Ectomesenchymal chondromyxoid tumor (ECT) is a rare benign tumor of uncertain lineage, which almost exclusively affects the anterior tongue. Herein, we report 2 cases of ECT occurring in 58- and 56-year-old males on the right and on the left side of the dorsum of the anterior tongue, measuring 18 mm and 10 mm, respectively. Despite positive resection margin in one case, none of the tumors recurred during follow-up of 6 and 5 years. Microscopically, both tumors had lobular architecture with a mixture of solid, microcystic, and chondromyxoid areas. The tumor cells were polygonal or elongated and showed mild atypia in one case. Immunohistochemically, both tumors showed diffuse expression of vimentin and focal positivity of CD10 and of smooth muscle actin. Regarding neural tissue-related markers, there was nearly diffuse expression of CD56 and neuron-specific enolase and focal positivity of PGP 9.5 in both cases and variable expression of CD57, synaptophysin, glial fibrillary acidic protein, and S-100 protein. Interestingly, we observed diffuse expression of SOX10 in one case. In both tumors, diffuse strong nuclear expression of cyclin D1 was present, without CCND1/IGH translocation or CCND1 amplification. The EWSR1 gene rearrangement was not detected. To the best of our knowledge, expression of SOX10, which may support neural crest origin of this peculiar lesion, has not been reported in ECT. The significance of strong cyclin D1 expression remains to be further investigated.
- MeSH
- cyklin D1 analýza biosyntéza MeSH
- hybridizace in situ fluorescenční MeSH
- imunohistochemie MeSH
- lidé středního věku MeSH
- lidé MeSH
- mezenchymom diagnóza patologie MeSH
- myxom diagnóza patologie MeSH
- nádorové biomarkery analýza MeSH
- nádory jazyka diagnóza patologie MeSH
- transmisní elektronová mikroskopie MeSH
- Check Tag
- lidé středního věku MeSH
- lidé MeSH
- mužské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH
- kazuistiky MeSH
V kazuistice prezentujeme případ neobvyklého mezenchymálního tumoru plic. Jedná se tzv. plicní „sugar“ tumor, patřící do skupiny tumorů z perivaskulárních buněk (PEComů). Popisujeme případ 53leté ženy s náhodně rentgenologicky zachyceným dvoucentimetrovým ložiskem, která byla následně indikována k torakotomii. Histopatologické vyšetření odhalilo dobře ohraničený tumor ze světlých buněk s velmi vysokým obsahem glykogenu, vodojasnou cytoplazmou a pozitivitou HMB-45, S100, melan-A a vimentinu. Následně budou rozebrána dostupná fakta o tomto málo známém tumoru.
We present a case of rare mesenchymal lung neoplasm called pulmonary sugar tumor, member of perivascular epithelioid tumor family. A case of 53-years old woman with rounded, two centimeters wide lesion in right lung is described. The lession was incidentally detected on A-P chest X-ray. The patient was subsequently refered to surgical ward for thoracotomy. Histopathological examination revealed well-bounded clear cell tumor with abundant content of glycogen, water-like bright cytoplasm a positive immunohistochemy for HMB-45, S100, melan-A and vimentin immunomarkers and negative cytokeratines. Subsequently we discuss available facts about this not very well known tumor.
