化学感受器瘤12例报告

本文报告１２例化学感受器瘤。男女之比为１：２。年龄１１－６１岁，平均３６岁。肿瘤发生在脑、眼、颈动脉体、纵隔、胸壁内、硬脊膜外及后腹膜。有３例曾行化感瘤切除术。临床表现随发病部位不同而异。因本病少见，确诊有赖于组织病理学检查。手术切除肿瘤是最有效的治疗方法。术中发现肿瘤的共同特征有：微红或暗红色，表面血管网极丰富，有薄色膜，有囊性感，穿刺易抽到血性液，与周围粘连紧密。作者认为，本病属低度恶性肿瘤。     

Fractionated stereotactic conformal radiotherapy in the management of large chemodectomas of the skull base

PURPOSE: To evaluate the role of fractionated stereotactic conformal radiotherapy (FSRT) as a noninvasive method in the management of large chemodectomas of the skull base. METHODS AND MATERIALS: Twenty-two patients with chemodectomas of the skull base were treated with FSRT at our institution. Ten patients received primary RT, and 12 patients were treated for recurrent or progressive disease after primary surgery (8 patients) or embolization (4 patients). The median total dose was 57.6 Gy, with a median of 1.8 Gy/fraction. The median target volume was 71.8 cm3 (range, 10.5-212.2 cm3). The most common symptoms at the initial diagnosis were pulsatile tinnitus (16 patients), hearing loss (14 patients), and balance disturbance (14 patients). Twelve patients had additional cranial nerve deficits. RESULTS: The median follow-up was 5.7 years (range, 19-177 months). The actuarial overall survival rate was 89.5% at 5 and 10 years. The actuarial local control rate was 90.4% at 5 and 10 years. Seven patients (32%) had a partial response and 13 (59%) had stable disease of the irradiated chemodectoma. Two symptomatic patients developed recurrence after 19 and 32 months. Neurologic dysfunction improved or completely resolved in 59% and stabilized in 32%; 9% of patients experienced impairment of preexisting neurologic dysfunction. No patient developed new neurologic deficits after FSRT. RT was interrupted in 1 patient because of a maxillary bone abscess. In all other patients, no acute or late adverse reactions greater than Common Toxicity Criteria Grade 2 were seen. CONCLUSION: Fractionated stereotactic conformal radiotherapy is an effective and well-tolerated noninvasive treatment for chemodectomas, with excellent tumor control rates and a low risk of morbidity. It is an option for patients at greater risk of microsurgical resection or with residual and recurrent tumors.     

Paraganglioma of the cavernous sinus. Case report

A case of paraganglioma arising from the cavernous area is presented. A 51-year-old woman presented with a parasellar mass causing decreased visual acuity, oculomotor nerve paresis and retro-orbital headaches without endocrinological dysfunction. Diagnosis was confirmed by histological appearance and electron microscopy. The patient was treated with surgery followed by radiation therapy consisting of 45 Gy. The clinicopathological features and the possible pathogenesis are discussed.     

Fine-needle aspiration of six carotid body paragangliomas

We report six carotid body paragangliomas diagnosed by fine-needle aspiration (FNA) in five patients. A total of eight aspirations were performed. The cytologic findings are characteristic: hemorrhagic background, hypo- to hypercellular smears (depending on the skill of the aspirator) with cells having delicate, illdefined, vacuolated cytoplasm, pleomorphic nuclei with distinct nucleoli, rare intranuclear cytoplasmic inclusions, and prominent rosette formation. No complications arose in any of the eight aspirations. FNA is a safe, accurate means of diagnosing carotid body paragangliomas. It can provide essential information for treatment planning and patient management.     

Paragangliomas of the parasellar region

Parasellar paragangliomas are rare tumors. As far as we know, only ten cases are described in the literature. Their clinical, pathological, and radiological features and possible origin are discussed in this article and a review of the literature is given. Additionally, we report a new case of a 51-year-old woman with paraganglioma growing in the anterior, middle, and posterior cranial fossa with extended destruction of the skull base. The patient had been suffering from long-standing headaches and facial nerve paresis. Preoperatively, this tumor was suspected to be a meningioma.     

Chemodectoma of the larynx

Summary The present case report is concerned with a clinico-pathological study, including ultrastructural investigation, of a rare and uncommon laryngeal tumour, a chemodectoma, in a 62 year old patient. There have been 23 cases of laryngeal chemodectomas reported in the literature, and only three of them, including our own report, were investigated by electron microscopy. The tumours arise from the superior and inferior laryngeal nonchromaffin paraganglia or possibly from Kultschitzky-cells of the normal bronchial mucosa.Ultrastructurally they have all the characteristics of apudomas whose parent cells (the APUD-cells), usually show endocrine function and probably have their origin in the neural crest.The tumours show an aggressive type of behaviour, despite usually benign histological features when compared to chemodectomas at other sites in the head and neck region. Surgery is thus the therapy of choice.     

Optimum dose of radiotherapy for chemodectomas of the middle ear

Forty patients with chemodectomas of the middle ear were seen at the University of Virginia Hospital from 1932 to 1978. Surgery, post-operative radiotherapy or radiotherapy alone were the treatment modalities employed depending on the extent of the disease. These have been reviewed with regard to the clinical presentation and results of treatment with long term follow-up of 1–30 years. An attempt was made to determine the optimum dose of radiotherapy based on our data and reported cases in the literature. The majority of patients complaining of tinnitus, otalgia and pulsation obtained significant if not complete relief of symptoms. Cranial nerve defects, however, often persisted after therapy. Tumor was considered to be controlled if there was no increase in its size or progression of symptoms. Tumor control was obtained in eight of 10 early patients but only in two of seven more patients with advanced disease with total resection. Control rate with post-operative radiotherapy after subtotal resection was 85%. Radiotherapy alone was used for inoperable or recurrent tumors and control was obtained in 88 % of them. In addition to our data, the radiation dose used in over 200 patients reported in the literature were analyzed. There was only a 2% recurrence rate in patients who received 4000 rad/4 weeks or higher. Twenty-two percent of patients treated with less than 4000 rad developed recurrence. The tendency is to use a lower dose of postoperative treatment and a higher dose for gross inoperable tumors. 4000 rad/4 weeks seems to be adequate for control of postoperative residual disease and no more than 5000 rad/5 weeks are required even for advanced inoperable cases. By keeping the dose below 5000 rad/5 weeks, the incidence of complications such as brain necrosis is greatly decreased.