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© 2000 American Society for Clinical Oncology
Case 2: Acute Respiratory Failure Secondary to Lymphangitic CarcinomatosisUniversity of Iowa Hospitals and Clinics, Iowa City, IA A 52-year-old white man with a 30-pack-year history of smoking presented with a 1-day history of increasing dyspnea, nonproductive cough, malaise, fever, myalgias, and decreased appetite. His past medical history was noncontributory. A chest radiograph revealed bilateral reticular infiltrates (Fig. 1). He was placed on azithromycin and levaquin for presumed community-acquired pneumonia. Overnight he developed worsening hypoxia and was transferred to our facility for further management.
Upon arrival, he was in significant respiratory distress with a temperature of 38.3°C, pulse rate of 110 beats per minute, respiratory rate of 28 breaths per minute, and blood pressure of 140/70. Chest examination revealed decreased breath sounds at the bases. Cardiovascular examination showed no jugular venous distension and normal heart sounds, with a soft ejection systolic murmur over the aortic area. The abdomen was benign and the extremities did not reveal edema or clubbing. Lymph nodes were not palpable. Arterial blood gas on 100% oxygen by facemask revealed a pH of 7.45, a partial pressure of carbon dioxide of 37 mmHg, and partial pressure of oxygen of 53 mmHg. Negative study results included legionella DFA in the bronchoalveolar lavage (BAL) fluid, legionella EIA in the urine, and serologies for Rocky Mountain spotted fever. A computed tomography scan of the chest revealed a bilateral lower lobe consolidative process with air bronchograms, a developing right upper lobe interstitial process, and small bilateral pleural effusions. There was no evidence of mediastinal lymphadenopathy or primary mass (Fig. 2). An echocardiogram showed normal left ventricular function.
The patient was electively intubated, and bronchoscopy with BAL and transbronchial biopsies was performed. The BAL revealed an RBC count of 3,710 x 1012/L and a WBC count of 110 x 1012/L (lymphocytes 75%, monocytes 6%, and macrophages 19%). All cultures for bacteria, viruses, acid-fast bacilli, and fungi were negative. Cytologic studies were negative for malignant cells. Biopsies revealed well-differentiated, nonkeratinizing squamous cell carcinoma (SCC) with submucosal involvement of the bronchial wall. He died 12 days after the onset of symptoms from progressive hypoxemia. Autopsy showed diffuse lung nodules involving all lobes (Fig 3). Microscopy revealed multiple foci of moderately differentiated SCC predominantly within the lymphatic spaces and spilling out into the alveolar and interstitial spaces (Fig 4; A, arteriole; B, bronchiole; T, tumor cells; hematoxylin and eosin stain x10). The tumor cells were composed of keratinizing squamous cells with abundant eosinophilic cytoplasm, prominent intercellular bridges, and nuclear pleomorphism (Fig 5; hematoxylin and eosin stain x40). No tumor was found in the peribronchial lymph nodes. No tumor emboli were seen. Immunochemistry analysis showed positive reactivity for CAM5.2, which supports an epithelial differentiation. Stains for S-100, chromogranin, and synaptophysin had no reactivity. Extensive dissection of the respiratory tract and the other organs failed to reveal a primary lesion.
