Synchronous gastrointestinal multiple primary tumors including gastric, colonic, and rectal cancers are rare. Moreover, it was a challenge to find an appropriate procedure without negatively impacting the overall outcome. We described the case of a 63-year-old woman who presented with a 4 month history of upper abdominal pain, acid regurgitation, and anemia. Gastroscopy with biopsy suggested early cancer of gastric antrum. Abdominal contrast-enhanced computerized tomography and colonoscopy revealed ascending colon and rectum tumors. She had no family history of malignancy. Endoscopic submucosal dissection was performed for gastric cancer, and the pathological result presented that it was poorly differentiated and invaded into deep submucosa. The laparoscopy-assisted radical surgery combined with distal gastrectomy, right hemicolectomy, and anterior resection of rectum was performed for these three tumors via eight ports and a 7 cm midline upper-abdominal incision. No other perioperative complications were encountered except postoperative ileus. The patient was discharged on the 12th postoperative day. The pathological results revealed gastric cancer (T1N0M0), right colonic cancer (T3N1M0), and rectal cancer (T2N0M0), indicating complete surgical resection. We reported that our laparoscopic approach for synchronous triple primary gastrointestinal malignant tumors was feasible and minimally invasive. The incidence of multiple primaries in cancer patients is reported in the range of 2–17% . Synchro22. A gastroscopy was performed and suggested an early-stage gastric cancer lesion located at the antrum with shallow ulcer formation, about 1.5 cm in diameter. The pathology confirmed gastric adenocarcinoma. Abdominal contrast-enhanced computerized tomography (CT) showed a space-occupying lesion of the ascending colon and thickening of the rectal wall with no metastatic lesions. Furthermore, colonoscopy with biopsy revealed ascending colonic cancer with stenosis as well as rectal cancer of 2.5 cm in diameter located 12 cm proximal to the anal verge. Laboratory tests showed elevated serum carbohydrate antigen (CA-199) (30.39 U/mL). Additionally, preoperative chest CT found no lung metastasis.A 63-year-old woman visited the Department of Gastroenterology because of upper abdominal pain with acid regurgitation and anemia for 4 months. Her past medical history included a hysterectomy for benign disease and lumbar disc prolapse. Her body mass index was 27.8 kg/mGiven the possible diagnosis of early-stage gastric cancer, endoscopic submucosal dissection was performed. However, the pathological result presented that it was poorly differentiated and invaded into deep submucosa. Therefore, it was required to manage three tumors with minimal adverse effects; and laparoscopic surgery was considered.Laparoscopic surgery was performed under general anesthesia, and the patient was placed in the lithotomy position. Pneumoperitoneum was established through a Veress needle which was inserted just above the umbilicus. The intra-abdominal pressure was maintained at the level of 12 mmHg. Then a 10 mm port (Port 1) was introduced at the point about 5 cm under the umbilicus for a 30 degree laparoscopic camera. After abdominal exploration, the patient was turned into lithotomy-anti-Trendelenburg position. The first 12 mm port (Port 2) and the first 5 mm port (Port 3) were introduced below the costal margin at the left and right anterior axillary lines, respectively. The position of Port 3 was a little closer to the foot side than Port 2. Then, another 5 mm port (Port 4) was introduced above umbilicus level at left midclavicular lines. Moreover, one more 5 mm port (Port 5) was introduced under the umbilicus level at the left midclavicular line. These five ports were used to perform the right-side approach laparoscopic distal gastrectomy with D2 lymphadenectomy . After tThen, the sac wrist of a glove was used to snare the disposable wound retractor. The glove’s thumb was cut off, and a 10 mm port (Port 7) was inserted into the thumb stump . After tThe duration of surgery was 330 min with a blood loss of 200 mL. The only postoperative complication was postoperative ileus. The patient’s first flatus and stool time were on day 4 and 6 after surgery, respectively. Because of distension, enteral feeding remained a low amount before day 7 and increased to about 80% of target needs on day 10 after surgery. Supplement parental nutrition support was started on day 5 and stopped on day 11 after surgery. The patient was discharged and returned home on day 12 with no other complications.The pathological results revealed stage T1N0M0 gastric adenocarcinoma, stage T3N1M0 adenocarcinoma of the ascending colon, and stage T2N0M0 adenocarcinoma of rectum. And deficient mismatch repair (dMMR) was not found on immunohistochemistry (IHC) testing, which indicated microsatellite stability. The patient received capecitabine and oxaliplatin as postoperative adjuvant chemotherapy. By the time of submission of this manuscript, 1 year after surgery, follow-up CT showed no recurrence, and the patient was capable of general housework.Ethics approval and consent to participate: This study was approved by the Ethics Committee of Tongji Hospital. Consent for this presentation is obtained from the patient in writing.Consent for publication: The patient provided written consent for publication of his case details and medical images.3In general, MPT was considered rare and described in case reports ,8. The iFor patients with resectable gastric, colonic cancer, as well as stage I and II rectal cancer, the surgical procedure was the first recommendation in clinical guidelines ,14,15,16For three tumors to resect, the steps of the whole surgery should be carefully planned. The gastrectomy and colectomy were relatively clean operations. And during these two operations, the monitor was positioned on the head side of the patient. Additionally, one single incision could be used for reconstruction for both the operations. Therefore, these two operations were performed first. In this case, the unit combined the wound retractor, glove, and the port, which was used for the laparoscopic camera and the pneumoperitoneum reestablishing in proctectomy. This simple unit could be applied in many other conditions in our center, like laparoscopic examination before the abdomen closes. In addition, the reconstruction of rectectomy was more accessible with a circular stapler. The upper abdomen incision could be used to excise the tumor and insert the anvil shaft when about 5 cm more proximal descending colon was dissociated. Hence, rectectomy was performed as the second step. For the reason of longer operative time, we did not choose the total laparoscopic approach. The laparoscopic-assisted approach for resection of three tumors would reduce operative time, leading to less complication and better prognosis . EventuaIn this case, the patient had postoperative ileus as the only complication after the surgery. It might be associated with extensive surgical trauma and postoperative inflammation . The posLynch syndrome was the common cause of multiple primaries in patients with colon cancer ,26. HoweWe reported a single case of gastrointestinal triple primary tumors treated by laparoscopic surgery. As long as the surgical procedure details are properly planned and the indications are carefully selected, laparoscopic surgery is safe and feasible as an MPT treatment even in synchronous triple primary gastrointestinal malignant tumors.MPTmultiple primary tumorsCTcomputerized tomographydMMRdeficient mismatch repairIHCimmunohistochemistry


Leiomyomatosis peritonealis disseminata (LPD) is a non-metastatic, homologous, multicentric benign disorder characterized by small leiomyomas scattered over the peritoneum and omentum. It is a rare and benign disease with invasive potential. LPD mainly attacks women of childbearing age but has also been reported in post-menopausal women, men, and young children. Non-specific clinical and imaging findings of LPD lead to difficult diagnoses and treatment.This study reports the case of a patient with recurrent LPD with endometriosis after multiple myomectomies and hysterectomy, who presented recurrent abdominal pain with progressive exacerbation. Imaging examinations showed irregular shadows in the pelvic cavity and multiple nodular changes in the peritoneum, which were considered malignant lesions. A solid mass sized 10 cm × 9 cm × 10 cm in the inferior pelvis and nodules scattered over the surface of pelvic and abdominal organs and the peritoneum were detected during the surgery. The patient was treated with cytoreductive surgery (CRS), peritonectomy, ovarian ablation, and hyperthermic intraperitoneal chemotherapy (HIPEC). The surgery was challenging, and the intraoperative bleeding reached 900 ml. However, the patient recovered well and achieved a tumor-free survival of 13 months.It was concluded that a combination of CRS, ovarectomy, and HIPEC might be one of the therapeutic strategies for recurrent LPD. Additionally, she had been experiencing an aggravation of this pain, accompanied by a sensation of abdominal expansion, for the past month. The patient had a cesarean in 2009 and a laparoscopic myomectomy in 2012 for a uterine fibroid (5 cm) at A hospital. She experienced right lower abdominal pain from 2013. In 2015 and 2018, she had surgeries to remove uterine fibroids and ovarian cysts at B and C hospitals, followed by six cycles of GnRH-a therapy. In November 2018, she received a hysteroscopic levonorgestrel‐releasing intrauterine system at D Hospital and underwent an ultrasound-guided endometriomas puncture in 2019. In 2020, she had a total abdominal hysterectomy, right adnexectomy, left salpingectomy, left ovarian cyst removal, and intestinal adhesion lysis at D hospital. Pathology post-surgery revealed a pelvic nodule, leiomyoma, uterine and cervical adenomyoma, right accessory endometriosis, and left ovarian endometrioma. Following three cycles of postoperative GnRH-a therapy, an ultrasound re-examination revealed a 4 cm solid mass.However, the surgery did not alleviate the patient's right lower abdominal pain. As the pain gradually increased, oral painkillers became necessary for relief. In August 2021, a pelvic MRI and a contrast-enhanced MRI were conducted. They revealed a larger pelvic lesion (9.3 cm × 8.6 cm × 8.6 cm) than before, encroaching on the anterior rectum. Additionally, several nodules in the peritoneum and mesentery raised suspicions of malignancy . A subsequent biopsy of the pelvic cystic lesion suggested a smooth muscle tumor. However, the evidence for malignancy was inconclusive  . The patExacerbating right lower abdominal pain, a heavy feeling, and sporadic back pain led the patient to seek help at the Aerospace Center Hospital. This pain lasted one to 7 h and could be alleviated with oral tramadol. Finally, it is noteworthy that the patient had a two-year history of smoking, consuming seven cigarettes daily. However, she quit smoking five years ago.Physical examinations showed the patient in visible pain with a forced posture. The abdomen was soft and tender in the lower part, with no rebound tenderness. A pelvic examination revealed no hymen or obstruction in the vagina, and the previously sutured end remained smooth. A 10 cm mass was palpable in the pelvic cavity, fixed in the anterior rectum. It had a clear boundary, hard texture, significant tenderness, and limited mobility. The