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Atypical Carcinoid of the Thymus with Initial Presentation of Diffuse Bone Marrow Infiltration

罕見胸腺非典型類癌合併瀰漫性骨髓轉移

摘要


Thymic neuroendocrine tumors (NET) are rare primary thymic neoplasms with neuroendocrine differentiation. They are characterized by relatively aggressive behavior with a high propensity for local invasion and distant metastasis. However, thymic carcinoids metastasizing to the bone marrow have been very rarely reported in past decades. We present a case of atypical carcinoid of the thymus who had an initial presentation of nonspecific low back pain, with magnetic resonance imaging (MRI) unexpectedly showed heterogenous signal intensities of the lumbar spine. An anterior mediastinal mass was later found by chest imaging; histopathology of the mediastinal mass and bone marrow both revealed atypical carcinoid tumors. The patient underwent mediastinal tumor excision, followed by cytotoxic chemotherapy. However, the follow-up bone marrow biopsy showed more extensive marrow replacement by NET cells. Systemic therapy was then shifted to everolimus with resultant disease control. This case emphasizes that a thymic carcinoid could spread to the bone marrow, and that MRI may play an important role in the investigation of marrow involvement in patients with non-specific musculoskeletal pain. Although the efficacy of systemic treatment for patients with metastatic diseases is unclear, there are systemic therapy options that may be effective in treating this rare thoracic malignancy.

並列摘要


胸腺神經內分泌腫瘤為非常罕見的前縱膈腔腫瘤,且在診斷時常已出現淋巴侵犯或遠端轉移。在此,我們報告一位53歲男性因下背痛四個月求診,在做脊椎核磁共振成像時意外發現瀰漫性骨髓病變,經進一步檢查後發現前縱膈腔的胸腺非典型類癌合併軟組織與骨髓轉移。病人接受手術切除前縱膈腔的主腫瘤,術後也銜接化學治療,但影像和骨髓病理切片都顯示骨髓轉移的程度在惡化。後來病人改接受everolimus治療,並用嗎啡類止痛藥控制疼痛。此案例提醒我們即使是下背痛如此常見的症狀,仍需要仔細評估病因,而核磁共振造影在偵測骨髓病變時具有優異的敏感性,是重要的評估工具。臨床上,目前仍欠缺針對已遠端轉移之胸腺神經內分泌腫瘤的有效治療,但可以參考其他部位之神經內分泌腫瘤的研究,提供患者治療的選擇。

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