BACKGROUND: Thymic neuroendocrine tumor as a cause of Cushing syndrome is extremely rare in children. CASE PRESENTATION: We report a case of a 10-year-old girl who presented with typical symptoms and signs of hypercortisolemia, including bone fractures, growth retardation, and kidney stones. The patient was managed with oral ketoconazole, during which she experienced adrenal insufficiency, possibly due to either cyclic adrenocorticotropic hormone (ACTH) secretion or concurrent COVID-19 infection. The patient underwent a diagnostic work-up which indicated the possibility of an ACTH-secreting pituitary neuroendocrine tumor. However, after a transsphenoidal surgery, the diagnosis was not confirmed on histopathological examination. Subsequent bilateral inferior petrosal sinus sampling showed strong indications of the presence of ectopic ACTH syndrome. Detailed rereading of functional imaging studies, including 18F-FDG PET/MRI and 68Ga DOTATOC PET/CT, ultimately identified a small lesion in the thymus. The patient underwent videothoracoscopic thymectomy that confirmed a neuroendocrine tumor with ACTH positivity on histopathological examination. CONCLUSION: This case presents some unique challenges related to the diagnosis, management, and treatment of thymic neuroendocrine tumor in a child. We can conclude that ketoconazole treatment was effective in managing hypercortisolemia in our patient. Further, a combination of functional imaging studies can be a useful tool in locating the source of ectopic ACTH secretion. Lastly, in cases of discrepancy in the results of stimulation tests, bilateral inferior petrosal sinus sampling is highly recommended to differentiate between Cushing disease and ectopic ACTH syndrome.
- MeSH
- Cushingův syndrom etiologie diagnóza chirurgie MeSH
- dítě MeSH
- ektopický ACTH syndrom * diagnóza chirurgie MeSH
- ketokonazol terapeutické užití MeSH
- lidé MeSH
- nádory brzlíku * komplikace diagnóza chirurgie patologie MeSH
- neuroendokrinní nádory * komplikace diagnóza chirurgie patologie MeSH
- thymektomie MeSH
- Check Tag
- dítě MeSH
- lidé MeSH
- ženské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH
- kazuistiky MeSH
BACKGROUND: Rebound thymic hyperplasia (RTH) is a common phenomenon caused by stress factors such as chemotherapy (CTX) or radiotherapy, with an incidence between 44% and 67.7% in pediatric lymphoma. Misinterpretation of RTH and thymic lymphoma relapse (LR) may lead to unnecessary diagnostic procedures including invasive biopsies or treatment intensification. The aim of this study was to identify parameters that differentiate between RTH and thymic LR in the anterior mediastinum. METHODS: After completion of CTX, we analyzed computed tomographies (CTs) and magnetic resonance images (MRIs) of 291 patients with classical Hodgkin lymphoma (CHL) and adequate imaging available from the European Network for Pediatric Hodgkin lymphoma C1 trial. In all patients with biopsy-proven LR, an additional fluorodeoxyglucose (FDG)-positron emission tomography (PET)-CT was assessed. Structure and morphologic configuration in addition to calcifications and presence of multiple masses in the thymic region and signs of extrathymic LR were evaluated. RESULTS: After CTX, a significant volume increase of new or growing masses in the thymic space occurred in 133 of 291 patients. Without biopsy, only 98 patients could be identified as RTH or LR. No single finding related to thymic regrowth allowed differentiation between RTH and LR. However, the vast majority of cases with thymic LR presented with additional increasing tumor masses (33/34). All RTH patients (64/64) presented with isolated thymic growth. CONCLUSION: Isolated thymic LR is very uncommon. CHL relapse should be suspected when increasing tumor masses are present in distant sites outside of the thymic area. Conversely, if regrowth of lymphoma in other sites can be excluded, isolated thymic mass after CTX likely represents RTH.
