A 36-year-old female with chronic watery diarrhea and persistent hypokalemia for more than eight months duration eventually being diagnosed as vasoactive intestinal peptide tumor (VIPoma) clinically and histologically is presented here. The patient achieved complete recovery after starting octreotide, a somatostatin analog. She underwent a distal pancreatectomy along with the removal of the tumor at Teaching Hospital Jaffna for the permanent cure. Vasoactive intestinal peptide tumor (VIPoma), also known as Verner-Morrison syndrome, is a rare type of neuroendocrine tumor. It was first reported in the literature in 1958 by Verner and Morrison [A 36-year-old Sri Lankan female, a mother of three children, presented with a history of chronic watery diarrhea for eight months duration. It was a secretory type of large voluminous watery diarrhea with a frequency of eight times per day. It was not mixed with blood or mucous and was not associated with abdominal pain. She had nocturnal diarrhea, and it was persisted despite fasting. There was no associated fever, facial flushing, vomiting, steatorrhea, alternating constipation, or fecal incontinence. Her past medical history was significant for bronchial asthma, which had been well controlled with inhaled corticosteroids. Other than chronic diarrhea, no other symptoms suggestive of hyperthyroidism were noted. She had an unintentional weight loss of 15 kg over a period of the last eight months due to persistent diarrhea. She has not been on any regular medication other than the inhaled steroid.2. Her pulse rate was 82 beats per minute, and her blood pressure was 110/70 mmHg. She had no signs of malnutrition, viz., glossitis, easy bruising, skin rashes, and peripheral edema. Examination of her abdomen revealed no organomegaly or any other masses or free fluid. The rest of the clinical examination was unremarkable.On examination, she was afebrile and not pale or icteric, and her body mass index was 23 kg/mHer initial investigations are shown in Table Vasoactive intestinal peptide-secreting pancreatic neuroendocrine tumor was made as a possible diagnosis after imaging and blood investigations. Serum VIP levels and somatostatin receptor scintigraphy were not done due to nonavailability. Octreotide 50 µg S/C twice daily was initiated as the therapy. After four days, she was able to maintain her potassium above 4.0 mmol/L with only oral replacement, and oral potassium was reduced and stopped completely during the course. She underwent laparoscopic distal pancreatectomy and removal of the tumor from the pancreatic body Figure . The bioVIPoma is a rare pancreatic neuroendocrine tumor causing chronic diarrhea. Most of them are malignant, and only 10% are benign . They coVIP is a 28-amino acid neuropeptide that causes large voluminous watery diarrhea by its vasodilator effect on intestinal smooth muscle and stimulating the production of CAMP in enterocytes, which induces the secretion of water and electrolytes, especially potassium and bicarbonate, along with water into the intestinal lumen .The differential diagnosis of large voluminous fasting diarrhea includes Zollinger-Ellison syndrome, laxative abuse, and rarely human immunodeficiency virus (HIV). VIPoma can be differentiated from Zollinger-Ellison syndrome by the absence of gastric hypersecretion and moderate loss of potassium in stool and metabolic acidosis . Serum gOther investigations include serum pancreatic polypeptide and chromogranin A. Functional PET imaging such as 68-Ga DOTA fusion PET/CT scans have a sensitivity of about 97% compared with CT scans . The WorThe definitive treatment for VIPoma is surgery; it improves disease-free survival in both primary and metastatic diseases. Treatment with somatostatin analog octreotide quickly resolves diarrhea. The main action of the drug is the direct inhibitory effect on the production of hormones from the tumor . InoperaAlthough VIPoma is rare, when a patient presents with chronic secretory diarrhea with persistent hypokalemia and metabolic acidosis, a diagnosis of VIPoma should be considered after excluding other possible causes. Early diagnosis prevents progression and complications. We made the diagnosis based on clinical and histological basis as the measurement of serum VIP level is not available in our country. Currently, the patient is symptom-free, and she is on regular six-month follow-up. The absence of metastasis, younger age, and complete resection of the tumor contributed to her good prognosis. We consider this case atypical presentation because of the absence of metastasis since most of the reported cases illustrate VIPoma that usually metastasizes to the liver or lung at the time of diagnosis.


A common presenting symptom for female patients is nipple discharge. Therefore having a background on how to manage is necessary for appropriately identifying and diagnosing the underlying etiology. The two most utilized imaging studies are diagnostic mammography and ultrasound. It can be difficult to identify a source/cause with mammography due to decreased sensitivity with also variable results seen with ultrasound. Advanced imaging such as MRI is becoming increasingly utilized limiting the need for ductography for diagnosis. In this case report, we discuss a rare case presentation of spontaneous nipple discharge. Nipple discharge is the third most common breast-related symptom, following palpable mass and breast pain . The maiThe patient is a 41-year-old female presenting for evaluation of spontaneous left-sided bloody nipple discharge for several months. The patient noted that discharge has been increasing in quantity and frequency. Family history of breast cancer in aunt. No palpable mass was identified on physical exam. The patient underwent imaging evaluation with representative images displayed in Figures The mammographic and sonographic findings can often be nonspecific. The most common presenting feature is ductal dilatation . MammogrPathologic nipple discharge can be a sign of breast cancer as illustrated in this case presentation. Careful history gathering and physical exam are extremely important in identifying the underlying etiology. Due to the wide spectrum of etiologies and differentials as potential causes varying from benign to invasive neoplasm, early detection is key. Both papillomas and neoplasms can have very similar imaging presentation when nipple discharge is the presenting symptom. Ductal carcinoma in situ has an overall good prognosis and when diagnosed early invasive neoplasms also have good outcomes. Imaging is very helpful and can serve as a powerful tool with breast MRI serving as a vital problem-solving examination. One should not stop investigating the underlying source of the pathologic discharge if imaging is negative but on the contrary, if no underlying abnormality is identified then the next step in management would be terminal duct excision since the presenting symptom is spontaneous nipple discharge.


Renal tumors during pregnancy are rare and the treatment requires evaluation of both the patient and the fetus. No consensus or guidelines has been proposed or verified in this field. We successfully managed three renal tumor cases during pregnancy and reviewed the relative literature.In the first renal cell carcinoma case diagnosed in the 21st week of pregnancy, laparoscopic retroperitoneoscopic partial nephrectomy was performed in the 26th week of pregnancy. In the second renal cell carcinoma case diagnosed in the 3rd week of pregnancy, laparoscopic retroperitoneoscopic radical nephrectomy was carried out after the abortion. In the third angiomyolipoma case who was diagnosed before pregnancy but received no treatment, we performed laparoscopic retroperitoneoscopic partial nephrectomy during the 17th week of pregnancy due to the rapid enlargement of the tumor.Although no consensus or guidelines for the management of renal tumors in pregnant patients has been proposed or verified, the general rules of kidney tumor management in non-pregnant patients and the guidelines for surgery in pregnancy could be referred to. Renal tumors found in pregnant patients require an individualized treatment regimen involving surgical timing, routes, techniques, and excision ranges, which should be decided by both the patients and the surgical teams. Previous publications have reported the estimated incidence of cancer diagnosed during pregnancy is 1 in 1000 pregnancies, among which the most common ones are breast cancer, melanoma, cervical cancer, and lymphomas . Renal cThree renal tumor cases managed during pregnancy were reported. Our patients’ demographics, clinical presentation, tumor size and location, management as well as pathological data are presented in Table cm mixed echoic signal mass with central vascularity in the middle of the left kidney on fetal ultrasound. Magnetic resonance imaging (MRI) was then performed and found the mass to be mixed intensity with multiple septa, suggesting a renal tumor and indicating there may exist bleeding and/or cystic change inside the tumor. No associated para-aortic lymphadenopathy or involvement of other organs was found. Referring to the current guidelines, radical nephrectomy is recommended for T2 (> 7 cm) tumors. However, some studies showed that partial nephrectomy for T2 or greater RCC may have a similar outcome to those of radical nephrectomy [2 insufflation was commenced and maintained at 12 mmHg. The kidney was exposed by opening Gerota’s fascia. The renal artery was dissected and clipped by Bulldog for 28 min. The operative time was 100 min, with an estimated blood loss of 150 ml. During the operation, the patient’s blood pressure remained stable. Fetal cardiac activity was monitored throughout the procedure, and fetal stability was ascertained at the end of the procedure. A pathological examination after surgery showed RCC (WHO grade III) with CA9 (+), EMA (+), and Vimentin (+) in immunohistochemical stain (pT2aN0M0). The surgical margin was clear. The patient did not require any adjuvant treatment during the postoperative period and was discharged 6 days after the surgery. The creatinine level was 51 μmol/L (eGFR = 126 mL/min·1.73m2) 1 month after the surgery, compared to 47 μmol/L (eGFR = 138 mL/min·1.73m2) before surgery. Subsequently, the patient delivered a healthy full-term baby 12 weeks after the operation.A 36-year-old female, Gravida 2, Para 1, at 21 weeks of pregnancy without remarkable history was incidentally found to have a 7.9 × 6.9 × 6.2hrectomy . The patcm mass with increased vascularity in the middle of the right kidney was incidentally found by ultrasound. The contralateral kidney was normal. There was no evidence of the involvement of any other organ. The RENAL score was 8P. After consultation with obstetricians and discussion with the surgical team, the patient decided to terminate the pregnancy before the kidney surgery. Three weeks after the abortion, a right retroperitoneoscopic radical nephrectomy was carried out under general anesthesia. A trocar for the camera was introduced on the mid-axillary line, 2 cm above the iliac crest. The other two trocars for manipulation were introduced on the anterior and posterior axillary line below the coastal border respectively. CO2 insufflation was maintained at 15 mmHg. The operative time was 90 min with an estimated blood loss of 50 ml. The right adrenal gland was removed. Postoperative recovery was uneventful and the patient was discharged 6 days after the surgery. Pathology of the resected tumor showed renal clear cell carcinoma (WHO grade I) with cystic change and hemorrhage, without invasion into the renal capsule and or pelvis (pT1bN0M0). Immunohistochemical stain showed positive AE1/AE3. The creatinine level was 65 μmol/L (eGFR = 94 mL/min·1.73m2) 1 month after the surgery, compared to 64 μmol/L (eGFR = 95 mL/min·1.73m2) before surgery.A 39-year-old female, Gravida 1, Para 0, at 3 weeks of pregnancy with a history of