Lung Metastases-Lymphangitic Carcinomatosis

Epidemiology

  • Presence of Lymphangitic Pattern in Patient with Known Malignancy: 70-80% probability of metastases being the cause

Etiology

General Comments

  • Metastatic Adenocarcinomas Account for 80% of Lymphangitic Lung Metastasis Cases

Breast Cancer (see Breast Cancer)

  • Epidemiology
    • In Autopsy Studies, Approximately 24% of Breast Cancer Cases Have Lymphangitic Lung Metastases
  • Mechanism
    • Mediastinal or Hilar Node Involvement with Retrograde Spread to Lung Lymphatics

Lung Cancer (see Lung Cancer)

  • Epidemiology
    • XXXXX
  • Mechanism
    • Mediastinal or Hilar Node Involvement with Retrograde Spread to Lung Lymphatics

Colon Cancer (see Colon Cancer)

  • Epidemiology
    • Case Reports (Medicine-Baltimore, 2019) [MEDLINE]
      • May Occur without Concurrent Liver Metastases (Medicine-Baltimore, 2019) [MEDLINE]

Gastric Cancer (see Gastric Cancer)

  • Epidemiology
    • Lymphangitic Carcinomatosis as Manifestation of Gastric Carcinoma is Rare (BMC Res Notes, 2012) [MEDLINE]
  • Prognosis
    • Prognosis is Poor (BMC Res Notes, 2012) [MEDLINE]

Testicular Cancer (see Testicular Cancer)

  • Mechanism
    • Spread from Distant Nodes Through Lymphatics to Lung Lymphatics

Choriocarcinoma (see Choriocarcinoma)

  • Mechanism
    • Spread from Distant Nodes Through Lymphatics to Lung Lymphatics

Hodgkin’s Disease (see Hodgkin’s Disease)

  • Mechanism
    • Mediastinal or Hilar Node Involvement with Retrograde Spread to Lung Lymphatics

Lymphoma (see Lymphoma)

  • Mechanism
    • Mediastinal or Hilar Node Involvement with Retrograde Spread to Lung Lymphatics

Prostate Cancer (see Prostate Cancer)

  • Epidemiology
    • Lymphangitic Lung Metastatic Pattern is Probably the Most Common Pattern Seen in Prostate Cancer Which is Metastatic to the Lung
    • This Pattern is Observed in About 58% of Cases
    • Prostate Cancer Metastases are Rarely Observed in Absence of Pre-Existing Lymph Node or Bone Metastases
  • Diagnosis

Hepatocellular Carcinoma (see Hepatocellular Carcinoma)

  • xxxx

Pancreatic Cancer (see Pancreatic Cancer)

  • Epidemiology
    • Case Reports (Cureus, 2019) [MEDLINE]

Thyroid Cancer (see Thyroid Cancer)

  • xxxx

Cervical Cancer (see Cervical Cancer)

  • xxxx

Laryngeal Cancer (see Laryngeal Cancer)

  • xxx

Renal Cell Carcinoma (see xxxx)

  • Epidemiology
    • Case Reports (Curr Probl Diagn Radiol, 2021) [MEDLINE]

Metastatic Adenocarcinoma of Unknown Primary

  • xxxx

Physiology

Lymphangitic Carcinomatosis is the Diffuse Infiltration and Obstruction of Pulmonary parenchymal Lymphatic Channels by Tumor

  • Spread of Malignant Cells to Lung Lymphatics from Any of the Following
    • Obstructed Lymph Nodes
    • Hematogenous Spread
    • Previously Seeded Pleural Fluid

Diagnosis

Chest X-Ray (CXR) (see Chest X-Ray)

  • Findings
    • Diffuse Reticulonodular Infiltrates: often with linear markings radiating out from enlarged hilar or mediastinal nodes
    • Kerley B Lines: thickened interlobular septa (seen at periphery of lung) due to pulmonary lymphatic obstruction
    • Coexistent Pleural Effusion
      • Present in 30-50% of Cases
      • Effusion Occurs more commonly in breast cancer cases with lymphangitic lung mets than those with non-lymphangitic lung mets

