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The article "Giant rhabdomyoma: an atypical cause of ST segment elevation in the newborn" published in the Archives of Cardiology of Mexico, describes the case of a newborn with multiple cortical masses diagnosed prenatally. An elevation of the ST segment was observed in certain leads on the electrocardiogram, and an echocardiogram revealed rhabdomyomas in various areas of the heart, one of them being giant. The final diagnosis was complex tuberous sclerosis, and the patient was managed conservatively with multidisciplinary follow-up. [Extracted from the article]

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A 33­year­old female patient with a history of tuberous sclerosis diagnosed at 3 years of age, went to the emergency room due to hematuria, distension, and intense abdominal pain, predominantly on the left side. Among the previous studies observed in the PACS, we have abdominal ultrasound from 10 years ago that reported innumerable round and hyperechogenic injuries in relation to an­giomyolipomas smaller than 4.0 cm. She was evaluated by the urology department, which revealed a soft, distended abdomen and an abdominal mass of solid. [ABSTRACT FROM AUTHOR]

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The tuberous sclerosis complex also called Bourneville disease is a phacomatosis characterized by neurological alterations with different degrees of intellectual disability and epilepsy as well as the formation of multiple hamartomas in different organs and systems, it has genetic and clinical diagnostic criteria which require imaging studies for proper diagnosis, surveillance and decision making. We present 3 cases with a final diagnosis of tuberous sclerosis complex in our center, sent for clinicalsuspicion of tuberous sclerosis whichwe consider show the most common lesions in the central nervous system and abdomen, mainly cortical tuberosities, subependymal nodules, subependymal giant cells astrocytoma andoangiomyolipomas. [ABSTRACT FROM AUTHOR]

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Introduction: Tuberous sclerosis is an autosomal dominant genetic disease with highly variable penetrance, characterized by skin lesions, mental retardation and the appearance of multiple tumors. Objective: To contribute to the knowledge of this disease with emphasis on the usefulness of clinical knowledge and imaging characteristics for diagnosis. Case report: We report a 3-month-old female infant, who at 2 months of age began to have paroxysmal events that started with interrupting activity, blinking of the right eye followed by cephalic version to the right, oro-alimentary automatisms and dystonic posture of both upper limbs, during short periods (30 seconds) with spontaneous recovery without sequelae. These events occurred several times a day. Hypomelanotic spots were found on the skin; as well as a confettishaped lesion at the upper dorsal level and left axillary region. Magnetic resonance imaging was performed showing the presence of subependymal nodules and cortical tubercles. Treatment with sodium valproate and Vigabatrin was started with positive evolution. Conclusions: The case reported is an example of a patient with tuberous sclerosis due to a de novo mutation, with a typical clinical-imaging picture, which allowed the diagnosis; with no history of affected relatives and with improvement favored by the treatment used. [ABSTRACT FROM AUTHOR]

Introduction: Tuberous sclerosis is a rare disorder with multisystemic clinical manifestations that can compromise vital organs such as the kidney, lung and heart, which requires early diagnosis to provide timely and targeted treatment, improving the prognosis and reducing the morbidity and mortality attributed to these pathologies. Objective: To establish the importance of the use of genomics and the phenotype-genotype correlation for the diagnosis, treatment, follow-up, prognosis, genetic counseling of tuberous sclerosis. Materials and methods: Case report of a 15 year old patient with body angiofibromas, retinal hamartoma, right angiomyolipoma and alterations in neuroimaging studies without seizures or neurobehavioral disorders, clinically suspected of tuberous sclerosis with genetic confirmation by having a pathogenic variant in heterozygosity in the TSC2 gene. Results: A pathogenic heterozygous deletion was found in which a cytosine is changed at position 2539 of the TSC2 gene cDNA (c.2539delC), leading to a premature stop codon at amino acid 893 (p.Leu847Cysfs*47) into a protein of 1,807 amino acids with pathogenic clinical significance. Conclusions: Tuberous sclerosis complex is an orphan disease for Colombia given the low population prevalence, with a high burden of morbidity and mortality due to multisystem involvement. Its confirmation is performed by molecular-genomic methods that allow establishing phenotype-genotype correlation given the variability in the variants reported in this gene and the different degree of phenotypic expression in individuals, which guides us to look for signs and symptoms of involvement of organs or systems possibly affected, approaching a personalized and precision medicine. [ABSTRACT FROM AUTHOR]

