scholarly journals Bilateral angiomyolipoma of the kidney in patient with tuberous sclerosis

2005 ◽  
Vol 133 (9-10) ◽  
pp. 433-437
Author(s):  
Radoje Colovic ◽  
Natasa Colovic ◽  
Nikica Grubor ◽  
Vladimir Radak ◽  
Marijan Micev ◽  
...  
Keyword(s):  
Tuberous Sclerosis ◽  
Complete Loss ◽  
Hla Typing ◽  
Uneventful Recovery ◽  
Loss Of Function ◽  
Kidney Tumors ◽  
Benign Nature ◽  
The Right ◽  
Slow Growing

Angiomyolipomas are relatively frequent tumors of the kidney. It is believed that about 10 million people worldwide have such a tumor. About 1/10 of these 10 million are patients who suffer from tuberous sclerosis. The tumors are frequently bilateral, slow growing, and usually a symptomatic, as well as being rare in children. Due to the benign nature of angiomyolipomas, surgical treatment and embolisation of the tumors are generally not recommended, unless renal function is endangered, the symptoms are severe, or the kidney in question becomes completely dysfunctional. This is particularly the case in patients with tuberous sclerosis in whom these tumors are either already bilateral or may become so. We present a 24-year-old woman with tuberous sclerosis in whom bilateral kidney tumors were diagnosed 7 years earlier and in whom we carried out a left nephrectomy of a 5300 gram angiomyolipoma, which caused pain and complete loss of function. Although timorous, the right kidney was functional, so it was left untouched. After an uneventful recovery, a close follow-up was recommended, as well as HLA typing, as it is highly probable that the right kidney will gradually become inadequate or completely dysfunctional, so that haemodialysis and/or kidney transplantation along with nephrectomy will become necessary.

scholarly journals Tinnitus With Unexpected Spanish Roots: Head and Neck Paragangliomas Caused by SDHAF2 Mutation

2020 ◽  
Vol 4 (3) ◽  
Author(s):  
Laura Maria Roose ◽  
Niels J Rupp ◽  
Christof Röösli ◽  
Nadejda Valcheva ◽  
Achim Weber ◽  
...  
Keyword(s):  
Genetic Testing ◽  
Head And Neck ◽  
Carotid Body ◽  
Loss Of Function ◽  
Negative Family History ◽  
Paraganglioma Syndrome ◽  
Plasma Free Metanephrines ◽  
The Right ◽  
Head And Neck Paragangliomas

Abstract It is estimated that up to 40% of all head and neck paragangliomas (HNPGL) have a hereditary background with the most common mutations being found in the succinate dehydrogenase (SDH) genes. SDHAF2 mutation leads to the rare paraganglioma syndrome 2. The authors present the case of a 15-year-old male patient with 2, non-secretory HNPGLs, presenting with left-sided, pulsatile tinnitus, and hearing loss. Imaging led to the suspicion of a jugulotympanic paraganglioma on the left, as well as a carotid body tumor on the right. After resection of the jugulotympanic tumor, histology confirmed the presence of a paraganglioma; immunohistochemistry furthermore suggested a loss of SDHB expression. Genetic testing revealed a rare germline, loss-of-function mutation in the SDHAF2 gene, previously described to cause hereditary paraganglioma syndrome 2. Twenty months after the first operation, the patient underwent a resection of the right carotid body paraganglioma. Plasma-free metanephrines/catecholamines always remained within the reference range; the patient is under regular follow-up, and his relatives will be screened. Our findings emphasize the relevance of genetic testing in patients with HNPGL, also with negative family history, especially when the patients present at a young age and with multiple lesions.

scholarly journals Symptomatic attachment of appendix vermiformis following a plug inguinal hernia repair

2019 ◽  
Vol 12 (9) ◽  
pp. e220408
Author(s):  
Willem AR Zwaans ◽  
Marc RM Scheltinga ◽  
Rudi MH Roumen
Keyword(s):  
Inguinal Hernia ◽  
Hernia Repair ◽  
Groin Pain ◽  
Abdominal Discomfort ◽  
Uneventful Recovery ◽  
Appendix Vermiformis ◽  
The Right ◽  
Plug Repair ◽  
Groin Exploration

We describe a case of attachment of an appendix vermiformis following an inguinal hernia plug repair according to Rutkow and Robbins. A 62-year-old man presented at our outpatient clinic with a progressive sensation of tightness in the right groin area, painful urge of miction and long-lasting nausea with abdominal discomfort. During an open groin exploration, the appendix was found attached to an intraperitoneally located plug. The appendix and plug were removed with an uneventful recovery. During follow-up, the patient was free of groin pain and miction had normalised. Surprisingly, his long-lasting nausea and abdominal discomfort had disappeared as well.

