Lung Ultrasound Findings in a Patient with Lymphangitic Carcinomatosis.

How to cite this article: Bhattacharjee S, Prasanna M, Maitra S, Ray BR. Lung Ultrasound Findings in a Patient with Lymphangitic Carcinomatosis. Indian J Crit Care Med 2024;28(1):82-83.

Is it possible to differentiate pulmonary sarcoidosis in tumor patients and pulmonary lymphangitic carcinomatosis caused by extrapulmonary tumors on 18F-FDG PET/CT images?

PURPOSE: Pulmonary sarcoidosis (PS) and pulmonary lymphangitic carcinomatosis (PLC) can be complications in tumor patients, and both involve the pulmonary interstitium and have similar imaging findings. Our objective was to distinguish PS and PLC on 18F-FDG PET/CT images. MATERIAL AND METHODS: The authors reviewed 18F-FDG PET/CT data of PS and PLC, diagnosed based on histopathology and imaging, in patients with tumors from July 2015 to January 2023. Three independent readers performed a blinded comparative analysis of 18F-FDG PET/CT signs in all patients. A multivariate logistic regression model was used to establish a differential diagnosis model. RESULTS: A total of 114 patients were included in the study: 56 patients with PS (mean age, 56 ± 11 [SD] years; 10 men) and 58 patients with PLC caused by extrapulmonary tumors (mean age, 51 ± 11 [SD] years; 21 men). For PS, breast cancer and cervical cancer were the most common primary tumors. For PLC, breast cancer and gastric cancer were the most common extrapulmonary tumors. The model constructed using multivariate logistic regression consisted of five factors: area of lymph node involvement, bronchovascular bundle diffuse thickening, interlobular septal thickening, pleural effusion, and subpleural hypermetabolic activity. The area under the model characteristic curve was 0.973 (95% CI 0.925-0.994), with a sensitivity, specificity, and positive and negative likelihood ratios of 87.50%, 98.28%, 50.75 and 0.13 respectively. CONCLUSION: There are detailed differences in 18F-FDG PET/CT manifestations of PS in tumor patients and PLC caused by extrapulmonary tumors, and the constructed diagnostic model has high clinical application value in differentiating the two.

Postmortem Diagnosis of Gallbladder Cancer Presenting as Pulmonary Lymphangitic Carcinomatosis With Concurrent Gallbladder Adenomyomatosis.

In pulmonary lymphangitic carcinomatosis, it can be difficult to identify the primary site of the cancer on computed tomography (CT) imaging. Here, we report a rare case of pulmonary lymphangitic carcinomatosis, which was difficult to diagnose as gallbladder cancer. An 81-year-old woman, previously followed up for gallbladder adenomyomatosis, presented with persistent cough. CT revealed multiple small nodular opacities, irregular interlobular septal thickening, and bilateral pleural effusions. Based on the CT findings and the presence of malignant cells in the pleural fluid, a presumptive diagnosis of pulmonary lymphangitic carcinomatosis was made, but the primary site was not identified. The patient died of respiratory failure in two months. Autopsy confirmed gallbladder cancer with pulmonary lymphangitic carcinomatosis and multiorgan metastasis. Clinicians should be aware that in patients with gallbladder adenomyomatosis, gallbladder cancer can present with rapidly progressive respiratory symptoms even in the absence of an evident mass or increased gallbladder wall thickening.

Genomic profiling of non-small cell lung cancer with the rare pulmonary lymphangitic carcinomatosis and clinical outcome of the exploratory anlotinib treatment.

