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Clinical Practice Points • Cutaneous lymphangitic carcinomatosis is a rare form of cutaneous metastatic disease which can appear to be a variety of different dermatologic conditions. Clinicians should maintain a high index of suspicion in order to avoid delays in diagnosis and treatment given the poor prognosis of cutaneous metastatic disease. • While the skin is an uncommon site of metastatic disease, it is important to remember that cutaneous metastases can be the sole presenting sign of malignancy or extra nodal disease. • Skin metastases more commonly present within the first 3 years after discovery of a primary tumor. However, they can occur at any time and have been reported up to 22 years after an initial cancer diagnosis. Clinicians should consider this with any new skin lesion in a patient with a history of malignancy.

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.

Lymphangitic carcinomatosis, the presence of tumor within the pulmonary lymphatics, occurs in the setting of malignant tumors and is associated with a poor prognosis. Here we describe a case of lymphangitic carcinomatosis in the setting of renal cell carcinoma and review the radiological manifestations of this disease.

The diagnosis of lymphangitic carcinomatosis is challenging due to the manifestation of nonspecific symptoms and radiographic abnormalities that bear similarity to those of interstitial lung disease. Herein, we report the case of a 53-year-old woman diagnosed with lymphangitic carcinomatosis from metastatic gastric adenocarcinoma, 3 months after her initial presentation.

Background: The CA 19-9 tumor marker is commonly used alongside imaging to trend chemotherapy response in patients with pancreatic ductal adenocarcinoma. Presentation: We describe an unusual clinical case of metastatic pancreatic cancer who achieved a marked decline in CA 19-9 but paradoxically developed widespread pulmonary lymphangitic carcinomatosis leading to rapid clinical decline and death. Conclusions: This case highlights the limitations of using the CA 19-9 tumor marker in isolation.

Pulmonary lymphangitic carcinomatosis denotes the infiltration of tumor cells into the lung parenchymal lymphatic channels. Breast, lung, stomach, and colon adenocarcinoma are the most common origin of this invasion pattern. The micropapillary variant of colorectal adenocarcinoma has a high rate of lymph node metastases and poor overall survival. A 49 year-old man with a 6 months history of persistent cough and a relevant occupational chemical exposure had a computed tomography that showed bilateral interstitial lung infiltrates. The lung biopsy demonstrated a micropapillary adenocarcinoma with diffusely obstruction of the lung parenchymal lymphatics. The immunohistochemistry confirmed a colorectal origin. The colonoscopy evidenced a mass with identical morphology. Colorectal micropapillary carcinoma with metastatic lung lymphangitic carcinomatosis can occur, as a persistent cough, as presenting symptom in extraordinarily rare cases. To the best of our knowledge, this is the first case of an alive patient with colorectal metastatic micropapillary carcinoma presenting with lymphangitic lung carcinomatosis.

Pulmonary metastases typically present as well-circumscribed solid nodules, often with a basilar and peripheral distribution due to hematogenous spread. When an atypical pattern of metastasis occurs, a lack of recognition may result in understaging or a delay in diagnosis. The purpose of this article is to review the imaging findings of atypical pulmonary metastatic disease in children. Atypical pulmonary metastatic patterns that can be seen in children include cavitary lesions, calcified pulmonary nodules, nodules with peritumoral halos, tree-in-bud or strial pattern secondary to tumor in peripheral pulmonary arterial branches, lymphangitic carcinomatosis, and miliary disease. An awareness of the spectrum of imaging findings of atypical pulmonary metastases along with an understanding of histopathological underpinnings will allow the radiologist to make an accurate diagnosis.