PHAEOCHROMOCYTOMA OF URINARY BLADDER

1962 ◽  
Vol 39 (1) ◽  
pp. 1-12 ◽  
Author(s):  
R. Mićić ◽  
M. Kičić ◽  
S. Adanja
Keyword(s):  
Blood Pressure ◽  
Urinary Bladder ◽  
Arterial Hypertension ◽  
Bladder Wall ◽  
Short Review ◽  
Uneventful Recovery ◽  
Urinary Bladder Wall ◽  
The Right ◽  
Catecholamines In Urine

ABSTRACT A case of phaeochromocytoma localized in the urinary bladder wall above the right ureter in a 19 years old male is described. The patient had a sustained arterial hypertension between 170/120 and 260/170, without haematuria, with regular headache and palpitations immediately after urination. Catecholamines in urine 409 and 340 micrograms per day. A partial resection of the urinary bladder wall was performed with extirpation of a tumour weighing 40 g. Size of the tumour 80 × 60 × 60 mm. The tumour contained 1.5 mg adrenaline and 0.03 mg noradrenaline per g of tissue. Postoperatively blood pressure 140/90, catecholamines in the urine normal, and the patient made an uneventful recovery. The last follow-up ten months after operation. A short review of previously published cases is given.

Extradural developmental dural root sleeve cyst presenting as a lumbar paraspinal mass with renal compression in an infant

2010 ◽  
Vol 5 (6) ◽  
pp. 586-590 ◽  
Author(s):  
Balaji Srinivas ◽  
Vivek Joseph ◽  
Geeta Chacko ◽  
Vedantam Rajshekhar
Keyword(s):  
Marked Reduction ◽  
Left Kidney ◽  
Lower Limbs ◽  
Resonance Imaging ◽  
Abdominal Organs ◽  
Left Lower Limb ◽  
Extradural Cyst ◽  
The Right ◽  
Slow Growing

Spinal extradural cysts do not normally present as a visible paraspinal mass or cause compression of the abdominal organs. The authors describe the case of a 9-month-old boy with multiple spinal extradural cysts. The largest of these cysts was along the right L-2 nerve root with significant extraspinal extension resulting in a visible slow-growing swelling in the right paraspinal region and radiological evidence of compression of the right kidney with hydronephrosis. Another large cyst along the left T-12 root caused radiologically evident compression of the left kidney but to a lesser degree. The patient also had monoparesis of the left lower limb and phenotypic features of Noonan syndrome. The authors performed marsupialization of the cysts, as well as repair of the fistula between the subarachnoid space and the cyst on the right side along the L-2 root and on the left side along the T-12 root. At 1-year follow-up, there was no paraspinal mass and the lower limbs exhibited normal power. Magnetic resonance imaging confirmed marked reduction in the size of the cysts and relief of the renal compression. To the authors' knowledge, their patient is the youngest reported in literature to have a spinal extradural cyst and also the first with the cyst presenting as a paraspinal mass.

scholarly journals Intractable Trigeminal Neuralgia Secondary to Osteoma of the Clivus: A Case Report and Literature Review

2022 ◽  
Author(s):  
Ebtesam Abdulla ◽  
Krishna Das ◽  
Joseph Ravindra ◽  
Tejal Shah ◽  
Sara George
Keyword(s):  
Trigeminal Neuralgia ◽  
Pressure Effect ◽  
Benign Tumors ◽  
Radiographic Images ◽  
Patient Reported ◽  
Magnetic Resonance Imaging Mri ◽  
The Right ◽  
Slow Growing ◽  
Serial Observation