rectal mucosa was smooth, with another hard mass palpable 7 cm from the anus. This mass was mobile and without tenderness or bleeding upon digital rectal examination.An MRI and CT scan of the pelvic area revealed an irregular mass and multiple nodules considered malignant A–G. DuriThe exploratory laparotomy was performed in October 2021 at Aerospace Center Hospital. There were no detectable free ascites. Miliary nodules were present beneath the original incision site. The peritoneum of various areas had scattered miliary nodules ranging from 0.2 to 0.5 cm. Denser nodules of 0.5–1.5 cm were seen in the descending colon and related areas. Neoplastic adhesions sealed the pelvic cavity. During the operation, tissue was collected for pathology. Results hinted at a recurrent LPD spindle cell tumor. The patient then underwent CRS. Multiple resections were performed, including of the pelvic peritoneum, vaginal stump, and others. Full cytoreduction was achieved, denoted by a CC score 0 E and F.The patient had extensive abdominal nodules. Frozen section pathology indicated potential malignancy. Following family consultation, HIPEC treatment with 80 mg cisplatin in 4000 ml saline occurred. It was a challenging seven-hour surgery, with a blood loss of 900 ml. The patient was moved to intensive care afterward. Postoperative pathology showed multiple spindle cell tumors across various areas, with sizes from 0.5 cm × 0.5 cm × 0.5 cm–10 cm × 8 cm × 8 cm. Other findings included a pelvic tumor from endometriosis, a corpus luteum and corpus albicans in the left ovary, and chronic appendicitis. The fibrofatty tissues in the ligamentum teres hepatis and tumors were not detectable. Immunohistochemistry showed positive results for CD34, SMA, desmin, focal CD10, ER, PR, and 2% Ki-67, but negative for CD117, S-100, and Dog-1 A–I.Fig. Eleven days post-surgery, the patient was discharged. She was followed up for 13 months, showing tumor-free survival H–I. Whil3LPD is a rare disease with invasive potential, but it is histologically benign. It mainly affects women of childbearing age, but it occasionally affects post-menopausal women ,3, youngThe pathogenesis of LPD has not been fully elucidated. LPD lesions shrank or regressed after GnRH-a therapy or treatment with aromatase inhibitors, further confirming the hormone dependence theory. In this case, postoperative positive estrogen and progesterone receptors and 13 months of tumor-free survival after ovarian ablation were consistent with the hormone dependence theory. However, short-term ovarian ablation with six cycles of GnRH-a after the fourth operation and three cycles of GnRH-a after the seventh unilateral oophorectomy failed to prevent the development of uterine fibroids, adenomyoma, and endometriosis. It could also not prevent the recurrence of LPD, which could not be explained by the hormone dependence theory. LPD also affects post-menopausal women, men, and infants, indicating the potential involvement of other theories.Second, LPD may be caused by the metaplastic potential of stem cells within the peritoneal cavity . The recThird, the iatrogenic theory is involved in the pathogenesis of LPD. The patient was treated with an unindicated cesarean section followed by a laparoscopic myomectomy. According to the operation time and the hospital, unprotected intraabdominal morcellation was not applied. Immunohistochemical staining showed that SMA and desmin were strongly positive, suggesting that LPD shares partial common molecular and cytogenetic characteristics with those of uterine leiomyoma. It cannot be ruled out that the two surgeries jointly contributed to LPD and endometriosis, which accelerated the generation of totipotent stem cells in the subcutaneous peritoneum.MED12 gene [Fourth, genetics theory is linked with the pathogenesis of LPD, and familial clustering of LPD has been reported . ChromosD12 gene  are invoIt is challenging to distinguish LPD from peritoneal implants of small malignancies based on preoperative imaging. Moreover, clinical manifestations of LPD are atypical and are mainly diagnosed through postoperative pathology. So far, global consensuses for treating LPD are scant . In thisCurrently, CRS achieved by peritonectomy procedures and en-bloc resection of the viscera has been widely applied to treat tumors on the surface of the peritoneum, significantly reducing the recurrent rate and enhancing long-term survival. Multiple miliary nodules were examined in the patient's pelvic cavity, peritoneum, and omentum. The assessment of tumor burdens during surgical exploration is of great significance and ensures the feasibility of peritonectomy .CRS is the most effective treatment for advanced epithelial ovarian malignancies, followed by intravenous paclitaxel and platinum chemotherapy. Systemic intravenous chemotherapy, however, is less effective in treating metastases due to poor peritoneal blood flow and less penetration of drugs into tumors. HIPEC has become a novel alternative that applies heated chemotherapy directly to abdominal tumors, causing cytotoxicity. HIPEC has emerged as an alternative to adjuvant or neoadjuvant therapy for advanced ovarian cancer and other malignant diseases with extensive peritoneal metastasis. In this case, we used HIPEC first in the world in benign-disseminated disorder because of potential malignancy indicated by intraoperative pathological diagnosis and fully informed consent from her family. Moreover, her clinical outcome has been great so far. The patient did not have HIPEC-induced adverse events, and the postoperative liver and kidney functions were normal. The use of HIPEC in benign-disseminated tumors needs cautious, and its effects are inconclusive.During the surgery, malignant peritoneal implantation and metastasis could not be determined by the intraoperative frozen section pathology. After full communication with family members, HIPEC was actively performed to clear residual small tumors and free tumor cells in the pelvic cavity as much as possible, even though her CC score was zero. Heat is directly cytotoxic, enhances chemotherapeutic drug penetration into tumors, and synergizes with cisplatin to induce apoptosis in residual leiomyoma cells, which minimizes systemic adverse events. The patient did not have HIPEC-induced adverse events, and the postoperative liver and kidney functions were normal. The therapeutic effect of HIPEC in benign-disseminated tumors needs further exploration.The study indicated the efficacy of CRS and ovarian ablation in recurrent LPD combined with endometriosis. This study reported, for the first time, the application of HIPEC to a patient with undetermined intraoperative pathology and extensive involvement of tumors in the abdominal cavity. This treatment may serve as a novel therapeutic strategy for recurrent LPD combined with endometriosis. The patient's quality of life after ovarian ablation deserves attention. Peri-menopausal symptoms and osteoporosis caused by low-level estrogen are the main adverse events after ovarian ablation in women of childbearing age. Moreover, low-level estrogen negatively influences bone metabolism, and the patient developed obvious pelvic pain after the surgery. Based on the add-back therapy for endometriosis after GnRH-a administration, the effect of low-dose estrogen supplements that improve the quality of life will be closely monitored . In addiAerospace Center Hospital. Written consent was obtained from the patient.The study was reviewed and approved by the Ethics Committees of the Written informed consent was obtained from the patient to publish this case report and any accompanying images. A copy of written consent is available for review by the Editor-in-Chief of this journal.None.All authors listed have significantly contributed to the investigation, development and writing of this article.Data will be made available on request.The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper.


Infantile fibrosarcoma is a rare pediatric soft tissue tumor and usually appears in children before one year of age. Distal extremities constitute the most frequently affected locations, and other tissues such as the trunk, head and neck, gut, sacrococcygeal region, and viscera are uncommon sites.We describe a rare case of infantile fibrosarcoma arising from the perineum. First, a cystic mass was detected using prenatal ultrasonography, and then an echo was changed in serial ultrasound examinations. A solid cystic lesion was found at term; a hypoechoic lesion occurred in the back. The tumor became so large that massive bleeding occurred, which then underwent surgical resection. Pathological examination confirmed infantile fibrosarcoma.Our report demonstrates not all ultrasonographic findings in cases of infantile fibrosarcoma exhibit a solid mass during the initial examination — an early-stage lesion may reveal a cystic echo. Infantile fibrosarcoma has a good prognosis and surgery constitute the main treatment, with adjuvant chemotherapy being received if necessary. Infantile fibrosarcoma is a rare malignant soft tissue tumor composed of mesenchymal fibroblasts, histologically is characterized by uniform spindle-shaped cells packed in a herringbone pattern. It usually occurs in superficial or deep tissues of distal extremities and then extends to other surrounding soft tissues , and othA newborn female infant was referred to our hospital due to a perineal tumor with dorsal metastasis on day 1. At 24 gestational weeks, prenatal ultrasonography depicted a cystic mass in the perineum that measured 2 × 1 cm in size Fig. a. Thick Infantile fibrosarcoma, a rare mesenchymal soft tissue tumor, is one of the non-rhabdomyosarcoma sarcomas, which usually appears in children before one year of age and affects about five neonates per 1 million infants . AccordiThey usually appear on ultrasounds as a well-circumscribed, hypoechoic soft tissue mass with rich blood flow signals , 13. In In the present case, infantile fibrosarcoma is huge and difficult to differentiate from rhabdomyosarcoma; nevertheless, ultrasonography could provide an efficient method for preoperative clinical evaluation. Perineal tumors near the sacrococcygeal region are often misdiagnosed as sacrococcygeal teratoma, one of the most common fetal tumors which arises from pluripotent embryonic stem cells. Sacrococcygeal teratoma typically presents with sacral mass, and on ultrasonography it appears as cystic, solid (most frequently), mixed. The majority grow slowly, but some with abundant blood supply can rapidly grow, which brings on fetal edema, polyhydramnios and fetal heart failure , 19. SacPathological examination is utilized to diagnose patients with infantile fibrosarcoma . Using mInfantile fibrosarcoma has a good prognosis, and the rate of metastasis is less than 10% , 7. DaniDaisuke Nonaka et al.  describeInfantile fibrosarcoma is a rare soft tissue tumor but is locally invasive, having an excellent prognosis and a low transfer rate. Previous reports have demonstrated that the tumor usually appears on ultrasounds as a hypoechoic soft tissue mass. In our study, a cystic mass was detected at first, which later became a solid cystic mass and finally exhibited a solid mass after birth. Although it is rather difficult to differentiate from rhabdomyosarcoma based on imaging studies, ultrasonography plays an important role in making an earlier diagnosis, monitoring the disease process, detecting metastasis, and guiding clinical decision-making.