OBJECTIVE. The purpose of this study was to perform a systematic review and meta-analysis regarding CT features of non–small cell lung cancer (NSCLC) with anaplastic lymphoma kinase (ALK) rearrangement. MATERIALS AND METHODS. The PubMed and Embase databases were searched up to February 20, 2019. Studies that evaluated CT features of NSCLC with and without ALK rearrangement was included. Methodologic quality was assessed using Quality Assessment of Diagnostic Accuracy Studies–2. The association between CT features and ALK rearrangement was pooled in the form of the odds ratio (OR) or the mean difference (MD) using the random-effects model. Heterogeneity was examined using the inconsistency index (I(2)). Publication bias was examined using funnel plots and Egger tests. RESULTS. Sixteen studies were included, consisting of 3113 patients with NSCLC. The overall prevalence of patients with ALK rearrangement was 17% (528/3113). Compared with NSCLC without ALK rearrangement, on CT images those with ALK rearrangement were more frequently solid (OR = 2.86), central in location (OR = 2.72), and 3 cm or smaller (OR = 0.57); had lower contrast-enhanced CT attenuation (MD = −4.79 HU); more frequently had N2 or N3 disease (OR = 5.63), lymphangitic carcinomatosis (OR = 3.46), pleural effusion (OR = 1.91), or pleural metastasis (OR = 1.81); and less frequently had lung metastasis (OR = 0.66). Heterogeneity varied among CT features (I(2) = 0–80%). No significant publication bias was seen (p = 0.15). CONCLUSION. NSCLC with ALK rearrangement had several distinctive CT features compared with that without ALK rearrangement. These CT biomarkers may help identify patients likely to have ALK rearrangement.

Bilateral lung parenchymal involvement is seen in infective as well as noninfective conditions, appearing as focal or diffuse lung disease. PET/CT with FDG helps in characterization (increased glucose utilization is seen by both inflammatory and neoplastic cells). In this article, we describe the spectrum of patterns of FDG uptake and associated CT changes involving bilateral lung parenchyma. Benign conditions described are aspiration pneumonia; pulmonary toxicity by bleomycin; infections, namely, sarcoidosis, miliary pulmonary tuberculosis, and pulmonary nocardiosis; and inflammatory conditions such as pulmonary Langerhans cell histiocytosis and pulmonary alveolar proteinosis. Neoplastic conditions described are bilateral pulmonary metastases and lymphangitic carcinomatosis.

The rationale of this study was to investigate the performance of high-resolution CT (HRCT) versus 18 F-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). 18 F-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 SUV max , SUV mean , metabolic tumor volume, and total lesion glycolysis, as well as peritumoral SUV max , SUV mean , 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 SUV max and 94%, 88%, and 0.96, respectively (95%CI, 0.92-1.00), for peritumoral SUV mean (all P ≤ 0.025). Conclusion: Qualitative evaluation of 18 F-FDG PET/CT and HRCT perform similarly for the diagnosis of PLC, with both being outperformed by 18 F-FDG PET/CT quantitative parameters.

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

Purpose Kerley A-lines are generally apparent in patients with pulmonary edema or lymphangitic carcinomatosis. There are two main thoughts regarding the etiology of Kerley A-lines, but no general agreement. Specifically, the lines are caused by thickened interlobular septa or dilated anastomotic lymphatics. Our purpose was to determine the anatomic structure represented as Kerley A-lines using 3D-CT lung segmentation analysis. Materials and methods We reviewed 139 charts of patients with lymphangitic carcinomatosis of the lung who had CT and X-ray exams with a maximum interval of 7 days. The presence of Kerley A-lines on X-ray was assessed by a radiologist. The A-lines on X-ray were defined as follows: dense; fine ( Results On chest X-ray, 37 Kerley A-lines (right, 16; left, 21) were identified in the 22 cases (16%). Of these, 4 lungs with 12 lines were excluded from analysis due to technical reasons. Nineteen of the 25 lines (76%) corresponded to the septal lines on CT. Of these, 11 lines matched with automatically segmented lines (intersegmental septa, 4; intersubsegmental septa, 7) by the workstation. Two lines (8%) represented fissures. Four lines corresponded to the bronchial wall/artery (3 lines, 12%) or vein (1 line, 4%). Conclusion Kerley A-lines primarily represented thickened and continued interlobular septal lines that corresponded to the septa between lung segments and subsegments.

