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Background: To investigate the predictable parameters associated with downgrading in patients with a Gleason score (GS) 8 (4+4) in prostate biopsy after radical prostatectomy., Methods: We retrospectively analyzed 62 patients with a GS of 4+4 on prostate biopsy who underwent robotic radical prostatectomy between 2017 and 2022., Results: 38 of 62 (61.2%) were downgraded. In multivariable logistic regression model, Ga-68 prostate-specific membrane antigen (PSMA) positron-emission tomography (PET)/computed tomography (CT) SUV max was independent predictor of downgrading (OR 0.904; p = 0.011) and a Logistic Regression model was constructed using the following formula: Y = 1.465-0.95 (PSMA PET/CT SUV max). The model using this variable correctly predicted the downgrading in 72.6% of patients. The AUC for PSMA PET/CT SUV max was 0.709 the cut off being 8.8. A subgroup analysis was performed in 37 patients who had no other European Association of Urology (EAU) high risk features. 25 out of 37 (67.5%) were downgraded, and 21 of these 25 had organ confined disease. Low PSMA SUV max (<8.1) and percentage of GS 4+4 biopsy cores to cancer bearing cores (45.0%) were independently associated with downgrading to GS 7., Conclusion: PSMA PET/CT can be used to predict downgrading in patients with GS 4+4 PCa. Patients with GS 4+4 disease, but no other EAU high risk features, low percentage of GS 4+4 biopsy cores to cancer bearing cores, and a low PSMA PET/CT SUV max are associated with a high likelihood of the cancer reclassification to intermediate risk group., (© 2024. The Author(s).)

Introduction: This study aimed to investigate the oncological outcomes and toxicity profile of 177 Lu-PSMA-I&T radioligand therapy (RLT) in patients with metastatic castration-resistant prostate cancer (mCRPC), as well as our initial experience in metastatic hormone-sensitive prostate cancer (mHSPC)., Patients and Methods: A total of 38 consecutive patients with metastatic prostate cancer (33 mCRPC and 5 mHSPC) received 177 Lu-PSMA-I&T RLT, with a median of 2 cycles per patient (range, 1-7). Response to RLT was evaluated based on prostate-specific antigen (PSA) changes and imaging response. Clinical progression-free survival and overall survival were used to report oncological outcomes. Toxicity was assessed using the Common Toxicity Criteria for Adverse Events criteria., Results: In mCRPC, 22 (69%), 18 (56%), and 11 (34%) patients achieved any PSA decline, PSA response of ≥30%, and PSA response of ≥50%, respectively. The clinical progression-free survival and overall survival after the first cycle of RLT were 6.3 and 21.4 months, respectively. In mHSPC, 177 Lu-PSMA-I&T RLT resulted in excellent PSA response (93.0%-99.9%) in all cases. Clinical progression and cancer-related mortality occurred in only 1 case. Toxicity profile was favorable in both mHSPC and mCRPC., Conclusions: 177 Lu-PSMA-I&T RLT demonstrated favorable PSA response (≥30%) in over half of the patients with mCRPC and excellent PSA response in all patients with mHSPC. Toxicity profile was favorable in both mHSPC and mCRPC settings. Further studies are needed to evaluate the role of 177 Lu-PSMA-I&T RLT in the management of metastatic prostate cancer., Competing Interests: Conflicts of interest and sources of funding: B.E. is supported by the European Urological Scholarship Programme through a 1-year research scholarship., (Copyright © 2023 Wolters Kluwer Health, Inc. All rights reserved.)

