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Experience of pulmonary lymphangitic carcinomatosis diagnosis

O.S. Kvasnitska, A.O. Bob, Z.A. Lubyanska

Abstract: 

Pulmonary lymphangitic carcinomatosis (PLC) is a metastatic lung disease characterised by diffuse infiltration and obstruction of the pulmonary lymphatic system by tumour cells. The authors describe the case of a 58-year-old woman who underwent broad-spectrum antibiotic therapy and anti-inflammatory medication (glucocorticosteroids) for the suspicion of sarcoidosis associated with interstitial pneumonia. Unfavorable evolution required additional investigations. High Resolution Computed Tomography (HRCT) was suggestive for the diagnosis of PLC. Bronchoscopy with bronchial aspiration could not confirm the disease. Digestive endoscopy and histopathology from gastric biopsy established the diagnosis of a stomach adenocarcinoma. The advanced tumor stage allowed only palliative treatment. Patients with suggestive thoracic HRCT imaging for PLC have to be thoroughly investigated in order to localize the primary tumor.

 

Keywords: 
pulmonary lymphangitic carcinomatosis  

Introduction

Pulmonary lymphangitic carcinomatosis (PLC) is a metastatic lung disease characterised by the diffuse infiltration and obstruction of the pulmonary paren-chymal lymphatic system by tumor cells(2,3). PLC is most commonly seen secondary to adenocarcinomas of breast, lung, stomach, colon, thyroid, prostate, and cervicx. The radiological features are similar to those of other inter-stitial lung diseases, which complicate the differential diagnosis. The thickening of bronchovascular bundles and interlobular septa, ground-glass opacities, pleural effusion, mediastinal lymphadenopathy, and nodular lesions are the most common radiologic findings.

Case presentation

A 58-year-old female patient presented to our clinic in February 2018 with complaints of severe shortness of breath, unproductive cough, and fever up to 38° C (started two weeks before presentation). She linked the occurrence of her symptoms to a viral infection of her family mem-bers. Several days, the patient had ambulatory treatment, after that she was hospitalized and treated with ceftriax-one and levofloxacin. Due to the absence of a clinical and radiological improvement, she was directed to our univer-sity clinic for further detailed examination and treatment.

Past medical history revealed chronic pyelonephritis and a peptic ulcer disease (last treatment in October, 2016). At the admission, the patient was in moderate distress, afebrile, with tachycardia (96-100 bpm), dyspnea and polypneea (26 breaths/min), and an oxygen saturation of 91% in the ambient air. Chest auscultation revealed crack-les in both lungs, mostly in lower lobes. Heart and abdo-men examination was without pathological elements. The chest X-ray revealed bilateral infiltrative opacities. Complete blood count revealed leucocytosis (12x109/L) and eosinofilia (12%), with ESR of 36 mm/hour. Biochemical blood tests were unremarkable. ECG showed tachycardia (110 bpm), with an incomplete right bundle branch block. The percutaneous transthoracic cardiac ultrasound revealed slight increase in the pulmonary artery pressure up to 23 mmHg. In accordance with the above data, we interpreted the case as a bilateral pneumo-nia, and we started the treatment with broad spectrum antibiotics (cefepime, spyramycin, and later meropenem). After a week, the patient’s condition showed no improve-ment. We extended the investigations. High Resolution Computed Tomography (HRCT) showed mediastinal and hilar bilateral lymphadenopathy, diffuse thickening of interstitium (interlobular septa), micronodules and a small pleural effusion (Figure 1a, 1b).

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The flexible bronchoscopy revealed diffuse hypere-mia of the mucosa and significant amount of serous sputum in the bronchial lumen. Cytological conclusion: on the background of RBC there are lots of epithelial cells, some leucocytes, represented by neutrophils. The initial suspicion of a sarcoidosis led to a treatment with prednisolone 60 mg/day and next the reduction of dos-age to 32 mg/day (methylprednisolone). After a week of treatment with corticosteroids, the patient was still in moderate distress, complained of unproductive cough and severe dyspnea. A new extended HRCT revealed the maintaining of the previous imaging and some areas of osteolysis in thoracic vertebrae (Figure 2). In this con-text, PLC was suspected and investigations continued with gastroscopy.

The esophagogastroduodenoscopy described chronic hypertrophic gastritis. Gastric biopsy confirmed the etiology of PLC: undifferentiated adenocarcinoma of the stomach.

Palliative care was proposed to the family who, understanding the severity of the diagnostic, requested the discharge of the patient (after 23 days of hospitaliza-tion), with follow-up appointments in oncology specialty as an outpatient. The patient died after two months.

Discussion

PLC is the permeation of malignant cells into lym-phatic vessels and obstruction, most commonly occur-ring within the chest. While 30% to 40% of malignancies will involve intrathoracic spread, only 6% to 8% of these cases comprise PLC(3). The most common primary tumors that cause PLC are breast (33%), stomach (29%), lung (17%), pancreas (4%), prostate (3%), cervix (3%) and large bowel (2%)(4). The clinical presentation is nonspe-cific, but nearly always includes dyspnea with or without cough, shallow tachypnea, muscle wasting, cyanosis, and pulmonary hypertension(4).

It has been reported that 30-50% of cases have no abnormalities on chest X-ray. A variety of chest radio-graphs and CT changes are reported and can mimic the aspect of chronic interstitial pulmonary disease, including sarcoidosis(8). Nodular thickening and ground-glass atten-uation are seen in 30-60% of the patients with sarcoidosis. Sarcoidosis lesions mainly involve central regions of the middle and upper lobes of the lungs. PLC changes usually occur in the lower lobes(7). Although imaging studies may suggest sarcoidosis, the diagnosis should be confirmed by biopsy, indicating noncaseating pleomorphic granulomas, and after the exclusion of other causes of granulomatous disease. The rapid onset and progression of symptoms, the asymmetrical enlargement of the lymph nodes, the predominant disease in the lower lobes of the lungs and the lack of response to steroids within 2-4 weeks should also alert the clinicians to take into consideration another diagnosis than sarcoidosis(1). Irregular and nodular thickening of the interstitial septum, subpleural nodules, prominent interstitial markings, ground-glass opacities, pleural effusion, and hilar and mediastinal lymphadenop-athy may be seen also in pulmonary edema or in other interstitial lung diseases, such as collagen disease and hypersensitivity pneumonitis(9). The high suspicion of a PLC arose from the severe decline of the clinical status and from the HRCT aspects.

Our patient’s physical examination did not reveal any signs of an oncologic process. The observation is consistent with the field literature that reports the fre-quent absence of physical features in PLC(3,9). Although the diagnosis was delayed for our patient, an earlier diagnosis may not have altered the outcome because of the extremely poor prognosis of most cases in this advanced stage. The prognosis of PLC is particularly grim, with approximately 50% to 85% of patients dying within 3-6 months of diagnosis(1,2,8,9).

Conclusions

Patients who present respiratory distress, unrespon-sive to antibiotics and anti-inflammatory treatment, in a high suggestive context of a lung interstitial syndrome, should be explored for PLC by extended investigations (especially endoscopies), for the diagnosis of the pri-mary tumor.

 

References: 
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