Incidental Pleural-Based Pulmonary Lymphangioma CASE REPORT
Total Page:16
File Type:pdf, Size:1020Kb
CASE REPORT Incidental Pleural-Based Pulmonary Lymphangioma Michael G. Benninghoff, DO William U. Todd, MD Rebecca Bascom, MD, MPH Adult benign thoracic lymphangiomas typically present as ondary forms develop in adults as a result of lymphatic channel incidental mediastinal lesions, or, more rarely, as solitary pul- obstruction caused by radiation, surgery, or infection.1 monary nodules. Symptomatic compression of vital struc- Patients with lymphangiomas can be asymptomatic for tures may require lesion resection or sclerotherapy. In the many years and often have symptoms only after vital structures present report, we describe the incidental finding of a soli- are compressed by the lesion. As such, asymptomatic lym- tary pleural-based pulmonary lymphangioma in a 38-year- phangiomas are typically found incidentally on chest radio- old woman with chronic arm and shoulder pain. Positron graphs or computed tomography (CT) scans without any emission tomography revealed that the lesion was highly unique visible characteristics. Although biopsies can identify fluorodeoxyglucose-avid. Biopsy exposed benign tissue malignant or benign lesions, empiric resection is often per- consistent with lymphangioma. After continued radio- formed as a precautionary measure. If surgical means are pur- graphic tests, the lesion was determined to be an unlikely sued, however, it is important to remove the entire lesion to source of the patient’s chronic pain. The present report is, to avoid tumor regrowth. our knowledge, the first published case of solitary pleural- In the present report, we describe a woman who had based pulmonary lymphangioma in the medical literature. chronic pain in her right upper arm and shoulder. A pleural- J Am Osteopath Assoc. 2008;108:525-528 based lesion was found incidentally and was initially sus- pected to be the cause of her pain. However, further exami- nation suggested otherwise. The present report is, to our ulmonary lymphatic disorders are rare and are often knowledge, the first published case of isolated pleural-based Pmistaken for serious pulmonary diseases. Among such pulmonary lymphangioma as well as a fluorodeoxyglucose disorders are pulmonary lymphangiectasis, which is often (FDG)-avid pleural lesion with this pathology. fatal in children; lymphangiomatosis, which comprises mul- tiple lymphangiomas and typically has multiorgan involve- Report of Case ment; and lymphatic dysplasia syndrome, which results in A 38-year-old woman presented to the outpatient Pulmonary peripheral lymphedema and pleural effusions.1 Pulmonary Clinic at the Penn State College of Medicine in Hershey, Pa, lymphangiomas, perhaps the most common of the four main complaining of chronic pain in her upper arm and shoulder on manifestations of pulmonary lymphatic disorders, are focal the right side. congenital malformations that consist of atretic nonfunc- The patient reported that while she was at work 3 years tional lymphatic tissue separated from the lymphatic drainage earlier, a forklift trapped her right upper arm and shoulder system.1 against a wall, resulting in immediate and persistent numbness Mediastinal lymphangiomas comprise 10% while intra- and tingling in the right hand. Initial surgical treatment pulmonary lymphangiomas make up less than 1% of all lym- included decompression of the right radial nerve and cervical phangiomas.2 More than 90% of thoracic lymphangiomas spinal fusion of vertebrae C4 through C7. However, chronic, occur in children younger than 2 years1 and may be more unrelenting pain persisted. The patient therefore sought neu- prevalent in those with superior vena cava syndrome.3 Sec- rosurgical consultation for possible brachial plexus surgery. The neurosurgeon ordered a thoracic CT scan, the results of which revealed a right apical pleural-based lesion. The neurosurgeon referred the patient to a community pulmonary specialist, who monitored the lesion. In 1 year, the lesion grew from 1.5 cm ϫ 1.2 cm to 1.6 cm ϫ 1.4 cm. A From the Penn State College of Medicine in Hershey, Pa. positron emission tomography (PET) scan confirmed the pres- Address correspondence to Michael G. Benninghoff, DO, Penn State Her- shey Medical Center, 500 University Dr, H041, Hershey, PA 17033-2360. ence of a right apical pleural-based nodule. The pulmonologist E-mail: [email protected] suspected a malignant primary lung mass and referred the patient to an osteopathic physician (M.G.B.) at the Penn State Submitted November 2, 2006; revision received January 9, 2007; accepted Jan- uary 10, 2007. College of Medicine. Benninghoff et al • Case Report JAOA • Vol 108 • No 9 • September 2008 • 525 CASE REPORT The patient denied fever, chills, night sweats, and weight chronic pain. Because no vital structures were obstructed, the loss. She also stated that she did not have chest pain, shortness lesion was not excised. However, as consensus dictated at the of breath, cough, wheezing, or hemoptysis. She had no history conference meeting, the patient continues to receive