Background: Treatment response is traditionally monitored using prostate-specific antigen (PSA) and conventional imaging in patients with metastatic prostate cancer (mPCa)., Objective: To assess the diagnostic performance of prostate-specific membrane antigen (PSMA) positron emission tomography (PET)/computed tomography (CT) when monitoring mPCa patients receiving systemic treatment and also to investigate the concordance between PSMA PET response according to the PSMA PET progression (PPP) criteria and biochemical response., Design, Setting, and Participants: A total of 96 patients with 68 Ga-PSMA-11 PET/CT-detected mPCa at baseline PSMA PET/CT (bPSMA) who underwent at least one follow-up scan after receiving systemic treatment were included in the study. PSA levels at bPSMA and follow-up PSMA PET (fPSMA) scans were recorded. The PPP criteria were used to define PSMA progression. Biochemical progression was defined as ≥25% increase in PSA. PSMA PET and PSA responses were dichotomized into progressive disease (PD) versus non-PD, and the concordance between PSA and PSMA responses was evaluated., Outcome Measurements and Statistical Analysis: The concordance between PSA and PSMA PET responses was presented using frequencies, percentages, and Cohen's kappa test., Results and Limitations: A total of 345 serial PSMA PET/CT (96 bPSMA and 249 fPSMA) scans were evaluated. The positivity rates of PSMA PET scans for PSA levels of <0.01, 0.01-0.2, 0.2-4, and >4 ng/ml were 55.6%, 75.0%, 100%, and 98.8%, respectively. PSA and PSMA responses showed moderate-to-high concordance (Cohen's κ = 0.623, p < 0.001). PSA-PSMA discordance was detected in 39 scans (17%). The most common cause of discordance was the discordant results between different metastatic lesions (16/28, 57.1%) in patients with PPP without PSA progression and local progression in prostate (n = 7/11, 63.6%) in patients with PSA progression without PPP., Conclusions: PSMA PET/CT showed very high detection rates of malignant lesions even at very low PSA values and showed significant concordance with PSA response when monitoring treatment response in patients receiving systemic treatment for mPCa., Patient Summary: This study describes that prostate-specific membrane antigen positron emission tomography (PSMA PET), a new sensitive imaging tool, can detect malignant lesions even at very low prostate-specific antigen values when monitoring metastatic prostate cancer. The PSMA PET response and biochemical response showed significant concordance, and the reason for discordant results seems to be the different responses of metastatic lesions and prostatic lesions to systemic treatment., (Copyright © 2023 European Association of Urology. Published by Elsevier B.V. All rights reserved.)

Purpose: The aim of this study was to evaluate the impact of SPECT/CT lymphoscintigraphy on targeted axillary dissection (TAD) in node-positive breast cancer (BC) patients who had undergone neoadjuvant chemotherapy (NAC)., Methods: Sixty-two female BC patients with biopsy-confirmed axillary nodal metastases underwent NAC, followed by breast surgery with TAD. A metallic clip was placed in the sampled LN before NAC. On the day of surgery, a periareolar intradermal 99m Tc-nanocolloid injection was administered, followed by SPECT/CT lymphoscintigraphy. The clipped nodes were localized on CT images, assessed for 99m Tc uptake before surgery, and confirmed during the procedure., Results: T1-4, N1-2 patients were enrolled in the study. All patients underwent sentinel lymph node (SLN) biopsy. The clipped node was the SLN in 54 (88.5%) patients. In 3 patients (4.9%), a clip was found in a nonsentinel lymph node. In 4 patients, the clips were not visible on SPECT/CT images, and lymph nodes were not found during the procedure. SPECT/CT correctly localized the clipped lymph node in all patients. The overall false-negative rate for TAD was 3.33%. The mean follow-up duration was 29 months, and there were no axillary recurrences., Conclusions: SPECT/CT lymphoscintigraphy can accurately localize clipped nodes and SLNs after NAC in patients with node-positive BC., Competing Interests: Conflicts of interest and sources of funding: none declared., (Copyright © 2023 The Author(s). Published by Wolters Kluwer Health, Inc.)

Purpose: In lung adenocarcinoma cases, 'spread through air spaces' (STAS) is a new indicator of invasion and directly related to disease survival. The aim of our study is to establish whether a preoperatively performed 18F-Fluorodeoxyglucose (FDG) PET/computed tomography (CT) imaging data can predict the presence of STAS in cases with lung adenocarcinoma and thus predict the decision for the type of surgery and adjuvant chemotherapy., Materials and Methods: Between 2000 and 2019, we retrospectively analyzed 63 patients with lung adenocarcinoma cases that had undergone lobectomy or pneumonectomy. Semiquantitative parameters were calculated and metabolic tumor volume (MTV)/CT volume (CTV) ratio was recorded from FDG PET/CT data. The pathological samples from these patients were evaluated for STAS. All these values were evaluated for their correlation with the alveolar spread., Results: There was no statistically significant correlation to be found between CTV, MTV, total lesion glycolysis (TLG), standardized uptake value (SUV)max, SUVmean and STAS (P > 0.05). However, MTV/CTV ratio above 1 had statistically more alveolar spread. In the group with an MTV ratio above 1, STAS positivity was 27 (75%), and 9 (25%) did not have STAS, whereas these were 6 (22.2%) patients who had STAS, and 21 (77.8%) did not have STAS in the group with below 1 (P < 0.001)., Conclusions: In the preoperative PET study inoperable lung adenocarcinoma cases, MTV/CTV ratio higher than 1 was found to predict STAS positivity. As a result, it was found that it provided significant clinical additional information regarding the need for a surgical approach (lobar resection instead of sublobar) and adjuvant chemotherapy., (Copyright © 2021 Wolters Kluwer Health, Inc. All rights reserved.)