High-Resolution Chest Computed Tomography (HRCT) (see Chest Computed Tomography)

  • Findings
    • “Beaded chain” or “string of pearls” thickening of interlobular septa -> producing polygonal shapes (outlining the lobule)
      • Interlobular septa is usually irregular or nodular, but may be smoothly thickened
        • In contrast, in interstitial pulmonary edema, interlobular septa is usually smoothly thickened
      • Intervening parenchyma between the interlobular septa is typically normal (unlike in IPF, where it is usually distorted or honeycombed)
    • Centrilobular peribronchovascular thickening
    • Preservation of normal parenchymal architecture at the level of the secondary pulmonary lobule
    • Thickening of the fissures as a result of the involvement of the lymphatics concentrated in the subpleural interstitium
    • Peribronchovascular thickening
    • Lung Nodules
    • Pleural effusion (30-50% of cases)
      • Effusion occurs more commonly in breast cancer cases with lymphangitic lung mets than those with non-lymphangitic lung mets
    • Mediastinal and/or hilar lymphadenopathy (30-50% of cases)
    • Above findings may be unilateral or bilateral, focal or diffuse, and symmetrical or asymmetrical
      • Focal, unilateral lymphangitic disease occurs in 50% of cases
      • Focal, unilateral pattern is seen commonly in cases due to lung cancer

Sputum Cytology

  • May be used to diagnose lymphangitic mets

Bronchoscopy (see Bronchoscopy)

BAL is more sensitive than sputum cytology in diagnosis of lymphangitic mets – BAL may be diagnostic in lymphangitic carcinomatosis cases due to lymphoma – However, lymphocytosis >35% may also be seen in sarcoidosis, berylliosis, hypersensitivity pneumonitis, drug-induced ILD, LIP, breast cancer-related lymphangitic carcinmatosis, and infrequently, in lung cancer-related lymphangitic carcinmatosis cases) – Normal BAL lymphocyte percentage: <15% – BAL lymphocytosis is seen in about 50% of breast cancer cases with lymphangitic lung mets (Chest, 1992) – TBB: may be diagnostic – TBB use in this setting represents one of the few high yield uses of TBB in the diagnosis of interstitial lung disease – Cytology: immunohistochemical stains may be useful in identifying germ cell and breast cancers

PET Scan (see xxxx)

  • 2005 Study: intensity of FDG uptake in the diseased lung is significantly greater than in the normal, contralateral lung [J Comput Assist Tomogr. May-Jun 2005;29(3):346-9]
  • 2006 Study: comparing PET with HRCT scans in 5 patients with lymphangitic carcinomatosis [Clin Nucl Med. Nov 2006;31(11):673-8]
    • FDG-PET scan activity distribution was identical to segmental, lobar, or diffuse areas of lymphangitic disease seen on HRCT
    • In segmental lymphangitic carcinmatosis, linear or hazy FDG uptake extending from the tumor may be seen

Clinical Manifestations

General Comments

  • Asymptomatic: often

Pulmonary Manifestations

  • Dyspnea (see Dyspnea)
    • Common
  • Orthopnea (see Orthopnea)
    • Common
  • Cough (see Cough)
    • Common: due to mucosal edema associated with airway lymphatic infiltration
  • Chest Tightness (see Chest Pain)
    • Chest pain (late in course, with pleural involvement)
  • Pleural Effusion (see Pleural Effusion-Exudative)
    • Occurs late in course

Prognosis

  • Presence of Bronchoalveolar Lavage (BAL) Lymphocytosis (range: 15-45% of total cells): portends better prognosis in breast cancer cases with lymphangitic mets (Chest, 1992)

Treatment

  • Treat Underlying Malignancy
  • Steroids: may provide some symptomatic relief
    • May decrease inflammatory response to tumor cells in lung
  • Opiates (see xxxx): may relieve dyspnea
    • Nebulized Morphine Sulfate: may be useful
  • Oxygen: may relieve dyspnea
  • Antitussives: as required

