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| CASE REPORT |
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| Year : 2023 | Volume
: 6
| Issue : 2 | Page : 72-75 |
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A rare case of lymphangitis carcinomatosis presenting as diffuse parenchymal lung disease
P Maheswari, K Krishnamoorthy, T Joseph Pratheeban, E Mathan, O M. Rahman Shahul Hameed
Department of Respiratory Medicine, Tirunelveli Medical College, Tirunelveli, Tamil Nadu, India
| Date of Submission | 23-Feb-2023 |
| Date of Decision | 15-May-2023 |
| Date of Acceptance | 30-May-2023 |
| Date of Web Publication | 13-Jul-2023 |
Correspondence Address:
P Maheswari
Department of Respiratory Medicine, Tirunelveli Medical College, Tirunelveli, Tamil Nadu
India
 Source of Support: None, Conflict of Interest: None
DOI: 10.4103/japt.japt_8_23
Lymphangitis carcinomatosis (LC) is the infiltration and inflammation of lymphatic vessel secondary to the spread of malignancy from a primary site. Most cases result from dissemination of adenocarcinomas. Its presentation may be unilateral or bilateral, it is asymmetrical and is limited to one lobe of the lung especially lower lobes, focal unilateral presentation is observed in 50% of patients, while only 6%–8% of lung metastases present as diffusely infiltrating pattern. In this case report, we present a case of adenocarcinoma with LC presenting as diffuse parenchymal lung disease which is a rare presentation. A 45-year-old female matchbox industry worker by occupation with no comorbid illness presented with 8-month history of cough with scanty mucoid expectoration and Grade II dyspnea according to the modified Medical Research Council along with history of loss of appetite and weight. CT chest shows parenchymal nodule with spiculated margin in the anterior segment of the upper lobe, nodular thickening of the Broncho vascular interstitium and pleura, diffuse interstitial septal thickening and randomly distributed nodules in all lobes. PET scan showed low grade metabolically active nodule in the anterior segment of the left upper lobe, metabolically active mediastinal, supraclavicular and retrocrural lymph nodes, mild pleural effusion and Low-grade diffuse metabolism was noted in the uterine endometrium. Low-grade diffuse metabolism was noted in the uterine endometrium. Abdominal ultrasound revealed an endometrial thickness of 8 mm. Visual inspection with acetic acid (VIA), visual inspection with lugol's iodine (VILI) and Papanicolaou smear was done which was suggestive of inflammatory smear, and Papanicolaou smear was done and was suggestive of inflammatory smear. Fiber-optic bronchoscopy was done, it was normal, and transbronchial lung biopsy revealed adenocarcinoma lung. The patient was referred to medical oncology, and the first cycle of chemotherapy was started with cisplatin and pemetrexed. Physicians should be aware of pulmonary lymphangitic carcinomatosis from lung adenocarcinoma and consider it in patients with pulmonary symptoms who are unresponsive to antibiotics. Despite establishment of diagnosis with cellular etiology even primary source, the condition is fatal. This rare condition needs to be considered in appropriate clinical settings.
Keywords: Adenocarcinoma, diffuse parenchymal lung disease, lymphangitis carcinomatosis
How to cite this article:
Maheswari P, Krishnamoorthy K, Pratheeban T J, Mathan E, Hameed O M. A rare case of lymphangitis carcinomatosis presenting as diffuse parenchymal lung disease. J Assoc Pulmonologist Tamilnadu 2023;6:72-5 |
How to cite this URL:
Maheswari P, Krishnamoorthy K, Pratheeban T J, Mathan E, Hameed O M. A rare case of lymphangitis carcinomatosis presenting as diffuse parenchymal lung disease. J Assoc Pulmonologist Tamilnadu [serial online] 2023 [cited 2023 Sep 30];6:72-5. Available from: https://www.japt.in//text.asp?2023/6/2/72/381421 |
| Introduction | |  |
Lymphangitis carcinomatosis is the infiltration and inflammation of lymphatic vessel secondary to the spread of malignancy from a primary site. Most cases result from dissemination of adenocarcinomas.[1] Microhematogenous spread to the periphery of the lung, with subsequent retrograde, centripetal lymphatic extension toward the hilar region, is the responsible mechanism in approximately 75% of patients. The remaining cases are due to retrograde extension from a hilar tumor or from an ipsilateral lung or breast carcinoma. In the latter settings, the lymphangitic spread is unilateral; in comparison, microhematogenous seeding is frequently bilateral. High-resolution computed tomography can detect lymphangitic tumor in up to 50% of patients who are symptomatic but have normal-appearing lungs on chest radiography. The imaging features of lymphangitic tumor are determined by the pattern of bronchovascular and lymphatic spread. Pulmonary lymphangitic carcinomatosis almost always represents the clinical form that denotes an end-stage malignancy.[2] The diagnosis may be difficult to make as the clinicoradiological picture is often confused with other interstitial lung disorders such as pulmonary congestion, interstitial pneumonitis, pulmonary fibrosis, bronchioloalveolar carcinoma, and granulomas. Physicians should be aware of pulmonary lymphangitic carcinomatosis from lung adenocarcinoma and consider it in patients with pulmonary symptoms who are unresponsive to antibiotics. Its presentation may be unilateral or bilateral, it is asymmetrical and is limited to one lobe of the lung especially lower lobes, focal unilateral presentation is observed in 50% of patients, while only[3] 6%–8% of lung metastases present as diffusely infiltrating pattern.