- Klíčová slova
- světlobuněčný tumor plic, perivaskulární epiteloidní buňky, PECom, mezenchymální plicní tumor,
- MeSH
- imunohistochemie MeSH
- lidé středního věku MeSH
- lidé MeSH
- mezenchymom diagnóza chirurgie patologie MeSH
- multimodální zobrazování MeSH
- nádory plic * diagnóza chirurgie patologie MeSH
- nádory z perivaskulárních epiteloidních buněk * chirurgie patologie radiografie MeSH
- rentgendiagnostika hrudníku MeSH
- torakotomie MeSH
- Check Tag
- lidé středního věku MeSH
- lidé MeSH
- ženské pohlaví MeSH
- Publikační typ
- kazuistiky MeSH
BACKGROUND: Oncogenous osteomalacia (OOM), which is also known as tumour-induced osteomalacia, is a rare condition associated with a neoplasm and a related systemic bone demineralization caused by renal phosphate wasting. OOM usually occurs in association with a variety of different mesenchymal tumours, and they were categorized into four distinct morphological patterns which they termed "phosphaturic mesenchymal tumour". Of its 4 histopathological subtypes, the mixed connective tissue variant is most commonly observed. Only 10% of cases appear in the head and neck regions and moreover, only 5 previously published tumors were localized in the sinonasal area. The authors describe a case of a man with a PMT originating from the frontoethmoidal region. CASE PRESENTATION: A 53-year-old man was referred to our ORL clinic due to a presence of a mass at the nasal root having been growing asymptomatically for 1 year. CT scans demonstrated a large (25 × 20 × 35 mm) bilateral frontoethmoidal mass with destruction of nasal bones. The tumor did not appear to invade to the anterior skull base. A selective angiography revealed a moderate hypervascularization of the tumour during early and late arterial phases. The tumour was removed from the external approach and the definitive histopathological diagnosis was a phospaturic mesenchymal tumor. Dual energy X-ray absorptiometry revealed a slight osteopenia of the first and second lumbar vertebrae and neck of the thigh bone. The serum and urinary levels of both calcium and anorganic phosphate were within normal limits. The patient is doing well three years after the operation, and the serum and urine levels of calcium and phosphate remain well within normal limits. CONCLUSION: PMT is rare in the sinonasal region, it can be rarely observed without the signs of osteomalacia.
- MeSH
- dospělí MeSH
- fosfáty krev moč MeSH
- lidé středního věku MeSH
- lidé MeSH
- mezenchymom diagnóza metabolismus patologie MeSH
- nádory kostí diagnóza metabolismus patologie MeSH
- nádory vedlejších dutin nosních diagnóza metabolismus patologie MeSH
- nádory z pojivové tkáně diagnóza metabolismus patologie MeSH
- počítačová rentgenová tomografie MeSH
- senioři MeSH
- Check Tag
- dospělí MeSH
- lidé středního věku MeSH
- lidé MeSH
- mužské pohlaví MeSH
- senioři MeSH
- ženské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH
- kazuistiky MeSH
- přehledy MeSH
Autoři popisují nález vzácného nádorového onemocnění ledviny - primárního leiomyosarkomu. Předoperační diagnóza byla stanovena dle zobrazovacích metod jako karcinom ledviny u 71 letého muže. V únoru 2001 byla provedena pravostranná transperitoneální nefrektomie. Nádor byl lokalizován v ledvinném parenchymu. Histologicky byla diagnóza uzavřena jako leiomyosarkom. Dalším vyšetřením byla vyloučena jiná primární lokalizace i případné metastázy. Za 6 a půl měsíce od operace se objevily 2 drobné metastázy na nativním RTG-snímku plic, které byly potvrzeny spirální počítačovou tomografií (SCT) plic. Pacient byl předán do péče onkologů, kde podstoupil 6 sérií systémové chemoterapie typu MAID (mesna, adriamycin, ifosfamid, dakarbazin), po které byla zaznamenána částečná remise choroby s následnou stabilizací. Pacient do poloviny prosince 2002 žil v dobrém stavu. V druhé polovině prosince došlo k progresi onemocnění v oblasti metastáz a úmrtí během 3 týdnů.
The authors describe the finding of a rare tumorous disease of the kidney - primary leiomyosarkoma. Pre-operative diagnosis done with the help of imaging methods was a carcinoma of the kidney in a 71-year old man. Right-sided transperitoneal nephrectomy was performed in February 2001. The tumor was localised in the parenchyma of the kidney. Histological diagnosis was leiomyosarcoma. Further investigations excluded other primary localisation or metastases. Two small metastases appeared on plain chest X-ray and were confirmed by spiral CT six and a half months after the surgery. The patient was referred to oncologist care, where he underwent six cycles of chemotherapy of MAID (mesna, adriamycin, ifosfamid, dacarbazine), after which partial remission with subsequent stabilisation was achieved. The patient lived in a good condition until December 2002. Progression of the disease in the site of meatastases appeared in mid-December and death followed in three weeks.
- MeSH
- leiomyosarkom diagnóza chirurgie MeSH
- lidé MeSH
- mezenchymom diagnóza MeSH
- nádory ledvin diagnóza MeSH
- Check Tag
- lidé MeSH
- mužské pohlaví MeSH
- Publikační typ
- kazuistiky MeSH
