Ground-glass opacity nodules (GGNs) are a computed tomography (CT) finding suggestive of lung cancer. Conventional bronchoscopy with brush cytology is a simple diagnostic modality but has a low diagnostic yield for peripheral lesions, especially peripheral GGNs. Therefore, maximizing the detection rate of bronchoscopic brushings should be a key objective. We report a case of a subpleural ground glass opacity (GGO) with a cytological diagnosis of adenocarcinoma by bronchoscopic brushing guided by manual mapping navigation.A 46-year-old man was hospitalized for GGO in the right lung. Follow-up CT revealed a subpleural nodule sized 1.2 cm × 0.9 cm in the superior segment of the right lower lobe.CT findings of the patient's nodule were suggestive of malignancy.The patient underwent conventional bronchoscopy combined with brushing guided by manual mapping navigation, with subsequent cytological diagnosis of adenocarcinoma. The patient then underwent right lower lobectomy with mediastinal lymph node dissection.There were no postoperative complications. Postoperative pathological examination showed lung adenocarcinoma with lepidic and acinar growth without visceral pleural invasion .Exfoliated cells present in peripheral GGNs are rarely detected on brush sampling. However, use of a manual mapping navigation system may help increase the sensitivity of conventional bronchoscopic brushing for the diagnosis of peripheral pulmonary lesions. Maximizing the detection rate of bronchoscopic brushings should therefore be a key objective. Although advanced technologies and innovations such as C-arm fluoroscopy, electromagnetic navigation bronchoscopy, radical endobronchial ultrasound, and virtual bronchoscopy navigation can significantly improve the sensitivity of bronchoscopy brush cytology of peripheral nodules, they are expensive, time consuming, require special equipment and experienced bronchologists, or involve radiation exposure.–8Ground glass opacity nodules (GGNs) are a computed tomography (CT) finding suggestive of lung cancer. This method improved the sensitivity of conventional brushing of malignant PPLs. In combination with the mapping method, the sensitivity of conventional brushing with mapping increased from 17.0% to 31.5%. After that, we used manual mapping for routine bronchoscopy with brushing. Herein, we present a case of a subpleural ground glass opacity (GGO) with a cytological diagnosis of adenocarcinoma by bronchoscopic brushing guided by manual mapping navigation.Starting in 2010, we used a manual mapping method to guide bronchoscope insertion for brushing. This method determines the route with a series of bronchial opening sketches and marks the leading bronchus at every bifurcation point based on thin-section CT.2A GGO nodule was identified in the right lower lobe of a 46-year-old man during incidental CT screening performed 5 months before his visit to our hospital. Before admission, the patient underwent additional CT screening, which indicated no significant changes in the lesion. Chest CT imaging revealed a 12 mm × 9-mm solitary part-solid nodule in the superior segment of the right lower lobe of the lung .3 This is mainly because the tumor cells in these situations are confined to the alveolar cavity without infiltrating into the lung interstitial tissues or without shedding from the alveoli for dissemination through the airways. However, there is evidence that other more invasive, non-lepidic growth patterns of adenocarcinoma, such as acinar and papillary patterns, have appeared as GGNs.,12 Sun et al reported GGNs (nodule diameter <20 mm and solid component diameter <50%) in a group of 408 patients, among which 22.8% (93 cases) were identified as an invasive adenocarcinoma, including 12 cases of papillary adenocarcinoma and 1 case of micropapillary adenocarcinoma. Moreover, CT images of pure GGOs also still show acinar, papillary, and lepidic components in the tumor tissues. In these cases, exfoliated cells that shed from the alveoli may spread along the airway and disseminate; therefore, as seen in this case, it is possible to obtain a cytological diagnosis of peripheral GGO through bronchoscopic brushing, but results from a large case series are required for confirmation.Herein, we present an unusual case of a subpleural GGO with a cytological diagnosis of adenocarcinoma obtained by bronchoscopic brushing. Generally, GGO observed on a chest CT is considered a lepidic component suggestive of adenocarcinoma in situ (AIS), minimally invasive adenocarcinoma (MIA), or lepidic adenocarcinoma. These findings are correlated with a favorable prognosis after surgical resection. However, the surgeons may face a dilemma when postoperative pathology suggests non-lepidic growth patterns of adenocarcinoma, such as acinar and papillary patterns, wherein sublobar resection may not be the best treatment of choice. Therefore, it is of interest to evaluate tumor characteristics through various diagnostic modalities preoperation. In this case, a cytological diagnosis of adenocarcinoma was obtained by bronchoscopic brushing, indicating that exfoliated cancer cells had disseminated through the airway. These findings are suggestive of an invasive adenocarcinoma. Therefore, sublobar resection was not enough, and lobectomy with mediastinal lymph node dissection was required. This is one of the interesting parts of using the preoperative bronchoscope brush in this case. However, whether or not routine bronchoscopy with brushing and selection of certain patients with peripheral GGOs are required should be unraveled in further research.Many surgeons choose sublobar resection (wedge resection or segmentectomy) for curative treatment of patients with GGNs together with a favorable prognosis.,13,14 One factor that accounts for the lower yield of conventional bronchoscopy brush is that the selection of bronchial routes to the lesion based solely on CT scans is likely to be inaccurate beyond the third- or fourth-generation bronchus and that bronchoscopes typically will not be able to reach the peripheral lung lesions. We previously reported through a retrospective cohort study that the sensitivity of conventional bronchoscopic brushing was 17% for malignant PPLs. For lesion sized <3 cm, the sensitivity of brushing as a diagnostic modality was as low as 11.1%. Currently, increase in the diagnostic sensitivity is possible when bronchoscopy is combined with navigation and imaging techniques such as radial-probe endobronchial ultrasound (EBUS) or navigational bronchoscopy.,8,15 However, these advanced bronchoscopy techniques are expensive and not feasible for implementation in every institution.,17Conventional bronchoscopy with brush cytology is a simple and specific diagnostic modality, but it has a low diagnostic yield regarding the detection of peripheral lesions. Using this mapping method, the generated two-dimensional map provides information regarding the actual relative position bronchoscope and allows the bronchoscopist to recognize the route easily, thereby increasing the chance of reaching the peripheral lesions. With the use of the mapping method, the sensitivity for detecting malignant PPLs using brushing can increase from 17.0% to 31.5%. In addition, the mapping method is especially suitable for cases with larger, solid-appearing lesions, cut-off signs at the targeted bronchus, lesions nearer to the lobar bronchial opening, and clear accessibility. However, the nodule of the patient did not have these characteristics. Given the anatomy and pathologic features of the present case, the positive preoperative cytology finding may be explained by the fact that exfoliated malignant cells were probably detected using brushing of the bronchi/bronchioles, which, otherwise, cannot be detected. Therefore, a hand-drawn “bronchial map” based on CT images benefited the pulmonologist, who could then perform precise brushing.This representative case was cytologically diagnosed using bronchoscopic brushing guided by manual mapping. As described previously, the principle of the manual mapping method is the translation of thin-section CT information into an endoscopic route.4This case provides evidence for the presence of peripheral GGOs diagnosed using brush cytology. Although further studies are required to generalize our findings, the manual mapping method used in this case may help improve the sensitivity and diagnostic yield when used in combination with bronchoscopy brushing in patients with findings suggestive of peripheral lung cancer. This modality can be used in most institutions that cannot offer advanced bronchoscopy techniques.Conceptualization: Lei Zhang, Guiqi Wang.Data curation: Lei Zhang.Funding acquisition: Lei Zhang.Supervision: Guiqi Wang.Writing – original draft: Lei Zhang.Writing – review & editing: Guiqi Wang.