Background: To evaluate the potential treatment for patients with non-small cell lung cancer (NSCLC) and rare malignant pulmonary lymphangitis carcinomatosis (PLC), our study provided a genomic profile and clinical outcome of this group of patients. Methods: We retrospectively reviewed patients with NSCLC who developed PLC. The genomic alterations, tumor mutation burden (TMB), and microsatellite instability (MSI) based on DNA-based next-generation sequencing were reviewed and compared in a Chinese population with lung adenocarcinomas (Chinese-LUAD cohort). Clinical outcomes after exploratory anlotinib treatment and factors influencing survival are summarized. Results: A total of 564 patients with stage IV NSCLC were reviewed, and 39 patients with PLC were included. Genomic profiling of 17 adenocarcinoma patients with PLC (PLC-LUAD cohort) revealed TP53, EGFR, and LRP1B as the three most frequently altered genes. EGFR was less mutated in PLC-LUAD than Chinese-LUAD cohort of 778 patients (35.3% vs. 60.9%, P = 0.043). BRIP1 was mutated more often in the PLC-LUAD cohort (11.8% vs. 1.8%, P= 0.043). Two patients presented with high tumor mutational burden (TMB-H, 10 mutations/MB). Combing alterations in the patient with squamous cell carcinoma, the most altered pathways of PLC included cell cycle/DNA damage, chromatin modification, the RTK/Ras/MAPK pathway and VEGF signaling changes. Fourteen of the participants received anlotinib treatment. The ORR and DCR were 57.1% and 92.9%, respectively. Patients achieved a median progression-free survival of 4.9 months and a median overall survival of 7 months. The adverse effects were manageable. In patients with adenocarcinoma, the mPFS (5.3 months vs. 2.6 months) and mOS (9.9 months vs. 4.5 months) were prolonged in patients receiving anlotinib treatment compared to those receiving other treatment strategies (P < 0.05). Conclusion: Patients with PLC in NSCLC demonstrated distinct genetic alterations. The results improve our understanding of the plausible genetic underpinnings of tumorigenesis in PLC and potential treatment strategies. Exploratory anlotinib treatment achieved considerable benefits and demonstrated manageable safety.

Pulmonary Lymphangitic Carcinomatosis: An Atypical Presentation Leading to Discovery of Multi-Organ Metastasis With Unknown Occult Primary Malignancy.

Pulmonary lymphangitic carcinomatosis is the spread of malignant cells to the lymphatic system in the lungs, which results in inflammation and lymphatic dilation. It is often found to coexist in patients with prior history of malignancy. The clinical presentation is usually related to respiratory symptoms, but atypical presentation can occur. Chest x-ray imaging can be non-revealing at initial stages, and abnormalities may only be appreciated when the disease gets to more advanced stages. Computed tomography imaging can reveal radiological abnormalities that were found to be associated with pulmonary lymphangitic carcinomatosis. More advanced imaging modalities and pathological tissue confirmation may be required for the diagnosis. However, once the diagnosis is made, prognosis remains poor and treatment efforts are geared towards the underlying malignancy. Here, we report on a rare case of pulmonary lymphangitic carcinomatosis in an adult male with no prior history of cancer, for whom his hospitalization led to the discovery of malignancy involving multiple organs.

Clinical outcome of pulmonary lymphangitic carcinomatosis in gynecologic malignancy: A single-institution experience.

OBJECTIVE: Pulmonary lymphangitic carcinomatosis (PLC) is a rare event of metastatic lung disease in advanced gynecologic malignancy. Nonspecific symptoms of patients and difficulties in detection of PLC often result in a delayed diagnosis. In this study, we evaluated the clinical outcomes of PLC in patients with gynecologic cancer. MATERIALS AND METHODS: We retrospectively reviewed electronic medical records of patients with gynecologic cancer who received care from January 1, 2008 through December 31, 2018 in Samsung Medical Center, Seoul, Republic of Korea. We based diagnosis of PLC on chest CT scan and analyzed clinical parameters of cancer type, International Federation of Gynecology and Obstetrics (FIGO) stage, histology, and patient survival. RESULTS: During the study period, 27 cases of PLC in patients with gynecologic malignancy were identified, including 11 cervical, 12 ovarian, and four uterine cancers. The most common histologic type at initial diagnosis was squamous cell in cervical (6/11, 55%), serous in ovarian (1/11, 92%), and serous in endometrial (2/4, 50%) cancer. The average survival time from diagnosis of PLC to death was a mean of 5.7 months (0.7-23.6 months) in all patients and 6.3, 6.6, and 3.6 months for cervical, endometrial, and ovarian cancer, respectively. CONCLUSION: This study showed that PLC results in extremely poor survival, from several days to a few months, in patients with gynecologic cancer. Clinicians must be aware of these clinical characteristics and consider other novel therapeutic strategies in the future.

Pulmonary lymphangitic carcinomatosis secondary to ureteral cancer.

