TP53 variant; however, the genetic underpinning of remaining cases has not been elucidated. In patients having germline TP53 variants, loss of maternal chromosome 11 and duplication of the paternal copy  occurs early in tumorigenesis and explains the overexpression of IGF2, the hallmark of pediatric ACT. Beckwith-Wiedemann syndrome (BWS) is also associated with overexpression of IGF2 due to disruption of the 11p15 loci, including segmental UPD. Here, we report six children with ACT with wild type TP53 and germline paternal 11p15 UPD. Median age of five girls and one boy was 3.2 years (range 0.5-11 years). Two patients met the criteria for BWS before diagnosis of ACT. However, ACT was the first and only manifestation of paternal 11p15 UPD in four children. Tumor weight ranged from 21.5 g to 550 g. Despite poor prognostic features at presentation, such as pulmonary metastasis, bilateral adrenal involvement, and large tumors, all patients are alive 8-21 years after cancer diagnosis. Our observations suggest that children with ACT and wild type TP53, irrespective of their age, should be screened for germline abnormalities in chromosome 11p15.Pediatric adrenocortical tumors (ACTs) are rare and heterogeneous. Approximately 50% of children with ACT carry a germline  TP53 mutations in 50% of cases  , atypical (patients with isolated features of BWS) and isolated lateralized overgrowth  are associated with germline of cases , 2. For  130650) , have beergrowth . Howeverergrowth . The varergrowth . Cancer ergrowth . Most ofergrowth , 8. HoweH19, which is maternally expressed in the embryo and placenta , a zinc finger protein with insulating activity, thereby preventing the expression of IGF2, and allowing transcription of H19 by downstream enhancers on the maternal chromosome  and mediates the silencing of several genes, including CDKN1C, which encodes the G1 cyclin-dependent kinase inhibitor (p57KIP2), that negatively regulates cell growth and proliferation. CDKN1C is maternally expressed in the embryo and placenta as well as postnatally throughout the body  are expressed. On the paternal chromosome, the KCNQ1OT1 promoter is not methylated, the transcript is expressed in the opposite direction of KCNQ1, and silences in cis genes of the centromeric domain on the paternal chromosome  at St. Jude Children’s Research Hospital (St. Jude). Written informed consent was obtained from parents or legal guardians for inclusion in the St Jude Children’s Research Hospital (St. Jude) International Pediatric Adrenocortical Tumor Registry . One patient (#1) was diagnosed with BWS at the time of cancer diagnosis. A second patient (#2) developed lateralized overgrowth and the ACT was found when surveilling for abdominal tumors. No features of BWS were seen during cancer diagnosis for the remaining four cases  but patients were subsequently found to harbor constitutional chromosome 11p15 alterations  which provides two outputs, one related to chromosome 11p15 copy number changes and the other methylation status of imprinting control 1  and ICR2  by the ratio of digested to undigested DNA. DNA from control individuals shows reduction of 50% of the MS-MLPA signal, corresponding to the presence of methylated alleles  and the contribution of both parental chromosomes  covering positions 1,061,991 to 4,539,851 at chromosome 11p15 (GRCh37/hg19) was performed by using a fluorescently labeled forward and conventional reverse primers as previously described  and histochemical criteria including chromogranin, cytokeratin, Cam 5.2, inhibin Figure 3The patient was an 8.5-year-old female with presumptive diagnosis of BWS who presented to the local physician in 2003 with new onset of herculean habitus, clitoromegaly, pubic hair, acne, and apocrine odor. Abdominal computed tomography imaging revealed synchronous bilateral adrenal masses. There was no evidence of disease elsewhere. The patient underwent laparotomy with excision of the left adrenal gland and tumor