Intravascular leiomyomatosis is a rare benign lesion with malignant potential. The cases are sporadic. Most patients have no clinical symptoms, and the preoperative diagnostic rate is low. Case 1 was misdiagnosed, passively managed during operation, recurred quickly, and underwent a secondary operation. We learned lessons from case 1 and treated the case 2 patient differently. The case 2 patient had a good prognosis. We hope the report will be helpful to other gynecologists.Case 1: a 49-year-old woman complained of dysmenorrhea. Traditional ultrasound showed adenomyosis and a solid mass 6 * 3 cm in the right appendix. After routine examination, the patient underwent transabdominal total hysterectomy + bilateral salpingectomy + IVL tumor resection, with both ovaries kept. No medication was used after operation. Routine ultrasound was performed every 3 months. The disease recurred, and the patient underwent a secondary surgery 9 months after the first time. So far, 25 months after the secondary surgery, there is no sign of recurrence. Case 2: a 41-year-old woman underwent a routine body examination, where a left adnexal mass 7 cm was found. The patient underwent contrast-enhanced ultrasonography and was diagnosed and prepared well preoperatively. The patient underwent transabdominal total hysterectomy + bilateral salpingectomy + IVL tumor resection. GnRH-a drugs were used after operation for 3 cycle. Now, there is no sign of recurrence after operation for 23 months.The incidence rate of IVL is low, and there are no typical clinical symptoms. It is easy to be ignored by gynecologists. Contrast-enhanced ultrasound is helpful to diagnose preoperatively and reduce misdiagnosis. Good preparation, full exploration of the pelvic and abdominal vessels, removal of lesions completely as much as possible, and anti-estrogen therapy after operation can reduce the recurrence of disease. Intravascular leiomyomatosis (IVL) is a special type of uterine myoma. The preoperative diagnosis rate is low because of no specific symptom, which leads to misdiagnosis. Inadequate pelvic exploration and missed lesions will increase the recurrence rate. This paper summarizes the experience and lessons from one misdiagnosed case; the second case is fully prepared and the treatment effect is good. We review the relevant literature, hoping to provide help to other clinical colleagues.Medical history of the two cases displayed in IVL mainly consists of benign smooth muscle tissue spreading and growing along the venous lumen. It is a rare special type of uterine myoma in gynecology. The onset age was 40–60 years, and 60% of the patients had a history of uterine myoma. The two patients we met had no previous history of hysteromyoma, but multiple hysteromyoma or adenomyosis was found in both cases during our operations. IVL invades not only the pelvic vein, inferior vena cava, renal vein, and pulmonary artery but also distant metastasis to the heart, lung, brain, and lymph nodes –3. The cIVL has no obvious clinical symptoms and signs, and the preoperative diagnosis rate is very low. It is often misdiagnosed as uterine myoma, adenomyosis, pelvic malignancy, and so on. Our first case was misdiagnosed as uterine subserosal myoma or ovarian solid mass before operation. We were passive in finding the IVL lesion during operation, almost missing it. We spent nearly an hour to search the mass and only gave the patient routine blood preparation before operation. IVL is an estrogen-dependent benign disease and is recurrent. According to the intraoperative findings and quick frozen pathology, for older patients, bilateral oophorectomy can be considered to reduce recurrence. In our case 1, because the possibility of IVL was not fully considered before operation, we felt passive during the operation and almost missed the focus. The families were not fully prepared for oophorectomy during operation. Bilateral ovaries were preserved, and there was no medication after operation; the disease recurred half a year after operation. 9 months after the first operation, the patient took a secondary operation and experienced physical, psychological, and economic trauma again. During the second operation, because of pelvic tissue inflammatory adhesion, the operation was very difficult, and the risk of injury of adjacent organs such as the ureter is also greatly increased.The advantages and disadvantages of common inspection methods are shown in Flammulina velutipes like, and bead like, which can twitch. The section is gray, pink white, and soft or hard. Microscopically, the tumor cells were spindle shaped, braided, or vortex shaped, and the surface was covered with a single layer of flat vascular endothelial cells. There was no or occasional mitotic image. Previous reports suggested specific immunohistochemical markers, including tumor cells vimentin (+), desmin (+), and SMA (+), and endothelial cells of venous vessels CD34 (+), especially ER and PR (+).In general, the uterus of IVL can be enlarged, most of which are complicated with uterine myoma or adenomyosis. Tumors can be seen in the uterine wall, parauterine ligament, and adnexal vein, most of which are cord like, IVL should be differentiated from uterine fibroids and ovarian cysts, as well as the following diseases under a pathological microscope (IVL is mainly treated by surgery. Because the tumor is diffuse and the recurrence rate is high, complete resection of the tumor is the key of surgery. Li et al.  sorted aKokawa et al. , 9 foundFor IVL patients, preserving of the uterus and ovary, unclean lesion resection, application of estrogen drugs, lesion infiltration into parauterine vessels, or distant invasion can increase the recurrence rate. The treatment of IVL should focus on surgery and remove the lesions as much as possible. The preferred surgical method is total hysterectomy with bilateral salpingo-oophorectomy + extrauterine lesion resection. For young patients, to preserve reproductive function, the IVL tumors should be removed completely as much as possible, and GnRH-a drugs should be supplemented for follow-up treatment to reduce the risk of recurrence.Flammulina velutipes-like changes during operation, the disease should be considered, the blood vessels in the operation field should be fully explored, and the lesions should be completely and fully removed as much as possible. GnRH-a drugs or other anti-estrogen drugs can be used after operation to reduce the risk of recurrence. Regular reexamination and close follow-up are needed after operation.In conclusion, although IVL is sporadic, there are still many cases reported in recent years. Because of its sporadicity, it is difficult to arouse the vigilance of gynecologists, and routine gynecological preoperative ultrasound is difficult to make a clear diagnosis from, which often leads to inadequate preoperative preparation, wrong selection of operation methods, passive treatment during operation, and lesions being easily missed. Our cases suggest that clinical gynecologists should be vigilant and not only rely on routine ultrasound to assist in diagnosis and treatment. If the veins in broad ligament, parauterine tissue, or adnexal area are found to be cord-like or XL is the first author. Y-SJ is the corresponding author. XL drafted the manuscript. N-YM collected the patients’ clinical data. YZ contributed to analyzing and interpreting the imagine findings. Y-SJ gave the patients’ operation and reviewed the literature. All authors contributed to the article and approved the submitted version.The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.All claims expressed in this article are solely those of the authors and do not necessarily represent those of their affiliated organizations, or those of the publisher, the editors and the reviewers. Any product that may be evaluated in this article, or claim that may be made by its manufacturer, is not guaranteed or endorsed by the publisher.


Endomyometriosis is a rare finding and it can be challenging to diagnose and to treat. It can arise in the uterus, in the ovary, in the broad ligament, in the peritoneal surface and in other pelvic structures. Usually patients with endomyometriosis are asymptomatic, but symptoms could occur due to large dimensions or site of the mass. We present a case of a 49-year-old woman with a symptomatic pelvic mass in the rectal wall, with no history of endometriosis, who underwent laparoscopic myomectomy 8 years earlier. Endomyometriosis is a benign and relatively rare disease first reported by Cozzutto et al. in the ovary in 1981  to definHere we report a case of a 49-year-old woman with a symptomatic pelvic mass in the rectal wall, with no history of endometriosis, who underwent laparoscopic myomectomy 8 years earlier.A 49-year-old nulliparous patient with history of hypertension and chronic otitis media came to our attention complaining 3 months of pelvic pain. She had undergone a laparoscopic myomectomy 8 years earlier. Routine laboratory tests were normal and a transvaginal ultrasound revealed a normoverted uterus, with normal size, typical signs of adenomyosis and showed a large retro-uterine mass measuring 59 × 31 × 42 mm with an increased vascularity. A contrast-enhanced computed tomography (CE-CT) scan of abdomen and pelvis showed, in the pre-sacral space, the presence of a multi-lobed mass (6 cm × 4.5 cm in diameters) characterized by both solid and cystic components with homogeneous enhancement in close proximity to rectal wall . A contrAn endoanal ultrasound suspected a tight connection of the mass to the rectum, therefore a colonoscopy was performed to investigate the possible intestinal origin of the tumor but it excluded any visible intraluminal lesions.An abdominal laparoscopic examination was then performed. It showed normal uterus and ovaries and the presence of a trilobed mass adherent to the anterior wall of the rectum and two 2 cm nodules in the omentum and in the abdominal wall, the latter in the site of the previous left port.In addition, pigmented endometriotic nodules were found in the pouch of Douglas and in the lateral abdominal wall.Since it was not possible to exclude the malignant nature of the rectal trilobed lesion, a laparoscopic low anterior resection of the rectum (LAR) with an end-to-end colorectal anastomosis without lateral ileostomy was performed. A partial omentectomy to remove the abdominal masses was also performed.Histological examination showed that the trilobate mass was composed of a thick muscular layer surrounding a discrete glandular epithelium. No sign of mitotic activity was seen in the myometrium or the glandular or stromal component of the mucosa. Immunohistochemistry demonstrated a diffuse nuclear progesterone and estrogen receptor expression in the glands, stroma, and muscle bundles, a diffuse nuclear expression of PAX8 in the glandular component of the mucosa and a diffuse expression of CD10 in the stromal component. The muscular component was diffusely positive for smooth muscle actin .The two 2 cm omental and abdominal wall nodules were both leiomyomas with no evidence of glandular component or mitotic activity.Post-operative days were uneventful and the drain was removed on third post-operative day and the patient was discharged on 6th day after surgery.A digital rectal examination and a CT scan of abdomen and pelvis, performed 6 months from surgery, have excluded a local recurrence and so far, almost 1 year post surgery, the patient doesn't refer symptoms.We described a very unusual lesion. According to the literature, few cases of endomyometriosis have been reported so far . In addiThe mechanism underlining the presence of endometrial and stromal tissues together with smooth muscle is still not clear. It has been proposed that sub-mesothelial multipotential stem cells can differentiate in smooth muscle cells, endometrial glands and stroma. The subperitoneal mesothelium is often defined “the secondary mullerian system.” During embryogenesis, the distal segments of the two müllerian ducts, which represent the “primary müllerian system,” fuse to form uterus, cervix, and proximal third of the vagina. The proximal müllerian ducts remain separated to become the fallopian tubes. Remnants of the most proximal müllerian ducts, that do not participate in organogenesis, constitute the secondary müllerian system. This system maintains its ability to differentiate into specialized epithelia or stroma into adult life . The oriEndometriosis is defined as the presence of endometrial tissue outside the endometrium. It usually consists of endometrial glands and stroma, however, smooth muscle metaplasia has been observed in various endometriotic foci. It has been showed, recently, that peritoneal endometriosis develops smooth muscle proliferation with expression, in addition to common markers of smooth muscle differentiation, of oxytocin and vasopressin receptor like in myometrial cells .Extensive amounts of smooth muscle in endometriosis have been classified as endomyometriosis . CozzuttIn our case the patient had a lobular peri-rectal mass with cystic aspect, a well-organized endometrium and a smooth muscular layer with irregularly arranged muscle fibers histologically identical to myometrium. In addition, two omental leiomyomas were classified as leiomyomatosis peritonealis disseminated and two pigmented endometrioid lesions were seen in the pelvis. We could speculate that all those processes are correlated to mullerian epithelium metaplasia, however, our patient had a medical history of laparoscopic myomectomy in 2012.Al-Talib and Tulandi  proposedOn a clinical point of view, rarely these lesions are asymptomatic incidental findings and most frequent symptoms are represented by dyspareunia, dysuria and menstrual constipation. These symptoms are often clinically unrecognized and patients are often treated for pelvic inflammatory disease .Endomyometriosis is a very rare disease and should be taken in account in patients with a previous history of myomectomy, and particular care is necessary during laparoscopic extractions of myomas.The original contributions presented in the study are included in the article/supplementary material, further inquiries can be directed to the corresponding author.Ethical review and approval was not required for the study on human participants in accordance with the local legislation and institutional requirements. The patients/participants provided their written informed consent to participate in this study.GL, PD, CG, EP, and MD drafted the manuscript. CC and LM edited the manuscript. All authors were involved in the clinical care of the patient and approved the final version of the manuscript at time of submission.The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.


