory canal metastases of asymptomatic primary pulmonary adenocarcinoma presenting as unilateral sudden sensorineural hearing loss and vertigo. We report a case of a 60-year-old male patient who complained of sudden hearing loss in the right ear and vertigo. Upon a physical examination, no definite neurological signs or nystagmus were observed. Pure-tone audiometry showed deafness in the right ear at all frequencies and high-frequency sensorineural hearing loss in the left ear. The video head impulse test suggested bilateral vestibulopathy. Magnetic resonance imaging of the brain (with gadolinium contrast) revealed bilateral internal auditory canal enhancement and a variable-sized nodular and peripheral-enhancing lesion in the cerebrum and the right cerebellum. A computed tomographic and bronchoscopic biopsy identified asymptomatic primary pulmonary adenocarcinoma in the left upper lobe of the lungs. This is a rare report of bilateral internal auditory canal metastases in an asymptomatic patient with primary pulmonary adenocarcinoma who initially presented with symptoms of unilateral sudden sensorineural hearing loss with vertigo.
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