enucleation of the right gland. The left tumor weighed 21.5 g and measured 5.5 × 1.5 × 0.7 cm, and right tumor measured 1.3 × 1.2 × 1.0 cm. Sections of both tumors showed similar cellular constituents. Tumors were composed of a uniform population of cells with ample acidophilic cytoplasm and moderate anisonucleosis that recapitulated the zona reticularis, consistent with the diagnosis of adrenocortical adenoma. The p53 staining was negative and Ki-67 labeling index (LI) was less than 2%. Inhibin-α was positive in a subset of cells. No chemotherapy was indicated. The patient remained asymptomatic until 2008, and then developed bilateral small breast nodules. The right breast mass measured 3.5 × 2.5 × 2.0 cm and the left inferior breast mass measured 3.0 × 2.0 × 2.0 cm and both were surgically removed. Histology of the first mass had features of tubular adenoma and that of the left mass of juvenile fibroadenoma. The patient remained clinically stable until the age of 18 years when she was taken off the protocol.Molecular findings: Blood and tumor DNA of this patient was examined by whole exome sequencing (WES)  and the SIRT1 variant was not reported. Therefore, both variants have been reported in the Genome Aggregation Database (gnomAD) originated from unrelated individual sequences as part of population genetic studies supporting lack of biological significance for both variants in the context of adrenocortical tumors. WES analysis of blood-derived DNA did not detect 11p15 UPD. However, a pattern of homozygosity at chromosome 11p15 was verified by microsatellite markers analyses and confirmed by MS-MLPA consistent with paternal uniparental disomy (UPD) in the germline sample (ng (WES) . Resultshe tumor . AdditioPatient was a 2.4-year-old male with a past medical history consistent with BWS (hemihypertrophy) who had an abdominal mass in the left adrenal gland during surveillance for abdominal tumors. In addition, a hepatic mass was noted. The patient underwent laparotomy with resection of the left adrenal gland and partial hepatectomy. The adrenal tumor weighed 56 g and measured 5.9 × 5.9 × 3.6 cm. The liver lesion weighed 73 g and measured 6.5 × 4.5 × 4.0 cm. Tumors in adrenal gland and liver were histologically identical. The immune profile of both tumors was positive for pan keratin, vimentin, synaptophysin, melan-A, and was negative for inhibin-α, chromogranin, and alpha-feto protein, anti-endomysial antibody (EMA), and calretinin. Liver and adrenal masses were also negative for p53, and Ki-67 LI was <5% in both tumors. There was vascular invasion in the adrenal tumor. Peri-aortic lymph nodes were negative. Tumor margins were free of tumor. The patient was not treated with chemotherapy and remains free of disease 11 years after the diagnosis of adrenocortical carcinoma (ACC).Molecular findings: Molecular studies were done for the germline sample and the patient was wild type for TP53. Microsatellite analysis covering the position 1,061,991 to 4,539,851 at chromosome 11p15 (GRCh37/hg19) revealed a heterozygous pattern for all studied markers but MS-MLPA revealed a pattern consistent with mosaic paternal UPD.The patient was an 8.4-year-old girl diagnosed with stage II ACC at age 7 months. In 2013, she went to the local pediatric endocrinologist as she had Cushing syndrome. Her medical history revealed that she was a full-term infant with a birth weight of 6lb 12 oz (~3kg). She did not have umbilical hernia, omphalocele, macroglossia, nevus flammeus or lateralized overgrowth. An ultrasound imaging study revealed an abdominal mass in the adrenal gland region. The mass, which measured 8.2 × 7.5 × 4.5 cm was completely resected and the diagnosis of ACC confirmed. Tumor was positive for vimentin and melan-A and inhibin-α was moderate in occasional tumor cells. The Ki-67 LI was 30%. The patient was not treated with chemotherapy and has remained disease-free.Molecular findings: Blood and tumor samples were available for