BackgroundPulmonary lymphangitic carcinomatosis (PLC) is a malignant lung disease characterized by metastatic tumor lesions in the lung lymphatic vessels with a poor prognosis. Early diagnosis is the key to improve the outcome. However, when PLC is the first manifestation of patients without a known malignancy, it often leads to delayed diagnosis and treatment, and even fatal consequences due to non-specific symptoms. Here, we describe a young male presenting PLC as the first and only manifestation followed by diagnosis of gastric adenocarcinoma and systemically summarized previously reported clinical properties of PLC cases in the literature.Case presentationA 32 years old male presented nonproductive cough for 20 days and progressive exertional dyspnea in a week. He was diagnosed with pneumonia and received antibiotics for almost 3 weeks without any improvement. Chest computed tomography showed multiple nodules and thickened interlobular septa in bilateral lung. PET/CT revealed a primary gastric tumor with PLC and the biopsy of the cervical lymph node confirmed a poorly metastatic differentiated gastric adenocarcinoma. The patient’s general condition deteriorated rapidly and he developed respiratory failure, requiring non-invasive mechanical ventilation on day 9 after admission. Chemotherapy was given to the patient on the tenth day. However, few hours after receiving the first dose, the patient died of respiratory failure. It was only one month from the onset of symptoms to death of our case.ConclusionsPatients presenting PLC as the first manifestation of an unknown malignancy carry a bleak prognosis. PLC be considered for differential diagnosis in patients who present with dyspnea, dry cough with or without loss of weight. Particular attentions need to be given to patients, especially young ones without histories of malignancy, whose chest imaging exhibits thickened interlobular septa. While the stomach, lung and prostate are the most likely origins of the primary tumors, patients with PLC derived from prostate cancer may have a better prognosis after anti-cancer treatment.

A 55-year-old man with cough, bone pain, and cervical lymphadenopathy underwent both 18F-FDG and 18F-PSMA-1007 PET/CT scans with clinical suspicion of malignant disease. Compared with 18F-FDG PET/CT, 18F-PSMA PET/CT showed intense tracer uptake in the prostate gland, lungs, osteogenic lesions, and multiple lymph nodes. Interestingly, we also found the high tracer concentration of pulmonary lymphangitic carcinomatosis, a very rare form of prostate cancer metastasis to the lung, on 18F-PSMA-1007 PET/CT images.

The most common modalities to image thoracic metastases are chest radiography, chest computed tomography (CT), and 18F-fluorodeoxyglucose positron emission tomography (18FDG PET). In certain patient populations considered low risk for thoracic metastases, some management guidelines recommend chest radiography to screen for thoracic metastasis (1,2). However, chest CT remains the most sensitive imaging modality to detect and delineate the patterns of thoracic metastases. If volumetric 1–2 mm axial reconstructions are not routinely performed, high-resolution chest CT (HRCT) provides better resolution of the interstitial architecture, which is particularly useful in cases of lymphangitic carcinomatosis. 18FDG PET imaging is well established for the diagnosis and staging of metastases in lung, colon, head and neck tumours, melanoma, breast, and lymphoma (3,4).

Background Eccrine porocarcinoma is a rare malignancy accounting for 0.005 to 0.01% of all cutaneous tumors. However, its etiology is not well established. Herein we are reporting the occurrence of metastatic porocarcinoma of the back in a patient with a history of multiple cutaneous malignancies and chronic immunosuppression. Case presentation A 79-year-old male with a history of long-term immunosuppressive therapy for left lung transplantation and multiple cutaneous malignancies including melanoma, presented with an enlargening plaque on the right upper back. Biopsy demonstrated an infiltrative epithelial tumor with aggressive histology and ductal formation. Immunohistochemical stains for p63, CK-5/6, and CEA were diffusely positive, while CK-7 and CK-20 were negative. A diagnosis of eccrine porocarcinoma was made. He underwent complete wide excision with negative margins of the right upper back in January 2018. In November 2018, he was found to have a right posterior shoulder mass, positive right axillary lymph nodes, superficial right upper back soft tissue masses and lymphangitic carcinomatosis involving the left lung. He was transitioned to comfort care and subsequently passed from metastatic disease in December 2018. Conclusion The occurrence of eccrine porocarcinoma in a patient on long term immunosuppressive therapy for lung transplantation suggests a possible role for chronic immunosuppression in the induction of a subset of eccrine porocarcinomas, as in other cutaneous malignancies.

With the advent of high-resolution computed tomography (HRCT), micronodular lung disease is a routinely encountered pathology in thoracic imaging. This article will review how to differentiate the three main micronodular patterns and review the differential diagnosis for each. Differential diagnosis of micronodular lung disease may be extensive, but by identifying the pattern and using additional clues, such as distribution, additional imaging findings, and clinical history, a radiologist can make an accurate diagnosis. First, three micronodular patterns - centrilobular, peri-lymphatic, and random - can be identified by using a simple algorithm based on the location of nodules. This algorithm requires understanding of the anatomy and function of the secondary pulmonary lobule. Each micronodular pattern offers a unique differential diagnosis. Centrilobular nodules can be seen with inflammatory, infectious, or vascular aetiologies; peri-lymphatic nodules with sarcoidosis and lymphangitic carcinomatosis; and random nodules with haematogenous metastases or infections.