AbstractSkull base osteomas (SBOs) are benign tumors that are frequently detected on radiographic images by coincidence. They are known for being slow-growing tumors and rarely symptomatic. The therapeutic approach for SBOs can differ substantially. Depending on the symptoms, size, and location of the tumor, this can range from serial observation to vigorous surgical extirpation. Clival osteoma is extremely rare. We report a case of clival osteoma, causing intractable trigeminal neuralgia due to the pressure effect on the trigeminal nerve at Meckel's cave. We also provide a review of pertinent literature. A 37-year-old woman presented with intractable trigeminal neuralgia. Cranial magnetic resonance imaging (MRI) demonstrated a large, lobulated, extra-axial lesion involving the right cerebellopontine angle and epicentering the clivus. Pathologically, the specimen was proven to be osteoma. The patient reported complete symptom resolution over a 4-year follow-up period. To the best of the authors' knowledge, this is the first clinical case of intractable trigeminal neuralgia due to clival osteoma.

scholarly journals Localized Castleman’s Disease in the Breast in a Young Woman

2016 ◽  
Vol 2016 ◽  
pp. 1-3 ◽  
Author(s):  
Rafael Parra-Medina ◽  
José Ismael Guio ◽  
Patricia López-Correa
Keyword(s):  
Lymphoproliferative Disorder ◽  
Histopathological Examination ◽  
Castleman’S Disease ◽  
Unknown Etiology ◽  
Castleman's Disease ◽  
Axillary Lymphadenopathy ◽  
The Right ◽  
Slow Growing ◽  
Hyaline Vascular Variant

Castleman’s disease (CD) is a rare lymphoproliferative disorder of unknown etiology. It typically occurs in adulthood but it may also develop in childhood. Clinically, this disease may be classified as localized (unicentric) or systemic (multicentric). Six cases of breast CD have been described in the literature, and all have been reported in adults. Herein we describe the case of a 15-year-old female who presented with a slow-growing tumor in the right breast. The tumor was excised and histopathological examination demonstrated hyaline vascular variant CD. After two years of follow-up, the patient was asymptomatic without evidence of cervical or axillary lymphadenopathy.

scholarly journals A rare case of a benign giant chondroid syringoma of nose

2020 ◽  
Vol 6 (8) ◽  
pp. 1549
Author(s):  
Neemu Hage ◽  
Jaimanti Bakshi ◽  
Mayank Rampal
Keyword(s):  
Neck Region ◽  
Needle Aspiration ◽  
Disease Entity ◽  
Head And Neck Region ◽  
Chondroid Syringoma ◽  
Large Size ◽  
Benign Nature ◽  
Slow Growing ◽  
Disease Free

<p class="abstract">Chondroid syringoma (CS) is a rare benign appendageal tumour of the skin, with histological similarities to pleomorphic adenoma of salivary glands. It typically presents as a slow growing nodular mass generally less than 3 cm, arising from the skin, predominantly in the head and neck region. We report a case of a 61-year-old male with a massive nasal mass attained over a period of 2 years. A fine needle aspiration of the lesion was suggestive of CS. It was excised completely under general anaesthesia. The postoperative histopathology was also consistent with CS. The patient was followed up on a regular basis and was rendered disease free till his last follow up at 4 months. Our purpose of reporting this case is to highlight the rarity of the disease entity, as well as the occasion of it acquiring a large size yet, maintaining its benign nature.</p>

scholarly journals Giant renal artery aneurysm: A case report

2015 ◽  
Vol 87 (2) ◽  
pp. 169 ◽  
Author(s):  
Luca Cindolo ◽  
Manuela Ingrosso ◽  
Piergustavo De Francesco ◽  
Pietro Castellan ◽  
Francesco Berardinelli ◽  
...  
Keyword(s):  
Renal Artery ◽  
Three Dimensional ◽  
Artery Aneurysm ◽  
Uneventful Recovery ◽  
Renal Artery Aneurysm ◽  
Renal Arteriovenous Malformation ◽  
The Right ◽  
Midline Approach ◽  
Safe Approach

