scholarly journals Interesting pancreatic tumour in the background of Tuberous Sclerosis

2019 ◽  
Vol 12 (8) ◽  
pp. e227292 ◽  
Author(s):  
JMV Amarjothi ◽  
Jeyasudhahar Jesudason ◽  
Villalan Ramasamy ◽  
OL Naganath Babu
Keyword(s):  
Tuberous Sclerosis ◽  
Tuberous Sclerosis Complex ◽  
Early Identification ◽  
Neuroendocrine Tumours ◽  
Surgical Excision ◽  
Complete Surgical Excision ◽  
Pancreatic Tumour ◽  
Pancreatic Neuroendocrine Tumours ◽  
High Index Of Suspicion ◽  
Abdominal Tumours

Tuberous Sclerosis Complex (TSC) is easily discernible by a myriad of manifestations, most notably dermatological. It is associated with well known and recognised intra-abdominal tumours like angiomyolipoma of the kidney. However, rarer tumours like pancreatic neuroendocrine tumours can occur in the setting of TSC. A high index of suspicion is necessary to identify and treat these lesions early in their natural course. Early identification augurs well with complete surgical excision and excellent survival.

scholarly journals Tuberous sclerosis with cardiac rhabdomyoma: a case report

2017 ◽  
Vol 4 (2) ◽  
pp. 666
Author(s):  
Sunita Arora ◽  
Harnoorjit Kaur Brar ◽  
Prabhjot Kaur Dhillon
Keyword(s):  
Tuberous Sclerosis ◽  
Tuberous Sclerosis Complex ◽  
Genetic Disorder ◽  
Clinical Manifestations ◽  
Female Child ◽  
The Body ◽  
Cardiac Rhabdomyoma ◽  
Cardiac Evaluation ◽  
Benign Tumours ◽  
High Index Of Suspicion

Tuberous sclerosis or tuberous sclerosis complex (TSC) is a genetic disorder, caused by mutations on either of two genes TSC1 and TSC2. Clinical manifestations are caused by growth of benign tumours in different parts of the body. Ten months old female child with four major criteria of tuberous sclerosis complex and asymptomatic cardiac rhabdomyoma is presented. A case of TSC warrants cardiac evaluation for the presence of cardiac rhabdomyoma and if a cardiac rhabdomyoma is detected on antenatal ultrasound or postnatal echocardiography, one should have high index of suspicion for the diagnosis of TSC. Continued research on this disease has unfolded many realities regarding its etiology as well as treatment.

scholarly journals Malignant pancreatic tumour within the spectrum of tuberous sclerosis complex in childhood

1999 ◽  
Vol 158 (4) ◽  
pp. 284-287 ◽  
Author(s):  
S. Verhoef ◽  
R. van Diemen-Steenvoorde ◽  
W. L. Akkersdijk ◽  
N. M. A. Bax ◽  
Y. Ariyurek ◽  
...  
Keyword(s):  
Tuberous Sclerosis ◽  
Tuberous Sclerosis Complex ◽  
Pancreatic Tumour

scholarly journals Nasal Septal Angioleiomyoma and Septal Sinus Surgery: A Review of 2 Cases

2021 ◽  
pp. 014556132199132
Author(s):  
Gabriele Noreikaite ◽  
Sihun A. Kim ◽  
Adelaide Horcher
Keyword(s):  
Smooth Muscle ◽  
Nasal Septum ◽  
Surgical Excision ◽  
Sinus Surgery ◽  
Rare Tumor ◽  
Imaging Findings ◽  
Nasal Surgery ◽  
Single Institution ◽  
Complete Surgical Excision ◽  
High Index Of Suspicion

Angioleiomyomas are smooth muscle masses originating from blood vessel tunica media. This is a rare tumor, comprising less than 1% of benign sinonasal cavity tumors. Failure to recognize and surgically excise nasal angioleiomyomas can result in lesion recurrence. We present 2 new cases of nasal septum angioleiomyoma at a single institution. Additionally, both cases underwent septal and nasal surgery. Due to the nonspecific clinical and imaging findings, a high index of suspicion is needed to diagnose nasal septum angioleiomyoma, often requiring histopathological verification. Preferred treatment is complete surgical excision. From our experience, concurrent corrective septal and sinus surgery can be completed with tumors less than 1.0 cm in size.