Radiotherapy was planned post-operatively.A 78-year-old woman presented to the emergency department with mild headaches and a sudden onset of blurred vision. Computerized tomography scan and magnetic resonance imaging showed what was described at first as a meningioma invading and occluding the torcular Herophili, the posterior third of the superior sagittal sinus and the proximal part of the right transverse sinus. Gross total resection of the tumor was performed without reconstructing dural sinuses. The patient was discharged home without new neurological deficit. Histopathology was in favor of a hemangiopericytoma Grade II World Health Organization (WHO). Total body positron emission tomography [ Hemangiopericytomas (HPCs) are a rare mesenchymal tumor. They represent approximately 1% of all intracranial tumors are thought to originate from Zimmerman's pericytes  with a tHPCs are more common in males and the mean age of diagnosis is lower compared with meningiomas . The extA 78-year-old woman was admitted to the hospital for sudden onset of bilateral blurred vision associated with mild headaches. Neurological examination showed no other neurological deficit. The ophthalmological report found no sign of papilledema and described a loss of central visual field bilaterally. A computerized tomography (CT) scan reported a medial occipital iso-dense multi-lobulated lesion with occipital bone erosion and intense contrast enhancement. Magnetic resonance imaging (MRI) imaging showed an invasion of the posterior third of the superior sagittal sinus (SSS), the confluence of sinuses (CoS), and the proximal third of the right transverse sinus (RTS) with bone invasion . The rad®. Total operative time was 215 minutes, and total blood loss was estimated at 2 L. Two red blood cell units were required during the operation. The immediate post-operative period was marked by a worsening of the vision followed by a progressive improvement during the following days. The patient stayed 2 days in the intensive care unit and was discharged on day 7 after surgery. Post-operative MRI showed complete resection and no recurrence at 3, 6, and 12 months /HPC have a higher diffusion coefficient, lack a dural tail, or present with a narrow-based dural attachment. They also tend to present with less peritumoral brain edema and show extensive serpentine flow void corresponding to higher vasculature. This study also showed fewer differences between angiomatous meningiomas and SFT/HPC .On diffusion imaging, the normalized Apparent Diffusion Coefficient (ADC) values (nADC) and degree of intratumoral susceptibility signal intensity (ITSS) were significantly higher in intracranial hemangiopericytoma than those in meningiomas . The thrIn MR spectrography myo-inositol values superior or equal to 6,347 in short echo-time were associated with the diagnosis of HPC/SFT brain tumors with high sensitivity and specificity [It has to be mentioned that the definitive diagnosis of an HPC can only be made after a histopathology study  but the Patients suffering from HPC have a lower life expectancy compared to those with meningiomas because of the higher rate of recurrence and the risks of extracranial metastasis . Gubian Comparatively, the 5-year survival rate for Grade I meningiomas is estimated at 91.3% . For GraPORT is a debated matter in the literature. Some authors showed real benefits after GTR and partial resection with a reduction in local recurrence , 20. OthDue to the high local recurrence rate and distant metastasis of HPCs an aggressive surgery should be attempted when possible. For hemangiopericytoma invading the CoS symptom severity depends largely on the efficiency of the venous drainage. To the best of our knowledge, only three cases of CoS HPCs with dural sinus reconstruction have been reported. The first was in 1994 by Nagashima et al.  where thAt first, we had some confidence that the tumor was an HPC so we preoperatively planned a GTR with the reconstruction of the sinus using the pericardial patch. During surgery, because of the heavy bleeding, we opted for an en-bloc resection. Our decision was supported by several pre- and post-operative findings. First, the tumor invaded the entirety of the sinus walls, leaving no intact tissue that could be salvaged in a reconstruction. Second, the patient had a chronic and complete obstruction of the CoS, SSS, and proximal third of the RTS with no signs of increased ICP or venous infarction. Furthermore, the angiogram showed efficient venous drainage as proven by the early opacification of the sigmoid sinus after cortical venous outflows. Moreover, after occlusion of the sinuses with silk per-operatively we encountered no brain swelling or venous infarction. Additionally, we encountered no venous drainage in the bone or the scalp, which further suggests a good intracranial venous outflow. Finally, since the patient was 78 years old we wanted to prevent high morbidity that is associated with an extended surgical time due to the reconstruction of dural sinuses. Good clinical result was achieved as shown by the rapid recovery of the patient and the absence of post-operative complications.The patient received a 60 Gy dose of radiation therapy for several reasons. Despite her age, she was in a very good health condition and only had a history of mild hypothyroidism. Moreover, since the topic is still very debated in the literature no real guidelines are established. Finally, our team wanted to prevent a local recurrence as much as possible and the patient agreed to the proposed treatment.The use of preoperative embolization is known to reduce preoperative bleeding as well as decrease mortality and morbidity related to hemangiopericytoma surgery , 24. UnfIn this report, we describe the resection of a CoS HPC without reconstruction of the SSS and the RTS. This surgical strategy is viable in very select cases where cerebral venous drainage is not compromised. We also described some of the radiological differences between HPC and meningiomas, which can be helpful in the decision-making process for the surgical strategy. A cerebral angiogram should be mandatory in cases involving dural sinuses for the assessment of arterial supply and venous drainage. Preoperative embolization can be of good use in reducing preoperative bleeding and cases that could benefit from it should be carefully selected. Finally, GTR should always be the first intention when possible and radiotherapy should be administered to reduce as much as possible the local recurrence.


Ureteral hernias are an uncommon entity that are usually incidentally discovered during inguinal hernia repair. However, when symptomatic, they could cause severe symptoms from the urinary system and even affect renal function. We aim to report a rare case of a 91-year-old male patient with urosepsis because of ureteral entrapment within an inguinal hernia, and further discuss proper management of such cases. Inguinal hernia repair is one of the most frequent surgical procedures, with ~20 million repairs performed worldwide annually . Common Therefore, we aim to describe a rare case of urosepsis because of ureteral entrapment in a large inguinal hernia and discuss on proper management.A 91-year-old patient with an indwelling urinary catheter presented to the Emergency Department complaining about flank pain, a feeling of pressure in the suprapubic area and fever (temperature = 39°C). His past medical history included benign prostate hyperplasia, type II diabetes mellitus, and arterial hypertension. There was no history of prior abdominal surgery. Physical examination revealed a soft, non-tender abdomen, with a non-tender, non-reducible right inguinal hernia. Laboratory workup was significant for marked leukocytosis (white cell count 22 × 103/ml), increased inflammatory markers, and elevated blood urea and creatinine. Ultrasonographic examination revealed a pelvicalyceal dilatation of the right kidney. The patient was admitted to the hospital for antibiotic treatment and further investigation.A computed tomography (CT) was ordered that revealed a significant pelvicalyceal dilatation and hydronephrosis of the right kidney consistent with obstructive uropathy and a medium-sized right inguinal hernia containing fat, bowel loops, and the right ureter .Following multidisciplinary consultation, a stent placement was considered too dangerous for ureteral injury because of acute angulation. Therefore, a primary repair of the inguinal hernia was decided. Because of the patient’s comorbidities, an open approach was favored. Intraoperative dissection revealed an indirect hernia with protrusion of a peritoneal sac containing small bowel loops and large amount of retroperitoneal fat . The herWe reported a rare case of inguinal hernia entrapping the ureter and causing hydronephrosis and urosepsis.Ureteral inguinal hernias more commonly occur in men during the fifth and sixth decades of their life although our patient was over 90 years old . UreteraAdditionally, ureteral hernias are predominantly indirect , and incarceration is relatively infrequent because of the hernia’s large size . Direct Ureteral hernias are of two anatomical types. A paraperitoneal type, where the ureter slides beside a peritoneal sac and constitutes part of the hernia wall, occurs in 80% of cases. Less commonly an extraperitoneal type occurs (20%), where the ureter is accompanied only by retroperitoneal fat and no peritoneal sac is present . Paraperet al. [Certain conditions, such as kidney transplants and obesity, increase the risk of developing these hernias. The tortuous nature of the ureter in ectopic kidneys makes them more susceptible to herniation and potentially obstruction. There have been cases with bilateral ureteral obstruction because of inguinal hernia as well . Howeveret al.  have proUreteral inguinal hernias comprise a significant challenge for the average surgeon. Although rare, clinical suspicion should be high in patients with both signs of obstructive uropathy and an inguinal hernia. Minimally invasive approaches can be challenging and should be attempted only by experienced surgeons. Preoperative ureteral stenting is recommended when feasible. An open repair is a safe option but careful sac dissection or intraoperative use of indocyanine green stain is necessary to avoid ureteral injury.