anemia and multiple uterine leiomyomas was admitted into our hospital. A 4.6 × 4.4 × 5.3cm. As a result, we couldn’t ensure the tumor growth rate. She repeated the urologic ultrasonography at 17 weeks of pregnancy and the mass was shown to be 25 × 13cm with mixed echoic signals. We did a particular body examination after the patient was admitted. There were no skin characteristics of tuberous sclerosis, including hypopigmented macules, angiofibromas, and shagreen patches. No family history was reported. Therefore, we took no consideration of the diagnosis of tuberous sclerosis. After consultation with obstetricians and discussion with the patient, at 17 gestational weeks, a left retroperitoneoscopic partial nephrectomy was performed. After general anesthesia, the patient was placed in the right lateral position. A trocar for the camera was introduced on the mid-axillary line, 2 cm above the iliac crest. Two trocars were introduced on the anterior and posterior axillary line below the coastal border respectively. A third trocar was placed between them for instruments and manipulation. CO2 insufflation was maintained at 15 mmHg. The renal artery was dissected and clamped by Bulldog for 30 min. The operative time was 110 min, with an estimated blood loss of 100 ml. The blood pressure of the patient and fetal cardiac activity was stable throughout the procedure. On the day of surgery and 2 days after the surgery, the patient was treated with 40 mg/day intramuscular progesterone. Postoperative recovery was uneventful and the patient was discharged 6 days after the surgery. Pathological analysis revealed renal angiomyolipoma. The creatinine level was 42 μmol/L (eGFR = 163 mL/min·1.73m2) 1 month after the surgery, the same as that before surgery. The patient gave birth to a baby uneventfully at 39 weeks’ gestation.A 30-year-old female, Gravida 1, Para 0, at 17 weeks of pregnancy presented with left lower quadrant discomfort. She was diagnosed with bilateral multiple renal masses a year before pregnancy without any treatment. However, the patient lost her primary radiological results and we could not find the detailed information of the tumor. Ten months later, the ultrasonography at 13 weeks of pregnancy showed the largest renal mass located in the lower pole of the left kidney, with the size of 16 × 14Although urological tumors diagnosed during pregnancy are rare, a timely diagnosis has prognostic benefits because the intervention would be much easier before the tumor progresses further and when the uterine is relatively small. Approximately 109 cases of renal malignancies have been reported so far, of which 91 are RCC . The maiNot all of the kidney tumors found during pregnancy need urgent intervention. The surgical decision should be made based on the condition of the fetus and the mother, the aggressiveness of cancer, and more importantly, the wishes of the pregnant women. If the surgery is unavoidable, the timing of treatment is critical. The general principle for localized RCC (stage I or II) is that surgical intervention should be avoided in the first trimester, whereas performing the surgery during the second trimester or after the fetal lung matures is usually recommended. However, for stage III or IV tumors found in any trimester, pregnancy termination is recommended and appropriate treatment of RCC should be the priority . In our Radical nephrectomy and nephron-sparing surgery are essential treatments for the management of RCC. According to National Comprehensive Cancer Network (NCCN) guidelines, the general principle for RCC management is that in stage I disease, partial nephrectomy is recommended for T1a renal mass, while for T1b tumors, partial nephrectomy shows a similar outcome to that of radical surgery. The curative therapy for patients with stages II and III disease is radical nephrectomy. Partial nephrectomy can only be performed in patients with locally advanced tumors if technically feasible and clinically indicated . In prevIn recent years, with the development of surgical techniques and surgeons’ experience, laparoscopy has become gradually acceptable in pregnancy. An evidence-based guideline on laparoscopy in pregnancy commissioned by the British Society for Gynecological Endoscopy recommends laparoscopic surgery in pregnancy be carried out in settings where adequate time, laparoscopic expertise and monitoring facilities are available . LaparosIn the case of RCC during pregnancy, timely diagnosis and management are crucial in predicting maternal-fetal prognosis. The creatinine level and urological ultrasound of these three patients were followed 1 month after the surgery, which all showed no anomalies. Reoccurrence was not found in all three patients during long-term follow-up. The patients in case one and three both delivered a full-term healthy baby.We also find some pregnant women who are diagnosed with renal angiomyolipoma are treated with conservative follow-up. A case was reported that a pregnant woman with a 10 cm mass suggestive of angiomyolipoma in the left kidney had a spontaneous rupture at the 20th week of her pregnancy, and the operation was performed until the 34th week . It was In conclusion, although no consensus or guidelines for the management of renal tumors in pregnant patients has been proposed or verified, the general rules of kidney tumor management in non-pregnant patients and the guidelines for surgery in pregnancy could be referred to. Every pregnant patient needs an individualized treatment involving surgical timing, routes, techniques, and ranges based on the condition of the mother, the fetus, the tumor stages as well as the experience of the surgical team.


Joint-preserving prosthetic reconstruction for massive bone defects has the potential to be a new and revolutionary treatment option. In this paper, we discuss the case of a 30-year-old female patient who presented with pain and swelling around the knee for three months. The patient underwent this procedure. Postoperative patient satisfaction, pain scores, and range of motion results were found to be promising. We believe that this method has the potential to be the next stage in the quest for better treatment options for this condition. Three-dimensionally (3D)-printed joint-preserving prosthetic reconstruction after malignant tumor resection has the potential to be a new treatment option for a select group of young patients. Conserving the articular surface and ligaments can preserve the normal kinematics of the knee, rather than trying to mimic it. Precisely reconstructing the anatomy of the patient can result in a better sense of proprioception and range of motion ,2. CarefReconstruction of massive bone defects after tumor resection poses a challenge for orthopedic oncologists. Among the reconstruction options for massive bone defects after tumor resection, allografts , autograIn this report, we share our preliminary experience with the 3D-printed joint-preserving prosthetic reconstruction and aim to emphasize the importance of protecting the ligaments and the articular bone component of the joint. This method of treatment has the potential to be a major weapon in the surgeon's arsenal as a salvage procedure in young patients.A 30-year-old female with no conclusive history of trauma presented with progressive complaints of pain and swelling in the proximal of the posterior region of the knee for three months. Our physical examination and subsequent imaging of the affected extremity revealed a cortex-destructing lesion located at the posterior part of the proximal tibia. Clinical examination revealed a tender palpable lump located below the popliteal area approximately 4 x 2 x 2 cm in size, with no overlying erythema. No signs of local infection were present.The patient did not have any predisposing risk factors such as smoking or alcohol consumption. Her general well-being and vitals were stable. She was diagnosed with familial Mediterranean fever (FMF) and was prescribed colchicine. She had no surgical history and no known medication allergies.3 , hemoglobin: 13.6 g/dL. C-reactive protein was 3.3 mg/L , and erythrocyte sedimentation rate (ESR) was 11 mm/h . Alkaline phosphatase (ALP) was 93 U/L , and lactate dehydrogenase (LDH) was 170 U/L .Her laboratory test results were within normal limits: WBC: 7,800/mmThe X-ray imaging showed a solid cortex-expanding mass located at the proximal tibia Figures , 1b. CT Materials obtained were evaluated by a specialized musculoskeletal pathologist; 9-mm tru-cut biopsy materials were processed. Histopathological features and MDM2 positivity in some of the spindle-fibroblastic cells suggested the potential for conditions such as "low-grade osteosarcoma" and "parosteal osteosarcoma".Preoperative planningDual-stage reconstruction was planned. The aim of the preliminary surgery was to successfully preserve the epiphysis of the proximal tibia while attaining a tumor-free tibia. To perform the ideal resection of the tumor, 3D modeling and cut guides were created before the operation Figure . The aimPreliminary surgeryFirstly, with the aid of radiological examinations and histopathological diagnosis of the biopsy, the lesion of the patient was staged and the indications for resection were confirmed; 3D CT scans of the bilateral lower extremities were performed to obtain a surgical margin negative wide resection. MRI scan was performed to detect tumor borders and surrounding reactive tissue. For these examinations, the parameters applied by the Dokuz Eylül University Hospital Department of Radiology (1-mm cross-section and matrix of 512 x 512) were used and scans were made accordingly with these parameters. Then, the image was saved in the Digital Imaging and Communications in Medicine (DICOM) format and transferred to the 3D Slicer software for the reconstruction of 3D geometric models. Virtual mechanical analysis of the solid model created was performed. According to the shape and structure of the tumor, and by using the Meshmixer 3.5 software , osteotomy guides were 3D-printed using polylactic acid (PLA). These guides ensured that the tumor was completely removed.After the necessary preparations, the operation was performed. An approximately 20-cm long longitudinal incision was made at the midline of the knee, extending from the superior pole of the patella to 10 cms below the tuberositas tibia. The biopsy tract was excised during the first stage of this procedure. Arthrotomy was performed to check the viability of the anterior and posterior cruciate ligament and the menisci. Tibial tubercle osteotomy was performed. A tumor-free 5-cm segment of the bone that included the tibial tubercle and a portion of the anterior crest of the tibia was elevated. Prothesis design houses an indentation for this bone fragment to be fixated. The tumor was removed successfully using 3D-printed resection guides. To preserve the length of the limb and to avoid complications like infection, a hand-made cement spacer was used. Preserved epiphysis of the proximal tibia was then relatively stabilized onto this spacer using a conventional tibia intramedullary nail  with computer navigation-assisted surgery in terms of accuracy and surgery time. Studies have shown that PSIs achieved an acceptable resection accuracy with a shorter surgery time ,16. Addi3D-printed joint-preserving prosthetics, designed and produced according to the patient's needs, can decrease the need for revision surgery. Preserving the key structures for movement can result in a better sense of proprioception  and restWith this paper, we wanted to emphasize that in a select group of young patients, joint-preserving prosthetic reconstruction using 3D-printing technology can be an effective weapon in the surgeon's hands as a salvage procedure. With the mass adoption and widespread use of emerging new technologies, cost and time efficiency can be greatly optimized and technology can once again illuminate the path of medical science.


