Ann Thorac Surg 2009;88:641-642. doi:10.1016/j.athoracsur.2009.01.017
© 2009 The Society of Thoracic Surgeons
Case Reports
Facial Flushing Due to Multifocal Tumorlets in the Lung With Bronchiectasis
Baoxing Liu, MD,
Qiang Pu, MM,
Lunxu Liu, MD,
Guowei Che, MD*
Department of Cardiovascular and Thoracic Surgery, West-China Hospital, Sichuan University, Chengdu, China
Accepted for publication January 8, 2009.
* Address correspondence to Dr Che, Department of Cardiovascular and Thoracic Surgery, West-China Hospital, Sichuan University, Chengdu, 610041, China (Email: cheguowei{at}yahoo.com.cn).
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Abstract
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Facial flushing attacks as a Carcinoid syndrome are quite a rare presentation in pulmonary tumorlets and bronchiectasis. We experienced a case in which a 43-year-old woman with bronchiectasis presented to our department with continuous facial flushing attacks for 3 years in the absence of bronchiectasis symptom. Computed tomographic scans revealed bronchiectasis of the left lower lobe and no tumor. A left lower lobectomy was performed, and multiple tumorlets were observed on microscopic examination among the bronchiectasis. Histologic and immunohistochemical examination revealed findings consistent with diffuse tumorlets and bronchiectasis in the lung.
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Introduction
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Tumorlets of the lungs are multifocal hyperplasias of endocrine cells that may develop through the nodular hyperplasia into peripheral, occasionally metastatic carcinoids [1]. They are observed with particular frequency in bronchiectasis. Patients with bronchiectasis are often symptomatic and present with cough, recurrent pulmonary infection, and hemoptysis. However, facial flushing as a symptom is quite a rare presentation in bronchial carcinoid [2]. It has not been reported that patients with bronchiectasis and pulmonary tumorlets present with primary facial flushing.
A 43-year-old woman was admitted to our hospital because of continuous facial flushing attacks for 3 years. The facial flushing was not related to fever and menopause. She denied the following symptoms, including the presence of hemoptysis, cough, hoarseness, and dyspnea. Her sputum was not dense and was yellow-white in color. She was a nonsmoker and had no exposure to any environmental fumes or dust. Her family history was unremarkable.
Physical examination showed nothing remarkable, except findings of coarse crepitations and decreased breath sound on the left side on auscultation. Other systems were normal. Routine laboratory investigations were within normal limits. Plasma serotonin and 5-hydroxyindoleacetic levels were within normal limits. Diagnosis of bronchiectasis was based on high resolution computed tomographic scans. These scans showed a bronchiectasic appearance in the left lower lobe (Fig 1A). The other lobes revealed no tumorlets. A literature search revealed one report of facial flushing attacks as a carcinoid syndrome in a presentation in bronchial carcinoid [2] and tumor of carcinoid diameter < 0.5 mm named pulmonary tumorlets. Although most carcinoids behave in a benign manner, all have the potential to metastasize and are considered malignant neoplasms. As in our case, the diagnosis of bronchiectasis with carcinoid in the absence of bronchiectasis symptoms had to be considered. Finally, facial flushing attacks have seriously restricted the patient's life. On the basis of the known association of peribronchial carcinoid tumorlets with bronchiectasis, and a previous report [2] that tumorlets can cause flushing, we operated with the preoperative diagnosis of possible carcinoid tumorlets causing both bronchiectasis and facial flushing. The patient received a left lower lobectomy.
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Fig 1. (A) Computed tomographic scan shows bronchiectasis in the left lower lobe. (B) Nests of tumorlet cells in the fibro-muscle tissue surrounding a brochious. (Hematoxylin and eosin stain; x100.) (C) Tumor cells demonstrate strong positive reaction with chromogranin A (labeled streptavidin biotin [LsAB] methods; x100). (D) Ki-67 was expressed in the tumorlet cell nuclei showing brown LsAB (LsAB; x100).
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The surgical specimen consisted of resected lobectomy material measuring 12 x 10 x 5 cm, and one hilum of lung lymph node. Upon macroscopic examination, the lobectomy material was pink and spongy. The bronchi demonstrated moderate dilatation and wall rigidity. The bronchial lumen was filled with mucin secretion. The lung parenchyma appeared firm. No distinct tumor was seen.