scholarly journals Vigabatrin-Induced Encephalopathy in a 5.5-Month-Old Girl with Infantile Spasms due to Tuberous Sclerosis

2019 ◽  
Vol 2019 ◽  
pp. 1-3 ◽  
Author(s):  
Eleni Klinaki ◽  
Ioanna Argyri ◽  
Georgia Amountza ◽  
Gerina Ioannidou ◽  
Despoina Maritsi ◽  
...  
Keyword(s):  
Tuberous Sclerosis ◽  
Tuberous Sclerosis Complex ◽  
Adrenocorticotropic Hormone ◽  
Infantile Spasms ◽  
High Index ◽  
Female Infant ◽  
Eeg Abnormalities ◽  
High Index Of Suspicion ◽  
Electroencephalogram Eeg

A 5.5-month-old female infant with tuberous sclerosis complex presented with infantile spasms and was treated with vigabatrin. As her condition did not improve, she was given adrenocorticotropic hormone (ACTH) intramuscularly which stopped the spasms and improved the electroencephalogram (EEG) abnormalities. However, she developed encephalopathy with apathy, drowsiness, and generalized slowing in the EEG. Discontinuation of vigabatrin quickly improved her symptoms and reversed the EEG slowing. A high index of suspicion is required in order to diagnose vigabatrin-induced encephalopathy, especially as the underlying disorders of these patients can be erroneously considered the cause of the observed encephalopathy.

scholarly journals Updated Principles of Surgical Management of Pancreatic Neuroendocrine Tumours (pNETs): What Every Surgeon Needs to Know

Cancers ◽  
2021 ◽  
Vol 13 (23) ◽  
pp. 5969
Author(s):  
Charles de Ponthaud ◽  
Fabrice Menegaux ◽  
Sébastien Gaujoux
Keyword(s):  
Surgical Management ◽  
Neuroendocrine Tumours ◽  
Pancreatic Neoplasm ◽  
Distant Metastases ◽  
High Volume ◽  
Invasive Approach ◽  
Pancreatic Tumour ◽  
Functional Consequences ◽  
Pancreatic Neuroendocrine Tumours ◽  
Selection Of

Pancreatic neuroendocrine tumours (pNETs) represent 1 to 2% of all pancreatic neoplasm with an increasing incidence. They have a varied clinical, biological and radiological presentation, depending on whether they are sporadic or genetic in origin, whether they are functional or non-functional, and whether there is a single or multiple lesions. These pNETs are often diagnosed at an advanced stage with locoregional lymph nodes invasion or distant metastases. In most cases, the gold standard curative treatment is surgical resection of the pancreatic tumour, but the postoperative complications and functional consequences are not negligible. Thus, these patients should be managed in specialised high-volume centres with multidisciplinary discussion involving surgeons, oncologists, radiologists and pathologists. Innovative managements such as “watch and wait” strategies, parenchymal sparing surgery and minimally invasive approach are emerging. The correct use of all these therapeutic options requires a good selection of patients but also a constant update of knowledge. The aim of this work is to update the surgical management of pNETs and to highlight key elements in view of the recent literature.

scholarly journals Utility of the Autism Observation Scale for Infants in Early Identification of Autism in Tuberous Sclerosis Complex

2017 ◽  
Vol 75 ◽  
pp. 80-86 ◽  
Author(s):  
Jamie K. Capal ◽  
Paul S. Horn ◽  
Donna S. Murray ◽  
Anna Weber Byars ◽  
Nicole M. Bing ◽  
...  
Keyword(s):  
Tuberous Sclerosis ◽  
Tuberous Sclerosis Complex ◽  
Early Identification ◽  
Observation Scale
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