We present two cases of AOT, the first case concerns a 23‐year‐old patient with an AOT located in the maxilla and the second case involves a 37‐year‐old patient presenting an AOT with mandibular localization. Adenomatoid odontogenic tumor is frequently misdiagnosed as a dentigerous cyst. However, it has a characteristic radiological appearance, consisting in a mixed image having a mostly liquid content and a peripheral calcification lining the wall. However, this aspect is often not detected on radiographs if the calcifications are too discreet. It was first described by Dreiblard in 1907 as a pseudo‐adenoameloblastoma.At first, it was considered as a variant of ameloblastoma, from which it gained its previous name adenoameloblastoma. In 1948, Stafne considered it as a distinct pathologic entity.It was only in 1969 that Philipsen and Burn presented a review involving 76 cases, in which they showed that AOT is a well‐defined entity, distinct from solid or multi‐cystic ameloblastoma, and they therefore suggested the current term AOT.Its clinical symptomatology often includes a painless slow‐growing bone swelling.The follicular variant associated with an impacted tooth is the most frequent type. It is therefore misdiagnosed as a dentigerous cyst, an ameloblastoma, or a kerato‐cystic odontogenic tumor.The presence of intra‐lesional calcifications is considered as a characterizing distinctive element of AOT, allowing to have a differential diagnosis from other cystic bone lesions. Those calcified deposits are observed in approximately 78% of AOT.Thus, searching for these calcifications in imaging finds its importance to help guide diagnosis. The aim of this work was to describe the clinical, radiological, and anatomopathological characteristics of this rare tumor, to discuss its differential diagnosis and especially to highlight the benefit of radiological examinations in its diagnosis.22.1A 23‐year‐old patient presented with a painless nose and jaw swelling in the right side of the face that was progressing for 1 year. The patient's questionnaire revealed no previous extraction, traumatism, or infection. Extra‐oral examination revealed the presence of nose and jaw swelling in the right side of the face.The integument had a normal aspect with no pain. Intra‐oral examination showed a vestibular swelling extending from the apical region of the right maxillary central incisor (11) to the right second premolar (15) with a normal mucosa.On palpation, the swelling was depressible and painless. Dental examination revealed the absence of the right maxillary lateral incisor (12) on the arch as well as the persistence of the right temporary lateral incisor (52).The maxillary right central incisor (11) was in vestibular position with a light mobility, and the remaining teeth were healthy. Diagnosis of a cystic or benign tumoral lesion having a cystic nature was made based on the absence of the right maxillary lateral incisor (12) on the arch and the presence of a painless and depressible slow‐growing swelling.Panoramic radiograph showed a well‐defined radiolucent image with a peripheral condensation border having an oval shape, occupying the right median and paramedian region, extending upward to the apical region of tooth15 and fitting downward between the right maxillary central incisor (11) and the right maxillary canine (3)  was impacted, and it appeared to have a close rapport with this image. A light axis change of the right maxillary canine (13) resulting from the effect of the lesion extension was also noted , impaction of the right maxillary lateral incisor (12), and persistence of the right temporary lateral incisor (52).A new essential element, consisting in the presence of micro‐calcifications within the radiolucent image, appeared Figure B.The diagnoses already suspected on the panoramic radiograph were therefore eliminated with the presence of this mixed aspect in the radiological image.Thus, our diagnosis was oriented toward a benign tumoral lesion having a cystic nature, most likely presenting peripheral calcifications. These characteristics are the same as those described in calcifying odontogenic cysts, or AOT.Faced with this uncertainty of diagnosis, computed tomography was performed. It revealed a well‐defined hypodense image with fluid nature, extending upward to the nasal fossa floor, downward to the apical region of the right first premolar (14) and the right second premolar (15), and fitting in the space between the right maxillary canine (13) and the right maxillary central incisor (11). Medially, the image extended up to the inter‐maxillary suture. Laterally, the external cortical bone was repressed and thinned without perforation.Within this image, impaction of the right maxillary lateral incisor 12) and the presence of peripheral intra‐lesional micro‐calcifications were confirmed  and the impacted right maxillary lateral incisor (12).Intra‐operatively, it was a well‐encapsulated lesion with fluid content. The clinical and radiological evolution after two weeks, one month, and one year were favorable without any sign of postoperative secondary infection or recurrence. Macroscopically, the anatomopathological examination showed that it was a cystic cavity comprising an impacted tooth. The cystic wall had variable thickness and presented budding areas. Histologically, the cavity contained eosinophilic material mixed with rare cellular debris. The budding areas of the wall were due to the presence of nodules formed of cylindric epithelial cells with regular nuclei arranged in rosette or in a pseudo‐glandular structure. In some locations, these cells formed anastomosing spans of isolated edematous connective tissue having a plexiform appearance.Outside these cell areas, the wall was bordered by few rows of squamous cells resting on a strip of fibrous tissues having variable thickness. Finally, diagnosis of AOT was retained.2.2A 37‐year‐old patient with no particular pathologic antecedents presented for mandibular swelling of the symphysis and the right parasymphysis regions that was evolving for several years.The patient's questionnaire revealed no particularity. No previous extraction or infectious episodes were noted. On palpation, a painless vestibular filling of renitent consistency was noted. The lingual cortex showed no deformity. Dental examination revealed the absence of the right mandibular canine tooth (43) on the arch. The lip and chin sensation was preserved. The teeth in relation to the swelling had a negative vitality test without being mobile.A pericoronal cyst in relation to the impacted right mandibular canine (43) was the suspected diagnosis given the absence of the tooth 43 on the arch without the notion of prior extraction.Panoramic radiograph revealed a well‐defined homogenous radiolucent image with clear contours measuring approximately 11 cm × 4 cm of large diameter and extending from the mesial root of the right mandibular first molar to the root of the left mandibular second premolar 35). It had a close rapport with the apices of the overlying mandibular teeth. Downward, it extended toward the basilar border that was thinned  was impacted in horizontal position and repressed toward the basilar border. The border of the image at this level was in relation to the crown and to a part of this tooth root.At this stage, diagnosis of a pericoronal cyst in relation to the impacted right mandibular canine 43) was made. In order to establish a more accurate assessment of extension, computed tomography scan (CT) was performed. It confirmed the presence of hypodense image of fluid density repressing and essentially thinning the vestibular cortex. In certain sites, the lingual cortex was slightly thinned. The crown of the impacted 43 was encompassed by this image. A new element of semiology, consisting in the presence of fine calcifications arranged in periphery at the level of the vestibular wall, appeared on CT scan examination .Preoperatively, the right mandibular second premolar 45) was mobile. It was therefore attached to the right mandibular first premolar (44) using a steel wire suture. Postoperative follow‐ups were simple , AOT is considered as a tumor integrated in the group of epithelial odontogenic tumors.The lesions are more prevalent in the maxilla than in the mandible, and it is more common in the anterior region than in the posterior region.In our two reported cases, the localization of AOT perfectly corresponds to the data available in the literature both for maxillary and mandibular localization. AOT usually measures 1.5 to 3 cm of large axis, but it can sometimes reach a more important volume that can be responsible for dental displacements as well as functional and/ or esthetic disorders.In the second reported case, the tumor reached a very important volume, measuring approximately 11 cm × 4 cm of large axis, resulting in necrosis of the involved teeth and a troublesome jaw swelling that was the major motive for the patient to seek treatment.In fact, the size of the lesion is correlated with increasing aging and it is significantly associated with features, such as increased root resorption, ill‐defined borders, expansion, cortex perforation, and lesions crossing the midline. These changes may reflect a longer duration of the lesion due to late diagnosis.Three clinic‐topographic variants of AOT are identified: Follicular, extra‐follicular, and peripheral.The follicular variant is the most frequent. It is an intra‐osseous lesion associated with an impacted tooth. Our two reported cases correspond to this type  in the first case and an impacted right mandibular canine (43) in the second one).A large study including 105 cases of AOT demonstrated that in 51 cases (48.5%), tumors were located in the anterior maxilla, representing the most common anatomic site, followed by 31 cases (29.5%) in the anterior mandible, 16 cases (15.2%) in the posterior mandible and 7 cases (6.6%) in the posterior maxilla.The extra‐follicular or extra‐coronal variant is intra‐osseous having no relation to an impacted tooth. Philipsen suggested four sub‐types for this variant, classified from E1 to E4.E1: tumor without any relation to the tooth.E2: tumor between two teeth roots.E3: tumor encompassing the tooth root apex.E4: tumor encompassing an intermediate part of the tooth root.The peripheral type which is extra‐osseous is localized at the gingival tissue. Clinically, it usually appears as a labial gingival mass overhanging the crown .This extra‐osseous variant is exclusively restricted to the anterior maxillary labial gingiva, with only 0.3% of cases being reported in the mandible.The study conducted by T Becker et al. showed that the extra‐osseous peripheral variant is the least frequent (5% of the studied cases). The intra‐osseous variant of the follicular type is more frequent on the maxilla; however, the intra‐osseous variant of the extra‐follicular type is more frequent on the mandible.The pathogenesis of AOT is still poorly known and uncertain, having various origins as mentioned in the literature. Yet, the resemblance between the cylindric cells and the ameloblasts, and the frequent association with impacted teeth suggest that the tumor could originate from the dental epithelium.This could explain the possible radio‐clinical confusion between AOT and follicular cysts.Adenomatoid odontogenic tumor is often discovered fortuitously, either during a routine dental examination or during a radiographic examination carried out for a check‐up . Sometimes, it is the development of a slow‐progressing, often isolated and painless bone swelling that alerts the patient and incites him to seek treatment. This swelling can sometimes be accompanied by dental