Cardiac tumors are rare in children, having a maximum reported incidence of 0.027% in prenatal diagnosis, increasing the incidence in necropsy diagnosis; rhabdomyomas are the most frequent cardiac tumors, some cases are associated with tuberous sclerosis. We present the report of two cases in our unit that were diagnosed prenatal with follow-up after birth and one of them was associated with tuberous sclerosis. Both cases were admitted in a third level center, uncomplicated, without requiring surgical treatment, and could be discharged. [ABSTRACT FROM AUTHOR]

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Introduction: Rhabdomyomas are the most prevalent benign heart tumors in the fetal stage and during childhood. Objective: Our objective is to make known our experience over 39 years in pediatric patients with rhabdomyoma in our institution, as well as to emphasize the importance of its detection, study and control due to the association it has with tuberous sclerosis (TS). Material and methods: We conducted a retrospective, descriptive and cross-sectional study, from January 1980 to March 2018. Twenty-four met our criteria, we collected information regarding the clinic and cabinet studies, as well as their evolution and the treatment given. They were called by telephone to know their evolution and current status. Results: We found 51 patients with a diagnosis of cardiac tumor, of which 24 were rhabdomyomas. The diagnosis was made prenatal in 8 patients, 5 at birth and in 11 during the first year of life. The most frequent clinical manifestations were the presence of murmur, arrhythmias, cyanosis, dyspnea, and diaphoresis. In 17 of them a diagnosis of TS was made. Half had follow-up by neurology, 10 by dermatology, 8 by ophthalmology and 4 with genetics. Half were left under surveillance, 7 were given medical treatment and 5 required surgery. Regarding the clinical evolution 17 patients remained stable, 5 presented spontaneous regression and 2 died. Conclusions: Rhabdomyoma is a rare benign tumor, its evolution can be malignant and associated with TS, it darkens the prognosis. [ABSTRACT FROM AUTHOR]

BACKGROUND: Köenen tumors are cutaneous manifestations of tuberous sclerosis, a genodermatosis with systemic involvement. The knowledge of these tumors is important for the correct and early treatment of this entity, that may be difficult to diagnose when the characteristic triad is not available. CLINICAL CASE: A 42-year-old female patient who reported the appearance of small tumors surrounding the nail at 16 years of age, which progressively grew until they were uncomfortable. Upon physical examination, multiple dermatoses were found in several fingers and toes, consisting of smooth, skin-colored neoformations, approximately 5 mm with little hyperkeratosis at the tip, white in consistency and in some with canaliform distribution of the nail plate. CONCLUSIONS: The existence of multiple nail fibroids is key to perform the corresponding approach in search of tuberous sclerosis, because they may be the first and only cutaneous manifestation of this disease. [ABSTRACT FROM AUTHOR]

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Objetivo: Describir las características clínico-patológicas de pacientes con Astrocitoma de Células Gigantes Subependimario (SEGA), en el INNN (2013-2022) y evaluar el perfil de expresión de nuevos marcadores mediante inmunohistoquímica (IHQ). Antecedentes: El SEGA es un tumor benigno de bajo grado, generalmente asociado al complejo de esclerosis tuberosa (TSC), un síndrome hereditario autosómico dominante, por mutación en los genes TSC1 o TSC2. Métodos: Realizamos un estudio descriptivo, retrospectivo y comparativo, bajo el protocolo 36/23, que incluyó a todos los pacientes con diagnóstico de SEGA que fueron sometidos a cirugía entre enero de 2013 y diciembre de 2022. Se obtuvieron datos clínicos y paraclínicos de expedientes. Se aplicó panel de IHQ (NF, GFAP, hamartina, tuberina, Ki67, nestina, OCT-4, INI1, STAT-6, CKAE1/AE3). Se realizó análisis estadístico de los datos recolectados. Resultados: Se incluyeron 4 pacientes, dos de los cuales (23 y 25 años) tenían diagnóstico previo de TSC (10 y 12 años) y dos (18 y 46 años) no presentaban datos clínico-radiológicos de TSC. Un caso de TSC experimentó recidiva y fue sometido a reintervención. Todos los casos fueron positivos para NF, GFAP y nestina en células tumorales, con Ki67 (-). Uno de los casos sin TSC y uno con, no expresaron tuberina ni hamartina, mientras que el otro caso sin TSC, mostró (+) homogénea citoplásmica para hamartina y en mosaico (tinción positiva y negativa) para tuberina. El paciente con TSC ostentó hamartina (+), pero tuberina (-). Para OCT-4, la positividad citoplasmica intensa se observó en las células tumorales de los 2 casos con TSC; en los otros, uno no expresó y en el otro, la tinción fue en células endoteliales, pericitos y escasos astrocitos en contacto con células neoplásicas. Todos los casos fueron positivo (citoplasma y en procesos) para STAT-6 y CK. Para INI1, los casos con TSC mostraron tinción nuclear intensa y tenue tinción citoplásmica; en los casos sin TSC, uno tuvo tinción nuclear y otro, solo citoplásmica. Conclusiones: SEGA expresa marcadores de células madre neuroepiteliales, lo que explica su naturaleza dual, y la capacidad, como se demostró, de expresar CK, un marcador epitelial. El estudio de IHQ mostró perfiles diferentes en SEGA asociado a TSC de los "esporádicos", se demostró por primera vez, positividad en OCT-4, STAT-6 e INI1 en estos tumores. La expresión de la proteina salvaje harmatina y tuberina fue variable. La expresión de OCT-4 que se sabe modula la via mTOR, puede ser un blanco terapéutico adicional, asociado a inhibidores de esta vía. [ABSTRACT FROM AUTHOR]