Background: To evaluate the additive role of Ga-68 PSMA PET as a primary staging tool in patients bearing prostate cancer in single PIRADS 4 or 5 index lesions., Methods: Eighty-one biopsy-naive patients with preoperative mpMRI and Ga-68 PSMA PET who underwent radical prostatectomy (RP) were evaluated retrospectively. Forty-nine patients had PIRADS 4 and 32 had PIRADS 5 index lesions. The localization, grade, and volumetric properties of dominant (DT) and non-dominant tumors (NDT) in RP were compared to the index lesions of mpMRI and Ga-68 PSMA PET., Results: The median age and PSA level were 62 (IQR; 59-69) years and 7 (IQR; 2-8) ng/ml, respectively. Ga-68 PSMA PET detected DTs in 100% of the patients including 13 patients in whom mpMR failed. In 45 patients an NDT was reported in RP. Ga-68 PSMA PET accurately detected NDT in 24 of 45 (53.3%) patients. Six patients (12.2%) in PIRADS 4 and 8 (25%) in PIRADS 5 group showed upgrading. In PIRADS 4, Ga-68 PSMA PET localized DT in all patients with upgraded tumors whereas mpMRI missed exact location in 2 of 6 (33.3%). In PIRADS 5 both mpMRI and Ga-68 PSMA PET accurately located all DTs. Overall detection rates of extracapsular extension (ECE) and seminal vesicle invasion (SVI) by mpMRI were 51.1% and 53.8%, respectively. Ga-68 PSMA PET detected ECE and SVI in 27.9% and 30.7%, respectively. When mpMRI and Ga-68 PSMA PET were used in combination detection rates of ECE and SVI increased to 65.1 and 61.5%. Ga-68 PSMA PET-detected six of ten patients with positive lymph nodes whereas mpMRI could not identify any., Conclusions: Ga-68 PSMA PET has a better diagnostic accuracy in detecting DT, NDT, upgrading, adverse pathology in patients with PIRADS 4 index lesions. However, mpMRI better predicted ECE and SVI than Ga-68 PSMA PET.

Background: Results from prospective trials have shown higher accuracy of prostate-specific membrane antigen (PSMA)-based positron emission tomography (PET)/computed tomography (CT) in detection of lymph node metastasis (LNM) compared to conventional imaging., Objective: To evaluate the accuracy of 68 Ga-PSMA-11 PET/CT for LNM detection in patients undergoing radical prostatectomy (RP) and extended pelvic lymph node dissection (PLND)., Design, Setting, and Participants: Between June 2014 and November 2020, 96 patients with 68 Ga-PSMA PET/CT for primary staging underwent RP and extended PLND., Outcome Measurements and Statistical Analysis: The results from 68 Ga-PSMA PET/CT were compared with histologic data from primary PLND in 96 patients. All 68 Ga-PSMA PET/CT scans were centrally reviewed., Results and Limitations: Of 96 patients, 15.6% (n = 15) harbored LNMs. The median prostate-specific antigen at 68 Ga-PSMA PET/CT was 8.0 ng/ml (interquartile range 5.5-11.7). The majority of patients had intermediate- (52.1%) or high-risk disease (41.7%). Biopsy grade group 4 and 5 was present in 22.9% and 15.6%, respectively. The 68 Ga-PSMA PET/CT scans identified eight of 15 patients (53.3%) as LN-positive (true positive). The calculated per-patient sensitivity, specificity, positive predictive value, negative predictive value, and accuracy of 68 Ga-PSMA PET/CT in the detection of LNM were 53.3%, 98.8%, 88.9%, 92.0%, and 91.7%, respectively. The per-patient sensitivity and specificity in the detection of LNMs larger than 2 mm were 61.5% and 98.8%, respectively. The main limitation is the retrospective design of the study., Conclusions: 68 Ga-PSMA PET/CT is accurate in lymph node staging and the results support its use for primary staging of prostate cancer., Patient Summary: We compared prostate-specific membrane antigen (PSMA)-based positron emission tomography (PET)/computed tomography (CT) findings with histopathology results after extended lymph node dissection and showed that it is accurate in detecting lymph node metastases. Our results support the use of PSMA PET/CT for primary staging of prostate cancer., (Copyright © 2021 European Association of Urology. Published by Elsevier B.V. All rights reserved.)