signs .Therefore, the main clinical findings are asymptomatic lesions causing facial asymmetry as a result of cortical bone expansion and diffuse swelling in the buccal and palatal/lingual vestibules.When the lesion is intra‐osseous, it causes thinning, and even a minimal rupture of the cortex which can clinically manifest through a palpable and depressible swelling that could result in facial asymmetry.In this form, it is difficult to clinically differentiate between AOT and other intra‐osseous benign lesions, such as dentigerous cysts, calcifying odontogenic cysts, and ameloblastomas.In most cases, the content of the lesion is liquid. However, for extensive AOT, a solid consistency may be present in the walls.The distinction between AOT in its follicular form and dentigerous cysts is essentially based on the rapport of the image to the crown or the root of the tooth involved. AOT is rather suspected in case of an image encompassing the tooth crown and root. However, in case of dentigerous cysts, the image would rather involve only the crown.Radiologically, intra‐osseous AOT presents a unilocular osteolytic image with well‐defined contours containing, in certain locations, discrete calcified foci. In the most advanced cases, more or less marked radio‐opaque foci can be found, constituting a useful diagnostic tool.The extra‐osseous, peripheral, or gingival types of AOT are rarely detected radiographically; however, slight erosion of the underlying alveolar bone cortex may be present.When calcifications appear in imaging, diagnosis of a dentigerous cyst is ruled out. Our diagnosis was therefore oriented toward AOT, calcifying odontogenic cysts, or fibro‐cemento‐osseous tumors.An essential clinical sign, namely cortical depressibility to palpation associated with elements of radiographic semiology, like fluid density at the CT scan examination and the peripheral location of the calcifications are in favor of diagnosing a cystic tumoral lesion, such as AOT or calcifying odontogenic cysts. In this case, calcifying epithelial odontogenic tumors and fibro‐osseous tumors are eliminated.These radiographic characteristics are the same as those observed in our two cases where intra‐lesional and peripheral fine calcifications lined the wall with fluid density at the scanner.The distinction between calcifying odontogenic cysts and AOT can only be performed anatomopathologically. The anterior maxillary localization is in favor of AOT.For AOT, the calcifications found at imaging are described as spotted areas or snowflakes, calcified areas having unequal size, calcified speculated areas, fine and scattered radio‐opacities, irregular radio‐opacities, amorphous radio‐opacities, and fine and discrete radio‐opacities.It is important to note that panoramic imaging is often insufficient to reveal the presence of these calcifications. However, intra‐oral radiographic techniques are more efficient thanks to their visualization.Maxillary occlusal radiograph clearly showed the presence of fine radio‐opaque calcifications, which directed our diagnosis toward a tumoral lesion.In fact, the perception of radio‐opacities in radiographs may depend on the amounts of calcifications and the radiographic techniques. Minimal calcifications (particularly a thin radio‐opaque line) might not be detectable on conventional images due to superimposition of the structures.Thanks to its high resolution and good spatial definition, sectional imaging (multi‐bar scanner or Cone Beam Computed Tomography) allows a better approach to the lesion density. The presence of intra‐lesional calcifications and their peripheral disposition on the image are well‐detected on the different slices. Sectional imaging is more efficient in the detection of these calcifications and in the analysis of their central or peripheral locations. The presence of these calcifications on the different slices eliminates the diagnosis of a cystic lesion, previously made based on standard radiographs.Analysis of the lesional content allows a better diagnostic orientation, essentially through the possibility of density measurement provided by the scanner.With the technological evolution, cone beam computed tomography (CBCT) is currently used in all the fields of dental medicine and maxillofacial surgery. The main advantage of CBCT radiography is the multi‐planar cross‐sectional images in various orientations and the three‐dimensional reconstructions based on a single scan of the fields of view, varying from a single tooth to the whole maxilla‐facial area. CBCT imaging is superior to panoramic radiography with regard to elimination of superimposition and the excellent contrast resolution for mineralized tissue, such as teeth, bones, and calcified spots. Therefore, CBCT is advantageous in terms of demonstrating the detailed internal structures of lesions, particularly when the calcifications are minimal or the superimposition is serious in the maxillary region. Moreover, CBCT provides a better display of the extent and the complex spatial relationship of the lesions with the surrounding structures.The diagnosis suspected based on clinical and radiographic data can only be confirmed by the anatomopathological study of the operative pieces.Macroscopically, it is a cavity of 1 to 5 cm in diameter, having a wall of variable thickness and presenting some small endocavity over elevations. Histologically, the epithelial cells are arranged in spans or compact lobules within a small connective stroma. In certain sites, they can adopt an ameloblastic cylindric aspect with the presence of pseudo‐glandular cavities devoid of mucous secretions (PAS and negative blue Alcian).Within the epithelial masses, small masses of eosinophilic hyaline material capable of being calcified are observed.The histo‐enzymologic activities of these cells are particular. In fact, these cells have an intense ATPase and alkaline phosphatase activity on the cytoplasmic membrane.In electronic microscopy, the rare cases studied so far have revealed two cell types: Some are polygonal or cylindric, rich in tonofilaments resembling preameloblasts, and others are smaller stellate cells recalling the structure of stratum intermediun and stellate reticulum of the dental bud.Concerning the exact nature of hyaline deposits, it is controversial. Some authors assimilate it to an amyloid‐like substance close to the enamel matrix because it contains a rich network of microfibrils. However, for others, it is a dysplasic dentine comprising calcifications corresponding to apatite crystals.On the therapeutic level, the treatment of choice consists in complete enucleation. In case of an impacted tooth in a favorable position associated with AOT, it can be preserved and placed on the arch using orthodontic means.Evolution is constantly favorable. Recurrences are exceptional, and no malignant transformation is reported.4The two cases are presented in great radiological details in an effort to share radiographic features that can assist pathologists and clinicians who, at some point, need to diagnose and treat patients with AOT.In fact, AOT is a benign odontogenic tumor of the maxillae. The clinical aspect depends on the intra‐osseous or extra‐osseous form of the tumor. Histopathology can confirm diagnosis.However, a characteristic radiological aspect, consisting in the presence of a mixed image with calcifications arranged in the periphery, may be in favor of this diagnosis.None.I. C, R.C, and T. A: contributed to the diagnosis of the cases, prepared, and drafted the manuscript; B.S and R. K: contributed to the anatomopathological diagnosis of the cases. J.B and H.K: contributed to the oral and maxilla‐facial surgery of the cases.This article does not contain any studies with human or animal subjects performed by any of the authors.A written informed consent was obtained in accordance with the journal's patient consent policy.


This case serves as a reminder to consider ectopic splenic tissue in the differential diagnosis of pancreatic masses. The literature shows a lack of awareness and overtreatment of this condition due to clinical and radiologic concern for malignancy, namely neuroendocrine tumors (NETs) identified on positron emission tomography (PET)-CT NETSPOT. Given the vast difference in management and prognosis of ectopic splenic anomalies and malignant neoplasms involving the pancreas, accurate diagnosis is imperative to avoid unnecessary invasive procedures such as Whipple or distal pancreatectomy and splenectomy, which are associated with increased morbidity and mortality. Splenosis and accessory spleen are both ectopic splenic anomalies. Splenosis or autotransplantation of splenic tissue occurs after trauma or surgery in locations outside of the spleen, most commonly in the abdominal and pelvic cavity . The accA 70-year-old male with a past medical history of melanoma, skin squamous cell carcinoma of the scalp, and gastroesophageal reflux disease presented for chronic vague epigastric discomfort and bloating associated with intermittent bilateral flank pain which was present intermittently since his cholecystectomy 12 years ago. He also endorsed weight loss due to poor oral intake associated with an increase in abdominal girth. Physical exam showed a non-distended abdomen with diffuse tenderness and normal bowel sounds. He underwent an upper GI endoscopy that revealed mild chronic inactive gastritis without Helicobacter pylori (H. pylori) organisms. A concurrent colonoscopy showed multiple adenomatous polyps and diverticulosis. Despite proton pump inhibitor therapy, his symptoms persisted, and an MRI abdomen/pelvis was obtained Figure . Given tPET-CT NETSPOT received FDA approval in 2016 for functional imaging detection of SSTR avid NETs due to its high sensitivity in detecting small-sized tumors compared to the previously utilized octreotide scan ,8,9. PETLiterature documents many case reports of false positives on PET-CT NETSPOT due to ectopic splenic anomalies -17. One This case highlights the importance of considering ectopic splenic anomaly in the differential diagnosis of pancreatic masses that are small, 1-3 cm in size, located in the pancreatic tail, and are positive on PET-CT NETSPOT. Certain features on CT and MRI can suggest the diagnosis of intrapancreatic accessory spleen over pancreatic tumors. CT and MRI show attenuation and enhancement of the accessory spleen similar to the spleen in all phases. In contrast, pancreatic tumors show greater attenuation in the arterial phase and less in the venous phase on CT. On MRI, splenic tissue in the pancreatic tail often shows low signal intensity in T1 and high intensity in T2 when compared to pancreatic parenchyma; moreover, intratumoral hemorrhage and necrosis are absent in splenic tissue and may be seen with neoplasms . In diffEctopic splenic anomalies should be considered in the differential diagnosis of pancreatic masses that are small, 1-3 cm in size, located in the pancreatic tail, and are positive on PET-CT NETSPOT. Ectopic splenic anomalies are incidental, asymptomatic benign processes that do not need surgical intervention. Unfortunately, due to misdiagnosis and concern for malignant tumors such as NETs, patients undergo unnecessary procedures and surgeries. If this differential diagnosis is considered, additional imaging techniques such as Technetium-99m heat-damaged RBC or sulfur colloid scans can be helpful. Alternatively, tissue diagnosis may be performed, such as in this case, before committing to surgical resection. While FNA procedure-related complications and false negatives can occur, in our patient’s case, the biopsy provided the correct diagnosis and saved the patient unnecessary surgery, treatment, and anxiety.