Solid testis tumors in post‐pubertal males usually represent germ cell malignancies; however, other uncommon or rare histologies must be considered.We present a case of an 18‐year‐old male undergoing attempted bilateral partial orchiectomies for suspected germ cell tumors. Tumor pathology, laboratory results, radiographic studies, and post‐surgical elevated adrenocorticotropic hormone levels supported the diagnosis of testicular adrenal rest tumors secondary to previously undiagnosed nonclassical congenital adrenal hyperplasia.Testicular adrenal rest tumors are rare in patients with nonclassical congenital adrenal hyperplasia and may be accompanied by adrenal insufficiency and hypogonadism, which can be treated with glucocorticoid therapy and testosterone replacement. Differential diagnosis of tumors is challenging but necessary for proper symptom‐based management. ACTHadrenocorticotropic hormoneAFPalpha‐fetoproteinCAHcongenital adrenal hyperplasia CBCcomplete blood countCTcomputed tomographyFSHfollicle‐stimulating hormoneH&Ehematoxylin and eosinLDHlactate dehydrogenaseLHluteinizing hormoneMRImagnetic resonance imagingNCAHnonclassical congenital adrenal hyperplasiaOARTovarian adrenal rest tumorTARTtesticular adrenal rest tumorReported here is a rare case of a male patient diagnosed and treated surgically for TARTs in the presence of previously undiagnosed NCAH. Elevated ACTH is the primary indicator and predictor for TARTs. Such tumors may be accompanied by adrenal insufficiency and hypogonadism, which can be treated with glucocorticoid therapy and testosterone replacement.CYP21A2 gene cause a deficiency in the 21‐hydroxylase enzyme.Mutations in the There are two main types of CAH, classical and nonclassical. Classical CAH is less common, occurring in one 1 of 16 000 live births worldwide.TARTs are a known sequela of CAH and are found in up to 94% of male classical CAH cases.vs TARTs was suggested.An 18‐year‐old man was referred for painful bilateral testicular tumors confirmed with scrotal ultrasonography. MRI revealed a 2.5 cm tumor and a 3.0 cm tumor in the rete testis regions of the right and left testicles, respectively Fig. . CBC, LHCT scans of the abdomen and pelvis revealed a soft tissue density measuring 2.1 × 1.7 cm in the left para‐aortic region Fig. . Also noA postoperative endocrine evaluation revealed markedly elevated ACTH levels (4322 pg/mL) yet normal cortisol levels. The patient’s clinical and radiographic findings, testis pathology, and elevated ACTH level, all supported a diagnosis of NCAH and the presence of TARTs. The postoperative endocrine evaluation also revealed an LH level of 21.5 mIU/mL, FSH of 22.9 mIU/mL, and a low testosterone level (81 ng/dL). Borderline adrenal insufficiency and moderate hypogonadism were effectively managed with hydrocortisone and testosterone supplementation. Larger than average doses of hydrocortisone were necessary to maintain the ACTH at a desired level (under 100 pg/mL).Follow‐up scrotal U/S and CT scans 2 years post‐surgery revealed a right testicular prosthesis, a partial left testicle containing no new masses, and a stable para‐aortic soft tissue mass Fig. .TARTs are far less prevalent in patients with NCAH compared to CAH. Although our patient was not tested for 21‐hydroxylase deficiency for confirmation, clinical findings supported this diagnosis. In addition, we suspected adrenal rest tissue in the left para‐aortic region, although an image‐guided biopsy was inconclusive. Females with CAH and NCAH have rarely experienced OARTs.The cells with potential to become TARTs originate during early gestation, when cells with the intended role of producing steroids in the adrenal cortex and gonads differentiate from the surrounding coelomic epithelium. Some cells that migrate to the gonads retain ACTH responsiveness, having the potential to become TARTs.Differentiating TARTs from Leydig cell tumors is necessary as prognosis and treatment are vastly different. Whereas TARTs are benign, Leydig cell tumors can have more serious implications, as 10% are malignant and uniformly fatal in the setting of metastatic progression.CYP21A2 mutations. Our patient’s family history was negative. However, when early diagnosis is possible, monitoring ACTH levels and optimizing glucocorticoid therapy may be the only way to effectively prevent TART development or size progression to minimize any negative effects on the reproductive system.Table Adrenal rest tumors are rare in patients with NCAH but can occur throughout the pelvic and abdominal cavities. TARTs typically do not present until late adolescence or adulthood and can be the first sign of NCAH. Differential diagnosis is challenging but necessary to direct management and surveillance. Elevated ACTH is a predictor of TARTs. Family history is also relevant. Prediction or early detection of TARTs could allow for treatment prior to infertility. TARTs can respond to glucocorticoid therapy used to manage adrenal insufficiency, and testosterone replacement has been successful for patients with hypogonadism.The authors declare no conflict of interest.


Background: Pediatric tumors can present with vascular extension to the inferior vena cava and right atrium, which impacts the surgical strategy and can be challenging during surgical treatment. Wilms tumor (WT) is the most common retroperitoneal tumor that can present with vascular extension, but also adrenal tumors, clear cell tumors from the kidney, and hepatoblastomas can present with this situation. Surgical aims include obtaining complete tumor resection without risk for patients, to avoid severe bleeding, cardiac arrest, and embolization, and to avoid cardiac bypass if possible.Objective: To describe and discuss the surgical strategies to deal with pediatric tumors with vascular extension and propose a protocol.Method: Retrospectivly review the experience of treating patients with vascular extension in a single institution, describing different scenarios and a decision making fluxogram based on the preoperative evaluation regarding the surgical techniques and the need for cardiac bypass that are adequate for each situation. Image studies are important to guide the surgical strategy. Depending on the quality of image available, computerized tomography (CT) or magnetic resonance imaging (MRI) can be enough to give the information needed for surgical decisions. Ultrasonography (US) with Doppler is helpful to confirm diagnosis and describes factors to guide the adequate surgical strategy, like the upper level extension and presence or absence of blood flow around the thrombus. Neoadjuvant chemotherapy is indicated in most cases, in order to reduce the upper level of extension (and avoid the need for cardiac bypass) and to lower the risk of embolization. The approach is based on the upper level of the thrombus and can include cavotomy or cavectomy, sometimes with cardiac bypass and cardiac arrest with hypothermia, when the thrombus reaches the diaphragmatic level or above. Pathology analysis of the thrombus can guide staging and the need for radiotherapy postoperatively.Results: A decision making fluxogram protocol is presented focusing on the surgical treatment of such condition.Conclusion: Surgery strategy is highly impacted by the presence of vascular extension in pediatric tumors. Surgeons should be aware of potential complications and how to prevent them. Such cases should be treated in reference centers. Vascular extension to the inferior vena cava and right atrium can be challenging for surgical treatment of pediatric tumors. Wilms tumor (WT) is the most common retroperitoneal tumor with vascular extension , but othThe management requires a detailed preoperative evaluation. Neoadjuvant chemotherapy is indicated in most cases, in order to reduce the upper level of extension, to lower the risk of embolization, and mainly to avoid cardiac bypass (CBP) .In cases of WT, despite the differences on the initial approach between the cooperative groups Societé Internationale d'Oncologie Pediatrique (SIOP) in Europe and Children's Oncology Group (COG) in North America, both agree with the use of preoperative chemotherapy in cases with vascular extension, in an attempt to reduce the level of the extension, minimizing the surgical risks and the need for cardiac bypass (CBP) –9. The nTherefore, there are some factors that should be mentioned along with the preoperative evaluation :Tumor histology and stage at presentationResponse to chemotherapyConfirmation of the vascular extension and its levelPresence of blood flow around the thrombusRisk of tumor embolizationThe extension of the thrombus can be classified in 3 levels, according to the hepatic veins:Level 1: infrahepatic thrombusLevel 2: retrohepatic thrombus (subdivided in infradiaphagmatic and diaphragmatic)Level 3: supra hepatic or atrial thrombusImage studies are of the essence to guide the surgical strategy. Depending on the quality of image available, either computerized tomography (CT) or magnetic resonance imaging (MRI) can be enough, but if there is doubt, ultrasonography (US) with Doppler should be performed to confirm the factors previously mentioned, like the upper level extension and blood flow , 6. TranAlthough there is controversy in the literature, preoperative anticoagulation should be indicated only when there is high risk of embolization, symptoms, and clinical impairment.We aim to describe lessons learned from dealing with such cases and suggest a protocol to manage it.In our institution, in the past 24 years, we have registered 40 patients whose tumors presented with vascular extension and were evaluated for surgery. The diagnoses included WT, hepatoblastoma, adrenocortical carcinoma (ACT), and primitive neuroectodermal tumor (PNET) of the kidney.Vascular extension was identified mainly after CT, but also US with Doppler was used in some cases to confirm the upper level and blood flow status. In one case, anticoagulation was performed, in order to prevent embolization, due to a pedicled tumor through the tricuspid valve and clinical symptoms. Anticoagulation was not indicated in other patients of the series.The surgical outcomes will be presented below.The surgical approach is based on the upper level of the thrombus and the presence or absence of blood flow around it. Usually, a midline laparotomy incision is recommended, which can be extended to the chest, if it is necessary, but associated sternotomy and transverse laparotomy provide good exposure as well. Since some patients may need CPB, it would be advisable to minimize muscular section, due to the need of