BACKGROUND: Pulmonary lymphangitic carcinomatosis (PLC) is a metastatic lung disease of malignant tumors that spread through pulmonary lymphatic vessels. Although prompt diagnosis and specific treatment of PLC are required due to the poor prognosis associated with this disease, it is often challenging to determine the primary cancer site. CASE PRESENTATION: A 67-year-old Japanese woman presented to our hospital with a 10-day history of cough and dyspnea on exertion. Chest radiography and computed tomography (CT) revealed diffuse nodular opacities with interlobular septal thickening. Both bronchoalveolar lavage (BAL) and transbronchial lung biopsy (TBLB) revealed carcinoma cells with unknown origin. Contrast-enhanced CT depicted a mass in the right ureter with hydronephrosis, and retrograde urography showed a narrowing of the right ureter. Urine cytology from her right ureter via ureteral catheter also revealed atypical cells, highly suggestive of malignancy. Immunohistochemical examination of lung specimens via TBLB showed results consistent with lung metastasis of ureteral cancer. Therefore, we arrived at a diagnosis of PLC secondary to ureteral cancer. CONCLUSIONS: This case encouraged multidisciplinary discussion and a whole-body examination, including TBLB with immunohistochemistry, to determine the origin of PLC.

Pulmonary lymphangitic carcinomatosis from recurrent gastric cancer 19 years after primary resection: a case report.

Pulmonary lymphangitic carcinomatosis is one rare pattern of pulmonary metastases in advanced cancers. Gastric cancer is one of the most common forms of cancer that causes pulmonary lymphangitic carcinomatosis. However, recurrent gastric cancer presenting as pulmonary lymphangitic carcinomatosis after surgery is extremely rare. Furthermore, recurrence is usually observed within 5 years. We present the first case of pulmonary lymphangitic carcinomatosis in a patient with recurrent gastric cancer, 19 years after resection. In patients with a history of gastric cancer and the presence of interstitial shadow, pulmonary lymphangitic carcinomatosis should be considered in the differential diagnosis even if several years have passed since surgery.

Prognosis of pulmonary lymphangitic carcinomatosis in patients with non-small cell lung cancer.

BACKGROUND: The eighth edition of the TNM classification for lung cancer does not provide a definite guideline for pulmonary lymphangitic carcinomatosis. The purpose of this retrospective case-control study is to evaluate the prognosis of pulmonary lymphangitic carcinomatosis in patients with non-small cell lung cancer compared with those with intrapulmonary metastases. METHODS: Non-small cell lung cancer (NSCLC) patients with pulmonary lymphangitic carcinomatosis detected on chest computed tomography scan during staging evaluation between 2000 and 2016 were included. The extent of pulmonary lymphangitic carcinomatosis was classified as being around the primary tumor (cLy1), at a distance from the tumor but confined to the same lobe (cLy2), in other ipsilateral lobes (cLy3), or affecting the contralateral lung (cLy4). Overall survival rates of the subjects were compared with those with intrapulmonary metastases. RESULTS: A total of 103 subjects with pulmonary lymphangitic carcinomatosis were analysed. The 5-year overall survival rates of the subjects with pulmonary lymphangitic carcinomatosis (n=103) and intrapulmonary metastases (n=111) were 33% and 21%, respectively. The 5-year overall survival rates of cLy1 (n=28), cLy2 (n=40), cLy3 (n=26) and cLy4 (n=9) were 54%, 35%, 12% and 11%, respectively. On multivariable analyses after adjusting for possible confounders, the subjects with cLy1 and cLy2 had better overall survival (adjusted hazard ratio for death, 0.34 and 0.49; 95% confidence interval, 0.24-0.73 and 0.30-0.80; P<0.001 and 0.004, respectively) and the subjects with cLy4 had worse overall survival (adjusted hazard ratio, 2.21; 95% confidence interval, 1.03-4.70; P=0.040) compared with those with intrapulmonary metastases. CONCLUSIONS: The subjects with cLy1/2 had better overall survival than those with cLy3/4 or intrapulmonary metastases. cLy1/2 seems to be a T descriptor (T3/4) rather than an M1 descriptor.

Initial diagnosis and successful treatment of pulmonary tumor embolism manifesting as the first clinical sign of prostatic adenocarcinoma.

Although pulmonary tumor embolism (PTE) is a well-recognized end-stage form of pulmonary metastases at postmortem examination, the entity is rarely the first clinical sign of prostate cancer. Diagnosis of this condition in patients who have no previous history of malignancy is a challenge. Herein, we reported a 79-year-old man presented with progressive, unexplained dyspnea on exertion. Microscopic PTE coinciding with pulmonary lymphangitic carcinomatosis were readily recognized based on the presence of multifocal dilatation and beading of the peripheral pulmonary arteries with thickening of the bronchial walls and interlobular septa on the initial thin-section chest CT images. Pathologic examination of the transbronchial lung biopsy specimen revealed tumor emboli occluding both the small muscular pulmonary arteries and lymphatic vessels. These tumor cells were positive for prostatic specific antigen on immunohistochemical staining. The final diagnosis of prostatic adenocarcinoma was confirmed. Remarkable clinical and radiographic improvement was achieved following bilateral orchiectomies and anti-androgen treatment.