Objectives: To evaluate the use and benefits of tumor navigation during performing robotic assisted radical prostatectomy (RARP)., Patients and Methods: Borders of the visible tumor(s) was/were and surrounding structures marked on multiparametric prostate magnetic resonance imaging (mpMRI) and 68 Ga-labeled prostate-specific membrane antigen ligand using positron emission computed tomography (Ga68 PSMA-PET/CT). Three dimensional (3D) reconstruction of the images were done that were transferred to virtual reality (VR) headsets and Da Vinci surgical robot via TilePro. Images were used as a guide during RARP procedures in five cases. Indocyanine green (ICG) guided pelvic lymph node dissection (n = 2) and Martini Klinik Neurosafe technique (n = 2) were also applied., Results: Mean patient age was 60.6 ± 3.7 years (range, 56-66). All VR models were finalized with the agreement of radiologist, urologist, nuclear physician, and engineer. Surgeon examined images before the surgery. All VR models were found very useful particularly in pT3 diseases. Pathological stages included pT2N0 (n = 1), pT3aN0 (n = 1), pT3aN1 (n = 2), and pT3bN1 (n = 1). Positive surgical margins (SMs) occurred in two patients with extensive disease (pT3aN1 and pT3bN1) and tumor occupied 30% and 50% of the prostate volumes. Mean estimated blood loss was 150 ± 86.6 cc (range, 100-300). Mean follow-up was 3.4 ± 1.7 months (range, 2-6). No complication occurred during perioperative (0-30 days) and postoperative (30-90 days) periods in any patient., Conclusions: 3D reconstructed VR models by using mpMRI and Ga68 PSMA-PET/CT images can be accurately prepared and effectively applied during RARP that might be a useful tool for tumor navigation. Images show prostate tumors and anatomy and might be a guide for the console surgeon. This is promising new technology that needs further study and validation., (© 2020 The Authors. BJUI Compass published by John Wiley & Sons Ltd on behalf of BJU International Company.)

SCA48 is a novel spinocerebellar ataxia (SCA) originally and recently characterized by prominent cerebellar cognitive-affective syndrome (CCAS) and late-onset ataxia caused by mutations on the STUB1 gene. Here, we report the first SCA48 case from Turkey with novel clinical features and diffusion tensor imaging (DTI) findings, used for the first time to evaluate a SCA48 patient. A 65-year-old female patient with slowly progressive cerebellar ataxia, cognitive impairment, behavioral changes, and a vertical family history was evaluated. Following the exclusion of repeat expansion ataxias, whole exome sequencing (WES) was performed. Brain magnetic resonance imaging (MRI), including DTI, and single-photon emission computed tomography (SPECT) were used to study the primarily affected tracts and regions. WES revealed the previously reported heterozygous truncating mutation in ubiquitin ligase domain of STUB1 (ENST00000219548:c.823&#95;824delCT, ENSP00000219548:p.L275Dfs*16) leading to a frameshift. Patient's cognitive status was compatible with CCAS. Novel clinical features different from the original report include later onset chorea, dystonia, general slowness of movements, apraxia, and palilalia, some of which have been recently reported in two families with different STUB1 mutations. CCAS is a prominent and often early feature of SCA48 which may be followed years after the onset of the disease by other complex neurological signs and symptoms. DTI may be helpful for demonstrating the cerebello-frontal tracts, involved in CCAS-associated SCA48, the differential diagnosis of which may be challenging especially in its early years.