anticoagulation. Most techniques are derived from the liver transplant surgical approach, which allows a good exposure and safe vascular control along the procedure .Based on preoperative information about thrombus level and blood flow around it, the following protocol was designed  Figure .For infradiaphragmatic thrombus, either infra or retro hepatic below the diaphragmatic transition, there is no need for CBP, heparinization, or cardiac arrest. IVC vascular control below the liver and distally, along with renal vein vascular is enough to achieve complete tumor resection, either by cavotomy or cavectomy. In this scenario, blood loss is minimal and the ischemic time to the kidney with this technique is uneventful .If there is blood flow around the thrombus, cavotomy is performed and the thrombus can be easily removed since there is no invasion to the vessel wall.In the case of blood flow absence around the thrombus, it is firmly adherent and invades the vessel wall, thus cavectomy is indicated and no anticoagulation is needed.Contralateral renal vein ligation is justified and safe due to the development of collaterals venous of the azygo's system while the tumor grows, which is responsible to keep adequate venous return to the contralateral kidney or adrenal , 4, 5. FFor tumors with thrombus in the diaphragmatic transition, it is advisable to have a cardiac surgery team backup, for the decision of CBP may be intraoperative. If CBP is not needed, the already described approach for infradiaphragmatic thrombus should be performed. In our initial cases with this scenario, we indicated resection with CBP, but we learned that this can be avoided in many cases.If the vascular control at the upper level is not secure, a small sternotomy should be made in order to achieve IVC control and a “half” CBP without cardiac arrest and without hypothermia is indicated. In this case, the patient receives heparinization (2 mg/Kg), blood is recovered from the surgical field, is filtered and warmed, and cannulation of the aorta or iliac artery is made, in order to provide rapid fluid replacement without CBP.Either cavotomy or cavectomy can be performed, depending on the presence or absence of blood flow around the thrombus, as described above, with or without CBP.Finally, for tumors with intracardiac extension, CPB is always needed, associated with cardiac arrest and hypothermia , 12. ThiThere were 40 patients identified with vascular extension at the initial investigation, six of them were excluded due to lack of data. From 34 patients included, 21 were WT, one hepatoblastoma, 11 adrenocortical carcinomas (ACT), and one PNET of the kidney.Only two patients with ACT were submitted to the surgical approach due to clinical conditions. In both of them, there was a thrombus reaching the atrium and they were submitted to cavotomy with CPB. One of them died due to bypass system obstruction with thrombus. The other one had neurological postoperative complications related to CBP and died 4 years after the procedure of progression of disease .Renal and hepatic tumors were first treated with preoperative chemotherapy. There was complete thrombus regression in six patients, five of them with WT and one of them with renal PNET, with no need for the vascular approach.Partial regression of the thrombus occurred in 17 patients, who needed vascular management. Among them, we performed eight cavotomies and five cavectomies. Four patients still presented atrial thrombus after chemotherapy and needed CPB.We achieved complete remission in 13 patients. Five patients lost follow up after ending adjuvant chemotherapy and radiotherapy, five patients had died due to complications not related to surgery, and one patient is still under treatment.Surgical management changes in the presence of vascular extension and the present paper proposes a fluxogram to guide the surgical treatment, that can be used both for retroperitoneal and intraperitoneal pediatric tumors with vascular extension . Some deIn renal cell carcinoma, vascular extension occurs in 10% of patients  and it iSome authors advise radiotherapy to treat the thrombus instead of surgical resection , but we In ACT, the IVC extension occurs in 20% of the cases. It is more frequent at the right side  and is rIn hepatoblastomas, vascular involvement adds risk to the stratification based on the European based International Childhood Liver Tumors Strategy Group (SIOPEL) Pre Treatment Extent of disease (PRETEXT), as shown in Pediatric Hepatic International Tumor Trial (PHITT). Vascular extension is one of the Annotation Factors and they are considered positive when there is: tumor involvement of all 3 hepatic veins or retrohepatic vena cava and/or tumor thrombus in any 1 or more of the main hepatic veins; tumor involvement of the portal bifurcation, both right and left portal veins; and/or tumor thrombus in either the left or right portal, and others related to the involvement to other organs . The vasPediatric tumors with vascular extension demand careful multidisciplinary discussions and preoperative preparation. They should be referred to high complexity surgical centers.In conclusion, surgery strategy is highly impacted by the presence of vascular extension in pediatric tumors. Despite the protocols, the best approach is determined case by case, considering the preoperative evaluation. The risks are justified because the tumor resection is of essence for treatment. Surgeons should be aware of potential complications and how to prevent them. Such cases should be treated in reference centers, always remembering the importance of a multimodal assistance, including the pediatric oncologist, pediatric radiologists, pathologists, and pediatric and cardiac surgeons.The original contributions presented in the study are included in the article/supplementary material, further inquiries can be directed to the corresponding author.The studies involving human participants were reviewed and approved by Comitê de ética e Pesquisa UNIFESP - n° 1015/2019. Written informed consent was not required for this study, in accordance with the local legislation and institutional requirements.All authors listed have made a substantial, direct, and intellectual contribution to the work and approved it for publication.The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.All claims expressed in this article are solely those of the authors and do not necessarily represent those of their affiliated organizations, or those of the publisher, the editors and the reviewers. Any product that may be evaluated in this article, or claim that may be made by its manufacturer, is not guaranteed or endorsed by the publisher.


Sertoliform cystadenoma is a very rare, benign lesion of the rete‐testis difficult to distinguish from other malignancies of the testicle.We present the case of a 42‐year‐old male who presented with a right testicular mass, asymptomatic for 1 year. Clinical examination revealed a palpable, painless, and well‐delimited right testicular superior pole nodule. Testicular ultrasound confirmed the nodule, whereas serum tumoral markers were normal. The patient underwent inguinal partial orchiectomy. Intraoperative excisional biopsy and frozen section pathology were performed, reporting undetermined tumoral origin with negative surgical margins. Ischemia time was 12 minutes. The final pathology report showed a Sertoliform cystadenoma of rete testis, with immunomorphology positive for AE1, CK7, and negative surgical margins.To our knowledge, this is the first report of testicular sparing surgery for Sertoliform cystadenoma, a very rare benign tumor of rete testis. All previously reported cases were managed by radical inguinal orchidectomy. AE1Cytokeratin AE1CAM5.2Cytokeratin CAM5.2CK7Cytokeratin 7CTcomputed tomographyEMAepithelial membrane antigenPAX8Paired‐box gene 8To our knowledge, this is the first report of testis‐ sparing surgery for Sertoliform cystadenoma, a very rare benign tumor of the rete testis. All previously reported cases were managed by radical inguinal orchidectomy. Partial inguinal orchiectomy with frozen section pathology could be an alternative to radical surgery in selected cases.Testicular tumors account for 1% of male cancers and approximately 5% of urogenital malignancies, with an increasing incidence due to the widespread use of ultrasound examination.N = 0.89‐ 8.78), beta‐human chorionic gonadotropin , and lactate dehydrogenase . A staging chest–abdominal–pelvic contrast‐enhanced CT scan was performed which did not show any evidence of visceral or lymph node metastasis.A 42‐year‐old male with no prior medical history presented with a palpable mass in the right testicle, asymptomatic, and without any increase in size for 1 year. The patient is married, without children. At clinical examination, a firm, painless, well‐delimited superior pole right testicular lesion was objectified. Scrotal ultrasound revealed a 1.7/ 0.5 cm mass in the upper pole of the right testicle, with normal contralateral testicle and normal testicular volumes  with a predominant tubular architecture, columnar to cuboidal tumor cells with eosinophilic cytoplasm, and basally located nucleus. Moreover, adenocarcinoma of rete testis is also a rare but aggressive finding, with an incidence peak around 70 years of age. The most important morphologic criteria required for diagnosis are the involvement of testicular mediastinum with the preservation of the parietal tunica vaginalis, in the absence of other lesions.Although current recommendations are in favor of considering every scrotal mass as malignant until proven otherwise, we propose in selected cases where clinical history is in favor of benign lesion and patients desire maximum fertility preservation the alternative of organ sparing surgery. Testis‐preserving surgery should always be accompanied by frozen section examination, due to the fact that it has a high concordance with the final pathology result.In conclusion, we report the first case of Sertoliform cystadenoma managed by conservative surgery—partial orchidectomy, without any early or late postoperative complications. Sertoliform cystadenoma remains a rare finding among intratesticular mass findings, with only 16 cases reported in the literature, all treated by radical inguinal orchidectomy.No funding was received for this study.The authors disclose no conflicts of interest.This study was approved by the local ethics committee of the Croix du Sud hospital (register number 57/2021) and was conducted in line with the principles of the Declaration of Helsinki.Not applicable.Not applicable.