follow-up of seasonal or perennial postnasal drip, rhinitis, sinusitis, CT scans for serial observation of the lesion. Gabapentin, pre- asthma, bronchitis, or pneumonia. She had intermittent scribed by the patient’s family physician, alleviated her chronic headaches but no history of seizures or skin lesions. The patient arm and shoulder pain, though the source of that pain was not had no history of alcohol or drug abuse, and though she never found. smoked cigarettes, she had exposure to secondhand smoke from both parents throughout childhood. Before the work Discussion injury, which left her disabled, she had worked as a laborer in Cases of lymphangioma have been reported in the form of a battery factory for 6 years, and before that, in a fabric factory. isolated parenchymal lesions,5 chest wall lesions,6 and multiple The patient also reported that she had consistently received age- cystic lesions throughout the thorax.7 However, lymphan- appropriate preventive health screening. giomas presenting as solitary pulmonary lesions are rare.8 On physical examination, the patient’s blood pressure Likewise, lymphatic abnormalities are typically found in was 120/84 mm Hg; heart rate, 72 beats per minute; respira- any region of the body where lymphatic drainage exists— tory rate, 16 breaths per minute; and body mass index, 30. most commonly, the head and neck, axilla, and abdomen.8 In She appeared healthy and in no distress, with neither cervical the chest, such lesions are most frequently found in the medi- nor axillary adenopathy. Cardiovascular examination revealed astinum, accounting for up to 4.5% of mediastinal tumors.8 Of a regular heart rate without murmurs. Her lungs were clear to the adult patients who have solitary pulmonary lesions, most auscultation with normal percussion notes and no point ten- are asymptomatic. Other clinical features, such as cough and derness with chest wall compression. Chest excursion and dyspnea, may be present if the patient’s vital structures are diaphragm descent were normal. The patient’s fingernails compromised. were normal, her fingers were not clubbed, and she had no Lymphatic dysplasia syndrome and lymphangioma are peripheral edema or skin or joint lesions. The results of her neu- the two most common diseases in their class, with 90% of rologic examination were normal except for 4/5 muscle lymphangiomas occurring in children younger than 2 years.1 strength in right shoulder abduction and diminished sensation In the present case, the absence of radiographic pleural effu- on the palmar aspect of the medial two fingers on her right sion excluded the presense of a chylothorax, therefore elimi- hand. nating the possibility that the patient had lymphatic dysplasia The initial CT scan showed a focal area of nodular thick- syndrome. With lymphangiomas, CT scans indicate the loca- ening measuring 60 Hounsfield units—similar to the density tion, size, and density of lesions, but they cannot establish the of muscle—located in the lateral right apical lung pleura. There diagnosis. While the lesion in the present report measured were no other lung or pleural-based masses, no mediastinal, 60 Hounsfield units, lymphangiomas are typically Ϫ4 to hilar, or axillary adenopathy, and no pleural effusion. The 34 Hounsfield units and are smooth cystic masses.9 Spicu- heart and great vessels were normal, as were the postsurgical lated lesions can also occur. The high density of the mass changes from the spinal fusion. described in the present report was unlike any previous lym- A second CT scan 3 months later revealed an increase in phangiomas found in the medical literature. lesion size. A PET scan taken immediately afterward showed Magnetic resonance imaging (MRI) is considered the most a single 1 cm ϫ 2 cm FDG-avid lesion at the same location. precise modality for characterizing lesion tissue and for deter- Three core biopsies under CT guidance yielded benign, CD31- mining tumor extension, particularly in the case of lymphan- positive tissue, which is consistent with lymphangioma giomas.1,9-12 Because the lesion in the present report was suf- (Figure 1).4 A CT scan with intravenous contrast administered ficiently delineated using a contrast medium with CT scans, an to the right antecubital fossa showed no evidence of struc- MRI was not ordered. However, a PET scan was ordered tural compression to right apical lymphatic flow (Figure 2). because the lesion appeared to be noncystic. The scan revealed The patient returned for a third CT scan 3 months later, that the lesion was FDG-avid, yet, to our knowledge, FDG at which point the lymphangioma measured 1.3 cm ϫ 2.2 cm avidity has not been exposed previously on the PET scans of between the lateral aspect of the second and third ribs on the lyphangiomas. While PET scans have been used to identify right side. Minimal dependent bibasilar atelectasis was present. malignant lesions, malignant degeneration has not been The bony structures were otherwise unremarkable, and no reported.13,14 To identify areas of increased glucose metabolism, right shoulder mass was seen on the margin of the images.