Ann Thorac Surg 2001;71:696-697
© 2001 The Society of Thoracic Surgeons
Case report
Recurrence of pulmonary mucinous cystic tumor of borderline malignancy
Gary N. Mann, MDa,
Sharon P. Wilczynski, MDc,
Kenneth Sager, MDc,
Frederic W. Grannis, Jr, MDb
a Department of General Oncologic Surgery, City of Hope National Medical Center, Duarte, California, USA
b Section of Thoracic Surgery, City of Hope National Medical Center, Duarte, California, USA
c Department of Pathology, City of Hope National Medical Center, Duarte, California, USA
Accepted for publication March 25, 2000.
Address reprint requests to Dr Mann, University of Washington Medical Center, 1959 NE Pacific St, Box 356410, Seattle, WA 98195
e-mail: gnmann{at}u.washington.edu
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Abstract
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Cystic mucinous tumors of the lung are recently described neoplasms whose histology is different from most lung adenocarcinomas, and represent a spectrum of malignant potential. Little is known of the behavior of the more malignant subtype. We present a cystic mucinous tumor of borderline malignancy that recurred locally following initial limited resection, and was treated with lobectomy.
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Introduction
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Primary mucinous cystic tumors of the lung are rare neoplasms, and span a spectrum of malignant potential. Histologically, they are similar to the more common mucinous tumors of the ovary and appendix [1]. The natural history and recurrence rates of these tumors are unknown. Their recognition and identification are important because of presumed differences in management and prognosis. The clinical presentation and treatment of a case of recurrent pulmonary cystic mucinous tumor of borderline malignancy is described, with recommendations for treatment.
A 66-year-old white female ex-smoker, presented with a round 3-cm mass in the left lower lobe of the lung. A margin negative wedge resection was performed. On initial frozen section, the lesion was felt to represent a benign cystadenoma, and a decision was made not to proceed with lobectomy. On permanent section pathologic examination, the final diagnosis was low-grade mucinous cystadenocarcinoma. A decision was made not to complete the lobectomy given that it was a margin negative resection, and because the literature at the time suggested a good prognosis for this pathological lesion. Four years following the primary resection, an asymptomatic left lower lobe lung nodule was found on follow-up chest roentgenogram. Computed tomographic (CT) scan of the chest confirmed a 2.6-cm left lower lobe mass without mediastinal adenopathy. She underwent repeat left thoracotomy and completion left lower lobectomy with mediastinal lymph node dissection. The histology of both the primary and recurrent tumor were identical and composed of multiloculated cysts lined predominately by stratified columnar cells with numerous goblet cells. Focal tufting of the epithelium and small clusters of tumor floating in the central mucin were found. The tumor nuclei were hyperchromatic with moderate atypia and occasional mitotic figures (Fig 1). Pathologic review confirmed this as a recurrent pulmonary mucinous cystic tumor of borderline malignancy, completely excised, without lymph node metastases. Gynecology consultation and normal abdominal and pelvis CT scans excluded an ovarian primary. Because this was a stage I tumor, no adjuvant therapy was given. Twenty-one months following surgery, the patient is doing well and is currently free of disease.
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Fig 1. Low (A) and high (B) power photomicrographs of H&E preparation of resected tumor, showing the cystic mass lined by columnar epithelial cells with mild-to-moderate nuclear atypia, and filled with mucin dissecting into the adjacent lung parenchyma. (Original magnification x100 in A; original magnification x400 in B.)
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Comment
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We describe the first report of a locally recurrent pulmonary cystic mucinous tumor of borderline malignancy. Pulmonary cystic mucinous tumors are very rare, with the first description by Kragel and colleagues in 1990 [2]. They constitute a morphologic and biologic spectrum that ranges from benign cystadenoma through borderline lesions to mucinous adenocarcinoma. Pulmonary mucinous cystadenomas are composed of cysts lined by well differentiated, histologically benign, columnar mucinous epithelium. The tumors of low malignant potential or borderline mucinous cystic tumors, as described in this report, have stratification of the epithelium with cytologic atypia consisting of hyperchromatic, pleomorphic nuclei that may have prominent nucleoli and mitotic activity. At the malignant end of the spectrum, mucinous adenocarcinomas are characterized by single and sparse groups of malignant cells suspended in pools of mucin. These are usually more cellular and less cystic than the tumors of low malignant potential.