Case Presentation. This article reports primary ovarian choriocarcinoma, likely gestational in a 25-year-old para 2 woman presenting with lower abdominal pain and swelling of two-week duration. Diagnosis was suspected by serum beta-human chorionic gonadotropin and confirmed histologically after surgery. Postoperatively, she was managed with multiple courses of chemotherapy using a bleomycin, etoposide, and cisplatin regimen, and the treatment was effective.  Primary pure ovarian choriocarcinoma is a rare aggressive tumor which can be nongestational arising from germ cells or gestational origin. Preoperative diagnosis of extrauterine choriocarcinoma is challenging due to nonspecific clinical presentation.  In patients with adnexal mass presenting with nonspecific symptoms especially with high Doppler blood flow of the mass on ultrasound evaluation, serum beta-human chorionic gonadotropin determination is recommended before laparotomy. In setups where the genomic test is not available, histological and clinical effort to differentiate gestational versus nongestational choriocarcinoma is useful for specific management decision. Primary pure ovarian choriocarcinoma is a highly aggressive tumor which arises from ovarian ectopic pregnancy as gestational choriocarcinoma or as nongestational choriocarcinoma from germ cells . The estA 25-year-old para 2 woman with last normal menstrual period three weeks back presented with lower abdominal swelling and pain of two-week duration. She has associated loss of appetite but no cough, shortness of breath, or vaginal bleeding. Her last delivery was 2 years back. She was not taking any form of contraception.On physical examination, she was acutely sick looking in pain with blood pressure of 130/80 mmHg, pulse rate of 108 beats per minute, respiratory rate of 22 breaths per minute, and temperature of 37.1 degree centigrade. Further examination revealed fourteen-week-sized mildly tender, firm, mobile abdominopelvic mass, bulged posterior vaginal fornix, and normal cervix.Ultrasound showed huge multiseptated adnexal mass with internal solid component, likely originating from the left ovary. The solid component and septum had blood flow on the Doppler study. There was also free peritoneal fluid. On chest the X-ray film, the costophrenic angle was blunted with pleural effusion.Her blood group was B-ve and had hematocrit of 29%, platelet of 768,000, negative urine human chorionic gonadotropin (hCG), and normal liver and renal function tests. Some available tumor markers were also determined: CA-125 (189.6 U/mL) and LDH (1044 U/mL).Exploratory laparotomy was decided with impression of the malignant ovarian tumor. Intraoperatively, about 200 mL bloody ascetic fluid was found in the peritoneal cavity together with 12 × 14 cm fragile, dark left ovarian mass attached to the uterine serosa and dome of the urinary bladder. There was also 10 × 6 cm cystic ipsilateral ovarian mass adjacent to the fragile tumor .Hemorrhagic fluid was sucked out, and the mass was excised with part of the dome of the bladder. Total abdominal hysterectomy and bilateral salpingo-oophorectomy were performed, and the bladder was repaired.All the tissues were submitted for histopathology and revealed solid proliferation of round to oval pleomorphic cells exhibiting an indistinct eosinophilic cytoplasm, coarse nuclear chromatin, prominent nucleoli, abundant multinucleate giant cells, extensive necrosis, and hemorrhage .A section from the cyst structure showed a fibrous convoluted cyst wall, cavity with calcification, and luteinized stroma. Sections from the uterus, cervix, and tubes showed normal appearance. No evidence of other germ cell element was noticed. The patient was stable postoperatively, and serum B-hCG was determined to be 1,000,000 mIU/mL.th month of follow-up being disease free and having no evidence of recurrence or metastasis.After three weeks of surgery, the patient received multiple courses of the bleomycin, etoposide, and cisplatin (BEP) regimen. The posttherapeutic period was smooth, and serum B-hCG becomes normal. Currently, the patient is at the 12Ovarian choriocarcinoma is a rare but highly aggressive tumor. This may arise from metastatic gestational choriocarcinoma from the uterus or tubes or as primary ovarian origin . Pure chAbdominal pain, detection of abdominopelvic mass, abnormal vaginal bleeding, or metastatic manifestations to the lung and brain could be the nonspecific presentation. Lesions which appear before menarche manifest with isosexual precocious puberty in about 50% of patients , 7. ThisConfirmed extrauterine gestational choriocarcinoma can be managed with methotrexate-based chemotherapy reserving excision of the local tumor and postoperative chemotherapy for a bulky or chemoresistant tumor. Confirmed nongestational choriocarcinoma preoperatively or intraoperatively in young patients, who desire their fertility, could be managed with comprehensive staging fertility preserving surgery. Lymphadenectomy was considered with suspected nodal metastasis , 9. In pPreoperative diagnosis of extrauterine choriocarcinoma is challenging due to nonspecific clinical presentation. In the present case, ovarian choriocarcinoma was diagnosed after surgery with histology and serum B-hCG. Primary ovarian choriocarcinoma is an extremely rare aggressive tumor. In patients with adnexal mass especially high Doppler blood flow, serum B-hCG determination is recommended before laparotomy. In setups where the genomic test is not available, histological and clinical effort to differentiate gestational versus nongestational choriocarcinoma is useful for specific management decision.


Cystic pancreatic lesions (CPLs) are frequently casual findings in radiological examinations performed for other reasons in patients with unrelated symptoms. As they require different management according to their histological nature, differential diagnosis is essential. Radiologist plays a key role in the diagnosis and management of these lesions as imaging is able to correctly characterize most of them and thus address to a correct management. The first step for a correct characterization is to look for a communication between the CPLs and the main pancreatic duct, and then, it is essential to evaluate the morphology of the lesions. Age, sex and a history of previous pancreatic pathologies are important information to be used in the differential diagnosis. As some CPLs with different pathologic backgrounds can show the same morphological findings, differential diagnosis can be difficult, and thus, the final diagnosis can require other techniques, such as endoscopic ultrasound, endoscopic ultrasound-fine needle aspiration and endoscopic ultrasound-through the needle biopsy, and multidisciplinary management is important for a correct management. CPLs are frequently detected lesions with different malignant potential, requiring different management.MRI with MRCP best allows detection of those imaging features helpful for lesion characterization.A CPL communicating with MPD is an IPMN.A CPL non-communicating with MPD is related to different pathologies .Differential diagnosis can be done using morphological appearance, location and demographic data.Cystic pancreatic lesions (CPLs) are quite common: Their frequency of detection ranges from 2.4 to 19.6%, and their prevalence as well as size and number increases with age (from 7.9 below 70 years to 40.2 over 70 years) . A preciHowever, difficulties in differential diagnosis of CPLs still exist because of the lack of specific clinical and laboratoristic signs and the overlap of imaging findings, and thus, the management of patients with CPLs remains complex . MoreoveMost CPLs can be considered “technopathies,” as they are more frequently detected in the last decades due to the widespread use and advancement in diagnostic imaging. The vast majority of these lesions will never threat the life of affected subjects, but due to their malignant potential will cause affected subjects to become patients, and followed up even for many years. Follow-up strategies rise a really challenging issue for radiologists, due to the high number of patients to be submitted. The role of Imaging is to differentiate benign from malignant or potentially malignant CPLs avoiding unnecessary surgery and, in potentially malignant CPLs, to early detect morphological changes related to malignant transformations in order to offer more chance of survival to these patients.Scope of this review is to offer a practical approach to the diagnosis of CPLs using mainly MR imaging findings, location and demographic data and thus drive their correct management. Differential diagnosis among different CPLs is also emphasized.According to data extrapolated from WHO classification, CPLs are classified in epithelial and non-epithelial and each of these categories is further subdivided in non-neoplastic and neoplastic (CPNs) Table  9]TableTable9]TaEpithelial CPNs are further divided in mucinous, always premalignant–malignant lesions and non-mucinous neoplasms, which include both benign (most frequent) and borderline and malignant lesions.In particular, mucinous CPNs include mucinous cystic neoplasm (MCN) and intraductal papillary mucinous neoplasm (IPMN), while non-mucinous CPNs include serous cystic neoplasm (SCN), solid pseudopapillary neoplasm (SPN), cystic neuroendocrine neoplasm (CPNET), the rarer acinar cell cystic neoplasm (ACCN), ductal adenocarcinoma with cystic degeneration and other rarer lesions Table .Many others CPLs can be included in this review, but due to their very low incidence, their discussion will be omitted .From the radiological viewpoint, the key factor to characterize a CPL is to establish whether the lesion communicates or not with the main pancreatic duct (MPD) Table .Table 2RCPLs non-communicating with MPD include non-neoplastic  as well as neoplastic cysts , 12. NeoNowadays, CPLs can be detected with most imaging techniques , but for a correct characterization MDCT and MRI are needed. Endoscopic ultrasound—EUS—is another useful imaging technique to correctly characterize CPLs, but its use is mostly managed by gastroenterologists, and thus, its description is beyond the scope of this paper.MRI with MRCP, thanks to its high contrast resolution and high sensitivity to static fluids on T 2 w sequences, is the best imaging technique to assess communication with MPD (which is the key factor to characterize a CPL); on the other hand, MDCT is the best imaging technique to demonstrate the presence, the intra-lesional localization and the size of eventual calcifications, helpful findings in the differential diagnosis; moreover, in elderly and uncooperative patients MDCT with curved multiplanar reconstruction (MPR) post-processing is a valid technique to assess communication with MPD. Both MRI with MRCP and MDCT have high diagnostic performance in differentiating benign from malignant CPLs, with an accuracy ranging from 73 to 81% for MRI and 75% to 78% for MDCT, respectively –16.Pancreatic pseudocyst is a pancreatic and/or peripancreatic fluid collection with well-defined walls containing pancreatic juice or amylase-rich fluid and essentially no solid material. It is considered a delayed  complication of interstitial edematous pancreatitis , 17.At MRI, pseudocyst is markedly hyperintense on T2w and hypointense