Pulmonary lymphangitic carcinomatosis (PLC) is defined as infiltration of the lymphatic vessels and perilymphatic connective tissue with tumor cells, which is secondary to malignancy. Therefore, it rarely appears as an initial finding preceding a diagnosis of malignancy. A 30-year-old male patient was hospitalized in our clinic with a pre-diagnosis of interstitial lung disease owing to the complaints of dry cough, progressive dyspnea, and acute respiratory insufficiency. He was diagnosed with signet ring cell carcinoma, which is a histologic subtype of adenocarcinoma, via gastroscopy, and lung involvement was consistent with PLC. Regardless of the patient age, PLC should be considered in differential diagnoses of progressive dyspnea, acute respiratory failure, and widespread interstitial lung involvement.

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., An 81‐year‐old man was admitted due to dyspnoea and haemoptysis. We suspected diffuse alveolar haemorrhage based on computed tomography (CT) findings of diffuse bilateral ground‐grass opacity and partial consolidation. Autopsy revealed pulmonary lymphangitic carcinomatosis of whole lungs and primary gallbladder cancer.

The diverse imaging manifestations of thoracic metastatic disease often challenge accurate interpretation. Malignant cells usually arrive via the bloodstream but find fertile substrates within the pulmonary circulation, lymphatic networks, branching airways, and pleural envelope. Unusual metastatic nodules cavitate, calcify, or hemorrhage. Widespread intravascular tumor may induce pulmonary hypertension. In lymphangitic carcinomatosis the deep and superficial lymphatic networks expand with tumorlets, with consequent parenchymal congestion. Nodal lymphomas can spread retrograde via lymphatic pathways, forming multilobar consolidations that mimic pneumonia. Sarcoid-like reactions occasionally develop in thoracic nodes and lung in settings of malignancy or immunotherapy, and are often confused with tumor recurrence.

Acute kidney injury (AKI) is a major complication in patients with cancer, associated with significant morbidity and mortality. Only two cases of kidney lymphangitic carcinomatosis associated with AKI have been reported, in gastric and colorectal adenocarcinoma. Here, we report on a 53-year-old man with pancreatic adenocarcinoma who developed AKI as a result of kidney lymphangitic carcinomatosis. The patient rapidly became anuric and required haemodialysis. Kidney lymphangitic carcinomatosis should be considered as a cause of AKI in patients with cancer and may become a more frequent clinical finding as patients with metastatic carcinoma survive for longer.

Patient: Female, 26-year-old Final Diagnosis: Adenocarcinoma with signet-ring cell features Symptoms: 32 week gestation with new onset chest pain and shortness of breath Medication: — Clinical Procedure: — Specialty: Obstetrics and Gynecology Objective: Rare co-existance of disease or pathology Background: Cancer in pregnancy is extremely rare, and gastric cancers are rarer still. Diagnosis is difficult in pregnancy due to overlapping symptoms with pregnancy such as nausea, pain, anemia, and fatigue. Case Report: A 26-year-old G1 woman at 32 weeks gestation with a past medical history of systemic lupus erythematosus presented with new-onset chest pain and shortness of breath. Computed tomography of the chest, electrocardiogram, and echocardiogram were normal. Laboratory evaluation revealed thrombocytopenia, proteinuria of 480 milligrams, and normal complement. She delivered on hospital day 3 due to worsening chest pain. During cesarean delivery, the patient became hypotensive and hypoxic and required intensive care unit admission after a cesarean hysterectomy. On postoperative day 2 she had a pulmonary embolus and was started on therapeutic anticoagulation. She clinically improved until postoperative day 4, when she was found unresponsive with pulseless electrical activity. After 38 minutes of Advanced Cardiac Life Support, death was pronounced. An autopsy was performed and the cause of death found to be complications of multi-organ system involvement of adenocarcinoma with signet ring cell features. Lymphangitic carcinomatosis was noted throughout the lungs. Conclusions: This patient had adenocarcinoma with signet ring cell features and associated lymphangitic carcinomatosis, which led to her postpartum death. Lymphangitic carcinomatosis is associated with an exceedingly poor prognosis, especially in pregnancy.

