scholarly journals Primary myxoid temporal bone tumor: A rare neurosurgical manifestation of Carney complex?

2020 ◽  
Vol 11 ◽  
pp. 166
Author(s):  
Tobechi Nwankwo Mbadugha ◽  
Kohei Kanaya ◽  
Tetsuyoshi Horiuchi ◽  
Mai Iwaya ◽  
Samuel Chukwunonyerem Ohaegbulam ◽  
...  

Background: Carney complex (CNC) is a rare autosomal dominant syndrome, manifesting mainly with cardiac, cutaneous, and mucosal myxomas. Osteochondromyxoma is known as an extremely rare bone lesion of CNC which usually appears early in life; however, there were no reports of primary bone myxoma of the skull in the patients with CNC. We present the first case of primary myxoid skull tumor in the patient with CNC. Case Description: We report the left temporal bone tumor with significant intracranial mass effect in a 58-year- old woman already diagnosed with CNC. Complete resection of the tumor with skull bone reconstruction was carried out. Pathological diagnosis was labeled the lesion as an atypical myxoid spindle cell neoplasm. The features were different from atrial myxoma and osteochondromyxoma which has been described in CNC. There have been no signs of recurrence in 9 years follow-up. Conclusion: To the best of our knowledge, there have been no reports of the primary myxoid tumors in the skull in the patients with CNC. This paper highlighted a possible important association between CNC and primary intracranial myxoid tumors.

2017 ◽  
Vol 20 (4) ◽  
pp. 329-333 ◽  
Author(s):  
Jarod L. Roland ◽  
Richard L. Price ◽  
Ashwin A. Kamath ◽  
S. Hassan Akbari ◽  
Eric C. Leuthardt ◽  
...  

The authors describe 2 cases of triventricular hydrocephalus initially presenting as aqueductal stenosis that subsequently developed tumors of the pineal and tectal region. The first case resembled late-onset idiopathic aqueductal stenosis on serial imaging. Subsequent imaging revealed a new tumor in the pineal region causing mass effect on the midbrain. The second case presented in a more typical pattern of aqueductal stenosis during infancy. On delayed follow-up imaging, an enlarging tectal mass was discovered. In both cases hydrocephalus was successfully treated by cerebrospinal fluid diversion prior to tumor presentation. The differential diagnoses, diagnostic testing, and treatment course for these unusual cases are discussed. The importance of follow-up MRI in cases of idiopathic aqueductal stenosis is emphasized by these exemplar cases.


2015 ◽  
Vol 2015 ◽  
pp. 1-4
Author(s):  
Remi Goupil ◽  
Annie-Claire Nadeau-Fredette ◽  
Virginie Royal ◽  
Alexandre Dugas ◽  
Jean-Philippe Lafrance

Lupus nephritis is a frequent manifestation of systemic lupus erythematous. Lupus nephritis usually presents with abnormal urinalysis, proteinuria, and/or renal insufficiency. We report a case of a 48-year-old woman who underwent partial nephrectomy for a fortuitously discovered solid enhancing left kidney mass. No neoplastic cells were found in the biopsy specimen; however, the pathology findings were compatible with immune complex glomerulonephritis with a predominantly membranous distribution, a pattern suggestive of lupus nephritis. The mass effect was apparently due to a dense interstitial lymphocytic infiltrate resulting in a pseudotumor. Further investigation revealed microscopic hematuria with a normal kidney function and no significant proteinuria. Antinuclear antibodies were negative, although anti-DNA and anti-SSA/Rho antibodies were positive. A diagnosis of probable silent lupus nephritis was made and the patient was followed up without immunosuppressive treatment. After two years of follow-up, she did not progress to overt disease. To our knowledge, this represents the first case of lupus nephritis with an initial presentation as a renal mass.


1994 ◽  
Vol 108 (12) ◽  
pp. 1115-1119 ◽  
Author(s):  
H. K. Leong ◽  
P. Y. Chong ◽  
R. Sinniah

AbstractChondroblastoma represents approximately one per cent of all primary bone tumours. It is even rarer in the temporal bone and so far only 34 cases have been reported. We report here two cases with chondroblastoma of the temporal bone. The first case was discovered as a small lesion of the attic and root of zygoma. It was removed via mastoidectomy and reconstruction of the bony defect achieved normal external ear canal anatomy and hearing post-operatively. The second case presented as an advanced tumour involving the infratemporal fossa and parapharyngeal space. It was treated surgically via the infratemporal fossa approach. As clear surgical margins were not obtained, post-operative radiotherapy was also given to minimize the chance of recurrence.