References

  • The value of bronchial washings and bronchoalveolar lavage in the diagnosis of lymphangitic carcinomatosis. Chest. Nov 1988;94(5):1028-30 [MEDLINE]
  • Pulmonary lymphangitic carcinomatosis: CT and pathologic findings. Radiology. Mar 1988;166(3):705-9 [MEDLINE]
  • Chronic diffuse infiltrative lung disease: comparison of diagnostic accuracy of CT and chest radiography. Radiology. Apr 1989;171(1):111-6 [MEDLINE]
  • Pulmonary microvascular cytology in the diagnosis of lymphangitic carcinomatosis. N Engl J Med. Jul 13 1989;321(2):71-6 [MEDLINE]
  • Pulmonary microvascular cytology in lymphangitic carcinomatosis. N Engl J Med. Jan 4 1990;322(1):59-60 [MEDLINE]
  • CT evaluation for pulmonary metastases in patients with extrathoracic malignancy. Radiology. Jul 1991;180(1):1-12 [MEDLINE]
  • Case report: lymphangitic carcinomatosis from cervical carcinoma–an unusual presentation of diffuse interstitial lung disease. Am J Med Sci. Mar 1992;303(3):174-6 [MEDLINE]
  • Specific diagnosis by CT and HRCT in six chronic lung diseases. Comput Med Imaging Graph. Jul-Aug 1992;16(4):277-82 [MEDLINE]
  • CT findings in lymphangitic carcinomatosis of the lung: correlation with histologic findings and pulmonary function tests. AJR Am J Roentgenol. Jun 1992;158(6):1217-22 [MEDLINE]
  • Paraseptal emphysema mimicking unilateral lymphangitic carcinomatosis: CT findings. J Comput Assist Tomogr. Sep-Oct 1993;17(5):810-2 [MEDLINE]
  • High-resolution CT of the lungs. Am Fam Physician. Sep 1 1993;48(3):493-8 [MEDLINE]
  • Imaging case of the month. Pulmonary lymphangitic carcinomatosis from adenocarcinoma of the prostate. Md Med J. Nov 1994;43(11):989-90 [MEDLINE]
  • Chest radiographic features of thoracic metastatic disease in adolescents with colon cancer. Pediatr Radiol. 1994;24(7):491-3 [MEDLINE]
  • CT of pulmonary metastases with pathological correlation. Semin Ultrasound CT MR. Oct 1995;16(5):379-94 [MEDLINE]
  • Recurrent squamous cell carcinoma of the cervix with pulmonary lymphangitic metastasis. Int J Gynaecol Obstet. Jan 1995;48(1):85-90 [MEDLINE]
  • Pulmonary lymphangitic carcinomatosis: chronicity of radiographic findings in long-term survivors. AJR Am J Roentgenol. Jul 1995;165(1):49-52 [MEDLINE]
  • Squamous cell carcinoma of the uterine cervix. Patterns of thoracic metastases. Invest Radiol. Dec 1995;30(12):724-9 [MEDLINE]
  • Lung metastases. Eur Radiol. 1996;6(5):596-606 [MEDLINE]
  • Lobar extent of pulmonary lymphangitic carcinomatosis. Tl-201 chloride and Tc-99m MIBI scintigraphic findings. Clin Nucl Med. Sep 1996;21(9):726-9 [MEDLINE]
  • Adenocarcinoma of the lung presenting as a diffuse interstitial process in a 25-year-old man. Lung Cancer. Sep 1996;15(2):239-44 [MEDLINE]
  • Transbronchial lung biopsy: an analysis of 530 cases with reference to the number of samples. Monaldi Arch Chest Dis. Aug 1997;52(4):324-9 [MEDLINE]
  • The ‘fragmented’ scintigraphic lung pattern in pulmonary lymphangitic carcinomatosis secondary to breast cancer. Respiration. 1998;65(5):406-10 [MEDLINE]