Hydatidiform mole, complete or partial (CHM/PHM), is the most common type of gestational trophoblastic disease (GTD), which is characterized by excessive trophoblastic proliferation and abnormal embryonic development. Some patients present with sporadic or familiar recurrent hydatidiform moles (RHMs), which are characterized by two or more episodes of the disease. A healthy 36-year-old woman was admitted to the Obstetrics and Gynecology Unit of Santa Maria Goretti Hospital, Latina, because of RHMs at 6 weeks of amenorrhea, with an obstetrical anamnesis of RHMs. We performed uterine dilatation and curettage with suction evacuation. The histological examination confirmed the diagnosis of PHM. The clinical follow-up was conducted according to recent guidelines on the diagnosis and management of GTD. After the return to the baseline values of the beta-human chorionic gonadotropin hormone, a combined oral contraceptive therapy was proposed, and the patient was invited to undergo in vitro fertilization (IVF) techniques, specifically oocyte donation, to reduce the possibility of similar future cases of RHMs. Although some etiopathogenetic mechanisms involved in RHMs are still unknown, all patients of childbearing age who are affected by this syndrome should be properly treated and directed towards a correct clinical path as IVF, to have a successful and safe pregnancy. Gestational trophoblastic disease (GTD) is a spectrum of cellular proliferations arising from the placental villous trophoblast including four main clinical forms: complete hydatidiform mole (CHM), partial hydatidiform mole (PHM), invasive mole, choriocarcinoma, and placental site trophoblastic tumour (PSTT), and epithelioid trophoblastic tumor (ETT) . The latThe GTD spectrum has recently been expanded to include atypical placental site nodules, which may coexist and develop into PSTT/ETT in 10–15% of the cases . The incUltrasound is the standard imaging modality for identifying molar pregnancy. Classically, a ‘snowstorm pattern' has been described, resulting from the presence of a complex vesicular intrauterine mass containing many ‘grape-like' cysts with no foetal tissue (CHM) or only partial tissue (PHM). Ultrasound evaluation of the adnexa can also reveal theca lutein cysts, due to ovarian stimulation by abnormally elevated beta-human chorionic gonadotropin (hCG) levels. Moreover, other different imaging techniques in the diagnosis and management of GTD, such as pelvic magnetic resonance imaging can represent a problem-solving tool to assess the depth of myometrial invasion and extrauterine disease spread in equivocal and complicated cases .CHM histology consists of hydropic villi in semitransparent vesicles of variable size with the absence of normal placenta tissue, and in some cases of early CHM, no clear evidence of abnormal villi could be detected. Otherwise, in the case of PHM, normal cytotrophoblast tissue or foetal/adnexal part could be detected. The cytogenetics of CHM and PHM are different: typically, CHM is diploid and has 46 XX chromosomes with both X chromosomes from the paternal line, whereas PHM is triploid with the maternal and paternal genetic origin . The recWe present a case of a 36-year-old Asian patient, gravida 3, para 0, at 5 weeks of amenorrhea, presented at our attention at the Obstetrics and Gynecology Unit, Santa Maria Goretti Hospital, Latina, in October 2022. The pregnancy was spontaneously conceived, the woman was in good global condition and was not affected by diseases or allergies. Obstetrical anamnesis of the patient reported two previous episodes of GTD, a PHM, and a CHM, interrupted at 14 and 8 weeks, respectively, and a spontaneous pregnancy loss at week 7 of amenorrhea . In her previous pregnancies, the patient had visited three different other hospitals and did not bring any related documentation to our attention for inspection. The patient also reports that she was adopted, therefore, we cannot trace her family history, especially relating to previous cases of repeated miscarriages or molar pregnancies in her family tree. All the previous pregnancies were conceived with the same partner. After our first transvaginal (TV) ultrasound evaluation at week 6 of amenorrhea . Then, wDilatation and curettage (D&C) with suction evacuation was performed on 20 October 2022, under ultrasound guidance. The procedure was performed in general anaesthesia with a 12–14 mm suction cannula and 10 IU of intravenous oxytocin. During and after the procedure blood loss was regular, without the need for blood transfusion, as well as Rhesus immune globulin prophylaxis was not performed because of the positive blood group of the patient. The woman was discharged from the hospital on the same day of the operation in global and local good condition, after performing a TV ultrasound showing a thin and regular endometrial line , and witGTD is still a complex and intriguing obstetrical disease, especially in the case of RHMs, where early diagnosis is still the best practice to correctly manage and treat this kind of disease. Ultrasound is the gold standard imaging technique to make a diagnosis, where a typical honeycomb appearance of a complete mole is rarely seen, especially in the first trimester, and there is an absence of foetal parts and cystic appearance of the placenta . TherefoMoreover, those patients should be encouraged to start as soon as possible with a contraceptive therapy, such as oral contraceptives, that have been proven to be safe and effective during post-treatment monitoring, reducing the incidence of post-molar GTD or altering the pattern of regression of hCG values .NLRP7 or KHDC3L gene mutations. Recently, PADI6 has also been identified to be responsible for RHMs [NLRP7 mutations have been implicated in 48–80% of RHMs cases, whereas mutations in KHDC3L are reported in 10–14% of these patients with no NLRP7 mutations [NLRP7 can have live birth with egg donation [Cases of molar pregnancy, although rare, are crucial to be early recognised and to be appropriately managed. Causes leading to a molar pregnancy underlying different etiopathogenetic pathways and complex genetic mechanisms, while clinically, patients with RHMs cannot be distinguished from non-recurrent sporadic moles, and the histological analysis is not specific in cases of RHMs. Indeed, HMs are of biparental origin, with familiar clustering of for RHMs , 15. It utations . Homozygdonation . Indeed,donation . Akoury donation . UnfortuNLRP7 gene. The IVF technique enabled a complication-free, singleton pregnancy that resulted in a healthy term live birth female [Furthermore, it appears that, in women who have at least two episodes of molar pregnancy, reproductive options are currently limited, while assisted reproductive technology may help to achieve normal fertilisation of oocytes . A recenh female . In our Finally, in cases of molar pregnancy, especially patients with repeated episodes, it is crucial an early diagnosis and to start appropriate treatment as soon as possible. Even though the mechanisms leading to a molar pregnancy are complex and not completely known, especially in cases of RHMs where familiar genetic clusters are involved, and no etiopathogenetic treatment is currently available, the option of oocyte donation is the best reproductive strategy, which can support this condition for a successful pregnancy.


Organ sparing resection of gastrointestinal stromal tumors (GISTs) located in the proximal stomach or esophagogastric junction can be challenging, resulting in proximal or total gastrectomy to facilitate a radical resection without tumor spill. We developed and evaluated a single incision surgical gastroscopy (SISG) procedure to provide a technically feasible alternative for the removal of gastric GISTs at these challenging locations. We developed an endoluminal resection of gastric GISTs through a small single abdominal incision and longitudinal ventral gastrotomy. Patients with a proximal tumor location, in whom a wedge resection was deemed challenging on pre-operative investigation were included in the current series. Safety, short-term oncological and surgical outcome were evaluated. We performed SISG in six consecutive patients with histopathological proven or suspected gastric GIST. In all patients, the procedure was performed successfully with no tumor rupture. The mean operative time was 61 min and there were no significant complications. Pathological examination showed a microscopically radical resection in all patients. Single incision surgical gastroscopy is a feasible technique with excellent short-term oncological and surgical outcomes. This technique serves as a good alternative for complicated resections for gastric GISTs at challenging locations. The majority of gastrointestinal stromal tumors (GIST) is found in the stomach (60–70%) and small bowel 20–25%) . In pati0–25% . IAll patients who underwent resection of a suspected gastric GIST using this technique in 2017, 2018, and 2019 were prospectively included. Standard diagnostic work-up involved endoscopy with tumor biopsy and computed tomography scan (CT scan) of the thorax and abdomen. Endoscopic ultrasonography and fluorodeoxyglucose positron emission tomography (FDG-PET) were performed on indication . Only patients with localized disease, preferably located in the proximal stomach  and an intraluminal tumor location within the gastric wall, were eligible for resection using SISG. Moreover, all patients were discussed in our multidisciplinary team meeting pre-*** and postoperatively. The Internal Ethics Committee approved this study and all patients gave informed consent.For this procedure, general anesthesia is used and the patient is placed in supine, split-leg position (French position). Standard antibiotic prophylactics are used. The surgeon stands between the patient’s legs with the assistant and the scrub nurse on the left and right side of the patient. A mini laparotomy is performed using a 4-cm subxiphoidal midline incision. Next, a dual ring wound protector is placed and the stomach is identified Fig. a. The stPost-operative complications were graded according to the five-point scale as proposed by Dindo and Clavien. Post-operative mortality was defined as in-hospital death and/or within 30 days after surgery. Tumor-free resection margins were classified as R0 resection. A microscopic non-radical resection was defined as R1 resection and a macroscopic non-radical resection was defined as R2 resection. Adjuvant treatment was installed according to the advice of the post-operative multidisciplinary team meeting.N = 2), low (N = 2), or moderate (N = 2) None of the patients received adjuvant therapy.In the study period, we performed the single incision surgical gastroscopy technique in six patients with a suspected GIST. Patient, tumor, and surgical characteristics are shown in Table In this report, a SISG technique to remove gastric GISTs at challenging locations is described. In a small prospective case series, the feasibility of this technique without compromising the oncological safety was shown. In the recently updated European Society for Medical Oncology (ESMO) guidelines, the treatment of localized GIST is primary surgical resection if a radical R0 resection seems feasible . ReporteNonetheless, due to the promising results of minimally invasive surgery for GISTs, a laparoscopic wedge resection has become a widely accepted procedure for localized gastric GISTs. A retrospective study by Khoo et al. showed that a laparoscopic wedge resection is also feasible and safe for larger (> 5 cm) gastric GISTs, with similar oncologic outcomes compared to open wedge resection  However,For smaller GISTs, several different endoscopic techniques have been described in recent literature  . One coOne of the first reports on laparoscopic intra-gastric surgery is by Ohashi, and describes a technique to remove early gastric adenocarcinoma by directly placing trocar ports through the abdominal and gastric wall into the gastric lumen . Tumor rIn our experience, the SISG technique described in the current paper is a useful technique for gastric GISTs located at challenging locations. The gastric insufflation gives an excellent overview of the exact tumor location and provides sufficient working space for working with laparoscopic instruments directly in the stomach. This is an advantage compared to earlier described combined laparoscopic/endoscopic techniques using endoscopic mucosal dissection for tumor removal . As a re


Diverticulitis is a very common cause of hospitalization in the United States with fistula formation being a common complication. However, a fistula formation between the sigmoid colon and an ovary is an exceptional rarity. We present a case of a 22-year-old female with a colo-ovarian fistula misdiagnosed as a tubo-ovarian abscess after a recent episode of diverticulitis. Initial workup, imaging studies, and treatment ending with Hartmann's procedure with eventual colostomy reversal are described. A review of similar cases within the literature and fistulas is also presented. Diverticular disease can be defined as symptomatic diverticulosis due to diverticulitis or its complications . DivertiFistulas are an abnormal connection between two surfaces caused by local inflammation or iatrogenic injury, and generally fistulas in diverticular diseases present with pneumaturia and fecaluria . AccordiWe present a case discussing a unique scenario - fistula formation between the colon and an adnexal structure following an episode of acute diverticulitis. This occurrence is quite uncommon, with very limited documented cases in the medical literature.3 with neutrophilic prominence, and a pregnancy test was negative. A physical exam showed an obese habitus, a soft but distended abdomen, and diffuse abdominal tenderness being worse in the left lower quadrant. A pelvic exam was done revealing a milky vaginal discharge with no suprapubic or cervical motion tenderness. The patient was not menstruating at this time. She reported regular cycles of 26-30 days and no sexual activity with no change in the character of menstrual blood. The patient's vital signs showed a blood pressure of 141/99 mmHg, a respiratory rate of 28 breaths/minute, a temperature of 99.6℉, a heart rate of 119 beats/minute, and an oxygen saturation of 99% on room air.A 22-year-old female with a past medical history of morbid obesity with a BMI of 48 and recent diverticulitis with a Hinchey classification of 1a, which was medically resolved three weeks prior, presented to the emergency department with severe abdominal pain, nausea, diarrhea, and vomiting for one week. Her white blood cell count was found to be 11,100 mmRadiologic images were collected to better determine the etiology. A contrast abdominal and pelvic computed tomography (CT) showed a left adnexal mass with surrounding inflammatory changes, internal air, and mild ascites Figure . Due to Obstetrics and gynecology was consulted for a suspected tubo-ovarian abscess, and she was taken to the operating room for a diagnostic laparoscopy under general anesthesia in which extensive adhesions and large amounts of green-yellow fluid were found. The decision was made to convert to an exploratory laparotomy, which found four quadrant feculent peritonitis, with approximately 1,000 cc of purulent fluid. Upon further exploration, a 1 cm colotomy was found in the sigmoid colon. A colo-adnexal fistula was found to be the source of the abscess. The pelvis was frozen due to intense inflammatory reactions with extensive adhesions.A decision was made to proceed