Pulmonary lymphangitic sarcomatosis and a review of the literature

Abstract

Intrapulmonary spread of a sarcoma via lymphatics is a rare cause of death in a young adult. A 31-year old man was admitted to our hospital complaining of dyspnea and malaise of 2 months' duration. A chest radiography revealed bilateral hilar enlargement, and reticulonodular infiltrations. Thoracic CT-scans demonstrated mediastinal lymphadenopathy, thickening of interlobular septa, polygonal lines, and thickening of bronchovascular bundles. The diagnosis was made by open-lung biopsy. The patient died within 3 months after diagnosis. Pulmonary lymphangitic sarcomatosis is a rare but important manifestation of an angiosarcoma. Optimal treatment of these patients is not well defined, but a trial of chemotherapy may be warranted.

Aggressive Presentation of Ewing Sarcoma Recurrence Demonstrates Rare Pattern of Pulmonary Lymphangitic Carcinomatosis

“…Such spread shares similar characteristics to PLC, but given the origin of malignancy, it is described as pulmonary lymphangitic sarcomatosis (PLS). [3][4][5] To our knowledge, no case of PLS from ES in a child has been reported, and only 1 such case has been reported in an adult, presenting in a 67year-old male. 5 Metastatic pulmonary lymphangitic infiltration is best evaluated with computerized tomography scans and classically appears as nodular interlobular septal thickening with preservation of secondary pulmonary lobule architecture.…”

“…Metastatic spread of sarcoma is typically hematologic 2 ; however there have been a few case reports describing lymphangitic spread. Such spread shares similar characteristics to PLC, but given the origin of malignancy, it is described as pulmonary lymphangitic sarcomatosis (PLS) 3–5 . To our knowledge, no case of PLS from ES in a child has been reported, and only 1 such case has been reported in an adult, presenting in a 67-year-old male 5 …”

“…PLS has a poor prognosis, with a 50% survival within 2 months following the onset of respiratory symptoms 3 . Given the scarcity of reported PLS cases, diagnosis may be delayed, and treatment recommendations are limited, though chemotherapy has shown to have modest survival benefits 3–5 …”

Pulmonary lymphatic involvement in metastatic uterine sarcomas: imaging and pathological appearance

“…According to a previous report, pulmonary lymphangitic carcinomatosis (PLC) most often coexists with adenocarcinoma (80%), while PLC induced by sarcomas are rare (3.6%) [2] . To the best of our knowledge, three previous reports have described the CT findings of the patients with PLS in detail; two patients with angiosarcoma, one with neuroectodermal/Ewing's sarcoma [ 1 , 3 , 4 ]. These findings included uneven thickenings of the interlobular septa and bronchovascular bundle, which were similar to the CT findings of PLC.…”

“…As opposed to carcinomas, sarcomas that shows metastases to lymph nodes account for only 2% to 5% [1] . Meanwhile, two of the three patients with PLS mentioned above showed hilar or mediastinal lymphadenopathy [ 1 , 3 ]. This difference may be explained by that PLS is more likely to be found in a more advanced state of the disease.…”