[Peripheral primitive neuroectodermal tumor of trachea: a case report and review of literatures].

OBJECTIVE: To improve the understanding of tracheal peripheral primitive neuroectodermal tumor (PNET). METHODS: A case of tracheal PNET diagnosed in July 2010 was reported and the related literatures were reviewed. The literature review was carried out respectively with "primitive neuroectodermal tumor", "peripheral" as the search terms in Wanfang med online and PubMed database by September 2011. RESULTS: A case of 63 year-old female patient, who had been misdiagnosed as having chronic pharyngitis, chronic bronchitis and bronchial asthma, was admitted to the hospital because of cough and sputum production for 50 days, and anhelation for 1 month. After admission, the chest computerized tomography showed a space-occupying lesion in the middle of the trachea. Bronchoscopy showed a pedicle neoplasm 4 cm under the subglottic, with integral capsule, smooth surface and rich vascellum. Subsequently, tumor resection under bronchoscope was performed. Pathology report after operation showed infiltration of flake small round malignant cells under bronchial mucosa. Immunohistochemistry showed CD(99)(+), Syn(+) and S-100(+). EWS-FLI-1 fusion transcript was detected by RT-PCR. Accordingly, it was diagnosed as PNET. The symptoms of cough and anhelation were disappeared after operation. So far, there was no local recurrence and distant metastasis with 14 months follow-up. A total of 111 literatures were received in Pubmed, including one of prospective study, one of review, 22 of retrospective study and 87 of case report. Forty literatures and 187 cases in all were received in Wanfang Med Online, including 24 of retrospective study and 16 of case report. But, there were no reports about tracheal PNET. CONCLUSIONS: PNET can occur in the trachea and is easy to be misdiagnosed. To make a definite diagnosis, histopathology and immunohistochemistry are needed and detection of EWS-FLI-1 fusion transcript is a reliable marker for molecular diagnosis. The tracheal pPNET may be different with the pPNETs in other parts, and has a lower-grade invasion and less distant metastasis.

[Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease) of the airway: a case report and review of the literature].

OBJECTIVE: To improve the awareness of sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease) involving the airways. METHODS: The clinical presentations, endoscopic findings in the airways, pathological characteristics, and diagnosis and treatment of a case of Rosai-Dorfman disease was reported, and related literatures were reviewed. RESULTS: A 60-year-old female patient was admitted to this hospital because of recurrent wheezing for 18 months and aggravated for 1 month on March 6, 2007. The diagnosis of "bronchial asthma" had been made and oral prednisolone and inhaled budesonide resulted in symptom improvement. One month ago, she had wheezing again with inspiratory dyspnea, which was more obvious at recumbent position.She had been found to have high blood pressure and left adrenal adenoma 23 years ago, and as the diagnosis of "primary aldosteronism" was made but underwent no surgery. Left parotid gland tumor and left submandibular lymph nodes had been found, and surgical resection implemented in 1999. Lacrimal gland tumor resection of her eyes had been performed in 2000. Multiple subcutaneous nodules, rising and disappearing spontaneously, had been demonstrated in 2001. After admission, physical examination revealed nodules of 3.0 cm x 2.0 cm in her left submandibular area, and soybean sized nodules at both arms, back, chest, abdomen, buttocks and thighs. Chest CT scan and tracheal reconstruction showed that there were multiple nodules in the tracheal wall with narrow lumen, with no obvious enlargement of mediastinal lymph nodes. Lymph node biopsy showed faintly stained areas and the formation of plasma cells and lymphocytes of the deeply stained area, presenting as a sinus-like structure, and plasma cells and lymphocytes were engulfed in the plasma of the histiocytes, consistent with the diagnosis of Rosai-Dorfman disease. CONCLUSIONS: Rosai-Dorfman disease involving the airway was a rare disease often misdiagnosed. Bronchoscopy was very helpful for the diagnosis. Histiocytosis with phagocytosis of plasma cells and lymphocytes was the pathological feature, and immunohistochemical staining positive for S100 protein and CD(68) was suggestive of the diagnosis. Surgical resection combined with corticosteroids or radiotherapy was effective treatment of the airway diseases.