4 research outputs found
Doege–Potter-szindróma a mellhártya óriás malignus szoliter fibrosus tumorával = Doege–Potter syndrome with giant malignant solitary fibrous tumour of the pleura
Absztrakt:
A mellhártya ritkának számító szoliter fibrosus tumora néhány százalékban
hypoglykaemiával társul, amit első két leírójukról Doege–Potter-szindrómának
neveztek el. Hatvanhárom éves férfi betegünk egy évvel korábban már
pulmonológiai kezelés alatt állt bal oldali mellüregi folyadékgyülemmel, bal
alsó lebenyi daganat CT-képével. Ismét fokozódó terhelési dyspnoe miatt végzett
újabb vizsgálatok során a most már óriásira nőtt tumor vastagtű-biopsziája low
grade sarkomát igazolt. Onkológiai konzílium műtéti elbírálás mérlegelését
javasolta. A beteg nyugalmi dyspnoe és ismétlődő hypoglykaemiás rosszullétek
miatt gyorsan romló állapotban az intenzív osztályról került a műtőbe. A tumor
eltávolítása és bal oldali pneumonectomia történt szövődménymentes gyógyulással.
A szövettan szoliter fibrosus pleuratumort igazolt a Doege–Potter-szindrómának
megfelelően. A műtét alatt észlelt pleuralis szóródás miatt adjuváns
kemoterápiában részesült. Egy évvel a műtét után helyileg kiújult tumort
távolítottunk el. A folytatódó kemoterápia ellenére lokális és ellenoldali
pulmonalis progressziót észleltünk. A beteg első műtétjétől számított túlélése
három év. Orv Hetil. 2018; 159(4): 149–153.
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Abstract:
Infrequent solitary fibrous tumours of the pleura are associated with
hypoglycaemia only in a few percent of the cases; this condition is called
Doege–Potter syndrome, named after its first descriptors. Our 63 years old male
patient has previously undergone clinical treatment for intrathoracic fluid
accumulation on the left side caused by a giant tumour-like mass in the lower
left lobe detected by CT scan. In the course of further investigations performed
due to increasing load-induced dyspnoea, lung core biopsy verified low grade
sarcoma in the tumour. Tumour board suggested surgery. The patient was
transferred from the intensive care unit into the operation theater due to
increasing dyspnoea and repeated hypoglycaemic periods in rapidly worsening
general condition. Pneumonectomy and removal of the tumour was performed on the
left side. Histology showed solitary fibrous tumour of the pleura corresponding
to Doege–Potter syndrome. The patient was discharged without complications and
underwent adjuvant chemotherapy due to pleural dissemination of the tumour
observed intraoperatively. One year after surgery the patient underwent surgical
removal of a locally recurrent tumour. In spite of repeated chemotherapy local
and multiplex contralateral pulmonary progression was observed. Three-year
survival was noted from the time of the first surgery. Orv Hetil. 2018; 159(41):
149–153
Unicentric mixed variant castleman disease associated with intrabronchial plasmacytoma.
Castleman disease (CD), described as a heterogeneous lymphoproliferative disorder, can be divided into different subtypes according to clinical appearance (unicentric and multicentric form) and histopathological features (hyaline vascular, plasma cell, mixed type, human herpesvirus 8-associated and multicentric not otherwise specified). Unicentric CD is known to be usually of the hyaline vascular variant, plasma cell and mixed type of this form are quite uncommon. Malignancies are mainly associated with the multicentric form. We report a rare case of unicentric mixed variant CD evolving into intrabronchial, extramedullary plasmacytoma.Intrabronchial mass with consequential obstruction of the left main bronchus, left lung atelectasis and mediastinal lymphadenomegaly was detected by chest CT in our patient suffering from cough and hemoptysis. Pulmonectomy was performed, histopathological and immunhistochemical analysis of lymph nodes revealed mixed type of CD with interfollicular monotypic plasma cell proliferation. The intrabronchial mass consisted of monotypic plasma cells confirming plasmacytoma. Systemic involvement was not confirmed by further tests.Although malignancies more often present in multicentric CD that usually belongs to the plasma cell subtype, this case confirms the neoplastic potential of the rarest, unicentric mixed variant of CD.Virtual slides: The virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/2872096831190851