In this case report, we present a case of adenocarcinoma with lymphangitis carcinomatosis presenting as diffuse parenchymal lung disease which is a rare presentation.
| Case Report | | |
A 45-year-old female matchbox industry worker by occupation with no comorbid illness presented with 8-month history of cough with scanty mucoid expectoration and Grade II dyspnea according to the modified Medical Research Council along with history of loss of appetite and weight. Computed tomography (CT) chest shows parenchymal nodule with spiculated margin in the anterior segment of the upper lobe, nodular thickening of the Broncho vascular interstitium and pleura [Figure 1] and [Figure 2], Diffuse interstitial septal thickening and randomly distributed nodules in all lobes was noted as shown in [Figure 1], [Figure 2], [Figure 3]. Serum angiotensin-converting enzyme level was taken and it was 73.1 U/L, rheumatoid arthritis factor was negative, and calcium level was within normal limits. She was further worked up with positron emission tomography scan which showed a low-grade metabolically active soft-tissue nodule with spiculated margins in the left upper lobe. Metabolically active mediastinal, supraclavicular and retrocrural lymph nodes, mild left pleural effusion and a low-grade diffuse metabolism was noted in the uterine endometrium [Figure 4] and [Figure 5]. positron emission tomography scan which showed a low-grade metabolically active soft-tissue nodule with spiculated margins in the left upper lobe [Figure 6]. On examination, the patient was conscious, oriented, afebrile, not dyspneic and tachypneic with stable vitals and Grade II clubbing. On auscultation, bilateral normal vesicular breath sounds were heard. positron emission tomography scan which showed a low-grade metabolically active soft-tissue nodule with spiculated margins in the left upper lobe [Figure 6]. | Figure 1: Nodule with spiculated margin in the anterior segment of the left upper lobe and nodular thickening of the bronchovascular interstitium with pleural effusion
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 | Figure 2: Nodular pleural thickening with diffuse interstitial infiltrates
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 | Figure 5: Active metabolic uptake in the mediastinal supraclavicular nodes and the soft-tissue nodule. Uterine endometrium also shows uptake
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 | Figure 6: PET-CT showing nodular uptake. PET: Positron emission tomography, CT: Computed tomography
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On examination, the patient was conscious, oriented, afebrile, not dyspneic and tachypneic with stable vitals and Grade II clubbing and Papanicolaou smear was done and was suggestive of inflammatory smear. Fiber-optic bronchoscopy was done, it was normal, and transbronchial lung biopsy revealed adenocarcinoma lung. The patient was referred to medical oncology, and the first cycle of chemotherapy was started with cisplatin and pemetrexed.
| Discussion | | |
Secondary neoplastic diseases of the lung frequently present as lymphangitis carcinomatosis.[1],[4] It most commonly originates from primary malignancy in the breast, lung, stomach, thyroid, cervix, colon, pleura, and prostate. The most frequent cell types of lung cancer causing lymphangitis carcinomatosis in patients with lung cancer include adenocarcinoma (80%) and small cell carcinoma. lymphangitis carcinomatosis secondary to squamous cell lung carcinoma is rare.[4] The spread of tumor cells to the pulmonary lymphatic system or the adjacent interstitial tissue results in thickening of the bronchovascular bundles and septa. The pulmonary interstitium is further thickened by a desmoplastic reaction caused by the proliferation of tumor cells and lymphatic dilatation.[3],[5] The spread of the neoplasm outside the interstitium and lymphatic spaces into the adjacent parenchyma can result in a nodular pattern.