with Hartmann's procedure (sigmoidectomy with end colostomy), and two drains were intentionally left in place. The patient tolerated the procedure well without any complications. Gross specimens were then sent to pathology, which revealed an 11 cm x 10 cm x 7 cm abscess with adherent gray exudate, a 2.5 cm hemorrhagic abscess cavity, and multiple mucosal outpouches consistent with diverticula. No masses or lesions were identified. The patient tolerated the procedure well and was discharged home on postoperative day eight. Her postoperative course was uncomplicated with no changes in menstruation and normal 26-30 day cycles. She returned nine months later for a colostomy reversal. A laparoscopic-assisted end colostomy reversal was performed under general anesthesia, which showed a large parastomal hernia, minimal adhesions, and a successful end-to-end colorectal anastomosis after rigid proctoscopy showed no air leaks. The patient tolerated the procedure well and was discharged home on postoperative day three.Our case here represents a colo-adnexal fistula, which is a rare complication of diverticular disease. As mentioned earlier, a colo-vesicular or a colo-vaginal fistula is more common after an acute episode of diverticulitis than a fistula between the colon and ovaries or the colon and fallopian tubes. Most research articles addressing colo-adnexal fistulas occur in patients with ovarian neoplasm, polycystic ovary syndrome, pelvic inflammatory disease, previous surgical procedures, radiation exposure history, or patients with Crohn’s disease . The casA case report and literature review published in the International Journal of Surgery Case Reports discussed two cases of colo-ovarian fistula after an episode of diverticulitis . The firIn our case, the initial clinical symptoms of milky vaginal discharge and radiological findings of an adnexal mass with inflammation, internal air, and free fluid in the pelvis pointed toward a tubo-ovarian abscess. Diagnostic laparoscopy was superior to imaging techniques for our case in diagnosing the fistula secondary to acute diverticulitis due to inconclusive results from computed tomography. Our patient’s clinical course, although different in age group, was similar to the second case discussed in the above literature. Our patient deteriorated despite percutaneous drainage by interventional radiology, and surgery had to be performed sooner with Hartmann's procedure. The first case described above had favorable conditions to delay surgery and perform the resection with a primary anastomosis. Few cases have been described of diverticulitis with colo-adnexal fistulas, which are described below in Table The many cases mentioned above were initially misdiagnosed as alternative pathologies, emphasizing the complex and variable presentations of colo-adnexal fistulas. Further complicating the diagnosis, conventional imaging techniques failed to definitively identify the presence of such fistulas. Instead, these modalities primarily detected diverticular disease. One recurring observation from the cases above, including our case, that could aid in the diagnosis of a colo-adnexal fistula is the presence of gas within the adnexa. Lastly, due to the rarity of the condition, diagnostic and treatment guidelines do not have a current systematic approach. From our review of the literature, the presence of recurrent vaginal discharge or urinary tract infections and a history of diverticulitis should raise suspicion and warrant further investigation for a possible colo-adnexal fistula. Lastly, with regard to treatment approaches, our review of cases has shown that initial treatment using conservative management or drainage-only techniques was met with failure. The treatment plan with the most favorable outcome in these cases has shown to be a laparoscopic resection of the fistulizing structures with end-to-end anastomosis. Hartmann’s procedure was used in emergency cases, especially in patients with sepsis, and had a favorable prognosis for colostomy reversal, as evidenced in our case as well as the cases mentioned above.Diverticulitis, although common in older adults, is a rarity in younger patients with only 10% of known cases being below the age of 40. Fistula formation between adnexal structures and the intestine is an exceptional rarity. Both of the above rarities cause misdiagnosis and delays in the management of these fistulas, especially in the younger population. As more cases of colo-adnexal fistulas arise within the literature, there is hope of finding a definitive guideline and approach to both diagnosis and treatment of these types of fistulas. Our case report could add to the few available cases within the current literature to help diagnose, treat, and minimize usual and unusual complications of colo-adnexal fistulas following diverticulitis.


Retinal astrocytic hamartoma (RAH) is a rare benign tumor originating from astrocytic cells located in the neural cell layer of the retina. It is commonly seen in patients with phakomatoses such as tuberous sclerosis complex or neurofibromatosis, rarely as an isolated retinal mass. This lesion is usually asymptomatic; however, these located in the area of the optic nerve, macula, or exhibiting the features of exudation, neovascularization may present visual disturbances and decreased visual acuity.We present a rare case of a 15-year-old boy, with no significant past medical history, whose cause of visual disturbances turned out to be isolated RAH.Based on the results of color images of the fundus, fluorescein angiography as well as the analysis of magnetic resonance imaging, the patient was diagnosed with RAH.Additionally an B-scan ultrasonography, static and kinetic perimetry were performed.Fundoscopic examination showed a unilateral yellowish, well-circumscribed, mulberry-like lesion with a wide base, located in inferosnasal quadrant, in the vinicity of the optic nerve. The patient underwent neurological, pediatric, and genetic evaluations that excluded other pathological findings or underlying systemic disease.The prognosis for RAH is generally good, however, the lesion requires regular ophthalmologic follow-up to rule out the progression of the tumor mass. The patient 7-year follow-up history is without evidence of tumor growth, local or general deterioration of the condition. This tumor is part of the retinal astrocytic tumor family that includes other, not always nonprogressive lesions from which it must be distinguished. RAH is usually associated with a systemic disease, either tuberous sclerosis complex (TSC) or neurofibromatosis (NF). Fundus examination reveals bilateral RAH in approximately 50% of patients with TSC. It rarely occurs as an isolated lesion, is usually an incidental finding during ophthalmic examination diagnosed later in life.–6The majority of RAHs are endophytic tumors growing toward the vitreous, but some can be endophytic subretinal lesions developing in retinal nerve fibers. Histopathologically, it is an eosinophilic lesion, made up of well-circumscribed, elongated fibrous astrocytes, with eosinophilic cytoplasm and round or oval nucleus. The tumor can be located peripherally or in the peripapillary area, and can be a yellowish, slightly elevated lesion; in some patients the nodule can be semitranslucent with central calcification. The majority of astrocytic hamartomas are slow-growing tumors that tend to calcify over time. They need to be differentiated from retinoblastoma, acquired retinal astrocytoma, reactive retinal astrocytic tumor or amelanotic choroid melanoma.A 15-year-old boy was referred to the Department of Pediatric Ophthalmology, the K. Gibiśski University Hospital Center, Medical University of Silesia, Katowice, Poland due to decline in distance visual acuity. His medical and family history did not reveal any eye disease or concomitant diseases. The eyeballs were normally positioned within orbit, with full mobility range and preserved binocular vision. Best-corrected visual acuity of the right eye was 1.0 with spherical correction of −0.75 and cylinder correction of −0.25 (axis 100º), and of the left eye 1.0 with spherical correction of −1.25. Near vision was within normal range. Intraocular pressure measured with the Goldmann tonometer was 15 mm Hg in both eyes. No abnormalities were detected in anterior segments of right or left eye. Fundocopic examination of the left eye revealed a yellowish, mulberry-like retinal mass with a wide base located in lower nasal quadrant, adjacent to the optic nerve disc Fig. . Static  Tumor that originate from astrocytes in the retina is called retinal astrocytic tumor. According to Rowley at al. they we differentiate its 3 subtypes.,9 Although they may share many similarities, particularly in fundoscopic appearance, each has its own unique morphologic and histologic characteristics, possibility of pathological progression and some are closely related to underlying primary disease. Reactive retinal astrocytic tumor is typically observed in the third decade of life and is neither genetic nor hereditary. It is thought to occur in response to retinal inflammation, degeneration or injury. Lesion is usually unilateral, single, peripherally located and has a venous component that gives it a yellow-pink tint. It is a progressive tumor with a high incidence of exudates that lead to retinal detachment.,11 Acquired retinal astrocytoma is also a solitary, benign lesion but may show locally aggressive features. It is not associated with TSC and is distinguished by a yellowish color related to numerous lipid exudates. This tumor can lead to a variety of intraocular complications including vitreous hemorrhages, retinal detachment and secondary glaucoma. Its precise and accurate diagnosis is crucial since this type typically requires prompt treatment, recently verteporfin photodynamic therapy has been recommended. RAH is usually a whitish-yellow lesion and is extremely often seen as part of TSC. Other clinical entities in which RAH has been observed include NF, Stargardt’s disease, Retinitis pigmentosa, and Usher’s syndrome. It constitutes one of the minor criteria for the diagnosis of TSC and it is also the most frequent retinal lesion observed in patients with this disease. When associated with TSC, the tumor is usually bilateral and multifocal. Typical RAH is described as single, flat, and semiopaque lesion. It is usually diagnosed in childhood and shows features of calcification. Progression of these lesions is not reported–it usually remains stable and does not require inclusion of treatment. Visual field restriction and a decline in visual acuity might be found depending on lesion location and potential pressure on the optic nerve. Clinical signs of retinal astrocytoma associated with tuberous sclerosis progress slowly. Zimmer-Galler et al examined 16 patients with a total of 37 RAHs. During follow-up that ranged from 6 to 34 years only 3 lesions showed progressive or new calcifications. Shields et al observed that peripheral astrocytomas did not exhibit growth during follow-up while those located near the optic disc did. Fast-growing lesions may cause retinal detachment, surrounding tissue necrosis, inflammatory response, macular edema, retinal vein occlusion, vitreous body hemorrhages or neovascular glaucoma and requires specific treatment To date, no cases of RAH evolving into a malignant form or generating metastases have been described.RAH is a benign tumor that infrequently reduces visual acuity and does not require treatment. It arises from astrocytes being an integral part neural retinal. Astrocytes are glial cells, providing support for retinal ganglionic cells, maintaining functional neuronal circuits and being part of the mechanism leading to reactive gliosis. These cells are located in the inner layers of the retina, where they migrate independently of blood vessels. Thus, they are involved in the retinal response to hypoxia and mediate the production of vascular endothelial growth factor.,18 Based on this classification, our case falls under type 2.We distinguish 3 subtypes of RAH subtypes based on morphological features. Type 1 is a small, flat, isolated, grayish lesion that is semitranslucent, with no evidence of calcification. Conversely, there is type II which is defined by its yellowish color and mulberries-like shape. It is nodular, modular and has many calcifications. It is nodular, contains multiple calcifications and is raised in relation to the retinal surface. Type 3 exhibits the characteristics of both of the previous 2.Differential diagnosis is always needed to exclude retinal malignancies. The most common lesions to be confused with are retinoblastoma, choroid uveal melanoma, and choroidal metastasis. Significant features which distinguish RAH from retinoblastoma are calcifications of a yellow tone and the absence of veins protruding into the lesion. Uveal melanoma has a resemblance of cavitation but rarely presents with calcifications. Choroidal metastasis is usually detected in older patients, usually with breast or lung cancer. They are distinguished by yellow masses located underneath the retina with large subretinal exudates that cause retinal detachment and consequently a decrease in visual acuity in 50% of cases. Retinal lesions develop within 2 to 6 years of TSC diagnosis in approximately 80% of patients with tuberous sclerosis, but can also be the first clinical symptom of the disease. Patients should undergo regular ophthalmic and neurological follow-ups. Each patient with RAH should undergo a thorough diagnostic process, including differential diagnosis. They also need regular follow-up examinations (every 3 to 6 months) and specialist consultations to rule out systemic diseases.High prevalence of astrocytoma among patients with tuberous sclerosis indicates a need for evaluation by internists to exclude characteristic skin lesions, kidney tumors, heart tumors, subependymal nodules and subpleural cysts.This case illustrates the importance of recognizing different types of retinal astrocytic lesions that may be associated with various clinical conditions and have different potential for malignancy. The lack of calcifications, the whitish-yellow tint of the lesion, and additional tests that ruled out TSC indicated a different type of tumor than the 1 we initially suspected. However, careful examination, frequent and regular follow-ups allowed us to observe changes that gave the tumor a clear morphologic picture, which is type 2 astrocytic retinal hamartoma. At the same time, the lesion did not change in nature, did not enlarge, did not cause complications, and most importantly did not decrease visual acuity, which was observed throughout the 7-year follow-up period.The authors are grateful to the patient and his family for their suport of this article.Conceptualization: Sebastian Sirek, Agnieszka Tronina, Erita Filipek.Data curation: Bogumiła Wójcik-Niklewska, Sebastian Sirek, Agnieszka Tronina.Formal analysis: Bogumiła Wójcik-Niklewska, Sebastian Sirek, Agnieszka Tronina.Investigation: Bogumiła Wójcik-Niklewska, Sebastian Sirek, Agnieszka Tronina.Methodology: Bogumiła Wójcik-Niklewska, Sebastian Sirek, Agnieszka Tronina, Erita Filipek.Project administration: Bogumiła Wójcik-Niklewska, Erita Filipek.Resources: Bogumiła Wójcik-Niklewska.Software: Bogumiła Wójcik-Niklewska.Supervision: Bogumiła Wójcik-Niklewska, Erita Filipek.Validation: Bogumiła Wójcik-Niklewska.Visualization: Bogumiła Wójcik-Niklewska, Sebastian Sirek.Writing – original draft: Sebastian Sirek.Writing – review & editing: Bogumiła Wójcik-Niklewska, Sebastian Sirek.