The geographical location and differences in tumor biology significantly change the management of gastric cancer. The prevalence of gastric cancer ranks fifth and sixth among men and women, respectively, in Turkey. The international guidelines from the Eastern and Western countries fail to manage a considerable amount of inconclusive issues in the management of gastric cancer. The uncertainties lead to significant heterogeneities in clinical practice, lack of homogeneous data collection, and subsequently, diverse outcomes. The physicians who are professionally involved in the management of gastric cancer at two institutions in Istanbul, Turkey, organized a consensus meeting to address current problems and plan feasible, logical, measurable, and collective solutions in their clinical practice for this challenging disease. The evidence-based data and current guidelines were reviewed. The gray zones in the management of gastric cancer were determined in the first session of this consensus meeting. The second session was constructed to discuss, vote, and ratify the ultimate decisions. The identification of the T stage, the esophagogastric area, imaging algorithm for proper staging and follow-up, timing and patient selection for neoadjuvant treatment, and management of advanced and metastatic disease have been accepted as the major issues in the management of gastric cancer. The recommendations are presented with the percentage of supporting votes in the results section with related data.

Bleomycin is an antineoplastic agent causing fatal pulmonary toxicity. Early diagnosis of bleomycin-induced pneumonitis is crucial to prevent irreversible damage. Pulmonary function tests are unreliable for identifying risk of bleomycin toxicity. Fluorodeoxyglucose PET/CT scanning can reveal inflammation secondary to pneumonitis but is not sufficiently specific for diagnosis. We retrospectively analyzed scans from 77 patients with Hodgkin lymphoma (median age 41 years, mean bleomycin dose 134 mg) to evaluate bleomycin-induced pneumonitis. We identified 13 patients with abnormal lung uptake of fluorodeoxyglucose. Tracer activity was predominantly diffuse, bilateral, in the lower lobes and subpleural areas. Interim scanning during treatment revealed pneumonitis in eight of 13 patients (asymptomatic in six). One asymptomatic patient died of bleomycin toxicity. For remaining 12 patients, bleomycin was discontinued and methylprednisolone given, all showed resolution of the pneumonitis. These findings suggest that routine interim or end-of-treatment FDG-PET/CT scanning could be beneficial for alerting clinicians to asymptomatic bleomycin-induced toxicity.

Introduction: Metachronous metastatic spread of clinically localized renal cell carcinoma (RCC) affects almost 1/3 of the patients. They occur most frequently in lung, liver, bone and brain. Isolated omental metastasis of RCC has not been reported so far., Case Presentation: A 62-year-old patient previously diagnosed and treated due to pulmonary sarcoidosis has developed an omental metastatic lesion 13 years after having undergone open extraperitoneal partial nephrectomy for T1 clear-cell RCC. Constitutional symptoms and imaging findings that were attributed to the presence of a sarcomatoid paraneoplastic syndrome triggered by the development this metastatic focus complicated the diagnostic work-up. Biopsy of the [18F]-fluorodeoxyglucose (+) lesions confirmed the diagnosis of metastatic RCC and the patient was managed by the resection of the omental mass via near-total omentectomy followed by targeted therapy with a tyrosine kinase inhibitor., Discussion: Late recurrence of RCC has been reported to occur in 10-20% of the patients within 20 years. Therefore lifelong follow up of RCC has been advocated by some authors. Diffuse peritoneal metastases have been reported in certain RCC subtypes with adverse histopathological features. However, isolated omental metastasis without any sign of peritoneal involvement is an extremely rare condition., Conclusion: To our knowledge, this is the first reported case of metachronously developed, isolated omental metastasis of an initially T1 clear-cell RCC. Constitutional symptoms, despite a long interval since nephrectomy, should raise the possibility of a paraneoplastic syndrome being associated with metastatic RCC. Morphological and molecular imaging studies together with histopathological documentation will be diagnostic., (Copyright © 2016 The Authors. Published by Elsevier Ltd.. All rights reserved.)