Pulmonary lymphangitic carcinomatosis from gallbladder cancer mimicking diffuse alveolar haemorrhage.

Diagnosis in cases with pulmonary lymphangitic carcinomatosis as a primary manifestation is difficult due to unawareness of the cancer. An 81-year-old man was admitted due to a one-week history of dyspnoea and haemoptysis. Chest computed tomography showed diffuse bilateral ground-grass opacity and partial consolidation. We suspected diffuse alveolar haemorrhage. High-dose methylprednisolone and cyclophosphamide did not improve his condition and he died from respiratory failure. Autopsy revealed pulmonary lymphangitic carcinomatosis of whole lungs and primary gallbladder cancer. We should consider pulmonary lymphangitic carcinomatosis in the differential diagnosis of patients with haemoptysis and diffuse lung opacity of unknown origin.

The value of virtual bronchoscopic navigation and radial endobronchial ultrasound-guided transbronchial lung cryobiopsies for pulmonary lymphangitic carcinomatosis.

BACKGROUND: Pulmonary lymphangitic carcinomatosis (PLC) is characterized by malignant infiltration into lung lymphatic channels from a primary site and is often observed in advanced malignant tumors. This study aimed to evaluate the diagnostic yield of transbronchial lung cryobiopsy in PLC guided by radial endobronchial ultrasound and virtual bronchoscopic navigation (VBN). METHODS: This prospective study enrolled 40 patients with clinical and radiologic features indicating PLC. The radial endobronchial ultrasound probe was initially advanced to the region of interest of the desired lobe near the pleura with guidance by VBN. Transbronchial lung biopsy and transbronchial lung cryobiopsy were both performed in the same ROI of all patients with the obtained samples being sent to the pathology laboratory for diagnostic analysis. Procedural complications were recorded. RESULTS: The average number of transbronchial lung biopsy and transbronchial lung cryobiopsy specimens were 4 (3 to 6) and 2 (1 to 3), respectively (t=10.43, P<0.01), with the corresponding mean diameters per biopsy being 3.7 and 8.7 mm (t=12.37, P<0.01). The diagnostic yields of transbronchial lung biopsy and transbronchial lung cryobiopsy were 70% (28/40) and 92.5% (37/40), respectively. The final positive predictive values of transbronchial lung cryobiopsy and transbronchial lung biopsy for PLC were 94.4% (34/36) and 77.8% (28/36), respectively (χ2=23.94, P<0.01). Further, 52.2% (12/23) and 81.5% (22/27) of the patients in the transbronchial lung biopsy and transbronchial lung cryobiopsy groups, respectively, were diagnosed with non-small lung cancer after further molecular analysis (χ2=19.56, P<0.01). Only 2 (5%) cases presented postoperative pneumothorax. Moreover, 0 (0%), 3 (7.5%), and 17 (42.5%) patients presented severe, moderate, and mild bleeding, respectively. There were no other adverse events or deaths. CONCLUSIONS: Transbronchial lung cryobiopsy with the guidance of radial endobronchial ultrasound and VBN without fluoroscopy has a good diagnostic yield for PLC; moreover, it allows one to obtain adequate and intact tissue samples for further molecular analysis.

Pulmonary Lymphangitic Carcinomatosis: Diagnostic Performance of High-Resolution CT and 18F-FDG PET/CT in Correlation with Clinical Pathologic Outcome.