Osimertinib is an oral, irreversible epidermal growth factor receptor inhibitor that is associated with various pulmonary manifestations including transient asymptomatic pulmonary opacities (TAPOs) and pneumonitis. We present a case of a 61-year-old female with Stage IV lung adenocarcinoma, who developed bilateral ground glass opacities on her chest-computed tomography (CT) three months after initiating osimertinib. Her imaging findings were thought to represent lymphangitic carcinomatosis responding to chemotherapy rather than drug induced toxicity, and she was continued on osimertinib. Conversely, we present a second case of a 57-year-old female with Stage IV lung adenocarcinoma who developed osimertinib-induced pneumonitis and was successfully rechallenged with osimertinib and glucocorticoids. These cases, described herein, illustrate examples of the range of pulmonary manifestations of osimertinib, as well as the safety of rechallenging patients with osimertinib and glucocorticoids following the development of pneumonitis.

Interstitial lung diseases (ILD) are a heterogeneous group of diseases and one of the differential diagnosis which have to be excluded during diagnostic procedures are malignancies. We will present four patients who were referred to our Department because of suspicion of interstitial lung diseases according to radiology finding. In one case only, one of the radiologist’s differential diagnosis was pulmonary lymphangitic carcinomatosis. All four patients had exertional dyspnea and dry cough which are nonspecific and can be first manifestation of ILD or obstructive lung diseases. After diagnostic evaluation in three cases, diagnosis was pulmonary lymphangitic carcinomatosis due to metastatic lung adenocarcinoma and in one due to metastatic adenocarcinoma of unknown primary origin. Patients with lymphangitic carcinomatosis have poor prognosis without treatment and usually die because of respiratory failure. With these four cases we want to highlight importance of thinking about malignancies when we have patients with suspicion of interstitial lung disease especially when reticular pattern is present on chest X ray. We also wanted to show how important is radiology finding and multidisciplinary approach, and how radiologist’s differential diagnosis can be very helpful in making decisions in further investigations and way of clinicians thinking.

Recent therapeutic advancements have prolonged the survival duration of patients with metastatic or recurrent colorectal cancer even during salvage treatment. Although treatment with regorafenib and trifluridine/tipiracil combination has exhibited apparent survival benefits, clear and objective evidence of a response to these drugs is scarce. Herein, the present study reports the case of a patient with rectal cancer refractory to multiple surgical interventions and standard chemotherapy. Treatment with regorafenib resulted in immediate improvement of respiratory failure caused by pulmonary lymphangitic carcinomatosis. This improvement persisted for over 3 months and was confirmed by radiology. Our findings suggest that regorafenib can reduce peritumoral edema via its interaction with the vascular endothelial growth factor receptor. Thus, regorafenib functions as a multityrosine kinase inhibitor to alleviate symptoms of lymphangitic carcinomatosis despite the low potency of the drug.

Breast cancer typically spreads primarily to regional lymph nodes and subsequently to distant sites via hematogenous routes. Occasionally metastasis can occur through lymphangitic spread, usually to the lungs, resulting in lymphangitic carcinomatosis. Lymphangitic spread of several malignancies have been reported at other sites in the body with varying degrees of clinical significance. In this case report, we describe a rare case of lymphangitic spread of invasive lobular carcinoma to the contralateral breast identified on imaging as significant background enhancement without a discrete suspicious mass.

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.