- MeSH
- dítě MeSH
- fluorodeoxyglukosa F18 terapeutické užití MeSH
- Hodgkinova nemoc * diagnostické zobrazování farmakoterapie komplikace MeSH
- hyperplazie thymu * diagnostické zobrazování etiologie MeSH
- lidé MeSH
- lokální recidiva nádoru diagnostické zobrazování farmakoterapie MeSH
- lymfom * farmakoterapie MeSH
- nádory brzlíku * diagnostické zobrazování farmakoterapie komplikace MeSH
- počítačová rentgenová tomografie MeSH
- pozitronová emisní tomografie metody MeSH
- radiofarmaka MeSH
- Check Tag
- dítě MeSH
- lidé MeSH
- Publikační typ
- časopisecké články MeSH
- MeSH
- antitumorózní látky terapeutické užití MeSH
- dospělí MeSH
- lidé MeSH
- myasthenia gravis * etiologie farmakoterapie MeSH
- nádory brzlíku * chirurgie farmakoterapie komplikace MeSH
- PET/CT metody MeSH
- protokoly antitumorózní kombinované chemoterapie MeSH
- thymektomie metody MeSH
- výsledek terapie MeSH
- Check Tag
- dospělí MeSH
- lidé MeSH
- mužské pohlaví MeSH
- Publikační typ
- kazuistiky MeSH
The authors report on a case of a 60-year-old female admitted to hospital with symptoms of bilateral cardiac failure. Upon ultrasonic examination of the heart, a massive pericardial exudate was diagnosed. Pericardial drainage was done to find the cause of pericardial effusion. Cells of malignant lymphoma were detected cytologically while immunophenotypization demonstrated a malignant lymphoma exudate. A computed tomography (CT) examination of the thorax disclosed a mediastinal tumour with infiltration of both lungs, vascular structures and dissemination on the chest wall. A CT-guided tumour biopsy was performed to confirm or exclude a lymphoproliferative process. Histopathologically, an invasive cortical thymoma was verified. The tumour was evaluated as stage III thymoma according to Masaoka. This case report highlights a rare malignant thymoma, its clinical symptoms, diagnosis, therapy and prognosis (Fig. 2, Ref. 10).
- MeSH
- elektrokardiografie MeSH
- lidé středního věku MeSH
- lidé MeSH
- nádory brzlíku komplikace diagnóza patologie MeSH
- perikardiální efuze etiologie MeSH
- srdeční selhání diagnóza etiologie MeSH
- thymom komplikace diagnóza patologie MeSH
- Check Tag
- lidé středního věku MeSH
- lidé MeSH
- ženské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH
- kazuistiky MeSH
- práce podpořená grantem MeSH
Incidence diabetu 1. typu (DM1T) se významně liší mezi jednotlivými národy a etnickými skupinami. Přesné mapování incidence DM1T a jejích trendů pomáhá při studiu interakce genetických a negenetických faktorů, které ovlivňují manifestaci a průběh onemocnění. Významný pokrok v porozumění mechanizmům autoimunitního diabetu přineslo studium genů u autoimunitních forem monogenně podmíněného diabetu.
- MeSH
- autoimunitní nemoci genetika imunologie MeSH
- autoimunitní polyglandulární syndromy diagnóza etiologie MeSH
- diabetes insipidus diagnóza etiologie MeSH
- diabetes mellitus genetika imunologie MeSH
- financování vládou MeSH
- Gravesova nemoc diagnóza etiologie MeSH
- lidé MeSH
- monoklonální gamapatie nejasného významu diagnóza etiologie MeSH
- nádory brzlíku diagnóza etiologie komplikace MeSH
- Wolframův syndrom diagnóza etiologie MeSH
- Check Tag
- lidé MeSH
The authors' own experience obtained from surgical management of the myasthenia gravis in the First Department of Surgery, Teaching Hospital, Palacký University in Olomouc, within 1963 to 1985 are analyzed. The total of 50 operations on the thymus were performed in this period. Five myasthenics were operated on. Four of them had the thymoma associated with MG, one had no thymoma either prior to or during the surgery. Once the MG was associated with a benign thymoma, three times with a malignant thymoma. All the thymomas could be radically extirpated. The late results of the treatment are given. Some more recent examination methods are evaluated. The conclusion sums up the results of surgical MG management, indication to operation and its tactics stressing the necessity of complex multidisciplinary care of MG patients.
- MeSH
- dospělí MeSH
- lidé středního věku MeSH
- lidé MeSH
- myasthenia gravis komplikace chirurgie MeSH
- nádory brzlíku komplikace chirurgie MeSH
- thymektomie * MeSH
- thymom komplikace chirurgie MeSH
- Check Tag
- dospělí MeSH
- lidé středního věku MeSH
- lidé MeSH
- mužské pohlaví MeSH
- ženské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH
- kazuistiky MeSH