molecular studies. No TP53 variants were observed in the germline or tumor  analysis of germline DNA revealed normal chromosome copy number but 50% mosaicism for chromosome 11p covering a 45.6 Mb region. A pattern consistent with mosaic paternal UPD was confirmed by MS-MLPA.or tumor . The tumPatient was a 28-year-old healthy female diagnosed with non-functional metastatic ACT at age 15 months. She did not have an antecedent of endocrine manifestations, and the tumor was discovered during the one-year routine visit to the pediatrician. She did not have a history of growth and developmental abnormalities. CT imaging of the abdomen and chest revealed a left supra-renal mass measuring 5 cm in the largest diameter and bilateral pulmonary nodules (Stage IV). She underwent resection of the primary tumor that measured 7.0 × 6.0 × 4.0 cm and weighed 130 g. The histopathological examination confirmed the diagnosis of ACC. She was transferred to St. Jude and treated with cisplatin, etoposide and mitotane. However, after four courses of chemotherapy, several pulmonary lesions remained. She underwent resection of the bilateral pulmonary metastatic nodules, and most of them showed viable metastatic tumor by histology. Chemotherapy was stopped and the patient was monitored for tumor progression. The pulmonary lesions regressed and remained very small for over 10 years. She continues to be free of disease and in good health to date, 26.5 years after the diagnosis.Molecular findings: No TP53 variants were observed in blood and tumor samples , calf circumferences (39 cm vs 37 cm), and a slight leg length discrepancy (88 cm vs 85 cm) were first noted during exams performed at St. Jude as part of a multidisciplinary rare endocrine tumor clinic when she was 18 years old, and led to additional testing subsequently confirming chromosome 11p15 UPD. She has been alive and tumor free for 17 years.The patient was a 21-year-old female with past history of stage 3 ACT diagnosed at 4.9 years. Her development was normal except for an incidental note per the family of longer and larger right upper and lower extremities throughout her life that had not been specifically investigated as far as the patient and her family were aware, but was associated with chronic left-leaning posture and asymmetric shoe size. She had been in her usual state of good health until the age 4 years when she complained of abdominal pain. She also had weight loss, increased headaches, decreased activity and energy, increased tiredness, and sweating. Given the persistence of symptoms, she was examined by a physician who palpated an abdominal mass and noted hypertension and slight androgen elevations. Magnetic resonance imaging showed a large abdominal mass in the right adrenal gland, with direct extension into the inferior vena cava and nearly completely filling of the right atrium. She underwent a partial sternotomy, and extraction of the caval portion of the adrenal tumor and resection of the right retroperitoneal tumor. The tumor weight was 550 g and measured 13.5 × 10.5 × 6.0 cm. The pathology confirmed ACC . There were large areas of necrosis, and vascular invasion with tumor penetration through the capsule. Immunohistochemistry analysis was negative for p53 and positive for beta-catenin. She was treated with the combination of cisplatin, doxorubicin, etoposide, and mitotane. Features of right-sided lateral overgrowth, including right-sided tongue enlargement as well as increased right Molecular findings: Blood DNA of this patient was analyzed by whole exome sequencing (WES). No reportable structural, single nucleotide variants, and indel or copy number changes were identified in APC, CDKN1C, MEN1, PRKAR1A, and TP53. Chromosome paternal 11p15 UPD was confirmed by MS-MLPA.The patient was a 23-year-old female who had hypertension during a routine medical visit in 2009 at age 11 years. Examination for causes of hypertension revealed a large, and well-defined mass in the left