Integrated positron emission tomography and computed tomography (PET/CT) scanning has become the standard for oncologic imaging. We sought to determine the role of PET/CT in mediastinal non-small cell lung cancer staging. One hundred and twenty-seven consecutive patients were enrolled in the study where PET/CT was performed due to pathologically defined non-small cell carcinoma from a single center. They all underwent complete resection with a thoracotomy and systemic lymph node dissection (SLND) between October 2005 and January 2007. Postoperative pathology results of lymph node stations regarding the nodal spread and stage were compared with clinical stage obtained by PET/CT. The sensitivity, specificity, accuracy, negative predictive value (NPV) and positive predictive value (PPV) of PET/CT in N2 cases were determined to be 72.0%, 94.4%, 92.7%, 97.7% and 49.2%, respectively. Maximum standard uptake (SUV(max)) cut-off value for mediastinal N2 involvement in PET/CT was obtained by applying 'receiver operating characteristic' (ROC) analysis that was set to 5.2. Correct stage with PET/CT was established in 76.3% of cases. Staging of non-small cell lung cancer (NSCLC), according to the PET/CT for which we determined 97.79% NPV, we consider that thoracotomy without preoperative mediastinal invasive staging in cases of negative mediastinal involvement in PET/CT can be certainly performed.

Background: The aim of the study was to assess the role of F-18 fluoro-2-D-deoxyglucose positron emission tomography ( (18)FDG-PET)/computed tomography (CT) in patients with undiagnosed pleural diseases and to compare the findings with those of invasive diagnostic procedures., Methods: The study included 83 patients with pleural lesions (63 with pleural effusion; 20 with pleural thickening) on CT scan performed between November 2005 and December 2007. The study group consisted of 63 males and 20 females; their median age was 47 years. PET-CT scan was performed for all patients before surgery. A maximum standard uptake value greater than 3.0 was accepted as positive for malignancy. For histopathological diagnosis, video-assisted thoracoscopic surgery was performed in 76 patients and a mini-thoracotomy was performed for the remaining 7 patients., Results: Postoperative histopathological examination revealed malignancy in 44 cases, 25 of which were malignant mesothelioma; the remaining 39 cases were benign. There were no false negative results, but two false positive results (tuberculosis). PET-CT scanning had 100 % sensitivity, 94.8 % specificity and 97.5 % accuracy., Conclusion: Our study suggests that PET-CT may be an effective tool for the differentiation of benign and malignant pleural diseases. We believe that PET/CT may prevent redundant surgical procedures in young patients who are SUVmax negative., (Georg Thieme Verlag KG Stuttgart New York.)

Unlabelled: The aim of this study was to evaluate the value of Tc-99m ethylenedicysteine exercise renography in patients with hypertension who were suspicious for renal artery stenosis and compare the results with captopril renography., Methods: Twenty-nine patients with hypertension who were highly suspect for having renal artery stenosis were included in the study. Basal captopril exercise renograms were performed within 1 week in all patients with Tc-99m EC. Exercise was performed with a bicycle ergometer as described in the literature. Interpretations of renograms were made according to the consensus report on angiotensin converting enzyme (ACE) inhibitor renography as high, low, or intermediate probability. The sensitivity, specificity, and positive and negative predictive values were also obtained on both the kidneys and a patient basis. The results were compared with renal angiography in 19 patients., Results: Twenty of 29 patients had concordant results with both captopril and exercise studies. All discordant tests were normal with captopril but positive with exercise renograms. Nineteen patients with 38 kidneys were included for statistical data analysis. Nine kidneys, which were either atrophic or abnormal during the basal study, were excluded from the kidney-based analysis. With contrast angiography, 8 patients had renal artery stenosis. When evaluated on a kidney basis, the sensitivity, specificity, and positive and negative predictive values for captopril scans were 87%, 100%, 100%, and 95%, and for the exercise scans were 100%, 90%, 80%, and 100% respectively. When calculated on a patient basis with regard to the captopril scans, the sensitivity, specificity, and positive and negative predictive values were 87%, 81%, 77%, and 88%, and for exercise scans were 100%, 54%, 61%, and 100% respectively. There were 2 false-positive exercise scans that were found to be the result of dilated minor collecting systems and changes in kidney contours as a result of motion during exercise., Conclusions: These results indicate that exercise renography has a similar sensitivity but lower specificity compared with captopril scintigraphy in the diagnosis of renal artery stenosis. Because of the lower specificity, its use in the screening of renovascular hypertension may be limited. Exercise renography may be more useful as an investigative tool for essential hypertension.

Noonan syndrome is characterised by a Turner-like phenotype and a normal karyotype. Although it is reported to be associated with abnormalities of the lymphatic system, involvement of the pulmonary lymphatics is rare. We present a case of Noonan syndrome where a whole body scintigraphy revealed lymphangiectasia of the lower extremities, abdomen and lungs.