Choriocarcinoma is a disease associated with uncontrollable proliferation and malignant change of cells of the placenta and belongs to the malignant end of the spectrum in gestational trophoblastic disease. These tumours are usually developed after molar pregnancies, and their incidence after full-term pregnancies is extremely rare.We present a very rare case of a 30-year-old lady, admitted with a five-month history of vaginal bleeding after a normal pregnancy. The human chorionic gonadotropin (hCG) was at a level of 209,566. A pelvic ultrasound scan revealed an endometrial thickness of 6 cm and the presence of an intra-uterine mass measuring 56 × 50 × 45 mm. After discussion with the regional gestational trophoblastic disease centre, we proceeded to a surgical evacuation of the uterus, which confirmed a post-partum choriocarcinoma  score 9). Care was continued in the specialised centre with multi-agent chemotherapy. The response was excellent, and the patient was subsequently discharged after 10 cycles of chemotherapy, and a 10-year follow-up was arranged.Choriocarcinomas after full-term pregnancies are a rare entity. Even when they happen, they are usually associated with pregnancy complications in the ante-natal period. The prognosis is usually very good, provided that prompt diagnosis and referral to a specialised centre are made. Low-risk patients are usually treated with methotrexate monotherapy, whereas high-risk women would normally require multi-agent chemotherapy.The diagnosis of choriocarcinoma might be proven challenging even for experienced clinicians. Women should be informed that the prognosis is usually excellent, provided that they receive the right treatment. Gestational trophoblastic disease (GTD) envelops a variety of diseases, from the pre-malignant conditions of complete hydatiform mole (CHM) and partial hydatiform mole (PHM), to malignant invasive mole, choriocarcinoma (CC) and more rarely, placental site trophoblastic tumour (PSTT) or epithelioid trophoblastic tumour (ETT). Collectively, the malignant forms are referred to as gestational trophoblastic neoplasia (GTN). The incidence of molar pregnancies (CHM/PHM) is reported to be 0.2-1.5 per 1000 births in Europe .A 30-year-old G1P1 female presented to a district general hospital with a five-month history of heavy post-partum bleeding. She reported having passed large clots in the preceding few days, prompting attendance at the accident and emergency department. Her general practitioner had inserted a Mirena IUS  at 12 weeks post-partum, which failed to cease the bleeding. The general practitioner then proceeded to prescribe norethisterone pills one month prior to admission.The index pregnancy was normal, with no associated complications. A healthy baby was born vaginally with a ventouse and was being exclusively breastfed. There had been no reported nausea, vomiting, cough, abdominal or pelvic pain, nor loss of consciousness associated with the erratic and heavy bleeding. The patient had only partaken in sexual intercourse once in the post-partum period, with the Mirena IUS in-situ. There was no notable family history. The patient had a previous appendectomy at age 19 and a borderline smear cytology result four years previously with no treatment.The general examination was largely unremarkable, with no abdominal tenderness; however, the uterus was palpable at 12 weeks' size. Normal vulva and vagina, with some large clots and blood noted in the vaginal canal. The cervical os was closed. There were no focal neurological signs nor other signs of any potential metastatic disease. One gram of tranexamic acid was administered, which slowed the bleeding. Blood results demonstrated haemoglobin of 121 and a beta-human chorionic gonadotropin (b-hCG) of 209,566.A trans-vaginal ultrasound scan demonstrated a thickened endometrium measuring 62 mm and a large mixed echoic mass in the cavity measuring 56 × 50 × 45 mm with some vascularity Figure . Mirena Her International Federation of Gynaecology and Obstetrics (FIGO) score was 9 (high risk): <40 years old (30 = 0), index pregnancy , time since delivery (five months = 1), b-hCG , size of tumour  = 2), metastases (none on CXR = 0).The case was discussed with the local GTD centre and it was agreed that the most likely differential was that of post-partum choriocarcinoma. The patient then proceeded to a surgical evacuation of the uterus on the advice of the local GTD centre. Products of conception (POC) were obtained and sent for histological analysis.Peri-operatively, the patient lost an estimated 1.5 l of blood and received two units of packed red cells. The POC were felt to be friable on palpation and likely molars.Histology demonstrated macroscopically a sac weighing 40.6 g, with no obvious vesicles nor fetal parts. Microscopically, it was comprised of large areas of haemorrhage with some necrosis and viable cells. The viable cells were in the form of atypical mononuclear intermediate trophoblastic cells in sheets that were surrounded by multinucleate syncytiotrophoblast tumour cells. There was no myometrial invasion, nor were chorionic villi visualised. The pattern was consistent with a diagnosis of choriocarcinoma. All cells stained strongly positive for programmed death ligand 1 (PDL1). There were some tumour-infiltrating lymphocytes (CD3 and CD8 positive).Following discharge, the patient presented with a further episode of blood loss; approximately a 120-ml sized clot.The patient was transferred to the local GTD centre with the diagnosis of post-partum choriocarcinoma and she received 10 cycles of chemotherapy , and actinomycin). She responded very well to chemotherapy and was advised that she could try to conceive again after one year. She will be followed-up in the tertiary centre for 10 years. Her b-hCG results are demonstrated in Figure Choriocarcinoma after term pregnancy is a rare entity , with anChoriocarcinomas, unlike our case, tend to be associated with pregnancy complications such as hydrops fetalis, fetal-maternal haemorrhage, intra-uterine fatal death, and irregular cardiotocography (CTG) patterns. In our case, the pregnancy was uncomplicated. Maternal complications may include intracranial haemorrhage and pulmonary embolism, thus choriocarcinoma should be considered as a possible diagnosis in women who present with these pathologies, especially in the post-natal period .The prognosis of choriocarcinoma is usually very good, provided that the appropriate referral to a tertiary centre is made and the appropriate chemotherapy regimen is started in a timely manner ,9. HowevIn regard to the long-term outcomes, these are generally promising, with approximately 80% of women achieving a further pregnancy following MTX treatment or multi-agent chemotherapy . Women, In summary, diagnosing CC can be challenging. However, combining the gynaecological history, elevated b-hCG levels and USS findings, usually leads to the diagnosis. Consideration should be given, as to whether or not a tissue biopsy is needed before starting treatment. Biopsy often leads to heavy bleeding, like in our case, and if the diagnosis is evident, it could be avoided. The overall prognosis is very good if a prompt diagnosis is made and care is provided in a centre with experience in the management of these cases.


Left innominate vein aneurysm is extremely rare, with a limited number of case reports present in the literature. Herein, we report a case of a 50-year-old female patient presenting with an incidental finding of an anterior mediastinal mass on chest radiography during a routine health examination. Contrast-enhanced computerized tomography (CT) of the chest showed a 4.8 × 4.6 cm anterior mediastinal mass with significant homogenous enhancement after injection of the contrast medium, suggesting a diagnosis of Castleman's disease, but not excluding thymoma. The patient underwent surgical resection of the anterior mediastinal mass under a thoracoscopic approach. Postoperative pathology confirmed the diagnosis of a left innominate vein aneurysm. This is the first case reporting a left innominate vein aneurysm resected under thoracoscopy. Despite this successful treatment experience, we need to emphasize that open thoracotomy or median sternotomy should be chosen as the first choice for surgeons who lack experience in thoracoscopic surgery, with the aim of avoiding intraoperative accidents. Mediastinal venous aneurysm is extremely rare and can be easily misdiagnosed . Its etiA 50-year-old female patient presented with the incidental finding of an anterior mediastinal mass on chest radiography during a routine health examination in October 2020. No related subjective symptoms were reported and the physical examination showed no abnormalities. Contrast-enhanced computerized tomography (CT) of the chest showed a 4.8 × 4.6 cm anterior mediastinal mass with significant homogenous enhancement after injection of the contrast , suggesting a diagnosis of Castleman's disease, but not excluding thymoma .The patient underwent surgical resection of the anterior mediastinal mass under a thoracoscopic approach. After the intubation of the single lumen tube with a bronchial obstruction balloon, single lung ventilation on the contralateral side was performed under general anesthesia. The patient was placed in the lateral position with a retroversion of 30°. The operation was performed via a single-utility port: the endoscopic port was a 1-cm incision at the 6th intercostal space in the midclavicular line, and the utility port was a 3-cm incision at the 4th intercostal space along the anterior axillary line. Intraoperatively, the anterior mediastinal mass was determined to be an aneurysm originating from the left innominate vein. Through careful surgical manipulations and adequate exposure of the operative field, the root of the aneurysm was fully dissected and ligated, and then the root vessel was divided with a cutter closure . PostopeMediastinal venous aneurysms are extremely rare and only a few cases have been reported in the literature, most of which occur in the superior vena cava . MediastLeft innominate aneurysms are most often identified on CT and may be difficult to distinguish from other anterior mediastinal masses such as thymoma . It is iTreatment modalities for mediastinal venous aneurysms include endovascular intervention, observation, and surgical resection. The role of endovascular interventions for mediastinal venous aneurysms is unclear . Follow-No specific guidelines exist guiding how to select the appropriate surgical approach when the resection of the aneurysm is required. It has been suggested that median sternotomy is the best surgical approach due to the ease of controlling bleeding, revascularization, and removal of the embolism during surgery . Fang etIn summary, we believe that this report emphasizes the necessary awareness of thoracic surgeons that left innominate vein aneurysm should be included in the differential diagnosis of anterior mediastinal mass. Thoracoscopic resection of the left innominate vein aneurysm is feasible under the hands of an experienced surgeon. Careful intraoperative handling and adequate exposure of the operative field are prerequisites for a successful surgical intervention. Undoubtedly, minimal surgical trauma contributed to the patient's rapid post-operative recovery. Nevertheless, we need to aware that open thoracotomy or median sternotomy should be selected as the first choice for surgeons who lack sufficient experience in thoracoscopic surgery, with the aim of avoiding intraoperative accidents.The original contributions presented in the study are included in the article/The studies involving human participants were reviewed and approved by Clinical Research Ethics Committee of the Second Xiangya Hospital. The patients/participants provided their written informed consent to participate in this study. Written informed consent was obtained from the individual(s) for the publication of any potentially identifiable images or data included in this article.YH drafted and edited this manuscript, assisted in the surgery, and analyzed the patient data. SR edited this manuscript and analyzed the patient data. CZ, JL, LW, PX, and FY analyzed the patient data. MZ assisted in the surgery. WL performed the surgery, edited this manuscript, and analyzed the patient data. All authors have read and approved the final manuscript.This work was supported by National Natural Science Foundation of China (81972638 and 81972195), Natural Science Foundation of Hunan Province, China (2019JJ30038), the Hunan Provincial Key Area R&D Program (2019SK2253), the Scientific Research Program of Hunan Provincial Health Commission (20201047), and the Clinical Medical Technology Innovation Guide Project of Hunan Province (S2020SFYLJS0311).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.