On microscopic examination, the bronchi demonstrated the classic histologic features of moderate brochiectasis and chronic bronchitis. In these cystically dilated bronchi, the lining epithelium varied from flattened to hyperplastic. The bronchial wall was fibrotic and infiltrated with lymphocytes and plasma cells. Within these fibromuscular areas, groups of tumorlets were displayed only under microscopy. These cells were arranged in clusters and foci of fascicles adjacent to bronchiectatic lesion. The tiny tumors consisted of short fusiform cells and small ovoid cells with generally uniform nuclei (Fig 1B). Neither mitotic figures, atypical cells, nor necrosis was found. Ki67 positive cells were less than 5% (Fig 1D). Intensive positive immunostaining for chromogranin A (Fig 1C), epithelial membrane antigen, and CD56 were detected. A weak positive for synaptophysin, low molecular weight cytokeratin were also observed. These findings were characteristic of diagnosis of multiple tumorlets. The lymph nodes were free of tumor cells.
The patient's postoperative course was uneventful. After the operation and 6 months without facial flushing, the patient was seen again. The final diagnosis, which was based on histopathologic examination and immunostaining, was multiple tumorlets with bronchiectasis in the left lower lobe.
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Comment
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Patients with pulmonary tumorlets are often asymptomatic or present with bronchiectasis symptoms, such as cough and recurrent pulmonary infection. Pulmonary tumorlets are usually an incidental finding during microscopic examination of the lung at autopsy or after surgery for other reasons [3]. Tumorlets are not seen on computed tomographic scan and on microscopic examination they are mainly arranged in clusters adjacent to the bronchiectatic lesion. Facial flushing as a carcinoid syndrome due to recurrent bronchial carcinoid has been reported [2], but it is quite rare and usually associated with recurrent carcinoid tumor in the presence of hepatic metastasis [4].
The histogenesis of pulmonary tumorlets has been described as tumorlet advancing to nodular epithelial hyperplasia, advancing to microscopic oat-cell carcinoma [5]. Their nature and significance remain controversial because of their potential link to pulmonary carcinoid tumors. Although most pulmonary tumorlets are benign, they all have a potential to metastasize and are considered malignant neoplasms [6]. Some authors have found argyrophilic granules in these tumor cells resemble those of the carcinoid tumor, which may account for the primary facial flushing [7]. However, bronchial carcinoids cannot provoke the syndrome, because pulmonary tissue contains large amounts of monoamine oxidase that inhibit serotonin, and they must be detected early and excised before they grow large enough to produce a large amount of serotonin. Metastasis to the liver produces the syndrome because the liver cannot metabolize these large amounts of serotonin [4].
Although there are reports of cases of carcinoid syndrome in bronchial carcinoid, the carcinoid syndrome will disappear after successful surgical excision of the primary tumor [8]. This is an interesting case of pulmonary tumorlets presenting with the facial flushing for 3 years without bronchiectasis symptom. Facial flushing attacks disappeared 6 months after the operation.
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References
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- Klinke F, Bosse U, Höfler H. The tumorlet carcinoid in bronchiectasis-changed lungs: an example of a multifocal, endocrine tumor Pneumologie 1990;44(Suppl 1):607-609.[Medline]
- Ganti S, Milton R, Davidson L, Thorpe A. Facial flushing due to recurrent bronchial carcinoid Ann Thorac Surg 2007;83:1196-1197.[Abstract/Free Full Text]
- Canessa PA, Santini D, Zanelli M, Capecchi V. Pulmonary tumourlets and microcarcinoids in bronchiectasis Monaldi Arch Chest Dis 1997;52:138-139.[Medline]
- Warner RR, Kirschner PA, Warner GM. Serotonin production by bronchial adenomas without the carcinoid syndrome JAMA 1961;178:1175-1179.[Abstract/Free Full Text]
- Nagai S, Katakura H, Okazaki T, et al. A pulmonary tumorlet with caseous granuloma associated with atypical mycobacterium Nihon Kokyuki Gakkai Zasshi 1998;36:464-468.[Medline]
- Davies SJ, Gosney JR, Hansell DM, et al. Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia: an under-recognised spectrum of disease Thorax 2007;62:248-252.[Abstract/Free Full Text]
- Churg A, Warnock ML. Pulmonary tumorlet: a form of peripheral carcinoid Cancer 1976;37:1469-1477.[Medline]
- Daddi N, Ferolla P, Urbani M, et al. Surgical treatment of neuroendocrine tumors of the lung Eur J Cardiothorac Surg 2004;26:813-817.[Abstract/Free Full Text]
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Abstract
Introduction