The differential diagnosis of mucinous tumors in the lung includes metastatic tumors as well as primary mucous gland adenoma, mucoepidermoid carcinoma, and colloid type of mucinous carcinomas. It may be very difficult to distinguish by morphology alone a primary lung tumor and a solitary metastasis from mucinous tumors of the gastrointestinal tract, testis, ovary, or breast. A careful clinical and radiologic evaluation to exclude a primary elsewhere is essential. Primary pulmonary mucous gland adenomas are composed of cystically dilated mucinous glands that protrude into the bronchial lumen and are confined to the bronchial wall without parenchymal involvement. Low-grade mucoepidermoid carcinomas can be differentiated by identification of focal sheets of intermediate squamous cells. In our patient, a negative metastatic workup and careful pathologic review confidently excluded metastatic and other primary pulmonary lesions.
To date, only a total of about 20 cases of pulmonary cystic mucinous tumors have been reported in case reports and small series. In those patients with mucinous cystadenoma or borderline malignant tumors, the prognosis would appear to be good following complete, margin negative, resection. Kragel and colleagues [2], and Roux and associates [3] each described 2 cases of resected mucinous cystadenoma. Their patients were alive without evidence of disease 1 to 15 years after thoracotomy. Graeme-Cook and Mark [4] reported survival of between 1 to 9.5 years in 11 patients following resection of mucinous cystic tumors of borderline malignancy. Little is known of the natural history of cystic mucinous adenocarcinoma because reported follow-up is limited: 4 to 10 months [5, 6].
As in the case with non-small cell lung cancer (NSCLC), it would appear that the optimal management of cystic mucinous adenocarcinoma is complete surgical excision by lobectomy. The role of adjuvant therapy in this tumor is undefined, but is probably limited. Reported cases have been treated with surgery alone, either lobectomy or limited resection. In a prospective, randomized trial of early stage NSCLC, the Lung Cancer Study Group [7] observed a 75% increase in recurrence rate attributable to a tripling of the local recurrence rate in those patients undergoing limited resection as opposed to lobectomy. There was a 30% increase in overall death rate in those treated by limited resection, although this did not reach statistical significance. Based on these results and our experience with this patient, we recommend formal anatomic lobectomy for early stage tumors, in those patients who can physiologically withstand it.
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References
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Seidman J.D., Elsayed A.M., Sobin L.H., Tavassoli F.A. Association of mucinous tumors of the ovary and appendix. A clinicopathologic study of 25 cases. Am J Surg Pathol 1993;17:22-34.[Medline]
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Kragel P.J., Devaney K.O., Meth B.M., Linnoila I., Frierson H.F., Travis W.D. Mucinous cystadenoma of the lung: a report of two cases with immunohistochemical and ultrastructural analysis. Arch Pathol Lab Med 1990;114:1053-1056.[Medline]
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Roux F.J., Lantuéjoul S., Brambilla E., Brambilla C. Mucinous cystadenoma of the lung. Cancer 1995;76:1540-1544.[Medline]
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Graeme-Cook F., Mark E.J. Pulmonary mucinous cystic tumors of borderline malignancy. Hum Pathol 1991;22:185-190.[Medline]
-
Davison A.M., Lowe J.W., Da Costa P. Adenocarcinoma arising in a mucinous cystadenoma of the lung. Thorax 1992;47:129-130.[Abstract/Free Full Text]
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Higashiyama M., Doi O., Kodama K., Yokouchi H., Tateishi R. Cystic mucinous adenocarcinoma of the lung: two cases of cystic variant of mucus-producing lung adenocarcinoma. Chest 1992;101:763-766.[Abstract/Free Full Text]
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Ginsberg R.J., Rubinstein L.V. Randomized trial of lobectomy versus limited resection for T1N0 non-small cell lung cancer. Ann Thorac Surg 1995;60:615-623.[Abstract/Free Full Text]
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Abstract
Introduction