The rationale of this study was to investigate the performance of high-resolution CT (HRCT) versus 18F-FDG PET/CT for the diagnosis of pulmonary lymphangitic carcinomatosis (PLC). Methods: In this retrospective institution-approved study, 94 patients addressed for initial staging of lung cancer with suspicion of PLC were included. Using double-blind analysis, we assessed the presence of signs favoring PLC on HRCT (smooth or nodular septal lines, subpleural nodularity, peribronchovascular thickening, satellite nodules, lymph node enlargement, and pleural effusion). 18F-FDG PET/CT images were reviewed to qualitatively evaluate peritumoral uptake and to quantify tracer uptake in the tumoral and peritumoral areas. Histology performed on surgical specimens served as the gold standard for all patients. Results: Among 94 included patients, 73% (69/94) had histologically confirmed PLC. Peribronchovascular thickening, lymph node involvement, and increased peritumoral uptake were more often present in patients with PLC (P < 0.009). Metabolic variables, including tumor SUVmax, SUVmean, metabolic tumor volume, and total lesion glycolysis, as well as peritumoral SUVmax, SUVmean, and their respective ratios to background, were significantly higher in the PLC group than in the non-PLC group (P ≤ 0.0039). Sensitivity, specificity, and area under the receiver-operating-characteristic curve for peribronchovascular thickening (69%, 83%, and 0.76, respectively; 95% confidence interval [95%CI], 0.67-0.85) and increased peritumoral uptake (94%, 84%, and 0.89, respectively; 95%CI, 0.81-0.97) were similar (P = 0.054). For detecting PLC, sensitivity, specificity, and area under the receiver-operating-characteristic curve were significantly higher, at 97%, 92%, and 0.98, respectively (95%CI, 0.96-1.00), for peritumoral SUVmax and 94%, 88%, and 0.96, respectively (95%CI, 0.92-1.00), for peritumoral SUVmean (all P ≤ 0.025). Conclusion: Qualitative evaluation of 18F-FDG PET/CT and HRCT perform similarly for the diagnosis of PLC, with both being outperformed by 18F-FDG PET/CT quantitative parameters.

A Case of Rapidly Deteriorating Lymphangitic Carcinomatosis in a Patient with Stage IV Pancreatic Cancer.

Pulmonary lymphangitic carcinomatosis (PLC) is defined as a tumor in the lung lymphatic vessels. It is rarely seen as a result of malignancy and generally carries a poor prognosis. Proper knowledge of the clinical presentation is important for physicians to be aware of in order to consider a diagnosis of PLC. We present the case of a 52-year-old Caucasian gentleman who initially came to the hospital with a three-week history of dyspnea. In the hospital, a diagnosis of Stage 4 pancreatic cancer with a lymphangitic spread of the tumor to the lungs was made. Despite optimal treatment, the patient required mechanical ventilation for acute hypoxic respiratory failure. PLC is a relatively rare manifestation of malignancy; yet, it generally carries a poor prognosis. It may occur during the course of malignancy or may represent as the first findings in malignancy. It is important for physicians to be aware of the clinical presentations of PLC in order to ensure timely treatment. Oftentimes, PLC can be diagnosed through clinical judgment alone without the need for radiological support and other invasive measures.

A Rare Case of Isolated Chronic Cough Caused by Pulmonary Lymphangitic Carcinomatosis as a Primary Manifestation of Rectum Carcinoma.

A 36-year old man was referred to our hospital due to isolated chronic cough that was refractory to anti-asthma medications, including inhaled corticosteroids/long-acting ß2 agonists. Chest X-ray showed diffuse nodular and enhanced vascular shadows with Kerley lines in both lungs. A blood analysis showed elevated serum carcinoembryonic antigen (CEA) and CA19-9 levels. A transbronchial biopsy revealed well to moderately differentiated adenocarcinoma, the origin of which was immunohistochemically suspected to be the gastrointestinal tract. Colonoscopy confirmed the diagnosis of primary rectum carcinoma. Pulmonary lymphangitic carcinomatosis was therefore regarded as the origin of the cough. Lymphangitic carcinomatosis is an uncommon diagnosis but important to consider in patients with persistent cough.

Gastric adenocarcinoma revealed by atypical pulmonary lymphangitic carcinomatosis.

Pulmonary lymphangitic carcinomatosis (PLC) is a well-known form of tumour metastasis to the pulmonary lymphatic system or to the adjacent interstitial tissue resulting in thickening of the bronchovascular bundle and septa. Another type of tumour metastasis to the lung involves the pulmonary vascular system and is known as pulmonary tumour thrombotic microangiopathy (PTTM). In this article, we will describe the unusual case of a young Chinese woman with gastric adenocarcinoma revealed by atypical radiographic lesions consistent with both PLC and PTTM. We will discuss the existing evidence and hypotheses about the pathophysiology of both conditions.

A Case of Lung Adenocarcinoma with Marked Improvement of Pulmonary Lymphangitic Carcinomatosis by Adding Bevacizumab to Cisplatin and Pemetrexed.