This case report shows that 125I seed implantation is a safe and effective means of delivering radiotherapy to young children with head and neck RMSs. It may be an option for children with RMSs for whom surgery or external radiotherapy is unsuitable.Rhabdomyosarcomas (RMSs) are highly malignant soft‐tissue sarcomas. Head and neck RMSs often pose unique challenges to treatment because of their closeness to important structures. We here report a rare case of a 1‐year‐old boy with a 1‐month history of right eye swelling and an eye mass. Biopsy of deep tumors in the maxillofacial region supports embryonal RMS. Postoperative positron emission computed tomography showed a 5.0 cm × 4.8 cm × 4.2 cm malignant tumor in the right maxillary region. In accordance with the international RMS study group guideline, the child was diagnosed with IIIa and TNM stage T2bN1M1 embryonal RMS. The child was treated with a combination of chemotherapy and  125I seed implantation radiotherapy without undergoing surgical treatment, resulting in long‐term remission.In this article, children with orbital rhabdomyosarcomas received chemotherapy combined with  CTcomputed tomographyIEifosfamide and etoposideRMSrhabdomyosarcomaVACvincristine, actinomycin D, cyclophosphamideVDCvincristine, doxorubicin D, cyclophosphamideVTCvincristine, topotecan, cyclophosphamide1125I seed implantation therapy. We will discuss the treatment of this rare case, including the long‐term effects of his local treatment.Rhabdomyosarcomas (RMSs) is the most common malignant tumor of the orbit in children, with about 10% occurring in the orbit . Althoug2A 1‐year‐old boy with a 1‐month history of eye swelling and an eye mass presented to our hospital and was found to have swelling of the right eyelid but no other abnormalities. He was diagnosed as having a stye. During 3 weeks of symptomatic treatment, the right eye mass enlarged to 10 cm × 5 cm × 3 cm. Orbital computed tomography (CT) showed localized bone resorption, destruction of the right maxilla, and a soft tissue shadow. The tumor had invaded the orbit superiorly. A biopsy of the deep maxillofacial tumor was obtained at a local hospital. A pathological examination of the biopsy sample revealed a small cell tumor of the right maxilla. After pathological consultation between three hospitals and immunohistochemistry, the final diagnosis was an EMR. Postoperative positron emission‐CT showed a 5.0 cm × 4.8 cm × 4.2 cm malignant tumor in the right maxillary region. There was extensive invasion and metastases in the right parapharyngeal lymph nodes and sternocleidomastoid muscles bilaterally. No other metastases were found. In accordance with the international RMS study group guideline [2 (VAC)/vincristine 0.05 mg/kg, topotecan 1.5 mg/m2, and cyclophosphamide 750 mg/m2 (VTC) were administered, followed by one course of ifosfamide 1.8 g/m2 and etoposide 100 mg/m2 (IE). This did not achieve significant shrinkage of the assessed tumors  for one course. This was followed by the implantation of 62 iodine‐125 seeds into the right maxillary tumor under CT guidance. The prescribed dose was 110 Gy and the seed activity was 0.65 mCi. The procedure was performed under general anesthesia, with seeds being implanted at 1 cm intervals. Subsequently, VDC and IE schemes were administered alternately for three courses, followed by VTC and IE for seven courses. This resulted in significant tumor shrinkage to 2.0 cm × 0.6 cm × 0.8 cm  strategy for medium‐risk RMSs, three courses of vincristine 0.05 mg/kg, actinomycin D 0.045 mg/kg, and cyclophosphamide 2.2 g/ms Figure . In accom Figure . Next, td Figure . The patd Figure . After 92 are all within the normal range for his age [In addition to assessing the primary tumor, we also conducted a long‐term follow‐up of changes secondary to the tumor and its treatment. The child is now 8.3 years old and growing normally. His height of 131 cm, weight of 28.4 kg, and BMI of 16.5 kg/m3RMSs are highly malignant soft tissue sarcoma . Surgery125I seed implantation can be performed under the guidance of CT and magnetic resonance imaging. A treatment planning system is used to determine the contour of the target tumor area, eyeball, and visual nerve. Next, the doses to the target area (tumor), eyeball, and optic nerve are calculated, and the dose, activity, quantity, and spatial positioning of 125I seeds are adjusted accordingly. Finally, a guided puncture template is constructed by 3D printing technology and used to accurately guide the insertion of the needle and implantation of the 125I radioactive seeds, ensuring that the target area is exposed to at least 95% radiation dose while the eyeball. Orbital CT scanning is performed 1–3 days after this procedure to confirm its quality [125I seed implantation radiotherapy, in which case seed implantation can be performed to treat subsequent local tumor recurrence. 125I seed implantation is recommended after three to four cycles of chemotherapy in patients in whom surgery and chemotherapy have achieved partial or complete remission. Seed implantation does not hinder the continuation of chemotherapy and therefore shortens the treatment time compared with external radiotherapy, which necessitates a pause in chemotherapy. Our patient underwent 125I seed implantation after completing four cycles of chemotherapy, the effect of which was similar to that of external radiotherapy.External radiotherapy significantly damages local tissues and has serious long‐term adverse effects . 125I se quality . The gre quality . Some ch4125I seed implantation radiotherapy is a safe and effective treatment approach in young children with head and neck RMS. It may be an option for children with RMS whose tumors cannot be managed with surgery or external radiotherapy.Overall, this case report shows that chemotherapy combined with Sidou He: Writing—original draft . Shuhang Tian: Writing—review & editing . Na Xu: Data curation ; Investigation . Jianguo Zhang: Methodology ; supervision . Chao Duan: Resources ; supervision . Xiaoli Ma: Project administration ; supervision .The authors declare that they have no conflict of interest.This case report was approved by the Ethics Committee of Beijing Children's Hospital, Capital Medical University (IEC‐C‐006‐A04‐V.06).Written informed consent was obtained from our patient's parents for publication of this case report, all information contained within it, and any accompanying images. Copies of the written consent are available for review by the Editor of this journal.