Hirokazu Toshima, Toshikazu Ikusue, Atsushi Hisamatsu, Kouji Kobayashi, Ken Shimada Medical Oncology, Showa University Koto Toyosu Hospital, Tokyo, Japan Abstract: Clinicians often encounter cases of pulmonary lymphangitic carcinomatosis when treating patients with cancer. When such a condition develops before the diagnosis of cancer, its diagnosis is often challenging. Herein, we report about two patients with colorectal carcinoma diagnosed after the identification of lymphangitic carcinomatosis, which achieved complete remission with combination anti-epidermal growth factor receptor (anti-EGFR) antibody therapy. In case 1, a 74-year-old woman presented with cough and dyspnea that had persisted for 1month. She had unresectable advanced carcinoma of the sigmoid colon with lymphangitic carcinomatosis. Her respiratory status gradually deteriorated due to the disease. Thus, FOLFIRI plus cetuximab therapy was initiated. Her dyspnea rapidly resolved with the treatment, and complete remission of lymphangitic carcinomatosis was achieved. In case 2, a 46-year-old man presented with fever and dyspnea that had persisted for 1month. He had unresectable advanced carcinoma of the transverse colon with lymphangitic carcinomatosis. FOLFOXIRI therapy was then initiated. However, his respiratory status did not improve. Therefore, his treatment was immediately switched to FOLFIRI plus panitumumab. His dyspnea rapidly resolved with the treatment, and complete remission of lymphangitic carcinomatosis was achieved. In oncologic emergencies, such as lymphangitic carcinomatosis, requiring an early response to treatment, the administration of anti-EGFR antibodies may be a highly effective treatment option. Keywords: anti-epidermal growth factor receptor antibody, early tumor shrinkage, depth of response, chemotherapy, Pan-Asian adapted ESMO consensus guidelines, tumor location

Trousseau's syndrome is characterized as an unexpected, cancer-related thrombotic event, such as a cerebral infarction or a deep vein thrombosis/pulmonary embolism. We describe the first reported case of Trousseau's syndrome with pulmonary pleomorphic carcinoma and aggressive features. A 74 year-old man presenting with a pulmonary mass, which was identified as pleomorphic carcinoma with extensive lymph node involvement, in the left lower lobe, underwent a left lower lobectomy. Immunohistochemical analysis revealed that neoplastic cells exhibited an extensive expression of tissue factors with a mucin-producing adenocarcinoma component. Three months postoperatively, diffuse infiltration rapidly appeared in the left lung, which was identified as lymphangitic carcinomatosis via bronchoscopy. Prior to treatment for cancer recurrence, the patient presented with a left hemiplegia due to a cerebral infarction via multiple thromboses, with no evidence of atherosclerotic or cardiogenic thrombi. Elevated D-dimer and carbohydrate antigen 125 levels and the presence of a fibrin thrombus retrieved from the occluded vessel suggested Trousseau's syndrome as the etiology of the brain infarction. A hypercoagulable state associated with the aggressive recurrence of pulmonary pleomorphic carcinoma, accompanied by cancer cell production of mucin and tissue factors may be a potential mechanism for cancer-related thrombosis.

e21064 Background: Pulmonary lymphangitic carcinomatosis (PLC) occurs in 6%-8% of intrathoracic metastases among malignant tumor. The median survival was only 2.0 months from time of pulmonary symptoms to death in cases during 2000-2018, which is a poor prognosis. Effective interventions were needed besides standard chemotherapy and symptomatic support. Anlotinib showed a critical effect on lymphangiogenesis, and lymphatic metastasis in mouse models of lung adenocarcinoma, it might be a therapeutic option for tumor lymphatic metastasis. In this study, we retrospectively analyzed the efficacy and safety of anlotinib for PLC in patients with Non-small Cell Lung Cancer (NSCLC). Methods: We retrospectively investigated NSCLC patients with PLC at our hospital between May 2018 and November 2020, who received anlotinib monotherapy or combined therapy for PLC. Data were analyzed for progression-free survival (PFS), overall survival (OS), objective response rate(ORR), disease control rate(DCR) and adverse events (AE). The impact of clinical and genomic factors on PFS and OS were also assessed. Results: A total of 14 patients were enrolled with a median age of 64 years. 10(71.4%) were male, 4(28.6%) has smoking history, 10(71.4%) of patients had a performance status of 2-3. 9, 3, 2 patients had TP53 mutation, EGFR mutation, ALK fusion respectively. 9(64.3%) patients received anlotinib monotherapy. Of 14 patients, 8 achieved partial response (PR), 5 presented stable disease (SD), 1 had progressed disease. The ORR and DCR were 57.1% and 92.9% respectively. The median PFS was 3.1 months (95% CI: 2.0-4.2), the median OS for 1, 2, ≥3 line were 13 months, 7.2 months, 5.2 months, respectively. Median PFS and OS (≥3 line) were significantly longer for patients with TP53-mutant tumors compared with those with TP53–wild-type tumors (median PFS: 7 vs. 1.1 months, median OS (≥3 line): 6.8 vs. 1.9 months). No difference of PFS and OS (≥3 line) was found between EGFR or ALK alteration and the corresponding wild type patients. The most frequently reported AEs were high blood pressure (11, 78.6%), hand foot syndrome (6, 42.9%), diarrhea (5, 35.7%), fatigue (4, 28.6%), hoarseness (3, 21.4%), proteinuria (2, 14.3%) and stomatitis (2, 14.3%). Conclusions: Anlotinib presented favorable efficacy in patients with pulmonary lymphangitic carcinomatosis and conferred considerable survival benefit compared with previous studies, especially in patients harboring TP53 mutations. The AEs were manageable. These indicated that anlotinib can be a promising therapeutic treatment of PLC. More clinical data is needed to validate this finding.