upper quadrant above the left kidney and deep to the spleen. The mass was approximately 8.7 × 9.7 × 9.7 cm and was relatively round. The kidneys and liver were normal in appearance. There was no regional adenopathy. Laboratory investigation showed remarkably elevated levels of deoxycorticosterone, aldosterone, and dehydroepiandrosterone. The mass was removed one month later, and weighed 388 g and measured 11 × 10 × 6 cm. There was a 3 × 3 cm defect on the surface suggestive of tumor rupture. The tumor was composed of nests of moderate-sized cells with round nuclei, prominent nucleoli, and moderate to abundant foamy to amphophilic non-foamy cytoplasm. There was only one mitosis per ten 400× fields and there was no atypical mitosis. However, the tumor demonstrated prominent venous invasion, necrosis, and extracapsular extension. Calcifications were noted. Immunohistochemical stains were positive for melan-A, vimentin, and cytokeratin . Epithelial membrane antigen, chromogranin, and inhibin-α were negative. Ki-67 LI was <5%. Occasional areas of the tumor showed moderate nuclear staining for p53. The patient was referred to St. Jude for treatment. At that time, her adrenal hormones had returned to normal levels. She underwent comprehensive imaging studies, including FDG-PET scans. There was no evidence of residual disease. The recommendation was observation. She remains disease free 12 years from diagnosis.Molecular findings: Blood and tumor DNA of this patient was analyzed by whole genome sequencing (WGS) (TP53 in both germline and tumor DNA (ATRX and acquired the p.G34E variant in CTNNB1 and p.R201H in GNAS (ng (WGS) . The patumor DNA , 17. ACT in GNAS . WES anaCDKN1C)  at chromosome 11p15. The association between chromosome 11p15 rearrangements and embryonal tumors, including pediatric ACT, in patients with clinical diagnosis of BWS/lateralized overgrowth is well established –8. WilmsCDKN1C) , 20. TheCDKN1C) , 20. ACTCDKN1C) . HoweverCDKN1C) , 22, 23.Remarkably, four of the patients with germline paternal 11p15 UPD , and three previously reported cases of ACT with hypomethylation at ICR2 , 25 did A diversity of adrenal gland lesions is observed in BWS, including adrenal hyperplasia, hemorrhage, cysts, neuroendocrine tumors, neuroblastoma, hemangioendothelioma, ovarian thecal metaplasia, and adrenal cortical tumors (adenoma and carcinoma) . MoreoveIGF2 expression is increased in virtually all cases of pediatric ACT, irrespective of the TP53 status, and is considered an early driver event in tumorigenesis of the adrenal cortex ]  have excellent outcomes ] . In thiserations . It is noutcomes –36 whereigenesis . As the igenesis . Conversric ACTs . Althougric ACTs .Recommendations for cancer surveillance of children with BWS have been suggested by consensus panels , 38. ForEGFR (p.E257K). A germline mutation in EGFR (p.D1080N) has been reported in another patient with bilateral ACT, but without overt clinical signs of BWS. Unfortunately, in the reported case ’ legal guardian/next of kin for the publication of any potentially identifiable images or data included in this article.All authors co-wrote the manuscript and are accountable for all aspects of the work. All authors contributed to the article and approved the submitted version.This work was supported by the American Lebanese Syrian Associated Charities (ALSAC), Speer Charitable Trust, and Cancer Center Support Grant CA21765. The content is solely the responsibility of the authors and does not necessarily represent the official views of the National Institutes of Health.The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.All claims expressed in this article are solely those of the authors and do not necessarily represent those of their affiliated organizations, or those of the publisher, the editors and the reviewers. Any product that may be evaluated in this article, or claim that may be made by its manufacturer, is not guaranteed or endorsed by the publisher.