EWSR1 gene (translocation 22q12). The patient’s treatment involved a multimodal approach, including perioperative chemotherapy, surgery, and postoperative chemotherapy, following which the patient remained in complete remission after 24 months. This case emphasizes the importance of considering rare malignancies such as ES in differential diagnoses for young patients with liver masses. It also accentuates the pivotal role of family physicians in early detection and holistic patient care, underscoring the need for comprehensive investigations when encountering persistent symptoms.Ewing sarcoma (ES) is primarily recognized as a primary bone tumor; however, its extraosseous variant is exceptionally rare and presents unique clinical challenges. In this article, we report the case of a 22-year-old male who initially presented with abdominal swelling. Diagnostic tests included abdominal imaging and a CT scan, revealing a solid liver mass. A thorough evaluation confirmed it to be an extraosseous ES, supported by liver biopsy and immunohistochemistry demonstrating positive expression for AE1/AE3 and CD-99, along with genetic analysis revealing a rearrangement of the  Ewing sarcoma family of tumors (ESFT) represents a rare and intricate group of malignancies originating from mesenchymal tissues . These tThe exceptional rarity of extraosseous ES adds a layer of complexity to its clinical presentation and management. ESFT, known for its inherent aggressiveness and tendency to recur and metastasize, often involving sites such as the lungs, bone marrow, brain, and lymph nodes, underlines the critical need for in-depth exploration of these unique tumor manifestations ,4. As thA 22-year-old man with no significant personal or family history (including an absent family history of cancer) came to the primary care consultation due to painful abdominal swelling and an unintentional 10 kg weight loss that had developed over the last three months. The patient reported having gone to the emergency department for two episodes of nausea, vomiting, diarrhea, and abdominal pain, which were diagnosed and treated as acute gastroenteritis two weeks before. Upon physical examination, a soft and depressible abdomen with tenderness in the right hypochondrium was observed. Deeper palpation revealed an enlarged liver with regular borders extended approximately 5 cm below the costal margin. Laboratory findings . These findings supported the diagnosis of ES. The case was presented to a multidisciplinary team to discuss the optimal therapeutic approach. Chemotherapy was initiated before surgery to reduce the size of the lesion consisting of six cycles of vincristine, doxorubicin, and cyclophosphamide followed by mesna (sodium 2-mercaptoethane sulfonate).The patient was then referred to the hospital, where a percutaneous biopsy of the liver mass was performed. The biopsy revealed fragments of malignant neoplasm, densely cellular, composed of small cells with a rounded, vesicular, or hyperchromatic nucleus. The cells were arranged in a towel-like pattern, mixed with a scarce inflammatory infiltrate within a dense, fibrous streak. Immunohistochemical analysis showed positive expression for AE1/AE3 and CD-99. In addition, a genetic analysis of the sample revealed a rearrangement of the After the third cycle, this scheme had to be alternated with ifosfamide and etoposide followed by mesna due to complications and toxicity . After completing six cycles of chemotherapy, there was a significant reduction in the lesion, measuring approximately 5 cm in size. A fluorodeoxyglucose (FDG) positron emission tomography/computed tomography (PET/CT) was then performed to confirm the complete metabolic response. The patient underwent laparoscopic resection of the hepatic segments IV B/V of the liver, which contained the tumor, along with laparoscopic cholecystectomy due to apparent sarcoma invasion.Post-surgery, the anatomopathological analysis revealed a neoplastic mass measuring 4.4 cm with a transmural invasion of the gallbladder wall. Histological examination revealed the presence of neoplastic cells in the surgical margins. Due to the patient’s history and preferences, the decision was made to opt for adjuvant radiotherapy without chemotherapy.The patient is currently under clinical surveillance and has remained in complete remission for 24 months.ES, particularly its extraosseous variant, represents a diagnostic and therapeutic challenge due to its rarity and often ambiguous initial clinical presentation .Although the primary bone form of ES is well-documented, extraosseous ES, as seen in our patient, can mimic other benign or malignant conditions and often leads to a delay in definitive diagnosis . ES freqThe role of imaging in diagnosing ES is crucial. Although imaging findings are often non-specific, certain patterns emerge based on the tumor’s location . ExtraosEWSR1 rearrangements in approximately 90% of ES cases [EWSR1 gene on chromosome 22 with the FLI1 gene on chromosome 11 ), creating the EWSR1-FLI1 fusion product, known for its oncoprotein functions [EWSR1 gene rearrangement, as seen in our patient, confirms the diagnosis of ES and distinguishes it from its mimickers.Small round blue cells, a feature found in ESFT, are not unique to this tumor and can also be observed in other malignancies such as lymphoma and rhabdomyosarcoma . The diaES cases . This trunctions ,10,11. TGiven the infrequent occurrence of ESFT, a dedicated staging system has not been developed. Instead, it is recommended to use the broader American Joint Committee on Cancer staging system designed for all soft-tissue sarcomas ,9. The rFollow-up and surveillance are critical, as the risk of local recurrence or metastatic spread persists. According to published guidelines, regular follow-up after therapy of primary ES includes CT of the chest (to detect lung metastases in asymptomatic patients) every three months for the first two years and every six months for the first five years. After the five-year surveillance, a CT of the chest is recommended once or twice per year up to 10 years after the completion of treatment. Long-term surveillance beyond 10 years should be performed based on clinical indications ,11.ES is a rare type of small round-cell tumor that lacks specific clinical or radiological features. Diagnosis requires histopathological, immunohistochemical, and cytogenetic examinations. Given the rarity of extraosseous ES, further studies and research are required to enhance the understanding of this condition and develop more effective treatment strategies. This case presentation significantly contributes to the medical literature, emphasizing the importance of maintaining vigilance toward uncommon symptoms, especially among young patients. The family physician plays a crucial role as the initial link in the healthcare chain, highlighting the significance of ongoing education and interdisciplinary collaboration in diagnosing and treating challenging medical conditions such as extraosseous ES. Early recognition remains a determining factor that can have a substantial impact on the prognosis of these patients.


Pilomatrixoma is a benign skin tumor typically presenting as a hard, slow-growing mass arising from hair follicle matrix cells. While most encountered in children, giant pilomatrixoma seldomly presents in adults. In the present case, a large subcutaneous, nonpainful and slow-growing mass was discovered in the infraclavicular region of a 52-year-old male. Biopsy confirmed the diagnosis of giant pilomatrixoma. Despite its benign nature, tumor size and location can result in significant morbidity and cosmetic deformity. This case highlights the importance of considering pilomatrixomas in patients with a slow-growing mass, especially after an inciting event, such as an insect bite. Timely diagnosis and proper management can result in successful tumor removal with minimal cosmetic compromise. Pilomatrixomas are benign skin tumors originating from hair follicles, with minimal malignant potential in sporadic cases . Some caTypically, pilomatrixomas present as an asymptomatic, firm, solitary, well-circumscribed tumor localized to the dermis or subcutaneous tissue, which usually arise in the head and neck regions. Tumors are described as giant when the tumor diameter exceeds 5 cm in its widest dimension . IncisioThe following case presents a giant pilomatrixoma arising in the infraclavicular region, after an insect bite, in an adult patient with successful surgical excision.A 52-year-old male presented for a mass in the left infraclavicular region which had been gradually increasing in size for the past two years. The patient reports that a few weeks prior to noticing the mass, a mosquito, described as having legs with white stripes, bit him in the same region. Upon physical exam, a large mass was palpated in the infraclavicular area. The mass was nontender, mobile and firm. The overlying skin was mildly erythematous with small, partially healed ulcers . There wAn en bloc resection of the mass was undertaken as follows. The patient was placed under general anesthesia, and the incision site was prepped with a local anesthetic. An elliptical incision was performed, followed by subcutaneous dissection. There was no infiltration past the subcutaneous layer as there was no need for fascial or muscular dissection. The mass was resected with 2 cm-wide margins and sent to pathology . The incMacroscopically, the mass measured 20.1 * 13 * 5.8 cm. When incised, it showed a combination of yellowish areas with a friable calcified appearance and solid uncalcified whitish areas . Histoloet al., pilomatrixomas are slightly more common in males [Our case matched the epidemiological profile of pilomatrixomas reported in the literature, which indicates a bimodal age distribution for this tumor, with the highest peak in the first and second decades and the second between 50 and 65 years of age . Accordiin males , 10, conin males , 12. In in males  However,The most significant case of a giant pilomatrixoma measured 34 cm × 21 cm × 17 cm . It was Our patient has a history of a mosquito bite a few weeks prior to noticing the mass. According to the patient's description, the mosquito could have been Aedes spp, the vector responsible for transmitting dengue, Chikungunya, Zika and other arboviruses. Like most cases described in the literature, the tumor in our case consisted of a solitary lesion. On the other hand, pilomatrixomas may be associated with Gardner syndrome, myotonic dystrophy, Steinert syndrome, xeroderma pigmentosum, Turner's syndrome or sarcoidosis , 8, 13.et al. and Pirouzmanesh et al., respectively [Pilomatrixomas lack specific clinical symptoms, which delay the diagnosis, primarily when it arises in unusual areas, especially following an inciting event regarded as innocuous. The accuracy of the clinical diagnosis of pilomatrixoma was 28.9% and 46%, according to Ciucā ectively . Imagingectively .Surgical excision is the treatment of choice due to a low relapse rate of 0.3% and appreciable cosmetic results . In addiPilomatrixomas are benign, slow-growing tumors; however, they can grow to giant sizes leading to skin lesions and cosmetic deformity, as in our case. Therefore, a high index of suspicion is required to make an accurate diagnosis, especially when encountered in unusual locations and with an associated indolent history.