on T1w , or patients with acute pancreatitis may have cystic tumors, so a previous acute pancreatitis cannot completely exclude the presence of a true cystic tumor.WON is an encapsulated collection of pancreatic and/or peripancreatic necrosis surrounded by enhancing walls of reactive tissue that occurs ≥ 4 weeks after onset of necrotizing pancreatitis as result of the organization of an acute necrotic collection . WON conCongenital cyst or “true cyst” is an extremely rare cystic lesion lined by a single layer of cuboidal epithelium; it mostly occurs in children or in patients with polycystic disorders  and is usually mistaken or finally diagnosticated by exclusion .Retention cyst is a cystic dilatation of a pancreatic duct, usually caused by calculi, mucin, chronic pancreatitis or pancreatic cancer.Retention cyst has mucinous mucosal lining and has imaging features similar to those of small MCN or of BD-IPMN . As it cAs retention cyst has no characteristic imaging features, it must be considered an indeterminate cyst whose management is different according to different guidelines, based on only the size  or balanThe most common cystic pancreatic neoplasms non-communicating with MPD are SCN, MCN, SPN, CPNET and ACCN, and these entities can be distinguished on the basis of their morphological features, location and demographic data.SCN is a benign lesions, and only very few cases of malignant degeneration have been published . It accoIt is composed by multiple cysts formed by glycogen-rich epithelial cells and mainly involves the head of the pancreas, although it can be located in any part of the gland .It primarily affects females in their 50 s , but it On 15–30% of cases, SCN is associated with von Hippel–Lindau disease (VHL) and in these cases tends to be multifocal and may involve diffusely the pancreatic gland  SCN is mostly (nearly 60%) asymptomatic and only when large can cause signs and symptoms of obstructive chronic pancreatitis such as unspecific abdominal pain, diabetes mellitus and pancreaticobiliary symptoms .At imaging, SCN may manifest with three main patterns, reflecting its morphological appearance: microcystic (or polycystic), honeycomb and macrocystic (or oligocystic) .On a multinational review on 2622 patients with SCA .Fig.Fig30].FSome authors observed that central scar had sensitivity (Se), specificity (Sp) and positive predictive value (PPV) of 32.4%, 100% and 100%, respectively, for diagnosis of SCA, while combination of microcystic appearance and lobulated margins had Se, Sp and PPV, respectively, of 68%, 100% and 100% , suggestSome other authors observed that on MDCT, also the presence of a central calcification and the “circumvascular sign”  is pathognomonic for SCN (100% Sp), and however, central calcification has a very low sensitivity (less than 30%), while circumvascular sign has a Se of 76.7% .The macrocystic pattern Fig.  is formeRarely also a pseudosolid variant of SCN has been described. Microscopically, the serous solid adenoma shows architectural and cytological characteristics similar to serous microcystic cystadenoma, where the small size of cystic spaces, made by round or ovoid structure formed of cuboid cells derived from the ductular epithelium, is related to the fact that the solid adenoma has no secretory activity, giving a more compact structure compared to cystic forms. Stromal components of these lesions are characterized by avid contrast enhancement, thus misleading to an incorrect diagnosis of neuroendocrine tumor at MDCT Fig. a; howeveOn a multinational review on 2622 patients with SCN ..37].Fig.Two main patterns of MCN are described: macrocystic multilocular and macrocystic unilocular . MacrocyRadiological signs statistically associated with malignancy are: papillary vegetations, nodules, septa and wall thickness > 3 mm, size > 7 cm, number of loculations > 4, hyperintensity of cystic content on T1w images, compression and/or infiltration of adjacent vessels or organs and metastases , 43. TheIn fit patients, resection is recommended also in MCN without radiological features of malignant degeneration, due to its high malignant potential. However, in elderly patients with comorbidities follow-up may be an option if lesion is smaller than 4 cm and if mural nodules are absent .Surveillance is recommended with MRI, EUS or a combination of both every 6 months for the first year and then annually if no changes are observed .SPN is a rare pancreatic cystic tumor  with a low malignant potential (more than 80% SPN are benign) and favorable prognosis .This tumor is solid and appears as a round, well-encapsulated mass with a variable amount of necrosis and hemorrhage, responsible of its frequent cystic appearance , and somThis lesion is usually discovered incidentally, as most patients with SPN are asymptomatic, but sometimes it may manifest with unspecific symptoms such as abdominal pain together with palpable mass , 47.At MRI, SPN appears as a well-defined round mass, with heterogeneous signal intensity on both T1 and T2w sequences due to necrosis and hemorrhage. Solid components are typically located in the periphery of the lesion, appear well vascularized and enhance later than pancreatic parenchyma Fig.  46]. Wh. Wh46]. When large cystic changes are present Fig. , SPN appACCN is a very uncommon benign cystic lesion characterized by serous content and prominent acinar cell differentiation of the epithelial lining, unrelated to the major ductal system .Its exact nature is still unclear: Initially described as a non-neoplastic entity such as developmental anomaly or post-obstructive glandular dilatation and referred as “acinar cystic transformation” (ACT), subsequent studies considered it a neoplastic lesion, so further studies are necessary to clearly demonstrate dystrophic versus neoplastic origin .It can manifest as unilocular and, more frequently, multilocular cyst which typically lacks solid areas of papillary projections and which can either be confined to an anatomic region or diffusely involve the entire gland –52.It can occur in a wide range of ages with a striking female predominance and causes clinical symptoms in half cases, in particular abdominal pain.Preoperative diagnosis is usually based on a combination of clinical features and radiological findings, but is often difficult because of similarity to other cystic pancreatic lesions. In particular, radiological findings of unilocular ACCN overlap with those of MCN and macrocystic SCN, while the main differential diagnosis of multilocular ACCN is IPMN. Delavaud et al. demonstrated that four radiological findings are significantly associated with ACCN  Fig.  and thatAs there have been no reports of malignant transformation, it is crucial to correctly characterize ACCN since it requires conservative treatment  –52.When diagnosis of ACCN is suspected, patients should undergo surgical biopsy under laparoscopy with frozen section examination  and once the diagnosis is confirmed, patients must be followed up, because the exact nature of ACCN (dystrophic or neoplastic) is not clearly defined yet .Cystic pancreatic neuroendocrine tumor (CPNET) is a distinctive subgroup of pancreatic neuroendocrine tumors (PNETs) with unique clinical, radiological and pathological features –56.Its etiology is not clear yet. On gross pathology, it usually appears as well-demarcated lesions with centrally or eccentrically located single thin-walled cystic area containing clear fluid which does not communicate with pancreatic ducts and which is separated by a thin fibrous band from neoplastic cells.Different authors demonstrated that compared with solid PNET, CPNET is more likely non-functioning, solitary and more likely associated with MEN1 syndrome. It is less likely to demonstrate tumor necrosis, perineurial invasion, vascular invasion, regional lymph node metastases and synchronous distant metastases and present with a lower pathological stage at diagnosis, with low Ki-67 index and mitotic count –56. MoreIt occurs over a wide range of ages, more often in adults (mean age: 53 years) and in men, and it accounts for 3.6–36.1% of resected PNETs .At CT or MRI, CPNET appears as non-communicating cystic lesion surrounded by a rim of well-vascularized tissue that enhances in the arterial phase  Fig. 1212. The mBecause of its indolent behavior, more conservative surgical procedures are proposed such as tumor enucleation and spleen preserving distal pancreatectomy, and in some selected patients, particularly those with purely cystic tumor < 2 cm, only surveillance is suggested –60. HoweIPMN represents a large and heterogeneous group of epithelial mucin-producing tumors communicating or involving the main pancreatic duct.It is the most common CPN, accounting for about 70% of all pancreatic cystic neoplasms, may be multifocal and have a malignant potential following an adenoma–carcinoma sequence. IPMN can display the full spectrum of histologic changes, from hyperplasia, adenoma, borderline tumor to in situ or invasive carcinoma, with a different incidence between main duct IPMN (MD-IPMN) and branch duct IPMN (BD-IPMN) , 63. MorAccording to the cell lineage of the papillary component, four histological subtype of IPMN with different clinical pathological behavior can be distinguished: gastric, intestinal, oncocytic and pancreatobiliary , 64.The gastric subtype, which accounts for the vast majority of BD-IPMNs, is typically of low grade with only a small percentage developing into carcinoma; however, if a carcinoma does develop, it is usually of the tubular type and behaves like a conventional PDAC.A significant portion of MD-IPMN is of the intestinal type, and large and complex intestinal-type IPMNs can have invasive carcinoma, typically of the colloid type with a relatively indolent behavior.The oncocytic type tends to be large, has relatively uncommon and limited invasion, seems to have a very good prognosis and tends to recur in the remaining pancreas years after the initial resection.The pancreatobiliary type is the least common and is regarded as a high-grade version of the gastric type. Invasive carcinoma associated with pancreatobiliary-type IPMN is usually tubular and aggressive.According to its site, three morphological types of IPMN have been described: the main duct type (MD-IPMN), the branch duct type (BD-IPMN) and the mixed type which meets both the criteria for MD-IPMN and for BD-IPMN. Frequencies of malignancy are significantly different according to the morphological types and have been higher for MD-IPMN/mixed type (mean: 61.6%) and lower for BD-IPMN (mean: 25.5%) , 64.IPMN is diagnosed at a mean age of 60 years, and it affects males slightly more frequently than females , 63.IPMN is mostly detected in asymptomatic patients, but sometimes impaired outflow of pancreatic juice induced by hypersecretion of mucin may cause pain, may induce laboratory test abnormalities of pancreatitis and may cause acute pancreatitis itself, and thus, an IPMN may be discovered after an episode of acute pancreatitis. More severe symptoms, such as jaundice, severe abdominal pain, anorexia, weight loss and diabetes, are more likely associated with malignant behavior .At imaging, IPMN appears as a cystic dilatation of the involved ductal segment, caused by mucin secretion. MRI is the most sensitive technique to characterize IPMN, and in particular, MRCP