Introduction The lymphangitic carcinomatosis (LC) pattern of metastatic malignancy is associated with a poor prognosis but is currently not well defined in malignant pleural mesothelioma (MPM). Here, we report the incidence and prognostic significance of the radiographic development of LC in MPM following extended pleurectomy/decortication (EPD). Methods Consecutive patients with biopsy-proven MPM undergoing EPD with intraoperative photodynamic therapy (PDT) at our institution from 2008 to 2014 were included in this retrospective study. Patients without available post-surgical clinical or imaging data for direct review were excluded. CT images were reviewed by an experienced, board-certified thoracic radiologist and confirmed by consensus review. Overall survival (OS) and progression-free survival (PFS) were calculated by Kaplan Meier methodology. Hazard ratios were compared with a cox proportional hazard model. Results 44 patients underwent EPD with PDT during the study period and had available clinical and imaging data. During the follow-up period (median 34 months), 17 patients (39%) developed LC at a median of 10 months after surgery (IQR 5–21 months). 16 of the 17 patients who developed LC (94%) died during the follow-up period, compared to 17 of the 27 who did not develop LC (63%). OS for the LC versus non-LC group was 53% versus 93% at 1 year and 18% versus 67% at 3 years. LC was significantly associated with a lower OS (HR 4.07; 95% confidence interval 1.44–11.48; p = 0.008). PFS for the LC group versus non-LC group was 8 months (IQR 5–9 months) compared to 17 months (IQR 11–24 months) (p Conclusion LC is a common form of failure in MPM following EPD and is associated with a poor prognosis. Thus, further studies are warranted to determine if any evidence of preoperative LC should be an absolute contraindication to EPD and may warrant an EPP or no surgery at all.

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 β2 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.

Clinicians generally suspect pulmonary tumor embolism (PTE) with uncontrolled carcinomas which often spread to lungs. We, however, experienced an autopsy case of diffuse microscopic PTE despite controlled liver metastases of transitional cell carcinoma (TCC). A 66-year-old man with progressing respiratory symptoms showed almost normal chest findings on computed tomography. Although liver metastases were successfully shrunk by chemotherapy, the patient died from aggressive respiratory failure. An autopsy revealed small pulmonary vessels showing diffuse tumor emboli. TCC can cause PTE even if liver metastases are controlled. We must therefore be aware that PTE can manifest as respiratory symptoms without any computed tomography findings.

Introduction:The thorax is a frequent site of metastasis from numerous non-pulmonary cancers. Intrathoracic metastatic disease may manifest in a variety of forms, including solitary or multiple pulmonary nodules, endobronchial tumor, lymphangitic carcinomatosis, or a pleural effusion.Material and Methods:This is muticentric, observational study conducted in Pulmonary Medicine, MIMSR medical college & Venkatesh chest hospital and critical care center, Latur during June 2012 to November 2016 to observe various thoracic manifestations of gynecological malignancies. Total 2300 cases of various gynecological malignancies were screened and finally 330 cases showing various thoracic manifestations were enrolled after subjecting those cases to inclusion and exclusion criteria. All study cases were subjected to chest radiological investigations and histopathological investigations whenever necessary to diagnose metastatic disease. Hospital’s ethical committee & IRB approval has been taken and written informed consent of patient was taken before enrollment.Results:Screened total 2000 cases of gynecological malignancies (ovary, endometrium, cervix & benign ovarian tumors) and selected 330 cases with various metastatic thoracic manifestations. Malignant gynecological tumors including endometrial, cervical & ovarian cancers predominantly observed in above 50 years of age while benign ovarian and uterine tumors in below 50 years of age (p

Lymphangitic carcinomatosis is a difficult to treat disease entity and usually portends a poor prognosis given the advanced state in which it is often diagnosed. Treatment is usually based on standard therapy of the primary malignancy, but in most cases, is palliative in nature. This case highlights the potential for PD-1 immunotherapy as both a rapid and durable treatment for lymphangitic carcinomatosis.