Prader-Willi syndrome (PWS) is a genetic disorder frequently characterized by obesity, growth hormone deficiency, genital abnormalities, and hypogonadotropic hypogonadism. Incomplete or delayed pubertal development as well as premature adrenarche are usually found in PWS, whereas central precocious puberty (CPP) is very rare. This study aimed to report the clinical and biochemical follow-up of a PWS boy with CPP and to discuss the management of pubertal growth. By the age of 6, he had obesity, short stature, and many clinical criteria of PWS diagnosis, which was confirmed by DNA methylation test. Therapy with recombinant human growth hormone (rhGH) replacement (0.15 IU/kg/day) was started. Later, he presented psychomotor agitation, aggressive behavior, and increased testicular volume. Laboratory analyses were consistent with the diagnosis of CPP . The patient was then treated with gonadotropin-releasing hormone analog (GnRHa). Hypothalamic dysfunctions have been implicated in hormonal disturbances related to pubertal development, but no morphologic abnormalities were detected in the present case. Additional methylation analysis (MS-MLPA) of the chromosome 15q11 locus confirmed PWS diagnosis. We presented the fifth case of CPP in a genetically-confirmed PWS male. Combined therapy with GnRHa and rhGH may be beneficial in this rare condition of precocious pubertal development in PWS. Prader-Willi syndrome (PWS), also known as Prader-Labhart-Willi syndrome, is a complex neurogenetic disorder characterized by neonatal hypotonia, psychomotor delay, early-onset hyperphagia, short stature, hypogonadism, sleep disturbance, learning disabilities, and behavioral and psychiatric disorders; it also represents the most common form of genetic obesity. Its incidence is estimated between 1:10,000 and 1:30,000 living births with the prevalence of 1:50,000. The expression of paternally active genes located on chromosome 15q11-q13 is lost in PWS, which may occur due to a deletion in this chromosomal segment (65-70%), maternal uniparental dissomy 25-30%), or imprinting defects (1-2%), even though rare gene mutation (< 0.1%) and balanced translocation (0.1%) can also be found  deficiency, and abnormal pubertal development . Usuallylocus 15q11 who experienced growth hormone deficiency and central precocious puberty (CPP). To the best of our knowledge, this is the first case of CPP reported in boys with PWS during GH replacement. Informed consent was obtained from the patient’s legal guardian for publishing this case report.Despite the common clinical picture of delayed or incomplete puberty, rare cases with true precocious puberty have been described in this syndrome . Here, wrd percentile, weight between the 25-50th percentile range, body mass index between the 90-95th percentile range with normal bone density and 37.9% total body fat. At presentation, he had no signs of sexual development (Tanner stage I): no pubic hair, nonpalpable left testis (1.05 cm3), normal right testis (1.53 cm3) (both measured by ultrasonography), and stretched penile length of 1.5 cm. Otherwise, his examination was normal, except for low ears implantation, relatively small hands and feet, valgus knee, and mild learning disabilities. The Holm and Cassidy criteria score was 9; the genetic test demonstrated only the methylated maternal allele, thus confirming the clinical diagnosis of PWS by the methylation-specific PCR (MS-PCR) performed ten years ago. The patient was born at term by operative delivery at 39 gestational weeks . His parents were healthy, non-consanguineous, and from Caucasian descent. Hypotonia and bilateral cryptorchidism were present at birth as well as poor sucking and low weight gain during the first month of life. Failure to thrive was diagnosed in the next months; no brain abnormalities were detected on the computed tomography scan. At the age of 2, he began to develop hyperphagia, followed by rapid weight gain and signs of mild psychomotor deficiencies; the clinical diagnosis of PWS was then first suspected. At the age of 3, he was submitted to surgical orchiopexy. At the age of 6, he was referred to our pediatric endocrinology unit for evaluation of obesity and short stature. His height was below the 3Initial laboratory analyses resulted in prepubertal hormone levels Table . The pea3, left and right respectively) and penis length (4.5 cm). Biochemical and imaging evaluation for peripheral precocious puberty revealed no evidence of congenital adrenal hyperplasia, human chorionic gonadotropin (hCG), or androgen-secreting tumors. At the age of 8  (depot leuprorelin acetate 3.75 mg i.m. every 28 days), and the rhGH dose was adjusted to 0.1 IU/kg/day. Patient’s response to treatment was satisfactory, as evaluated under pubertal blockade by GnRHa  at the aHere, we described the case of a boy presenting common features of PWS including obesity, short stature, hypotonia, and hypogonadism ; after the onset of rhGH therapy, he surprisingly presented early signs of pubertal development. According to a large database of PWS children, growth hormone deficiency was present in 80% of patients, and 86.7% were treated; hypogonadism was present in 49% of patients . Our patSNRPN, NDN, MAGEL2, and MKRN3 is associated with PWS. Mapping of microdeletions associated with PWS has identified a 4.3 kb region of the SNPRN gene that appears to be required for establishment and/or maintenance of the paternal epigenotype across the imprinting center domain (MKRN3)  . In thislial CPP ,14. HoweThe natural course of PWS is a significant increase in fat mass over the years that can be worsened by GH deficiency . Beyond Despite the fact that precocious puberty has been recognized in few PWS cases, there is controversy whether treatment with GnRHa should be used. Patients with PWS commonly fail to complete puberty, and, as a consequence, there was no final stature loss, in spite of slight bone age advance . In the The rare manifestations of CPP in patients with PWS have been attributed to brain lesions ,10. More


Pancreas divisum (PD) can be one of the causes of unexplained chronic abdominal pain. In PD, the dominant duct drains the majority of the pancreas via the minor papilla, which can be conveyed in the imaging as crossing duct sign.We report a case a of 16‐year‐old man who presented with unexplained chronic abdominal pain. Contrast‐enhanced computed tomography and magnetic resonance cholangiopancreatography showed a bulky pancreas, but his pancreatic enzymes were normal. The crossing duct sign was prominent in imaging, which confirmed the diagnosis of PD. Contrast‐enhanced computed tomography,  and magnetic resonance cholangiopancreatography (T2‐weighted) show the dominant dorsal duct crossing the intrapancreatic common bile duct giving crossing duct sign. This confirms the diagnosis of Pancreas divisum. The incidence of PD in the population at autopsy series, endoscopic retrograde cholangiopancreatography (ERCP), and magnetic resonance cholangiopancreatography (MRCP) is 4%–14%, 3%–8%, and 9%, respectively.Although most patients with PD are asymptomatic, PD may be the cause of chronic abdominal pain, acute, and recurrent pancreatitis. The major dominant dorsal pancreatic duct drains into the relatively smaller or stenotic minor papilla, which causes inadequate drainage of pancreatic secretions and transient obstruction of flow. This cause an increase in intraductal pressure and distention of the dorsal duct, which may lead to abdominal pain and pancreatitis.Herein, we report a case that presented to us with epigastric pain and vomiting, and imagings were consistent with PD. This case report is presented with the aim of highlighting the radiological finding in the PD as well as reminding the reader that PD is one the causes of unexplained abdominal pain, as an early radiological diagnosis can prompt early surgical intervention if necessary.2A 16‐year‐old man presented to the emergency department with dull aching epigastric nonradiating pain for a day, which was increasing in severity. He also had two episodes of nonbilious vomiting and loose stool without fever. He had a past history of similar epigastric pain requiring inpatient care 4 years ago that responded to conservative management. The report of the computed tomography (CT) scan done at that time mentioned mesenteric lymphadenopathy only. He had been evaluated for similar pain of lesser magnitude many times elsewhere and was treated intermittently with oral proton pump inhibitors and analgesics. The patient gave no history of smoking or drinking.On examination, his vitals were normal. No pallor or icterus were noted, and the patient was not dehydrated. An abdominal examination showed mild tenderness over the epigastric region. His laboratory reports showed normal liver function tests and pancreatic enzymes. A complete blood count was normal. The ultrasonography examination was normal. Based on clinical symptomatology and tests, he was managed in the line of acute gastroenteritis and was given antacids, analgesics, antispasmodics, and intravenous fluids. During the course of treatment, he developed increasingly severe pain, requiring stronger analgesics (opioids) with increasing frequency. In view of increasing pain, contrast‐enhanced CT (CECT) of the abdomen was done, which was suggestive of a bulky pancreas. The dominant dorsal pancreatic duct was draining into the minor papillae rather than the major papillae, suggesting pancreas divisum, and the main pancreatic duct was crossing the intrapancreatic common bile duct, which was further confirmed with MRCP as shown in Figure 3Pancreas divisum has three major types. Type I, or classic PD, is where there is a complete failure of the dorsal and ventral buds to fuse. In type II PD, there is no ventral duct as in our case; hence, the minor papilla drains the entire pancreas and the major papilla drains some of the common bile duct. Finally, type III has a small remnant of communication between the dorsal duct and ventral duct.Pancreas divisum, though mostly asymptomatic, may present as the etiology of acute or recurrent pancreatitis, chronic pancreatitis, or chronic abdominal pain.Endoscopic retrograde cholangiopancreatography is the modality of choice for diagnosing PD; however, the effects related to it—a likely invasive test requiring sedation, 10%–15% complication rate, and up to 10% post‐ERCP pancreatitis—cannot be overlooked.The predominant pancreatic duct drains into the minor papilla, which occurs at a level superior to the level of the bile duct opening. The bile duct drains into the minor papilla. This appearance of the dorsal duct running across the intrapancreatic common bile duct can be depicted as a crossing duct sign. This indicates PD, which can be seen on MRCP images. Sometimes, the duct of the ventral system may not be visible due to its small caliber. A focal dilation of the terminal duct of Santorini  can be seen and is called Santorinicele.Similarly, with the advent of multidetector CT scanners and high‐spatial‐resolution thin‐section imaging, PD may be routinely seen with the use of CT as well.For patients with abdominal pain as a result of PD, there is a corrective surgical option available. The patient may elect to receive endoscopic stenting or sphincterotomy of a minor papilla.4In this case report, we attempted to highlight the radiological findings in the patient with PD. The patient did not have biochemical and radiological evidence of pancreatitis, but this is surgically correctable in symptomatic patients; hence, early radiological detection is important. Looking for the crossing duct sign in the coronal thick slab images in CT as well as MRCP is sufficient to diagnose PD.Prakash Kayastha: Conceptualization; supervision; writing – original draft; writing – review and editing. Biraj Pokhrel: Resources; supervision; writing – original draft; writing – review and editing. Uma Bhatta: Conceptualization; writing – original draft; writing – review and editing. Roshan Pathak: Conceptualization; resources; writing – original draft; writing – review and editing. Goody Jha: Writing – original draft. Sharma Paudel: Supervision; writing – review and editing. sundar suwal: Supervision. Dosti Regmi: Conceptualization; writing – original draft; writing – review and editing.The authors declare that there is no conflict of interest regarding the publication of this paper.Need for ethics approval waived. Consent from the patient deemed to be enough.Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent will be available for review by the editor‐in‐chief of this journal if requested.


Lichen planus pigmentosus (LPP) is a rare form of lichen planus that typically affects middle-aged people with darker-pigmented skin. LPP is associated with a longer clinical course than classical lichen planus, which distinguishes it clinically. Its occurrence in children is uncommon, with few reported cases in this population in the literature. We report a rare presentation of unilateral blaschkoid LPP in a seven-year-old Saudi Arabian female patient. Lichen planus pigmentosus (LPP) is an uncommon variant of lichen planus (LP) . It is cA medically free seven-year-old female presented with a history of left thumb trauma that had occurred three months ago, followed by the appearance of an asymptomatic purple papular skin rash over her left thumb Figure .A month after the initial trauma, she had started to develop the same skin rash over the dorsum of her left hand Figure , which tThe rash exhibited an unclear boundary and did not affect the right side of the body, ceasing precisely at the midline in both the anterior and posterior parts of her trunk. Apart from the thumb trauma, she had no history of drug intake, topical application, sun exposure, or contact with chemicals or plants. Liveo-violaceous, S-shaped, and linear-patterned macules and papules were observed during the dermatological examination, covering the right half of her abdomen and extending to the right lumbar region. There was also a tendency for coalescing macules and papules, which were observed extending from the left abdomen to the left lumbar area, stopping in the midline. There were no indications of involvement on the scalp, nails, or mucous membranes. Furthermore, there was no sign of lymphadenopathy. All her routine laboratory tests were within normal limits. The final diagnosis was unilateral blaschkoid LPP. The patient was given topical steroids, but she was lost to follow-up.LPP usually manifests as mottled or reticulated areas of increased pigmentation, appearing as dark brown spots on the areas of the skin exposed to the sun. These macules can also appear on flexural folds on the face and neck, as well as the axillae and inguinal regions . BasicalAccording to the histopathological analysis, the condition is associated with hyperkeratosis, dermal atrophy with loss of rete pattern, focal basal cell vacuolization, and sparse dermal infiltrate . The disThe pigmentation may be perifollicular, diffuse, reticulate, or blotchy . The disBhutani et al. first described Blaschko's lines, which are lines of typical cell development . Dossi CTo the best of our knowledge, this is the first reported case of unilateral Blaschko-linear LPP in a pediatric patient in Saudi Arabia. Due to the condition's rarity and ambiguous symptoms, it should be taken into account in the differential diagnosis of macular hyperpigmentation. The exact cause of the disease remains uncertain, emphasizing the need for further research into its etiology, pathogenesis, and treatment.