is the most important sequence to demonstrate the involvement or the communication of MPD which are the key findings for the appropriate diagnosis of IPMN. Even endoscopic retrograde cholangiopancreatography (ERCP) may fail in demonstrating mucin-filled secondary duct dilatation .MD-IPMN appears as segmental or diffuse dilatation of the MPD (> 5 mm) without other causes of obstruction . In diffSegmental MD-IPMN may be difficult to diagnose because it appears as a non-specific segmental dilatation without an obstruction and without a previous pancreatitis (which can lead to a post-inflammatory stenosis). The affected segment is enlarged, sometimes with dilatation of collateral ducts because of pancreatic juice outflow impairment. In these cases, also upstream dilatation of MPD may be present, thus misleading to an incorrect diagnosis of diffuse MD-IPMN Fig. . In MD-IMixed-type IPMN appears as a segmental or diffuse dilatation of MPD and branch ducts along its course Fig.  68]. Ho. Ho68]. BD-IPMN appears as unifocal of multifocal cystic lesion communicating with MPD. Cysts may be uni- Fig.  or multiThe main differential diagnosis of unifocal BD-IPMN is pseudocysts, MCN and SCN, since sometimes communication with MPD cannot be correctly assessed , 68, whiIPMN has heterogeneous malignant potential, and thus, the International Association of Pancreatology (IAP), with Fukuoka consensus in 2012, proposed two-tier criteria to predict malignancy .First tier is represented by “worrisome features,” a group of imaging findings suggesting that the lesion is not malignant yet, but could evolve in malignant, and thus requires further work-up by EUS, to better risk-stratify the lesion, and strict follow-up Table .Table 3FSecond tier is represented by “high-risk stigmata,” a group of imaging findings suggesting the possibility that the lesion is malignant, thus requiring surgical resection if the patient is fit Table .2012 Fukuoka consensus guidelines had been widely accepted and had high sensitivity to predict invasive carcinoma and high grade dysplasia, but relative low specificity, leading to a high number of unnecessary pancreatic resections, so in 2017 revised Fukuoka consensus has been proposed  Table 33. HoweveTo date, management of IPMN is still controversial; to simplify, according to 2017 Fukuoka revised consensus, patients with IPMN with high-risk stigmata have to undergo resection if fit, patients with IPMN with worrisome features need further workup and patients with IPMN without worrisome features and without high-risk stigmata need follow-up at different intervals depending on the size of the largest cyst Table .Table 4FEuropean guidelines distinguish absolute and relative indications for surgery Table . PatientMany other guidelines for management of IPMN or CPNs including IPMN have been published , but nonIn our opinion, the IAP guidelines, in which treatment and follow-up strategies are decided on the basis of the presence of “high-risk stigmata” and “worrisome features,” and the European guidelines are the most comprehensive and practical for decision making; however, the final decision on treatment should be individualized and should depend not only on the risk of malignancy and on the presence of symptoms, but also on patient’s life expectancy, comorbidities, operative risk, cyst location and extent of surgery.In particular, it is important to remember that the only possible treatment is surgery, and even in high-volume centers, risk of mortality and morbidity for pancreatic surgery is high  ; moreoveAll patients “fit for surgery” with high-risk stigmata should undergo surgery , 71, butPatients fit for surgery without high-risk stigmata or worrisome features will undergo surveillance both for early detection of malignant progression of IPMN and for early detection of concomitant PDAC; surveillance for PDAC is needed also in patients who have undergone resection, even for noninvasive IPMN , 74.The interval and period of surveillance are still controversial: IAP guidelines recommend intervals stratified by the cyst size, which can be lengthened after 2 years of stability; other guidelines recommend discontinuation after 2 years (American College of Radiology 2010) or 5 years (American Gastroenterology Association 2015) of stability , becauseTada et al. , in a stThus, all patients with IPMN who are fit for treatment, even those who have been resected for a noninvasive IPMN, need indefinite surveillance which should be stopped only when the patient becomes unfit for surgery. The interval of follow-up is still a matter of discussion; however, it should be kept in mind that a long (more than 1 year) interval is dangerous, as after such a long follow-up a new appearing lesion could already be unresectable at diagnosis .While follow-up strategies for early detection of malignant degeneration of IPMN are well established on the basis of dimensional criteria, there are not screening criteria for early detection of concomitant PDAC yet. Different studies have demonstrated that IPMN is a major risk factor for PDAC  –82, but A short interval follow-up should be considered in these categories, in order to avoid that a PDAC arising in the interval between two studies becomes unresectable, in particularly in young patients, as the cumulative risk of developing PDAC can rise up to 38% at 10 years . As suggThis follow-up strategy of course rises the big issue of a large number of asymptomatic patients overloading radiological centers, due to the high prevalence of CPLs, especially in older patients (up to 40%) , 87. To Some authors have already established that a short MR protocol provides information equivalent to a more time-consuming and costly comprehensive protocol in the detection of significant changes of cystic lesions as well as in the detection of mural nodules . MoreoveConsidering the morbidity and mortality rate of pancreatic surgery , 73, it A combination of the clinical history, gender and imaging findings sometimes is not enough in order to obtain a correct diagnosis. In such situations, EUS, EUS-FNA, EUS-TTNB can be helpful diagnostic tools: cytology, cyst fluid analyses of carcinoembryonic antigen (CEA), amylase and molecular biomarkers as well as cyst wall biopsy, allow distinction of mucinous vs non-mucinous cystic neoplasms and permit identification of the specific histological types , 63.Analysis of cyst fluid obtained under EUS guidance permits the measurement of tumor markers and pancreatic enzymes, makes possible cytologic evaluation and allows to perform molecular DNA sequencing techniques which may help to distinguish serous versus mucinous cysts and to grade the epithelium of mucinous cysts .Although accuracy of morphological findings of EUS in the differential diagnosis of PCLs is extremely operator dependent, ranging from 40 to 96% , more inEUS is operator dependent and cyst fluid analysis very complex, so EUS with FNA and cytological and molecular analysis should be performed only in reference centers.Elevated CEA allows distinction between mucinous and non-mucinous cysts , and a cutoff value of ≥ 192–200 ng/ml is 79% accurate for the diagnosis of a mucinous cyst with moderate sensitivity (73%) and specificity (84%), while a cutoff value of < 5 ng/ml is the most valuable to exclude mucinous neoplasm and suspect SCN , 91.Elevated amylase levels in the cyst fluid are highly specific for pseudocyst, and a cutoff value of 250 U/L is associated with the diagnosis of pseudocyst with a sensitivity of 44% and a specificity of 98%, and thus, amylase level < 250 U/L virtually excludes the presence of a pseudocyst , 92.Cytological analysis of cyst’s fluid obtained under EUS guidance has good specificity (up to 83%) for diagnosing mucinous cysts, but very low sensitivity (< 50%) because of the small amount of cells dispersed in the fluid, and moreover is difficult to interpret , 93–95.Molecular analysis of the cyst fluid for diagnosis is still evolving; detection of KRAS gene mutation more accurately supports the diagnosis of a mucinous but not necessarily a malignant cyst 96% Sp) , 96–98, % Sp , 96Cytology brush is not indicated because of the high rate of complications  and of technical failure (27%) with conflicting results , 101–103Tissue acquisition is the ideal method for diagnosis and risk stratification in CPLs. Barresi et al. , in a muDistinguishing invasive from noninvasive IPMNs preoperatively remains challenging. Khoury et al. in a cohort of 478 pancreatectomies performed for IPMN in a single year in over 100 US institutions found that invasive carcinoma or high-grade dysplasia was identified only in 23% of resected lesions, so improved biomarkers are needed to aid in surgical selection .In the last years, different studies have demonstrated that quantitative imaging and radiomics applied to MDCT pancreatic protocols can provide markers for reliably discriminating benign from malignant IPMNs (low- and intermediate-grade dysplasia versus high-grade dysplasia and invasive carcinoma), thus allowing objective risk stratification of these lesions –108. In p < 0.05), 2 of which confirmed to be significant predictors of malignancy also at multivariate analysis. Moreover, when adding texture variable to MRI findings, diagnostic performance for predicting malignant IPMN improved from 0.80 and 0.78 to 0.85 in both reviewers (p < 0.05) [MRI-based radiomics is much more difficult because MRI suffers from less standardization than CT with a large variety in the multiple parameters related to scanner properties, acquisition settings and image processing that could hamper radiomics analysis . However < 0.05) .CPLs are a heterogeneous group of frequently detected lesions with different malignant potential and different prognosis, requiring different therapeutic and follow-up strategies; therefore, it is fundamental to correctly characterize them. MRI with MRCP sequence is the most useful technique to detect imaging features helpful for lesion characterization.Differential diagnosis of these cysts is based on the presence of communication or not with MPD, on their morphological features, on their location as well as on the basis of clinical history and demographic data.Once pseudocyst and WON have been excluded, a CPL communicating with MPD is an IPMN, while if not communicating could be a SCN, a MCN or a SPN and differential diagnosis, certain or presumptive, can be done on the basis of demographic data, location of the lesion and its morphological appearance.In some cases, differential diagnosis is still challenging and may require EUS-FNA and/or cyst-wall biopsy.MCNs and IPMN with high-risk stigmata must be resected if patients are fit for surgery; in patients with long life expectancy and IPMN with worrisome features, resection is suggested.Patients with IPMN without high-risk stigmata and patients who have been resected for an IPMN need follow-up till they are fit for surgery, in order to early detect malignant degeneration or concomitant pancreatic ductal adenocarcinoma.When the diagnosis of a CPL remains indeterminate, in our opinion it should be managed as an IPMN, as they are the most frequent CPLs with a potential malignant behavior.New imaging applications, with quantitative analysis thanks to texture analysis, have the potential to help to better stratify IPMNs, thus allowing a more correct management.