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BACKGROUND: Tuberous sclerosis is a rare disease which main manifestations are dermatological and neurological. In our region, there are no descriptive studies of tuberous sclerosis. OBJECTIVE: To determine the clinical and epidemiological characteristics in patients diagnosed with tuberous sclerosis and to compare our findings with the global literature. MATERIAL AND METHOD: A retrospective, descriptive and observational study of patients who met diagnostic criteria of tuberous sclerosis, who attended the Dermatology clinic of the University Hospital Dr. José Eleuterio González from April 2012 to November 2017. The epidemiological characteristics, chief complain, main clinical manifestation, topography and comorbidities were collected. RESULTS: A total of 11 patients were identified. The dermatological lesions were the first clinical manifestation. Hypomelanotic macules appeared in 100% of the cases; the most frequent form was ash leaf (63.6%). The least common skin lesions were periungual fibromas (18.2%). Seizures were referred in 36.4% and were the first neurological manifestation. CONCLUSIONS: Hypomelanotic macules, due to their high prevalence, have an important diagnostic value; however, they are not specific. These lesions together with angiofibromas were the most common findings reported. The findings within our study are similar to those reported in international literature. [ABSTRACT FROM AUTHOR]

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Tuberous sclerosis is an autosomal dominant syndrome characterized by a predisposition to the formation of benign tumors. It is caused by mutation in the TSC1 and TSC2 gene. It is more common in women 2:1, with a prevalence of 1:12,000. Approximately 80 - 90 % develop a renal manifestation in adulthood, it represents the principal cause of death after 30 years. Angiomyolipomas are benign tumors that have rich in dysmorphic blood vessels, fusiform cells and mature adipocytes. They present high risk of bleeding especially in those > 4 cm. We present a female patient of 39 years of age, history of tuberous sclerosis, seizures since childhood, she present irregular, slow and progressive increase in abdominal circumference of one year of evolution, causing gastrointestinal symptoms. We diagnose bilateral renal angiomyolipoma with righ the morrhagic cyst. Right radical nephrectomy was performed. Treatment for angiomyolipoma in tuberous sclerosis should be the most conservative as possible. Indications for radical nephrectomy are massive tumors, central tumors, presence of necrotic areas, spontaneous bleeding or suspected malignancy. Recent literature suggests that mTOR inhibitors are safe and effective in controlling tumor burden of angiomyolipoma, as well as preserving renal parenchyma. [ABSTRACT FROM AUTHOR]

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Tuberous sclerosis complex (TSC) has been described as the disease most commonly transmitted by autosomal dominant inheritance. It represents a set of highly variable neurocutaneous manifestations often causing multiple organ failure. The most affected organs are: skin, brain, kidneys, lungs and heart. His clinical features are characterized by a triad consistent in demonstrations of mental retardation, epilepsy and sebaceous adenomas, being epilepsy the most common. However, the clinical manifestations vary between patients, and therefore, there exist consensus diagnostic clinical and radiological criteria for diagnosis. The accurate early diagnosis may possible improve survival and reduces morbidity and mortality of these patients. [ABSTRACT FROM AUTHOR]

Tuberous sclerosis, also called Bourneville Pringle disease, is a phakomatosis with potential dermal, nerve, kidney and lung damage. It is characterized by the development of benign proliferations in many organs, which result in different clinical manifestations. It is associated with the mutation of two genes: TSC1 (hamartin) and TSC2 (tuberin), with the change in the functionality of the complex target of rapamycin (mTOR). MTOR activation signal has been recently described in systemic lupus erythematosus (SLE) and its inhibition could be beneficial in patients with lupus nephritis. We report the case of a patient who began with clinical manifestations of tuberous sclerosis complex (TSC) 30 years after the onset of SLE with severe renal disease (tipe IV nephritis) who improved after treatment with iv pulses of cyclophosphamide. We found only two similar cases in the literature, and hence considered the coexistence of these two entities of great interest. [ABSTRACT FROM AUTHOR]