  • Lymphangitic carcinomatosis from prostate carcinoma. J Comput Assist Tomogr. Sep-Oct 1999;23(5):761-3 [MEDLINE]
  • Unusual appearance of thromboembolism on perfusion lung imaging. Clin Nucl Med. Sep 1999;24(9):684-6 [MEDLINE]
  • Comparison of high resolution CT findings of sarcoidosis, lymphoma, and lymphangitic carcinoma: is there any difference of involved interstitium?. J Comput Assist Tomogr. May-Jun 1999;23(3):374-9 [MEDLINE]
  • Endobronchial metastasis from stomach carcinoma. Monaldi Arch Chest Dis. Feb 2000;55(1):6-8 [MEDLINE]
  • Pulmonary lymphangitic sarcomatosis from cutaneous angiosarcoma: an unusual presentation of diffuse interstitial lung disease. Jpn J Clin Oncol. Jan 2000;30(1):37-9 [MEDLINE]
  • Unilateral pulmonary edema after talc pleurodesis. J Thorac Imaging. Apr 2001;16(2):99-102 [MEDLINE]
  • Lymphangitic spread of breast carcinoma: scintigraphic pattern with chest x-ray and computed tomography correlation. Clin Nucl Med. Sep 2005;30(9):615-6 [MEDLINE]
  • Fluorodeoxyglucose positron emission tomography pattern of pulmonary lymphangitic carcinomatosis. J Comput Assist Tomogr. May-Jun 2005;29(3):346-9 [MEDLINE]
  • Pulmonary lymphangitic carcinomatosis (PLC): spectrum of FDG-PET findings. Clin Nucl Med. Nov 2006;31(11):673-8 [MEDLINE]
  • Clinical significance of small pulmonary nodules with little or no 18F-FDG uptake on PET/CT images of patients with nonthoracic malignancies. J Nucl Med. Jan 2007;48(1):15-21 [MEDLINE]
  • Bronchoalveolar lavage in malignancy. Semin Respir Crit Care Med. Oct 2007;28(5):534-45 [MEDLINE]
  • Unusual thoracic CT manifestations of osteosarcoma: review of 16 cases. Pediatr Radiol. 2008 May;38(5):551-8. doi: 10.1007/s00247-007-0735-3 [MEDLINE]
  • FDG PET/CT in assessment of pulmonary lymphangitic carcinomatosis. AJR Am J Roentgenol. Jan 2010;194(1):231-6 [MEDLINE]
  • Pulmonary lymphangitic carcinomatosis as a primary manifestation of gastric carcinoma in a young adult: a case report and review of the literature. BMC Res Notes. 2012 Nov 16;5:638. doi: 10.1186/1756-0500-5-638 [MEDLINE]
  • Pulmonary lymphangitis carcinomatosis: systematic review and meta-analysis of case reports, 1970-2018. Postgrad Med. 2019 Jun;131(5):309-318. doi: 10.1080/00325481.2019.1595982 [MEDLINE]
  • Pulmonary lymphangitic carcinomatosis without concurrent liver metastasis from colon cancer detected using 18F-FDG PET/CT: A case report. Medicine (Baltimore). 2019 Oct;98(41):e17446. doi: 10.1097/MD.0000000000017446 [MEDLINE]
  • A Case of Rapidly Deteriorating Lymphangitic Carcinomatosis in a Patient with Stage IV Pancreatic Cancer. Cureus. 2019 Apr 9;11(4):e4421. doi: 10.7759/cureus.4421 [MEDLINE]
  • Prostate Cancer Lymphangitic Pulmonary Carcinomatosis: Appearance on 18F-FDG PET/CT and 18F-DCFPyL PET/CT. Clin Nucl Med. 2020 Sep;45(9):727-729. doi: 10.1097/RLU.0000000000003109 [MEDLINE]
  • Pulmonary Lymphangitic Carcinomatosis From Renal Cell Carcinoma. Curr Probl Diagn Radiol. Jan-Feb 2021;50(1):104-107. doi: 10.1067/j.cpradiol.2018.07.008 [MEDLINE]