Pulmonary lymphangitic carcinomatosis is a special type of diffuse metastasis of carcinoma in the lymphatic vessels of the lungs. Lymphangitic carcinomatosis is commonly observed in malignancies of the breast, lung, pancreas, colon and cervix as a strong marker for poor prognosis. Presenting with common respiratory symptoms, it may be easily misdiagnosed as other pulmonary interstitial diseases. Pulmonary lymphangitic carcinomatosis is a rare manifestation of metastatic gastric cancer. The presented case describes a patient with pulmonary lymphangitic carcinomatosis in the course of stomach cancer. The primary diagnosis was established based on the exclusion of other interstitial lung diseases, with the use of imaging techniques as well as biochemical, microbiological and cytological findings. The patient's general condition was very severe, rendering him bedridden and therefore not eligible for any treatment, including chemotherapy. He died suddenly before final verification of the diagnosis. Pulmonary lymphangitic carcinomatosis should be suspected in patients with advanced gastric cancer, presenting with symptoms and signs of respiratory disease. Imaging techniques are mostly helpful to establish the diagnosis.

Lymphangitic carcinomatosis refers to the rare process of diffuse malignant invasion of the pulmonary lymphatics. We describe the first reported case of lymphangitic carcinomatosis visualized with gallium-68 prostate-specific membrane antigen positron emission tomography and its clinical significance in a 53-year-old man with prostate carcinoma. This case highlights the ability of gallium-68 prostate-specific membrane antigen positron emission tomography for prostate carcinoma characterization and the importance of always considering atypical patterns of metastatic disease.

A novel pathogenesis of pulmonary lymphangioleiomyomatosis (PLAM) is presented incorporating a review of clinical and pathology research on the disease together with a review of research in the field of senescence. In this model of pathogenesis it is proposed that: 1) the primary site of origin for this disease is the Mullerian smooth muscle found in the female reproductive tract and this accounts for the striking female predominance of the disease; 2) the tumor spreads to the lungs via a mechanism similar to lymphangitic carcinomatosis and this accounts for its universal symmetric and bilateral distribution; 3) there is a mutational basis for a senescence-associated growth arrest which helps explain the absence of enlarging tumor masses in the lung despite years of disease duration; and 4) PLAM cells have a senescence-associated secretory phenotype with implications for the pathogenesis of the cystic transformation of the lungs. The secretory phenotype of PLAM cells is reviewed and the overlap with the senescence associated secretory phenotype products of known senescent cells is documented. The role of the switch to glycolytic metabolism (Warburg state) by PLAM cells is described. Finally, the “contradictory signals” hypothesis for the induction of the senescent state is explored and its role in PLAM described.

Collecting duct carcinoma is a rare neoplasm of the kidney, accounting for only 1–2% of renal tumors. These tumors arise from the principal cells of the renal collecting ducts of Bellini. The majority of patients have lymph node involvement and metastases to lungs, liver, bone, adrenal glands, and brain. We present a case of a 48 year old woman who came to the hospital with a clinical presentation suspicious for pneumonia. One week later her symptoms aggravated. A CT chest and abdominal imaging showed bilateral pulmonary infiltrates, retroperitoneal lymphadenopathy, and left hydroureteronephrosis. She expired after developing acute respiratory failure. An autopsy was performed which revealed chylopericadium of 150 cc; bilateral reticular pattern on the surfaces of the lungs; neck, mediastinal and retroperitoneal lymphadenopathy, and a 5.1 cm left kidney mass located in the mid portion medulla. The kidney tumor was a rhabdoid collecting duct carcinoma. The lungs showed diffuse subpleural lymphangitic spread of the carcinoma. We report a rare case of chylopericardium due to lymphangitic carcinomatosis from a 5.1 cm rhabdoid collecting duct carcinoma not suspected clinically or radiologically. This case highlights the importance of performing autopsies in an era when clinicians heavily rely on high-tech imaging diagnostic modalities.