Vagal paraganglioma is a rare finding that develops from paraganglionic tissue found around the vagus nerve; it has a prevalence of 0.012% of all tumors. It is the third most common paraganglioma of the head and neck but still accounts for less than 5% of these tumors, and it has a well-established female prevalence. It is a difficult tumor to identify early based on its symptoms alone and only a thorough investigation can help solidify its diagnosis. In this report, we discuss a presentation of this phenomenon that is not only unique in its manifestation but also a very difficult diagnosis due to its deceptive presentation and multiple extensions. These masses need a good surgical regime to be removed properly and postoperative complications are very frequent in most of these cases. Vagal paragangliomas are rare findings that develop from paraganglionic tissue found around the vagus nerve . They arA 31-year-old male presented to the vascular surgery clinic with a history of multiple neck swellings and intermittent fever for six years. Initially, it had been just one swelling on the right lateral neck but over time, the swellings had increased in size and number. They were painful and tender to the touch and the pain intensity had increased over the years. The pain was non-radiating and was not associated with any nausea or vomiting, no difficulty in breathing, or weight loss. However, the swellings were associated with a fever of 101-102 °F. The fever was intermittent in pattern, was not associated with any rigors and chills, any cough, or sore throat. The fever was relieved upon taking acetaminophen, and the patient's workup for other causes responsible for the fever was unremarkable.The patient had woken up one day with a stiff neck and had been unable to rotate his neck with only slight movements possible. He had experienced excruciating pain in the neck swelling, which he described as an 8/10 in intensity. This pain he described had radiated from his throat into his right ear. He had also complained of pain in his right shoulder but no loss of shoulder movement. He had also started experiencing pain in the ipsilateral ear along with tinnitus. Along with the pain, he had experienced dysphagia. There had been no loss of hearing, no fainting spells, or hoarseness of voice. This had been accompanied by a loss of control of his right lower lip.For this episode of neck stiffness, he had gone to the local general practitioner where an ultrasound of his neck had been performed, which had revealed a right carotid body tumor. The general practitioner had prescribed pain medications and referred him to the hospital. He had been referred from one hospital to the other until he had reached a tertiary care hospital where an MRI with contrast of the neck had revealed a mass, measuring 50 x 26 x 28 mm on the right side of the neck extending superiorly up into the base of the skull and inferiorly at the level of the thyroid cartilage, as shown in Figure The patient was referred to the vascular surgery department of our institution in June 2021. His case was discussed in a multidisciplinary committee with the radiologists, which recommended surgical excision.Under general anesthesia, an incision was made in front of the anterior border of the sternocleidomastoid muscle, extending to the tragus. We noticed a globular, cystic, non-pulsatile mass adherent to the carotid sheath. Upon opening the sheath, the mass had ramifications between the common carotid artery and internal jugular vein, reaching up into sphenoid recess superiorly and inferiorly; the tumor was found encasing the vagus nerve, as shown in Figure On the immediate postoperative day, the patient developed hoarseness, difficulty in swallowing, and deviation of the angle of the mouth to left. This was attributed to the marginal mandibular nerve being injured during the operation, despite careful dissection. He also lost his ipsilateral nasolabial folds. He was put on nasogastric feeding, which was switched to semisolid food on the third postoperative day. His hoarseness settled on the second postoperative day. On indirect laryngoscopy, ipsilateral vocal cord paralysis was confirmed. His hoarseness was temporary as the damaged vocal cord was compensated for by the adjacent vocal cord. He was discharged home on the fourth postoperative day on a semisolid oral diet. The histopathological report confirmed the suspicion of vagus nerve paraganglioma and indicated an encapsulated tumor with zellballen architecture. The sample stained positive for S100, synaptophysin, and chromogranin. The lymph nodes examined showed no evidence of tuberculosis or malignancy. In conclusion, a paraganglioma of the vagus nerve with reactive hyperplasia of the neck nodes was present.Vagal paragangliomas usually arise in the parapharyngeal space though there is evidence of cervical paragangliomas growing along the cervical course of the vagus nerve . They frThe association of head and neck paragangliomas with catecholamine hypersecretion is a rare incidence, and hence, urinary catecholamine screening is often performed only in the presence of symptoms like tachycardia, hypertension, or, as in our case, a family history of paraganglioma . Just liGenetic tests are recommended in patients with any of the following risk factors: >50 years of age, family history, extra-adrenal tumors, multiple and/or metastatic tumors, and/or elevated dopamine and methoxytyramine levels. In the patient presented in this report, genetic testing was not performed owing to a negative personal, medical, and family history of paragangliomas or other neuroendocrine tumors [Preoperative diagnosis of vagal paraganglioma, even though difficult, can be made by combining clinical findings and radiographic studies . These tThe differential diagnosis in our patient involved vagal paraganglioma, schwannoma, neuroma, jugular meningioma, carotid body paraganglioma as well as jugular and tympanic paragangliomas. Due to their similar clinical presentation, they were high in our differentials. However, schwannoma shows moderate enhancement while paragangliomas reveal avid enhancement on imaging. Histologic characteristics of schwannomas include alternating Antoni A and B regions, which are distinctly different from paragangliomas. Displacement of the common or internal carotid arteries is also characteristic of these tumors. They do not demonstrate flow voids ,13. HencVagus paraganglia are morphologically identical to the carotid body with glomus cells (type I), located within the nerve or adjacent to the nerve. Both show the characteristic “zellballen” pattern, which is a reticular network of interlacing neuroepithelial sheets of varying thicknesses, separated by a rich microvascular bed. Microscopically, this “zellballen” pattern takes the appearance of roundish or elongated oval clusters of cells ,14. The Vagal paraganglioma in the parapharyngeal space has a rich blood supply and is closely related to the internal and external carotid arteries, internal jugular vein, and posterior cranial nerves. Surgical resection should be initially considered. The key to a successful outcome is a calculated approach to obtain excision of the tumor while paying full attention to protect the internal carotid artery. It can often be difficult to separate the nerve from the tumor capsule . SurgicaSurgical morbidity associated with vagal deficits is unavoidable. The literature clearly shows that vagal function cannot always be preserved even when the nerve is anatomically intact . ReportsParaganglioma of the head and neck is a rare finding, especially in such an age as our patient's. Clinically, it is extremely difficult to differentiate between swellings of the neck as most of them have a similar presentation. CT and MRI are the imaging modalities of choice. Despite performing preoperative radiographic studies , the vagal origin of the tumor was a diagnostic surprise. Surgical exploration was performed based on clinical suspicion of a carotid body tumor. Angiographic signs and findings were not accurately analyzed due to the fact that the swelling was behind the carotid bifurcation rather than involving it. Moreover, histologically it is very difficult to differentiate between carotid body paraganglioma and a vagus nerve paraganglioma, as they both have positive stains due to their similar embryological origin. Only the actual position of the swelling can reveal the exact nature of involvement. Histology is extremely useful in deciding if the swelling is metastatic or not. These masses need a good surgical regime to be removed properly and postoperative complications are unavoidable in most cases. However, with good surgical acumen and technique along with a routine follow-up, the prognosis can be easily improved.