Lymphatic filariasis is a major health problem in tropical regions especially in India. A large number of patients tend to be asymptomatic. Ovarian filariasis is an extremely rare manifestation of lymphatic filariasis. This is a case report of bilateral ovarian filariasis presenting as ovarian mass with associated lower abdominal pain, weight loss and chyluria. This is a very rare diagnosis, more so as it was diagnosed preoperatively by ultrasound and managed with anti-filarial drugs and confirmed by biopsy. Most cases of ovarian filariasis reported in literature are incidental diagnosis on histopathological examination of postoperative specimen. Lymphatic filariasis is a mosquito-borne parasitic disease caused by round worms of the genera Wuchereria and Brugia. Lymphatic filariasis represents the second leading cause of permanent disability worldwide according to the World Health Organization . Pelvic We report a case of ovarian localization of filariasis in a 26-year-old patient diagnosed before surgery and treated with anti-filarial drugs. The diagnosis was confirmed on histopathology examination of the specimen post-surgery.A 26-year-old Indian lady attended outpatient department with history of pain abdomen, weight loss and anorexia for three months and history of chyluria for one month.She was primipara with last childbirth 14 months back. Her menstrual cycles were irregular, at 40-45 days interval with 5-6 days of bleeding. She was extremely thin built with a BMI of 16.2 at the time of examination. Per abdominal examination was normal. On per vaginal examination, uterus was normal in size. A firm mass was felt in the anterior fornix just above the uterus.Ultrasound showed complex multi-loculated cystic lesion in pelvis and bilateral adnexa of size 9.1 x 6.5 cm on right side and 4.5 x 6.5 cm on left side. The cystic areas within the mass showed classical ‘microfilariae dance’ or movement of microfilaria. A well-defined echogenic lesion was seen in left ovary of size 3.2 x 3.7 cm suggestive of dermoid cyst. Contrast enhanced computerized tomography (CECT) showed bilateral enlarged cystic ovaries with a dermoid cyst on the left with retroperitoneal lymphadenopathy  100 mg thrice daily and advised to return for follow-up after one month. Once on DEC, her general condition improved. Her chyluria was relieved.Ultrasound was repeated after one month of starting DEC. As the ‘micro-filarial dance’ was still visible on ultrasonography, DEC was continued. Repeat ultrasound after three weeks showed absence of ‘micro-filarial dance’. She was then taken up for laparotomy. On laparotomy, both ovaries were enlarged and fleshy Figure . Left ovThe histopathology report of right ovarian tissue showed focal presence of microfilariae larvae in the ovarian stroma, surrounded by dense mixed inflammation, predominantly eosinophils Figure . Left ovMalaria and lymphatic filariasis are two of the most common mosquito-borne parasitic diseases worldwide which can occur as concomitant human infections while also sharing common mosquito vectors . LymphatThese parasites are grouped as nematodes (roundworms) of the family Filarioidea. Most infections worldwide are caused by Wuchereria bancrofti. In Asia, the disease can also be caused by Brugia malayi and Brugia timori .Sethi et al. reported two cases of filariasis, one in the ovary and the other in the mesosalpinx. In the first case, the patient underwent pan-hysterectomy and in the second case, right ovarian cystectomy with right salpingectomy was performed. Both patients presented with complaints related to gynecological problems and not filariasis . The diaAdditionally, the diagnosis of ovarian filariasis is rarely made in preoperative condition because clinical symptomatology simulates that of a functional cyst . Thus, iThis is a rare case report because the diagnosis was made pre-operatively based on the clinical picture and radiological investigations. The patient received anti-filarial drugs and the micro-filarial dance seen on ultrasound disappeared after around two months of specific therapy. The diagnosis was finally confirmed by histopathological examination.Surgery and medical therapy primarily help to avoid recurrences ,5. The pWe conclude that ovarian filariasis is a possibility in endemic areas. The diagnosis must be considered in the differential diagnosis of tubo-ovarian or ovarian masses. This can be diagnosed radiologically and easily treated with anti-filarial drugs. Histopathological report confirms the diagnosis. The patients recover and have good prognosis.


Myeloid sarcoma is a rare pathology with important clinical implications. In this paper, we report the case of a 95-year-old gentleman with an orbital mass, which was later confirmed to be a myeloid sarcoma. We also discuss the role of radiotherapy in regard to this diagnosis in patients. Myeloid sarcoma  is a rare extramedullary manifestation of acute myeloid leukaemia (AML) with an estimated incidence throughout the literature of under 1% ,3.Our patient was a 95-year-old gentleman from a nursing home background who presented with a large, fixed, non-tender mass around his left orbit, which progressed quickly over the two-week period prior to presentation. His vision was intact, but the pressure of the mass was causing discomfort to the patient. He had obvious proptosis of the left eye and associated diplopia as the mass progressed in size.The patient had a history of several non-melanomatous skin cancers in the past, which had been managed by his general physician, but no history of any haematological issues or other relevant medical history.When he first presented, it was felt to be an orbital cellulitis and treated with IV antibiotics, which did not result in any clinical improvement. A CT was then performed, which showed a mass that was invading his left orbit. It was noted that the mass extended into the left orbit from the medial side, displacing the eye and medial rectus muscle laterally Figure . No optiThe decision was made to admit the patient to hospital for further investigations. A punch biopsy was performed, which showed extensive infiltration of the dermis, subcutis, and skeletal muscle by a neoplastic infiltrate of epithelioid cells, with decohesion. The appearance of the biopsy and the immunohistochemical profile were in keeping with myeloid sarcoma.The patient’s case was discussed with both an ophthalmic surgeon as well as a haematology consultant. The conclusion was that this was in keeping with a manifestation or prelude to AML. It was agreed, in conjunction with the patient, that the best approach to treatment in this 95-year-old gentleman would be palliative radiation to the affected site. It was also decided not to proceed with further invasive investigations such as a bone marrow biopsy. Further CT scanning for staging showed no evidence of primary malignancy, metastases, or lymphoma. Blood tests showed no abnormalities except a mild hyponatraemia. Blood film was unremarkable.It was decided to treat him with a course of 30 Gy in 10 fractions using volumetric modulated arc therapy (VMAT) with bolus.Throughout the treatment, he showed a marked clinical improvement with a visible decrease in degree of proptosis and size of mass Figure . The patTwo months after his first treatment, he developed a mass on in his parotid gland, which was imaged and later biopsied. The biopsy confirmed a recurrence of his myeloid sarcoma in his parotid gland Figure . Due to This case demonstrates an interesting case of myeloid sarcoma presenting as an ocular mass. As myeloid sarcoma is rare throughout clinical practice and literature, diagnosis remains a challenge. It is often misdiagnosed as abscesses or haematomas as they can present with a clinically similar picture. It is also histologically similar to Hodgkin’s lymphoma and Ewing sarcomas, which can lead to misdiagnosis. Literature suggests that up to 50% of patients with myeloid sarcoma are misdiagnosed ,5.Most patients with a diagnosis of myeloid sarcoma but with no concurrent evidence of AML at presentation will go on to develop leukaemia with research estimating the interval to progression of around 10.5 months . The proLimited literature is suggestive that radiotherapy does not aid overall survival in patients who are also receiving chemotherapy. It has been suggested that radiotherapy can increase the failure-free survival but not the median overall survival . SeveralHowever, even low-dose radiotherapy can achieve excellent local response and control, which can be particularly useful when the myeloid sarcoma is causing local symptoms or in palliation, such as in the case discussed. Myeloid sarcomas are radiosensitive, and complete response rate was found to be 89% where over 30 Gy was used, dropping to 43% when under 20 Gy was used . TherefoThis case of a gentleman presenting with an orbital mass, which led to a diagnosis of myeloid sarcoma, highlights the difficulty in diagnosing this rare pathology. Due to its rarity, it is often misdiagnosed, and research surrounding myeloid sarcoma remains limited. Diagnosis of this pathology in a timely manner is critical to aid prompt patient management and prognosis. It is also important to note that it is radiosensitive and low-dose radiation can achieve excellent local control and alleviation of symptoms.