Mediastinum primary small cell neuroendocrine carcinoma

Neuroendocrine carcinoma, which was first reported in 1907, is a rare disease [1]. This cancer most commonly originates in the gastrointestinal tract, although it can occur in the lungs, thymus, parathyroid gland, ovaries, and biliary system. However, mediastinal primary small cell neuroendocrine carcinoma (MPSCN) is extremely rare. Its origin is not clear [2]. The diagnosis of MPSCN requires a combination of pathological and immunohistochemical examination. The survival of patients with MPSCN was reported to be very poor, no more than 12 months [3]. Because of its rarity, only a few cases with therapy are reported in the literature. Herein, we present a case of MPSCN and the patient was alive more than three years after combined local resection, radiation, and chemotherapy.
A 36-year-old female presented to Jinan Central Hospital Affiliated to Shandong University with complaints about chest distress, dizziness, and red face for one and a half months duration. Superficial varicose veins could be seen on face, neck, double upper limbs and upper chest. These symptoms were aggravated when she was lying and relieved when she sat. A chest radiograph and computed tomography (CT) scan (Fig. 1A, B, C, D, E) showed an irregular soft tissue mass located in the upper-mid mediastinum and infiltrating the right porta pulmonis, which was with uniform density and unclear border, surrounding and oppressing the superior vena cava and right innominate vein and part of the left innominate vein. All serologic and microbial tests were negative for infectious agents. Tumour markers were not elevated. A bronchoscopy was performed, which reported negative for malignancy. Patient was not found in any other parts of the lesions or lymph node enlargement. Whole-body bone imaging examination reported negative. Based on all of the above, the mass was diagnosed as a mediastinal tumour.
Because the mediastinal tumour had severely oppressed superior vena cava, right innominate vein, and part of the left innominate vein, we took resection via sternum medisect to remove the vascular compression and to make sure of diagnosis and guide further treatment. The operation showed that the tumour was located at the lower back of the left innominate vein and on the inside of right innominate vein and superior vena cava. The mass surrounded the superior vena cava and partly the left anonymous veins and right anonymous veins. There was a lymph node located behind the superior vena cava,...


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