A 27 year-old woman was initially referred to the division of nephrology because of polyuria and the finding of renal cysts. She did not have nervous system manifestations. According to the "2012 International Tuberous Sclerosis Complex Consensus Conference" the diagnosis of Tuberous Sclerosis Complex (TSC) was completed. TSC is an autosomal dominant inherited multisystem disease with nearly complete penetrance. Clinical manifestations can vary widely even in patients belonging to a same linage. The morbidity and mortality depends on renal or respiratory complications on adulthood. This report highlight the need of a high level of suspicions in patients with manifestations compatible with this disease and shows the importance of a multidisciplinary team working together to arrive to the appropriate diagnosis. [ABSTRACT FROM AUTHOR]

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Intracardiac tumors are uncommon in children, and to a lesser extent in newborns. Most of these tumors are rabdomiomas, which usually vary in size and number. The rabdomioma is located most of the times in the ventricles, and rarely in the atrium. The rabdomiomas can be associated with tuberous sclerosis from 56% to 86% of the cases. Nowadays, intracardiac fetal tumors can be detected through prenatal ultrasound, after the second gestation trimester. The tumor location and the hemodynamic effects will be the most important aspects that determine the severity. Serial prenatal ultrasound monitoring reveals the tumoral growth speed. It's believed that, due to an in utero maternal stimulation, an accelerated growth rate is presented during the second and third trimester, and it will slow down after the 35th week. This is a case-report of a patient with the prenatal diagnosis of an intracardiac mass, referred to the neonatology department of the Instituto Nacional de Pediatría. [ABSTRACT FROM AUTHOR]

The tuberous sclerosis complex is a phakomatosis, characterized by cutaneous lesions, epilepsy and mental retardation; with variable affectation in brain, kidneys, heart and other organs. With dominant autosomic character, there are between 60 to 70% de novo mutations. Renal lesions can be identified in up to 57.5% of patients. We report the case of a 20 years old male patient from the Hospital NacionalArzobispoLoayza. He presents cuteanousangiofibromas, Shagreen patches, subependymal calcifications, renal angiomyolipomas and mild mental retardation; along with an intense nephrotic syndrome associated with acute renal injury. No family history of importance. Literature is revised along with the diverse forms of clinical-pathologic presentations of tuberous sclerosis complex. [ABSTRACT FROM AUTHOR]

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Rhabdomyomas are the most common cardiac tumours in children. They are strongly associated with tuberous sclerosis disease. They usually regress spontaneously but sometimes they need surgical intervention because the children symptoms due to tumour location. We described here two neonates with tuberous sclerosis diagnosis and big cardiac rhabdomyomas in unusual situation. [ABSTRACT FROM AUTHOR]

Lymphangioleiomyomatosis (LAM) is a rare lung disease of unknown etiology, described since 1918 associated with tuberous sclerosis complex (TSC-LAM) and are reported sporadically (S-LAM). It is classified within the group of interstitial lung diseases (ILD) and currently the European Respiratory Society (ERS) has published guidelines for diagnosis and treatment. The objective is to evaluate the clinical presentation of two patients, evolution, management, and review of current treatment. Both patients admitted in our hospital for examination, presenting with spontaneous pneumothorax. Their CT scan shows characteristic cystic lesions and thoracotomy with lung biopsy was performed because lack of expansion and to confirming the diagnosis. Despite the controversy about the optimal management of these patients, there is evidence about the use of progesterone routinely in patients with rapid deterioration of respiratory function when it was provided for a period of at least 12 months. Due to the rareness of the disease, it requires a patient registry to evaluate the use of experimental drugs or include them in research protocols to improve their prognosis. [ABSTRACT FROM AUTHOR]

The first descriptions of tuberous sclerosis were reported in 1880 by Desire Maloire Bourneville, who determinates thereafter the descriptions of the cerebral patology and the neurologic signs. It was until the beginnings of the XX century that were aquisited more imagine and clinic information about the tuberous sclerosis. The association of cerebral, renal, cardiac and dermatologic damages was recognized in 1905, making that way the Vogt triads, that consists of epilepsy, cognitive alterations and behavior changes. They generate cerebral disorders like cortical tubers, subependimarious nodules, subependimarious astrocytoms and white substance anomalies. The tuberous sclerosis is a genetic disease, with defects in the production of hamartin (TSC1) in the locus of chromosome 9, and of tuberin (TSC9) in the locus of chromosome 16. The hamartin and tuberin have an inhibitor effect in the growing cells. We reported 3 cases with tuberous sclerosis, a 7 years boy who has uniquely sebaceous adenoms and subependimaria astrocytoma, a 31 years old woman who presents sebaceous adenoms, large evolution seizures and subepedimarious astrocytoma, and 35 year old man who presents seizures, sebaceous adenom and psychomotor deficit. [ABSTRACT FROM AUTHOR]