2018 ◽  
Vol 27 (1) ◽  
pp. 52-54 ◽  
Author(s):  
Fazal Wahab Khan ◽  
Ibrahim Zahid ◽  
Sarosh Moeen ◽  
Sulaiman Bashir Hasan

Chondrosarcoma is a cancer of cartilage cells, and despite being a common primary bone tumor, tracheal chondrosarcoma is rare with only 18 cases reported in the literature prior to mid-2016. A 60-year-old gentleman presented with progressively increasing cough, severe stridor, and production of phlegm for approximately 2 years. On admission to our tertiary care hospital, he developed complete obstructive apnea within an hour, and was intubated. A tracheal biopsy was performed, followed by resection. Histopathology confirmed chondrosarcoma of the trachea. The patient tolerated the procedure very well and is currently symptom-free on follow-up, with no signs of recurrence.


2018 ◽  
Vol 79 (04) ◽  
pp. e79-e82
Author(s):  
Buse Sarigul ◽  
Ece Uysal ◽  
İdris Avci ◽  
Halil Peker ◽  
Suat Celik

AbstractEwing's sarcoma (EWS) is the second most common primary bone tumor seen in children and adolescents, first being osteosarcoma. Skull lesions are seen in 6 to 9% of cases. The tumor tends to reside most frequently on frontal and parietal bones. In the literature, majority of papers on calvarial EWS present cases with intracranial involvement; extracranial extension is reported in only eight cases. The case that we are presenting in this report is a 23-year-old male patient with multiple infiltrations in femur, costal bones, and calvarium which had been diagnosed radiologically and histopathologically as EWS. The calvarial metastasis had reached a very huge size with dimensions of approximately 7.5 × 7.5 × 9.5 cm and was successfully excised totally. Twelve months of follow-up revealed no recurrence in the surgical site showing that total removal of giant cranial EWS may improve morbidity of these patients.


Author(s):  
Valentina Testini ◽  
Laura Eusebi ◽  
Willy Giannubilo ◽  
Vincenzo Ferrara ◽  
Francesco Bartelli ◽  
...  

Abstract Background Adrenal hemangioma is a rare and non-functional benign tumor. Since the first case in 1955, approximately 90 cases have been reported in literature frequently presenting as a large and incidentally discovered retroperitoneal mass or as a result of hemorrhage caused by spontaneous rupture. Case presentation A 69-year-old man was admitted to our hospital for right hypocondrium pain and anemia. A computed tomography (CT) scan was performed and a large right adrenal mass measured 18 cm was found. The patient underwent laparoscopic right adrenalectomy. Histopathological diagnosis revealed a cavernous adrenal hemangioma. Conclusion Cavernous hemangiomas are uncommon tumors. We reported a case of adrenal cavernous hemangioma incidentally discovered by ultrasound and CT. In the case of finding adrenal masses, cavernous hemangiomas, although rare, should be considered a possible diagnosis. The appropriate treatment is surgical resection to rule out any potential for malignancy, to relieve symptoms secondary to the mass effect, and to prevent complications such as retroperitoneal bleeding.


2017 ◽  
Vol 6 (1) ◽  
pp. 39-42
Author(s):  
Jerzy Kuczkowski ◽  
Wojciech Brzoznowski ◽  
Tomasz Nowicki ◽  
Jolanta Szade

The aim of this paper is to present the case of a 70-year-old women with endolymphatic sac tumor and temporal bone destruction treated at Otolaryngology Department of MUG. The patient was admitted to our Department due to a 3-year history of hearing loss, dizziness and ear pain. The first diagnosis was temporal bone tumor connected with von Hippel-Lindau syndrome (VHL). The patient was surgically treated. During intraoperative examination, a neoplasm was determined. The tumor was excised via transmastoid approach with sigmoid sinus skeletonization. After treatment, her pains disappeared. Histopathological and immunohistochemical examination revealed endolymphatic sac tumor. Follow-up CT showed no tumor remission.


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