A 40-year-old man with a diagnosis of lung adenocarcinoma (cT4N3M1c, stage IVB) experienced worsening of lymphangitic carcinomatosis in the right lung and right pleural effusion after receiving 1 cycle of first-line chemotherapy consisting of cisplatin and pemetrexed. Bevacizumab was thus added from the second cycle of the cisplatin-pemetrexed regimen, leading to a marked improvement in pulmonary lymphangitic carcinomatosis and a decrease in pleural effusion. Subsequently, maintenance therapy consisting of pemetrexed and bevacizumab was continued, successfully leading to long-term progression-free survival. Generally, pulmonary lymphangitic carcinomatosis shows poor prognosis because of poor response to chemotherapy. However, recent studies have been elucidating the role of the vascular endothelial growth factor A (VEGF-A)/VEGF receptor-2 pathway in pulmonary lymphangitic carcinomatosis. Therefore, bevacizumab is expected to be beneficial in the treatment of this pathological condition.

Fluorodeoxyglucose Uptake in Advanced Non-small Cell Lung Cancer With and Without Pulmonary Lymphangitic Carcinomatosis.

AIM: To assess the correlation between advanced non-small cell lung cancer (NSCLC) with or without pulmonary lymphangitic carcinomatosis (PLC) and fluorodeoxyglucose (FDG) uptake and its effect on survival outcomes. PATIENTS AND METHODS: We retrospectively reviewed 157 patients with NSCLC. The mean and maximum standardized uptake values (SUVmean and SUVmax, respectively), metabolic tumor volume (MTV) and total lesion glycolysis (TLG) were evaluated for their effect on overall survival (OS) and progression-free survival (PFS). RESULTS: The PLC group included 55 patients and the non-PLC group included 102 patients. The SUVmean, SUVmax, MTV and TLG values were lower in the non-PLC group. In the PLC group, primary lung tumor TLG was a significant predictor of PFS, while whole-body TLG was found to be a significant predictor in non-PLC patients. CONCLUSION: Primary lung tumor TLG was a good predictor in PLC patients. Whole-body TLG could be a useful predictor only in patients without PLC.

Pulmonary lymphangitic carcinomatosis from head and neck squamous cell carcinoma.

Pulmonary lymphangitic carcinomatosis (PLC) secondary to mucosal head and neck squamous cell carcinoma (HNSCC) is extremely rare, difficult to diagnose in the pre-symptomatic phase, and is rapidly fatal. We describe two cases of fatal PLC secondary to squamous cell carcinoma in whom a review of pre-treatment imaging (computed tomography of the chest) changes reported as unspecific were retrospectively felt to be consistent with pre-symptomatic PLC. Case 1, a 73-year-old male with T2N2bMx poorly differentiated squamous cell carcinoma of the right tonsil, died 6 weeks after chemoradiotherapy with curative intent. Case 2, a 65-year-old female with T4aN2bMx of the right body of the mandible, died within 6 weeks of radical surgery including free tissue transfer. A review of the literature showed that PLC secondary to HNSCC occurs in an older cohort of patients: mean age 69 years vs. other tumour groups 50 years. PLC secondary to HNSCC can behave in distinctly different ways, demonstrating similarity to either gastric adenocarcinoma or bronchogenic squamous cell carcinomas.

Pulmonary Lymphangitic Carcinomatosis due to Renal Cell Carcinoma.

Renal cell carcinoma is an aggressive disease with a high rate of mortality. It is known to metastasize to the lung, liver, bone and brain. However, manifestation through lymphatic spread to the lungs is rare. Lymphangitic carcinomatosis is commonly observed in malignancies of the breast, lung, pancreas, colon and cervix. It is unusual to observe lymphangitic carcinomatosis of the lungs due to renal cell carcinoma. Lymphangitic carcinomatosis of the lungs may result in severe respiratory distress and may be the direct cause of death. Currently, there are no known modalities of preventing or slowing lymphangitic carcinomatosis besides treating the primary tumor. However, early detection may change the course of the disease and may prolong survival. This is compounded by the difficulty involved in diagnosing lymphangitic carcinomatosis of the lung which frequently involves lung biopsy. Immunohistochemical studies are often used in conjunction with regular histochemistry in ascertaining the primary tumor and in differentiating it from pulmonary metastasis. In this case report, we describe the presentation and clinical course of renal cell carcinoma in a patient which manifested as lymphangitis carcinomatosa of the lungs. The patient underwent surgical resection of the primary tumor with lymph node resection but presented with a fulminant lymphangitic carcinomatosis of the lungs within two weeks. Immunohistochemistry of the tissue obtained by the biopsy confirmed the diagnosis which was subsequently corroborated during his autopsy. This case illustrates the necessity of an urgent follow-up of chemotherapy and immunotherapy in such patients.