The diseases that affect the thalamus are heterogeneous in their etiologies, including infectious, inflammatory, vascular, toxic-metabolic, and neoplastic causes. It is often difficult to make the clinical differentiation between different entities. Within this context, computed tomography and magnetic resonance imaging have come to be of fundamental importance for defining the etiology and planning the treatment. In this pictorial essay, we will illustrate the main causes of diseases affecting the thalamus, discussing the possible differential diagnoses, as well as the most relevant imaging aspects. There are numerous etiologies associated with thalamic lesions, and it is essential to investigate the clinical and biochemical history of the patient before requesting an imaging examination. Recent studies in the radiology literature of Brazil have highlighted the importance of imaging examinations for improving the diagnosis of neurological diseasesIn this study, we will discuss the imaging findings of thalamic lesions on computed tomography (CT) and magnetic resonance imaging (MRI), organized by etiology, including infectious/inflammatory, vascular, toxic-metabolic, and neoplastic causes.. The characteristic clinical finding is a rapid decline in cognitive function. The “pulvinar” and “hockey stick” signs are typical of the variant form and are characterized by restricted diffusion on diffusion-weighted functional MRI (DfMRI), with hyperintense signals in T2-weighted and fluid-attenuated inversion recovery (FLAIR) sequences in the posterior thalamus and posteromedial thalamus, respectively , together with contrast uptake, which can be nodular or peripheral, the latter potentially presenting the eccentric target sign (Caused by get sign , a commo.Numerous viruses can cause encephalitis, and thalamic involvement is quite common in flavivirus infection. MRI can demonstrate symmetrical or asymmetric thalamic involvement, characterized by hyperintensity in T2-weighted sequences, together with enhancement and variable restricted diffusion on DfMRI. There can be foci of bleeding, even in cases of dengue encephalitis. It can be accompanied by a longitudinally extensive spinal cord lesion. Acute necrotizing encephalopathy in childhood is another immune-mediated disease that goes into the differential diagnosis of bilateral thalamic involvement in pediatric patients.Acute disseminated encephalomyelitis is a demyelinating, immune-mediated disease that is classically monophasic. It is most common in children and is usually preceded by viral infection or vaccination. On MRI, it presents as small or swollen, asymmetric lesions that are hyperintense in T2-weighted sequences, with variable contrast enhancement, which may have a peripheral and discontinuous appearance, thalamic involvement  being seDeep cerebral venous thrombosis can occur at any age, with a predilection for the female sex, being more common in women using oral contraceptives, as well as in women who are pregnant or have recently given birth. The clinical presentation is variable, although the evolution is more rapid than in cases of superficial venous thrombosis. When it affects the internal cerebral veins and the straight sinus, it usually causes a volume increase in the thalamus, due to edema, which appears on MRI as an area of hyperintensity in T2-weighted and FLAIR sequences , potenti. Thalamic infarcts can give rise to numerous clinical syndromes, depending on the affected region, the most common site being the inferolateral region, the involvement of which can manifest as severe pain not relieved by analgesics, sensory loss, and ataxic hemiparesis. In acute cases, the CT findings can be normal, later evolving to areas of hypodensity in the affected region (The arterial supply of the thalamus is highly variable, its anteroinferior aspect typically receiving its blood supply from the perforating arteries of the anterior circulation, the remainder receiving its blood supply from the posterior circulation, mainly from branches of the P1 and P2 segments of the posterior cerebral arteryd region . MRI is d region ..Posterior reversible encephalopathy syndrome is an entity of varying etiology. Its pathophysiology is characterized by endothelial injury and dysfunction in the mechanisms of cerebral autoregulation. The clinical picture is acute/subacute, classically characterized by headache, a decreased level of consciousness, visual disturbances, and seizures. The typical MRI finding is bilateral symmetrical hyperintensity in T2-weighted and FLAIR sequences, affecting the cortex and the subcortical region, with a predilection for the parieto-occipital region. Involvement of the thalamus is rare, being most common in the central variant, as is involvement of the cerebellum, brain stem, and nucleocapsular region. In adults, most cases manifest as cardiopulmonary arrest, and CT may show diffuse cerebral edema with loss of differentiation between the white and gray matter, together with reversal of the normal attenuation of the white/gray matter and white cerebellum, the latter two having a worse prognosis. MRI is the most sensitive method, the DfMRI sequence showing changes in the first hours.Hypoxic-ischemic encephalopathy can occur at any point in life. In infants, neuroimaging presents different aspects, depending on the severity, the duration of hypoxia, and the age of the child. Thalamic involvement is common in cases of severe hypoxia and in infants at 32-36 weeks of age, typically being accompanied by nucleocapsular involvement and a reduction in white matter volume , probabl.Wernicke’s encephalopathy is an acute neuropsychiatric condition that manifests as ophthalmoplegia/nystagmus, ataxia, and mental confusion secondary to thiamine deficiency, classically associated with alcoholism and malnutrition. On MRI, the typical findings are symmetric areas of hyperintensity in T2-weighted and FLAIR sequences in the paraventricular regions of the thalamus, mammillary bodies, hypothalamus, periaqueductal region, and floor of the fourth ventricle . There c. The most common alteration on MRI is hyperintensity in T2-weighted and FLAIR sequences affecting the cortical gray matter or the subcortical white matter, which may be accompanied by contrast enhancement and restricted diffusion on DfMRI. There can also be involvement at other sites, such as the hippocampus and thalamus, the latter typically being affected in its pulvinar region (Status epilepticus is characterized by prolonged or recurrent seizures, lasting approximately 30 min. In up to 50% of cases, it occurs in patients without a previous history of seizures; it may be related to numerous factors, including hypoxia, encephalitis, and eclampsiar region .,13-15: involvement of the pons, with preservation of its periphery and corticospinal tracts, is indicative of osmotic demyelination syndrome; subcortical calcifications and dentate nuclei are indicative of Fahr’s disease; infarct-like lesions, together with a lactate peak on proton magnetic resonance spectroscopy, are indicative of mitochondrial encephalopathy; restricted diffusion on DfMRI in the thalamus, brainstem, and cerebellum is indicative of maple syrup urine disease; and a hyperdense thalamus on CT is indicative of gangliosidosis.Other diseases that can affect the thalamus include osmotic demyelination syndrome, Fahr’s disease, mitochondrial encephalopathy, maple syrup urine disease, gangliosidosis, and neurofibromatosis type 1 . In patiMany tumors can affect the thalamus. The main primary neoplasm involving the thalamus is diffuse midline glioma with a histone H3-K27M mutation, typically being characterized on MRI as hyperintense lesions in T2-weighted and FLAIR sequences, with restricted diffusion on DfMRI and variable contrast enhancement on gadolinium contrast-enhanced images .Metastases constitute the main malignant condition affecting the brain, being most common at the cortico-subcortical junction, due to the greater vascularization in that region, and less common in the thalamus. On CT and MRI, metastases are characterized by heterogeneous lesions with contrast uptake and signs of hyperperfusion . They shImaging is a very useful tool in patients with thalamic involvement and can add fundamental information for the diagnosis, treatment planning, and follow-up of such involvement. Radiologists should be aware of the neuroimaging patterns in order to be able to contribute to the clinical decision-making process.


Hemangiomas of the small intestine are rare, usually present with symptoms such as anemia, gastrointestinal bleeding or abdominal pain and are resected. We report resection of an incidentally identified cavernous hemangioma of the small intestine that did not present symptoms referable to the hemangioma. Although it was a large lesion, it was resected using laparoscopy and a mini-laparotomy.A 29-year-old otherwise healthy man was referred for evaluation of ileal wall thickening found on a contrast-enhanced computed tomography scan obtained for the workup of chronic diarrhea. Double balloon enteroscopy (DBE) showed a cavernous hemangioma of the small intestine. The lesion was 15 cm and resected using laparoscopy and a mini-laparotomy to prevent future bleeding. The histopathological diagnosis was a cavernous hemangioma of the ileum.Though there have been no reports of the asymptomatic patients of the disease, the recent spread of double balloon enteroscopy and capsule endoscopy will allow us to diagnose more asymptomatic hemangiomas like this patient. Also, this large lesion was able to be resected through a small incision due to its compressible nature.Future studies in asymptomatic patients of the disease may help to determine the optimal management for these patients. Even large hemangiomas are compressible, facilitating minimally invasive resection. •Hemangiomas of the small intestine are rare disease.•They have always been diagnosed with symptoms such as anemia and bleeding so far.•An asymptomatic hemangioma of the small intestine was incidentally identified.•It was big, but laparoscopic resection was possible due to its compressible nature.•To determine the optimal treatment for asymptomatic lesions is a task in the future. Though it was a large lesion, resection using a minimally invasive approach with laparoscopy and mini-laparotomy was possible. This work is reported in line with the SCARE 2020 criteria 2A 29-year-old healthy man was referred because a contrast-enhanced computed tomography scan obtained to evaluate a two-year history of diarrhea showed wall thickening in the ileum . The patDiagnostic laparoscopy was performed first, which revealed a 15 cm × 6 cm red-purple lesion along the serosal surface of the ileum. The lesion was easily extracted manually through a 5 cm incision and a functional end-to-end anastomosis performed after resection . Immedia3There were two notable things regarding this patient. First, the patient had no symptoms referable to the hemangioma. Second, this large lesion was resected through a small incision.In order to review previous reports, PubMed was searched for reports of hemangiomas of the small intestine published after 2000 using the following search terms: “hemangioma”, “small intestine” and “small bowel”. A manual search was then performed based on the references from eligible articles. The language was limited to English. A total of 45 cases were retrieved and reviewed. Most of the 45 patients presented with anemia, gastrointestinal bleeding or abdominal pain. No lesions were found incidentally, and no patient presented with diarrhea.The relationship between diarrhea and the hemangioma in the present patient cannot be known with certainty but given that the diarrhea persisted after resection and that no previously reported patients had diarrhea, the diarrhea had another etiology and the hemangioma was not causing symptoms. Among patients previously reported, none of them were asymptomatic, and we felt that operative resection was indicated to prevent future bleeding. Endoscopic therapy is one of the treatment options and among 45 cases we reviewed, it was performed for 3 lesions Diagnosis of hemangiomas of the small intestine was difficult in the past because there was no way to view the small intestinal mucosa non-invasively. DBE and capsule endoscopy are now widely available and the lumen of the small intestine can be easily imaged. This suggests that there may be more reports of asymptomatic patients with small bowel lesions such as hemangiomas in the future, and the optimal management of these patients will be established.This 15 cm lesion was resected through a small incision in this patient. The diameter of previously reported lesions varied from 0.8 cm to 50 cm with an average size of 7.0 ± 8.8 cm. The lesion in this patient is larger than most but was easily extracted manually through a 5 cm incision. This is likely due to the compressible nature of a hemangioma since they are not true solid tumors but just venous malformations. This large lesion compressed easily at the time of extraction like sponges. In the literature review, there were 3 lesions greater than 15 cm 4An incidentally identified 15 cm hemangioma of the small intestine was resected to prevent future bleeding. Previous reports have only included symptomatic patients, but the advent of imaging of the small intestinal mucosa may lead to the identification of more such lesions. Future studies in asymptomatic patients may help to determine the optimal management for these patients. Even large hemangiomas of the small intestine are compressible, facilitating minimally invasive resection.Authors had no sources of funding.Not applicable.Informed consent for the publication of this work was given by the patient. Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal on request.Not applicable.Takeshi Kano MD.Not commissioned, externally peer-reviewed.TK and KM gathered the patient's data and wrote the manuscript. KM was responsible for the in-patient optimization. SF, RO, YM, AKL and KM reviewed the manuscript. All authors approved the final manuscript.There are no conflicts of interest to be declared.