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Tuberous sclerosis was first described in 1880 and corresponds to an autosomic dominant disorder, where the mutation has been identified two genes responsible for disease. It triggers a systemic disease characterized by seizures and skin manifestations, as well as kidney, lung, and heart involvement. The central nervous system involvement causes high morbidity in affected patients, usually in the presence of cortical tubers, and the medium-term development of cognitive abilities depends on the early management of them. Clinical diagnosis is made using major and minor established criteria, in addition to imaging and molecular studies to identify TSC1 and TSC2 mutations. Surgical management of lesions improves the clinical course and appearance of the patient. [ABSTRACT FROM AUTHOR]

Introduction: Renal angiomyolipoma is an infrequent benign tumor that is made up of blood vessels, smooth and lipomatosous muscle. The solitaire is more frequent in Introduction: Renal angiomyolipoma is an infrequent benign tumor that is made up of blood vessels, smooth and lipomatosous muscle. The solitaire is more frequent in women and associated to tuberous sclerosis (disease of Pringle-Bourneville). The syndrome of Wünderlich was described like "spontaneous apoplexy of the renal capsule". Aetiology corresponds more of 50% to tumors. The Tomography is the method of election to demonstrate perirrenal hemorrhage. The treatment is based on the size and the state of the contralateral kidney. Summary of cases: Three cases taken care of in our Unit appeared in a period of relatively short time of 1 month. They varied in its clinical presentation and were quiet different one from each other. The association of the three cases occurred with data of haemodynamics instability reason for which they had to be operated as emergencies. The tomographic and transoperatories findings were similar in the three cases and the histopatologic result agreed with angiomyolipoma. Conclusions: The syndrome of Wünderlich must be taken into account when necessary elements like clinical data of shock, hypotension or tachycardia besides pain or hypersensitivity in flank as well as concrete mass are present and in some cases exist micro or macrocospic hematuria. The election study must be the axial tomography with a sensitivity of 100% for perirenal collections and if it is carried out at the precise moment of the active hemorrhage it determines the site of bleeding when it is due to angiomiolipoma. The treatment must be individualized in each case following the haemodynamics stability, the state of the contralateral renal unit, as well as the size of the mass. women and associated to tuberous sclerosis (disease of Pringle-Bourneville). The syndrome of Wünderlich was described like "spontaneous apoplexy of the renal capsule". Aetiology corresponds more of 50% to tumors. The Tomography is the method of election to demonstrate perirrenal hemorrhage. The treatment is based on the size and the state of the contralateral kidney. Summary of cases: Three cases taken care of in our Unit appeared in a period of relatively short time of 1 month. They varied in its clinical presentation and were quiet different one from each other. The association of the three cases occurred with data of haemodynamics instability reason for which they had to be operated as emergencies. The tomographic and transoperatories findings were similar in the three cases and the histopatologic result agreed with angiomyolipoma. Conclusions: The syndrome of Wünderlich must be taken into account when necessary elements like clinical data of shock, hypotension or tachycardia besides pain or hypersensitivity in flank as well as concrete mass are present and in some cases exist micro or macrocospic hematuria. The election study must be the axial tomography with a sensitivity of 100% for perirenal collections and if it is carried out at the precise moment of the active hemorrhage it determines the site of bleeding when it is due to angiomiolipoma. The treatment must be individualized in each case following the haemodynamics stability, the state of the contralateral renal unit, as well as the size of the mass. [ABSTRACT FROM AUTHOR]

Primary cardiac tumors are rare in childhood and are mostly benign (97%). Rhabdomyomas are the most frequent benign primary tumors with an incidence of 45% in autopsy series and 19% clinical series. In the pediatric population it incidence is 0.27%. They are benign tumors in view of their histological features. However, they may give rise to clinical manifestations depending on their location and on the structures they involve, obstruct or invade. They are associated with tuberous sclerosis has been observed up to 81% of the patients. We report a case of a newborn male who presented at birth with congestive heart failure (CHF), bradycardia. He was diagnosed as tuberous sclerosis with intracardiac rhabdomyoma which resolved spontaneously. Patient is currently asymptomatic one year after the initial diagnosis. [ABSTRACT FROM AUTHOR]

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