Solitary Pulmonary LymphangiomaChad Wilson, Frederic B. Askin, MD, and Richard F.Heitmiller, MD
Departments of Surgery and Pathology, Johns HopkinsHospital, Baltimore, Maryland
Lymphangioma is an abnormal collection of lymphaticsthat are developmentally isolated from the normal lym-phatic system. Lymphangioma rarely presents as a soli-tary pulmonary lesion. We present a case of solitarypulmonary lymphangioma and review the literature on itspathogenesis, clinical features, and radiographic findings.
Lymphangioma is an abnormal collection of lymphat-ics that are isolated from the normal lymphatic
system. It results from an abnormality in developmentallymphangiogenesis. Only rarely does lymphangiomapresent as a solitary pulmonary “coin lesion.” Wepresent a case of solitary pulmonary lymphangioma andreview the literature on its pathogenesis, clinical features,and radiographic findings.
We treated a 51-year-old white man who fell, sustainingblunt left chest wall trauma. An emergency room evalu-ation included PA and lateral chest and selective left ribfilms. No rib fractures, pneumothorax, or pleural fluidwas identified. However, a 1.8-cm peripheral right lungmass was identified, which was interpreted as suspiciousfor a nonsmall cell tumor (Fig 1). Aside from the left chesttrauma, the patient was asymptomatic. Medical historywas remarkable for a 60 pack-year smoking history andgastroesophageal reflux. A chest x-ray 16 years earlierwas normal. Computed tomography (CT) of the chestand abdomen showed a solitary 1.8-cm peripheral, non-calcified lesion in the right upper lobe anterior segment(Fig 2) without hilar or mediastinal adenopathy. Pulmo-nary function included a forced expiratory volume in 1second of 3.8 L, and a single breath deffusing capacity forcarbon monoxide (DLCO) of 75% predicted levels. Wechose to resect the lesion because of concern that it mightbe a primary lung cancer. A right lateral thoracotomy wasused. A solitary lesion was identified in the upper lobe,anterior segment. The lesion was removed in a wedgefashion. Intraoperative pathologic evaluation found abenign lesion that initially was thought to be a muco-coele. Final pathology results showed it to be a benignmass consisting of contiguous, interconnecting lymphaticspaces (Fig 3). The surgical margins were negative. Thediagnosis of pulmonary lymphangioma was made by oneof the authors (F.A.), who has described this entitypreviously [1]. The postoperative course was uncompli-
cated. The patient is asymptomatic and without recurrentlung nodules 20 months postoperatively.
Comment
Lymphangioma is an uncommon lesion that rarely re-sults in a solitary pulmonary lesion. The term lymphangi-oma refers to a benign collection of contiguous, intercom-municating lymphatics that do not communicate with thenormal lymphatic system (Fig 2). As lymph is secretedinto this sequestered space, the lesion grows. This lesionis thought to be the result of a developmental abnormal-ity in lymphangiogenesis. Hilliard and colleagues [2]
Accepted for publication Sept 19, 2000.
Address reprint request to Dr Heitmiller, Department of Surgery, JohnsHopkins Hospital, Osler 624, 600 Wolfe St, Baltimore, MD 21287-5674;e-mail: [email protected].
Fig 2. Chest computed tomography through the level of the lesiondemonstrating a peripheral, noncalcified lesion in the anterior seg-ment of the upper lobe.
1337Ann Thorac Surg CASE REPORT WILSON ET AL2001;71:1337–8 PULMONARY LYMPHANGIOMA
emphasized that lymphangioma is part of a spectrum ofdevelopmental lymphatic disorders that share a commonpathogenesis, including lymphangioma (localized le-sions), lymphangiomatosis (multifocal lesions), lymphan-giectasis (dilated lymphatics in their normal location),mixed vascular lymphatic angiomas, and combinations oflymphatic and other tissues (including the syndromelymphangiomyomatosis). Recent immunohistochemicaldata confirmed a common origin of these separate syn-dromes [3]. All of these abnormalities are thought to beneoplastic, but clinically they can exhibit a wide range ofbenign or malignant characteristics.