Mature teratomas are unique and generally benign neoplasms. They are derived from embryonic tissues and typically located within the gonadal region. Primary retroperitoneal teratomas are uncommon in adults and often challenging to treat, given their location and size. Here, we offer a rare case of a large primary retroperitoneal mature cystic teratoma, detected on abdominal ultrasound during the work-up of abdominal bloating and nausea and treated with robot-assisted laparoscopic excision in a 58-year-old male. In this report, we sought to describe the evaluation, treatment, and follow-up of this condition, as well as review the associated literature. Within the realm of neoplasms, teratomas are a rarity and consist of two or more cell types from the three germ layers  . TeratomA 58-year-old male initially presented to his primary care provider for evaluation of abdominal bloating, early satiety, nausea/vomiting, gastroesophageal reflux, and occasional intermittent flank pain. The patient was referred to gastroenterology, and an esophagogastroduodenoscopy (EGD) and colonoscopy with biopsies revealed chronic inflammation on pathology. As the patient had no improvement on medical therapy, an abdominal ultrasound was obtained which revealed a large renal cyst. No other abnormalities were noted at that time. A CT abdomen and pelvis with and without contrast was obtained, revealing what appeared to be a large exophytic right renal cyst measuring 10.1 x 8.8 x 7.0 cm Figures . The patThe patient had a past medical history significant for a left, non-toxic thyroid nodule discovered on ultrasound in February 2021. He had no history of abdominal surgery or genitourinary operations. The patient did not have any family history of genitourinary malignancies or other significant cancer history. At initial presentation, vital signs and physical exam were normal. There were no abdominal masses and his genitourinary exam was negative for scrotal masses. Prior to surgery, his creatinine was 0.92 mg/dL. No tumor markers were drawn, and the patient was scheduled for a right robot-assisted laparoscopic cyst excision/decortication.Right robot-assisted laparoscopic cyst excision began in the typical fashion. Upon encountering the cystic mass, it was noted to have an abnormal tan/brown appearance. In addition, the mass appeared to originate from the retroperitoneum rather than the right kidney, although it significantly displaced the kidney anteriorly. As we proceeded with our dissection, the kidney was noted to have both a superior and inferior vascular supply. The inferior renal artery/vein was significantly distended and unable to be easily mobilized, inhibiting our dissection. Thus, the inferior vascular supply was ligated in order to complete tumor removal. Overall, the dissection was difficult due to the anterior displacement of the right kidney and significant distention of the inferior renal vessels. Once the mass was free from adjacent structures, it was placed into an Endocatch bag and removed. As the bag was drawn through the incision, the cyst wall of the mass ruptured and a thick, yellow fluid with hair was noted. In the end, we performed a right robot-assisted excision of a cystic retroperitoneal mass.The patient tolerated surgery well and discharged on post-operative day 1. After surgery, his creatinine was 0.95 mg/dL, and he was followed closely for two weeks with no significant complications or complaints. Pathology assessment revealed a cyst wall including focal smooth muscle and squamous epithelium with keratinization Figure . The finTeratomas are masses composed of two or more cell types from the three germ layers and may contain any tissue type. Cystic teratomas may contain fully mature elements, such as hair and sebaceous tissue, and they are typically benign compared to their solid counterparts. These neoplasms most commonly arise from the gonads and sacrococcygeal region; but in rare instances, they can arise from the retroperitoneum. The incidence of retroperitoneal teratomas peaks once during the first year of life 43%-55%) and again in early adulthood. Primary retroperitoneal teratomas are exceedingly rare tumors in adults, with few cases presented in the literature . Researc5% and agBenign teratomas are typically asymptomatic and often incidental findings on imaging; however, as these tumors increase in size, various obstructive symptoms can develop. These include back pain, abdominal pain, gastrointestinal symptoms, genitourinary symptoms, lower extremity swelling, and abdominal fullness. In addition, a midline abdominal mass may be palpated on exam . In our As it relates to diagnosis for these masses, radiographic assessment is essential to pre-operative evaluation. Historically, X-rays helped distinguish between calcified components of teratomas; however, ultrasound and CT scans are now used to provide extra information on the mass and its constituents ,2. MRI iSurgical resection is critical to both the diagnosis and treatment of these tumors, whereas radiation and chemotherapy play minor roles in the overall treatment strategy for benign teratomas ,11. HoweSurvival of patients with primary retroperitoneal teratomas is best when surgically managed, with virtually 100% survival rates after five years for benign teratomas . After sPrimary retroperitoneal teratomas are extremely rare tumors in adults. They are usually asymptomatic and incidentally found but may present with abdominal symptoms if large enough. While the diagnosis can be implicated preoperatively on imaging, definitive diagnosis and treatment both require surgical resection of the tumor. Robot-assisted laparoscopic excision is a safe and effective method of surgical management for large primary retroperitoneal mature cystic teratomas. Finally, patients should undergo yearly follow-up for detection of recurrence.


Nowadays, neither imaging nor pathology evaluation can accurately predict the aggressiveness or treatment resistance of pituitary tumors at diagnosis. However, histological examination can provide useful information that might alert clinicians about the nature of pituitary tumors. Here, we describe our experience with a silent corticothoph tumor with unusual pathology, aggressive local invasion and metastatic dissemination during follow-up. We present a 61-year-old man with third cranial nerve palsy at presentation due to invasive pituitary tumor. Subtotal surgical approach was performed with a diagnosis of silent corticotroph tumor but with unusual histological features . After a rapid regrowth, a second surgical intervention achieved successful debulking. Temozolomide treatment followed by stereotactic fractionated radiotherapy associated with temozolomide successfully managed the primary tumor. However, sacral metastasis showed up 6 months after radiotherapy treatment. Due to aggressive distant behavior, a carboplatine-etoposide scheme was decided but the patient died of urinary sepsis 31 months after the first symptoms. Our case report shows how the presentation of a pituitary tumor with aggressive features should raise a suspicion of malignancy and the need of follow up by multidisciplinary team with experience in its management. Metastases may occur even if the primary tumor is well controlled. Most pituitary tumors are benign although they often invade surrounding structures. However, pituitary carcinomas are extremely rare accounting for only 0.1-0.2% of all pituitary tumors. This prevalence may be likely underestimated due to the challenge in diagnosing pituitary carcinoma  without a defined cut-off for Ki-67, mitotic count or p53 immunoreactivity. Thus, the description and definition of risk characteristics are highly relevant but without a separate denomination. Raverot et al. argued for a clinicopathological classification that includes aggressiveness and invasiveness, with aggressive and invasive tumors being more prone to metastatic dissemination (up to 10% of these cases)  scan of the brain region confirmed a 25-mm pituitary mass lesion extending into both left and right cavernous sinuses (Knosp grade 3)  revealed a tumor remnant (20x7x20 mm)  imaging of thorax and abdomen and MRI of the spine revealed no metastatic lesions.2/day for 5 of every 28 days and was well tolerated by the patient. After four cycles, a MRI was performed and revealed size stabilization of the size of the lesion. This led our multidisciplinary team to decide to additionally treat the patient with fractionated stereotactic radiation therapy. Thus, after the eighth temozolomide cycle treatment and 13 months after the initial diagnosis, radiation therapy was administered at a total dose of 50 Gray in 28 fractions and temozolomide dose was switched to 75 mg/m2/day. MRI was performed three months after starting radiation therapy that did not show growth of the tumor remnant. CT of the thorax and abdomen was negative for metastatic disease. Six months after the start of the radiation therapy, the imaging tests were repeated, and the tumor lesion remained stable and campimetric improvement was also achieved. However, the CT scan now showed a mass at S1-S3 level  concomitant with radiation therapy, as in the Stupp protocol. However, the identification of metastasis during the follow-up led us to restart temozolomide monotherapy at the highest recommended dose. Temozolomide treatment was largely ineffective though that combined with the rapid progression of the tumor forced us to consider an alternate oncological treatment. Unfortunately, the literature about second and third line treatments for temozolomide-resistant pituitary tumors is scarce and the reported chemotherapeutic agents have been very heterogeneous. Indeed, the recent ESE guidelines were unable to provide recommendations in this regard. We decided initiated chemotherapy with a combination carboplatin and etoposide [as standard treatment for certain poorly differentiated neuroendocrine tumors  for the publication of any potentially identifiable images or data included in this article.PR-R collected and analyzed the data, and wrote the report. EV-M atended the patient, ED-F atended the patient. JC made literature review. IF atended the patient. MA atended the patient. MJ-R performed the histological examination. FR interpreted MRI image. EF interpreted MRI image. AK performed surgery. ECR-V performed surgery. DC analyzed the data and wrote the report. AS-M coordinated the research, analyzed the data and wrote the report. All authors contributed to the article and approved the submitted version.This work was supported by grants from the ISCIII-Subdirección General de Evaluación y Fomento de la Investigación co-funded with Fondos FEDER (PI16/00175 to AS-M and DC) and the Sistema Andaluz de Salud .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.