Lymphangitic carcinomatosis, intrapulmonary spread of metastatic neoplasm via lymphatics, and adjacent connective tissue is frequently seen with lung, breast, stomach, pancreas, and prostate cancers. A de novo presentation of lung cancer with extensive lymphatic spread is rare. The most striking presenting feature is progressive, unrelenting dyspnea that is often disproportionate to the initial abnormalities seen on chest radiographs. Nonproductive cough is often present and chest pain may occur. Physical examination reveals tachypnea; tachycardia and occasionally cyanosis may be present. Auscultation of the chest may reveal crackles with no evidence of consolidation.1,2 Radiographs usually show a coarse reticulonodular pattern, which is more obvious in the lower zones with or without Kerley B lines. The presence of Kerley B lines in the absence of congestive heart failure is very suggestive of lymphangitic carcinomatosis.1 Other radiographic features include pleural effusions, hilar, and mediastinal adenopathy. In about half the cases, the chest radiograph may be completely normal.1,3-6 Computed tomography scans show nodular thickening of the bronchovascular bundles, thickened interstitial lines, polygonal lines, and beaded interlobular septae; discrete lung nodules are uncommon.1,6 BAL and bronchial washings may yield a diagnosis,7,8 but flexible bronchoscopy with transbronchial biopsy is the diagnostic procedure of choice.9 Cytologic studies performed on blood samples obtained from a wedged pulmonary artery catheter have been shown to yield a diagnosis in most patients in a small series.10 Microscopically, neoplastic cells are seen in clusters or cords within the lymphatic spaces and in the peribronchovascular and interlobular interstitium. Tumor emboli are frequently present in adjacent small arteries and arterioles and may be associated with a thrombus.1,11,12 It is thought that most cases of lymphangitic spread occur as a result of tumor microembolization.1,11,12 Other potential mechanisms include retrograde spread from involved lymph nodes, local extension from a primary pulmonary tumor, and transdiaphragmatic spread. Tumor emboli to the adjacent vessels may account for the severe hypoxia and dyspnea observed in these patients.11 Other factors include compression of the adjacent pulmonary vessels by the distended lymphatics and a vasculopathy that develops as a result of the lymphangiatic carcinamatosis.12,13 Moreover, progressive plugging of the lymphatics causes the lymphatic lattice around the alveoli to become rigid, thereby increasing the work of breathing and resulting in profound dyspnea.3 This case is unique because the acute presentation without prior complaints or constitutional symptoms and the subsequent fulminant course are both atypical for lymphangitic carcinomatosis. Second, lymphangitic carcinomatosis as the de novo presentation of SCC of the lung is extremely unusual. SCC of the lung commonly arises in the segmental or lobar bronchus. Tumor invasion of the submucosal and peribronchial connective tissue with concomittant extension into the airway lumen is typically seen, and airway obstruction is almost invariable.1 Although lymphangitic spread is a well-described complication of SCC, to our knowledge, this is the first reported case of lymphangitic carcinomatosis as a de novo presentation of SCC. Third, the radiographic findings of dense consolidations with air bronchograms as a manifestation of lymphangitic carcinomatosis have not been previously described. Fourth, the profound hypoxemia noted in our patient could not be explained by evidence of tumor emboli or vasculopathy. It is possible that compression of the adjacent vessels by the distended lymphatics and the dense consolidation is the reason for the hypoxia. REFERENCES 1. Fraser RG, Pare JAP, Pare PD,et al: Diagnosis of Diseases of the Chest (ed 3). Philadelphia, PA, WB Saunders, 1991 2. Hauser TE, Steer A: Lymphangitis carcinamotosis. Ann Intern Med 34:881, 1951
3.
Goldsmith HS, Bailey HD, Callahan EL, et al: Pulmonary lymphangitic metastases from breast carcinoma. Arch Surg 94:483-488, 1967 4. Alkalay I, Farifax CW, Bullard JC: Lymphangitic carcinamotosis of lung with normal appearing chest x-ray films. Chest 62:229-230, 1972 5. Green N, Kern W, Levis R, et al: Lymphangitic carcinomatosis of the lung: Pathologic, diagnostic and therapeutic considerations. Radiat Oncol Biol Phys 2:149-153, 1977
6.
Munk PL, Muller NL, Miller R, et al: Pulmonary lymphatic carcinomatosis CT and pathologic findings. Radiology 166:705-709, 1988
7.
Levy H, Horack DA, Lewis MI: The value of bronchial washings and bronchoalveolar lavage in the diagnosis of lymphangitic carcinomatosis. Chest 94:1028-1030, 1988
8.
Fedullo AJ, Ettensohn DB: Bronchoalveolar lavage in lymphangitic spread of adenocarcinoma to the lung. Chest 87:129-131, 1985 9. Arando C, Sidhu G, Sasso LA, et al: Transbronchial lung biopsy in the diagnosis of lymphangiatic carcinamatosis. Cancer 42:1995-1998, 1978[Medline] 10. Masson RG, Krikorian J, Lukl P, et al: Pulmonary microvascular cytology in the diagnosis of lymphangiatic carcinomatosis. N Engl J Med 321:71-76, 1989[Abstract] 11. Gonzales-Vitale JC, Garcia-Bunuel R: Pulmonary tumor emboli and cor pulmonale in primary carcinoma of the lung. Cancer 38:2105-2110, 1976[Medline] 12. Soares FA, Pinto AP, Landell M, et al: Pulmonary tumor embolism to arterial vessels and carcinomatous lymphangitis. Arch Pathol Lab Med 117:827-831, 1993[Medline]
13.
Altemus LR, Lee RE: Carcinomatosis of the lung with pulmonary hypertension. Arch Int Med 119:32-38, 1967
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Copyright © 2000 by the American Society of Clinical Oncology, Online ISSN: 1527-7755. Print ISSN: 0732-183X
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