Lymphangiomas can occur in any region of the body inwhich there is lymphatic drainage; however, they aremost common in the head and neck, abdomen, and axilla.The single most common site of occurrence is in the neck,where the lesion is referred to as a cystic hygroma. In thechest, lymphangiomas are most commonly found in themediastinum, where they account for 0.7% to 4.5% of allmediastinal tumors [4]. Localized pulmonary lymphangi-omas have been described but are rare [5, 6]. They havebeen found in patients over a wide age range (6 monthsto 67 years). In a review of 20 patients with thoraciclymphangioma (including mediastinal), Shafer and asso-ciates [4] noted a slight predominance in women. In ourreview, all adult patients with intrapulmonary lym-phangioma were men, including our patient. Symptomsvaried widely depending on patient age and extent ofdisease. In neonates and infants, cystic lymphangiomaoften present with pneumothorax and respiratory dis-tress. Holden and colleagues [7] treated a patient withboth intrapulmonary and mediastinal lymphangiomawho presented with hemoptysis. Most adult patients,however, present with an asymptomatic lung lesion sus-picious for primary lung tumor. The CT and magneticresonance imaging findings of lymphangioma have beendescribed, but these findings do not appear to be suffi-ciently characteristic to differentiate lymphangioma fromnonsmall cell lung cancer reliably. The most common CTappearance of lymphangioma is that of a cystic mass withsmooth margins; however, spiculations and calcificationhave also been noted.
The natural history of intrapulmonary lymphangiomais not known. Surgical resection should be considered inlight of the neoplastic nature of these lesions and thedifficulty in clinically differentiating them from primarylung cancer. The available data support the use of limited(wedge) lung resection. Minimally invasive techniqueshave been used successfully [6]. No recurrence of com-pletely resected intrapulmonary lymphangioma has beenreported.
This work was supported by the Lawrence R. Katz ThoracicInternship Award.
References
1. Langston C, Askin FB. Pulmonary disorders in the neonate,infant, and child. In: Thurlbeck WM, Churg AM, eds. Pathol-ogy of the lung. 2nd ed. New York: Thieme Medical Publish-ers, 1995:151–94.
2. Hilliard RI, Mckendry JBJ, Phillips MJ. Congenital abnormal-ities of the lymphatic system: a new clinical classification.Pediatrics 1990;86:988–94.
3. Brown M, Pysher T, Coffin CM. Lymphangioma and congen-ital pulmonary lymphangiectasis: a histologic, immunohisto-chemical, and clinicopathologic comparison. Mod Pathol1999;12:569–75.
4. Shafer K, Rosado-de-Christenson ML, Patz EF, Young S,Farver CF. Thoracic lymphangioma in adults: CT and MRimaging features. AJR Am J Roentgenol 1994;162:283–9.
5. Takahara T, Morisaki Y, Torigoe T, Sano S, Nakamura E,Fujino K, et al. Intrapulmonary cystic lymphangioma: reportof a case. Surg Today 1998;28:1310–2.
6. Takemura T, Watanabe M, Takagi K, Tanaka S, Aida S.Thoracoscopic resection of a solitary pulmonary lymphangi-oma: report of a case. Surg Today 1995;25:651–3.
7. Holden WE, Morris JF, Antonovic R, Gill TH, Kessler S. Adultintrapulmonary and mediastinal lymphangioma causing he-moptysis. Thorax 1987;42:635–6.
Dysphagia Caused by a Fetus-In-Fetu in a 27-Year-Old ManMalek G. Massad, MD, Lawrence Kong, MD,Enrico Benedetti, MD, Daniel Resnick, MD,Luna Ghosh, MD, Alexander S. Geha, MD, andHerand Abcarian, MD
Departments of Surgery and Pathology, The University ofIllinois at Chicago, Chicago, Illinois
Mechanical obstruction of the distal esophagus by afetus-in-fetu is an extremely rare condition that has notbeen previously reported. We present the case of a 27-year-old man who presented with dysphagia caused by fetus-in-fetu contained within a retroperitoneal cystic cavity. Thetumor, noticed since childhood, did not cause any symp-toms until a year before presentation when symptoms ofdysphagia developed. We propose including this entity inthe differential diagnosis of a retroperitoneal mass.
Address reprint requests to Dr Massad, Division of CardiothoracicSurgery (M/C 958), The University of Illinois at Chicago, 840 S Wood StCSB, Suite 417, Chicago, IL 60612; e-mail: [email protected].
Fig 3. Photomicrograph of the solitary pulmonary lymphangiomashowing a benign collection of interconnecting lymphatics. (Hema-toxylin and eosin stains. Original magnification: left, 3 40; right,3 160.)
1338 CASE REPORT MASSAD ET AL Ann Thorac SurgDYSPHAGIA DUE TO FETUS-IN-FETU 2001;71:1338–41