The pathologic features of pulmonary lymphangitis carcinomatosis include infiltration of cancer cells and interstitial edema in and around lymphatic vessels as well as infiltration of inflammatory cells caused by lymph node metastasis in the lung.[1] The metastatic cancer in the mediastinal and pulmonary hilar lymph nodes may obstruct lymphatic drainage, resulting in retrograde migration of cancer cells into terminal lung tissues via lymphatic vessels or anterograde migration of cancer cells in the pleura into the pulmonary hilar lymph nodes through intrapulmonary lymph vessels. In addition, a cancer embolus may form in the terminal vessels of the lung due to hematogenous metastasis, which can invade the surrounding lymphatic vessels. Thus, hilar and mediastinal lymph node metastasis may be present or absent in pulmonary lymphangitis carcinomatosis, depending on the route of metastasis of the primary cancer.
Pulmonary lymphangitis carcinomatosis can present with dyspnea and cough, and this may precede the diagnosis of the primary tumor.[4] Even if[2] histologically confirmed, the chest radiograph is normal in 30%–50% of cases. A variety of chest radiographs and CT changes are reported and can mimic those of sarcoidosis. Nodular thickening and ground-glass attenuation are seen in 30%–60% of patients with sarcoidosis.[1] The nodules in sarcoidosis mainly involve the central regions of the middle and upper lobes of the lungs. In contrast, changes usually occur in the lower lobes in pulmonary lymphangitis carcinomatosis. Although imaging studies may suggest sarcoidosis, the diagnosis should be confirmed by biopsy results indicating noncaseating granulomas and by the exclusion of other causes of granulomatous disease.[6],[7]
Rapid onset and progression of symptoms, asymmetrically enlarged lymph nodes, predominant disease in the lower lobes of the lungs, and lack of response to steroids within 2–4 weeks also should[3] alert clinicians to a diagnosis other than sarcoidosis. The characteristic findings are thickening of the interlobular septa, with beading caused by perilymphatic nodules, polygon or polygonal arcade formation, and thickening of the central bronchovascular structures.
In spite of the extensive involvement, the lung parenchyma is not distorted. The absent distortion distinguishes lymphangitic spread from sarcoidosis, which can otherwise produce similar findings but usually with less conspicuous thickening of interlobular septa.
Thickening of the interlobular septa and peribronchovascular interstitium without a nodular pattern may be seen in other conditions, such as pulmonary edema and idiopathic pulmonary fibrosis.
The prognosis for patients with PLC is extremely poor with less than half surviving the past 3 months. However,[3] platinum-based chemotherapy has led to transient remissions in some cases. Nebulization chemotherapy was introduced by[1] Tatsumura et al. in the treatment of inoperable non-small lung cancer. Aerosolized particles of the chemotherapeutic drug, 5-fluorouracil (FU) along with a solvent, were delivered using an ultrasonic nebulizer through the mouth. 5-FU administered by nebulization accumulates mainly in the trachea, bronchi, and regional nodes. Nebulization allows direct and prolonged action of high concentration of 5-FU on the tumor tissue.
This study also demonstrated negligible systemic absorption of 5-FU. The nebulized drug is retained for a considerably longer period in tumor tissue than in normal tissue resulting in high antitumor activity with negligible or no systemic adverse effects.
| Conclusion | | |
Despite establishment of diagnosis with cellular etiology even primary source, the condition is fatal. Marked weight loss, rapidly worsening dyspnea, dry cough, in the background of diffuse reticulonodular lung lesion in the imaging are the clues to suspect lymphangitis carcinomatosis.[1],[8] This rare condition needs to be considered in appropriate clinical settings.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
| References | | |
| 1. | Albhaisi SAM, Luqman N. Pulmonary Lymphangitic Carcinomatosis due to Metastatic Adenocarcinoma of the Lung: A Case Report. Clin Med Rev Case Rep 2016;3:099. 10.23937/2378-3656/1410099.  |
| 2. | Descombes E, Gardiol D, Leuenberger P. Transbronchial lung biopsy: An analysis of 530 cases with reference to the number of samples. Monaldi Arch Chest Dis 1997;52:324-9. |
| 3. | 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;46:491-4. |
| 4. | Gilchrist FJ, Alton H, Brundler MA, Edwards L, Plunkett A, Rao S. European Respiratory Review 2011;20:208-10. DOI: 10.1183/09059180.00000911. |
| 5. | Biswas A, Sriram PS. Getting the whole picture: lymphangitic carcinomatosis. Am J Med 2015;128:837-40. |
| 6. | Levy H, Horak DA, Lewis MI. The value of bronchial washings and bronchoalveolar lavage in the diagnosis of lymphangitic carcinomatosis. Chest 1988;94:1028-30. |
| 7. | Prakash P, Kalra MK, Sharma A, Shepard JA, Digumarthy SR. FDG PET/CT in Assessment of Pulmonary Lymphangitic Carcinomatosis. Am J Roentgenol 2010;194:2316 |
| 8. | |
[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6]
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