A rare presentation of Pulmonary Lymphangitic Carcinomatosis in cancer of lip: case report
© Babu et al; licensee BioMed Central Ltd. 2011
Received: 8 March 2011
Accepted: 14 July 2011
Published: 14 July 2011
Squamous cell carcinoma of lip is a common malignancy in Indian subcontinent. Metastatic spread is infrequent. Although advanced tumours spread to lymph nodes in the neck, it does not typically present with lung metastasis or with lymphangitic carcinomatosis. We describe a patient who developed cough and increasing dyspnoea while on treatment for carcinoma of lip. Chest x-ray and computed tomography were consistent with lymphangitic carcinomatosis. Lymphangitic carcinomatosis occurs with many different primary tumours and can rarely occur in oral cancers. This is the first report from carcinoma of lip.
The common site of metastasis from most of the solid malignancies is lung. They usually appear as nodular lesions in radiologic images. In some patients, metastasis presents with interstitial spread and it is referred to as Pulmonary Lymphangitic Carcinomatosis (PLC). Head and neck cancers very rarely have lung metastasis in the form of PLC. Oropharyngeal and hypopharyngeal cancers have been reported to have such type of metastasis . Cancer of lip is a common malignancy in Indian subcontinent mainly due to tobacco chewing and that these cancers are detected in early stages due to its visible location, a spread to lung is rare and they are of typical nodular metastases. PLC has not been reported till date from lip cancers in English literature. Here we report a case of PLC arising from cancer of the lower lip.
Lung metastasis from malignant tumours usually present as nodular lesions and rarely as Pulmonary Lymphangitic Carcinomatosis (PLC). PLC is characterised by diffuse spread of malignancy in the lung, causing inflammation of the lymph vessels. The first reported case of PLC was by Gabriel Andral in 1829 . The diffusely infiltrating pattern of metastasis as seen in PLC occurs in 6-8% of lung metastases . 80% of them are from adenocarcinomas. The common sites of primary from which PLC occurs are cancers of breast, bronchus, and stomach [4, 5]. The other described sites with PLC are cancers from colon, pancreas, kidney, cervix, thyroid, larynx and hypopharynx [6–8].
The cancers of head and neck rarely show this type of metastasis. The exact reason is unknown. The described sites in head and neck region are larynx, hypopharynx and thyroid. Metastasis to lymph nodes from advanced cancers of lip is seen in about 44%. Metastasis to lung is reported to be very low. There is no available report suggesting a PLC from oral cancers. PLC as metastatic feature as seen in the case described in this manuscript is an extremely rare presentation.
The pathophysiology is that the tumours spread by haematogenous route to the lung and then through the lymphatics within the lung. The lymphatics in the lung are seen in the peribronchovascular, centrilobular, interlobular and sub pleural regions. The tumour obstructs these lymphatic channels. The dilated lymphatic vessels due to oedema fluid, tumour secretion and the desmoplastic reaction by the tumour cells, produces interstitial thickening which is seen as streaks in imaging studies. The nodular pattern is due to the spread of tumour into the lung parenchyma as seen in usual lung metastases.
The clinical features of PLC are dyspnoea and nonproductive cough with crepitations and without features of consolidation. Chest X-ray shows septal lines (Kerley A and B lines). The differential diagnosis is interstitial lung disease, primary malignancy in the lung, pulmonary sarcoidosis and hypersensitivity pneumonitis. HRCT is the modality of choice for confirmation of the diagnosis. The findings in CT scan are - thickening of interlobular septa, fissures and bronchovascular bundles. These findings may be seen as limited or diffuse and may involve unilateral or bilateral lungs. The radiologic picture may be symmetric or asymmetric in both lungs. The other findings are nodularity in pleura and ground glass opacity . The possibility of interstitial lung disease is to be considered and ruled out. Prakash P et al described the use of PET/CT in diagnosing PLC. In a study of 35, they found that PET/CT has high specificity in detection of pulmonary lymphangitic carcinomatosis .
Histopathological examinations show interstitial oedema and fibrosis along with malignant cells and are found usually on postmortem biopsy. Since the radiological finding in a patient with malignant disease elsewhere is suggestive, a biopsy of the lung is not mandatory.
PLC often presents in the late stages of malignancy and it indicates poor prognosis. The treatment option in PLC is with chemotherapy. Cisplatin have been found to be effective .
Pulmonary Lymphangitic Carcinomatosis may also occur rarely in patients with oral cancers as seen in our patient and its prognosis is very poor even with treatment with chemotherapy.
Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.
- Zieske LA, Myers EN, Brown BM: Pulmonary lymphangitic carcinomatosis from hypopharyngeal adenosquamous carcinoma. Head Neck Surg. 1988, 10 (3): 195-8. 10.1002/hed.2890100308.View ArticlePubMedGoogle Scholar
- Doyle L: Gabriel Andral (1797-1876) and the first reports of lymphangitis carcinomatosa. J R Soc Med. 1989, 82 (8): 491-3.PubMed CentralPubMedGoogle Scholar
- Bruce DM, Heys SD, Eremin O: Lymphangitis carcinomatosa: a literature review. J R Coll Surg Edinb. 1996, 41 (1): 7-13.PubMedGoogle Scholar
- Yamagishi Y, Akiba Y, Izumiya M, Higuchi H, Iizuka H, Takaishi H, Nagata H, Hibi T: [A case of advanced gastric cancer with lymphangitis carcinomatosa after operation of Krukenberg tumor treated by TS-1 plus CPT-11 as third-line chemotherapy]. Gan To Kagaku Ryoho. 2005, 32 (8): 1167-70.PubMedGoogle Scholar
- Gupta PR, Joshi N, Meena RC, Ali M: Asymptomatic lymphangitis carcinomatosis due to squamous cell lung carcinoma. Indian J Chest Dis Allied Sci. 2005, 47 (2): 121-3.PubMedGoogle Scholar
- Thomas A, Lenox R: Pulmonary lymphangitic carcinomatosis as a primary manifestation of colon cancer in a young adult. CMAJ. 2008, 179 (4): 338-40. 10.1503/cmaj.080142.PubMed CentralView ArticlePubMedGoogle Scholar
- Kirk JE, Kumaran M: Lymphangitis carcinomatosa as an unusual presentation of renal cell carcinoma: a case report. J Med Case Reports. 2008, 2: 19-10.1186/1752-1947-2-19.PubMed CentralView ArticleGoogle Scholar
- Yang SP, Lin CC: [Pulmonary lymphangitic carcinomatosis]. Taiwan Yi Xue Hui Za Zhi. 1968, 67 (9): 361-74.PubMedGoogle Scholar
- Zhang K, Huang Y: [Clinical features and diagnosis of pulmonary lymphangitic carcinomatosis]. Ai Zheng. 2006, 25 (9): 1127-30.PubMedGoogle Scholar
- Prakash P, Kalra MK, Sharma A, Shepard JA, Digumarthy SR: FDG PET/CT in Assessment of Pulmonary Lymphangitic Carcinomatosis. AJR Am J Roentgenol. 2010, 194 (1): 231-6. 10.2214/AJR.09.3059.View ArticlePubMedGoogle Scholar
- Kikuchi N, Shiozawa T, Ishii Y, Satoh H, Noguchi M, Ohtsuka M: A patient with pulmonary lymphangitic carcinomatosis successfully treated with TS-1 and cisplatin. Intern Med. 2007, 46 (8): 491-4. 10.2169/internalmedicine.46.6363.View ArticlePubMedGoogle Scholar
This article is published under license to BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.