A case of a 12 cm giant renal artery aneurysm (RAA) in an 59-year-old woman is reported. The patient was referred to our hospital for flank pain and spot hematuria. Ultrasonography (US) revealed some wide lacunar areas in her right kidney and a thin cortex. Three-dimensional computed tomography (3D-CT) revealed a giant right renal arteriovenous malformation (AVM). AngioCT scan showed a pervious right renal artery. The cavities of the right kidney were dilated and the parenchyma was markedly reduced. Two months later the patient underwent an open resection of the aneurysm and a right nephrectomy. She had an uneventful recovery and a healthy status (last follow-up: 9 month). In this particular case, a safe approach is the transabdominal approach since the aneurysm was very large, friable, and located on the right side. This report confirms the opportunity of a planned nephrectomy once there is adequate renal reserve in the opposite kidney using a midline approach.

scholarly journals Schwannoma of the extracranial portion of the accessory nerve presenting as spinal adenopathy

2019 ◽  
Vol 12 (5) ◽  
pp. e228647
Author(s):  
Mohamed Mehdi El Fakiri ◽  
Meryem Lahjaouj ◽  
Mohammed Roubal ◽  
Mohammed Mahtar
Keyword(s):  
Sternocleidomastoid Muscle ◽  
Accessory Nerve ◽  
Histopathological Analysis ◽  
Rare Entity ◽  
Transcervical Approach ◽  
Benign Tumours ◽  
The Right ◽  
Slow Growing ◽  
Growing Mass

Schwannomas are benign tumours arising from Schwann cells in the peripheral nerve. The schwannoma of the accessory nerve is a very rare entity. We report a case of Schwannoma of the extracranial accessory nerve. A 22-year-old man presented with a slow-growing mass, located on the right upper neck. The patient did not have any neurological deficit. CT scan showed a hypodense mass behind sternocleidomastoid muscle. The suspected diagnosis was an adenopathy of the accessory spinal chain. Surgery was done via transcervical approach. The histopathological analysis concluded with a diagnosis of schwannoma. No recurrence was noted at the follow-up examination 29 months after surgery.

scholarly journals A Case Report of the Reconstruction of a Bone Defect Following Resection of a Comminuted Fracture of the Lateral Clavicle Using a Titanium Prosthesis

2021 ◽  
Vol 8 ◽  
Author(s):  
Sahar Ahmed Abdalbary ◽  
Sherif M. Amr ◽  
Khaled Abdelghany ◽  
Amr A. Nssef ◽  
Ehab A. A. El-Shaarawy
Keyword(s):  
Case Report ◽  
Bone Defect ◽  
Radiographic Evaluation ◽  
Loss Of Function ◽  
Limb Weakness ◽  
Element Analysis ◽  
Lateral Clavicle ◽  
Comminuted Fracture ◽  
The Right

Introduction: This case report describes the reconstruction of a severe comminuted fracture and bone defect in the lateral half of the clavicle using a novel titanium prosthesis. This unique prosthesis has been specifically designed and three dimensionally printed for the clavicle, as opposed to the Oklahoma cemented composite prosthesis used in common practice. The aims of this study were to: (1) describe the prosthesis, its stress analysis, and its surgical fixation and (2) to demonstrate the results of the 2-year follow-up of the patient with the lateral clavicle prosthesis.Patient's Main Concerns: A 20-year-old, right-handed woman complaining of severe pain in the right shoulder was admitted to our hospital following a traffic accident. Physical examination revealed pain, swelling, tenderness, limb weakness, asymmetric posturing, and loss of function in the right shoulder.Diagnosis, Intervention, and Outcomes: Radiographic evaluation in the emergency room showed complete destruction with a comminuted fracture of the lateral half of the right clavicle and a comminuted fracture of the coracoid. We designed a new prosthesis for the lateral half of the clavicle, which was then tested by finite element analysis and implanted. Use of the new prosthesis was effective in the reconstruction of the comminuted fracture in the lateral half of the clavicle. After 2 years of follow-up, the patient had an aesthetically acceptable curve and was able to perform her activities of daily living. Her pain was relieved, and the disabilities of the arm, shoulder, and hand score improved. Active range of motion of the shoulder joint and muscle strength were also improved.Conclusion: This novel prosthesis is recommended for reconstruction of the lateral half of the clavicle following development of bony defects due to fracture. Our patient achieved functional and aesthetic satisfaction with this prosthesis.

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