Several cases of the ectopic supernumerary tooth in the nasal cavity have been reported; however, an eruption of the primary maxillary tooth in the nose following trauma is extremely rare. Clinical evaluation and discriminating features on CT imaging, particularly with bone window setting, are sufficient to confirm the diagnosis. We discuss a case of deciduous central maxillary incisor in the nasal cavity and specifically focus on its clinical and radiological presentation. None of the previous authors have discussed probable theories of the post-traumatic intranasal tooth; we consider two possible theories which might be responsible for the ectopic intranasal tooth after trauma. Additionally, we discuss pertinent features which help distinguish ectopic deciduous tooth from the supernumerary tooth. The first case of the ectopic intranasal tooth was described by Albins in 1754 . Most ofA four-year-old boy presented with complaints of recurrent epistaxis and a bony white mass in the right nostril, in November 2020. The child’s mother initially noticed a small intranasal mass in March 2020, which she said has gradually increased to the current noticeable size. She did not visit a hospital earlier because of the coronavirus disease-19 (COVID-19) pandemic. However, the epistaxis episodes became frequent in the last few days. The child’s mother gave a history of loss of a maxillary central incisor following a dental trauma in November 2019, due to a fall. The mother was not able to find the tooth and was considered lost. A few weeks later, she felt a small and hard bump beneath the right nasolabial skin, which gradually kept drifting nasally, until she noticed a white mass in the right nasal cavity. On endoscopic nasal examination, a bony-hard, white mass was seen erupting from the floor of the right nostril and medial to the anterior edge of the inferior turbinate. A dome of granulation tissue was seen surrounding the base of the mass  and 1500-HU window width (WW), the lesion was noted to have a homogeneous high attenuation equivalent to that of the teeth and its root was buried in the mucosa of the nasal floor but was not surrounded by any bony socket. A slit-like cavity in sagittal cut and a focal central radiolucency in coronal cut, both correlating to the pulp cavity was seen Figure . These fThe patient underwent endoscopic removal of the nasal tooth. Gross examination of the excised tooth showed a root/crown ratio of ∼2, and the mesiodistal diameter of the crown exceeded its cervico-incisal length Figure . Both thEctopic teeth may be present in various sites of the maxillofacial region including maxillary sinus, palate, mandibular condyle, coronoid process, orbit, nasal cavity, or through the skin . In mostClinically, nasal teeth might be asymptomatic and detected incidentally, or they may present with a variety of signs, symptoms, or complications: nasal obstruction, nasal discharge, epistaxis, rhinitis caseosa, oro-nasal fistula, facial pain, mild fever, septal abscess, or external nasal deviation .A clinical evaluation combined with a CT scan  is usually sufficient to diagnose an ectopic primary/permanent nasal tooth. Clinically, an intranasal tooth may be seen as a white mass and surrounded by granulation tissue and/or debris. CT scan may show a radio-opaque lesion with the same attenuation as that of the oral teeth (bone density corresponds to +1000 HU). With the bone window setting, the central pulp space may be seen as a radiolucent slit or spot, depending on the inclination of the teeth . NeverthRemoval of nasal teeth is generally recommended to relieve the symptoms and prevent complications. An endoscopic-endonasal, a conventional-endonasal, or a transoral approach is advocated, depending on the age of the patient, depth of eruption, presence/absence of any bony socket, and surgeon’s experience in the endoscopic nasal surgery ,7-10.Deciduous maxillary tooth in the nasal cavity following trauma is extremely rare. Characteristic clinical features and discriminating imaging findings on CT scan are sufficient to diagnose an ectopic intranasal primary tooth. Gradual nasal growth after trauma-induced inversion of the oral denture (inversion theory) is a possibility in a patient with a remote history of trauma, and loss of denture which was later not found after trauma. Gross examination of the excised mass further confirms the type of deciduous tooth and distinguishes it from a supernumerary tooth.


Pelvic exenteration (PE) is a radical surgical procedure for treating locally recurrent or uncontrolled pelvic malignancies. The consequent postoperative pelvic dead space presents a challenge to extirpative surgeons. Many methods have been utilized for pelvic floor reconstruction to reduce related postoperative complications, however, none of them have been widely accepted.Here, we report 3 cases of patients who underwent PE. Case 1 was a 36-year-old woman who presented to our hospital with abnormal vaginal bleeding. Case 2 was a 50-year-old woman with recurrence of stage IIB squamous cell carcinoma of the cervix. Case 3 was a 54-year-old woman with uncontrolled stage IIB adenocarcinoma of the cervix. The last 2 patients were both treated with radiotherapy and chemotherapy previously.Biopsy results revealed adenocarcinoma of the vagina, squamous cell carcinoma of the cervix, and adenocarcinoma of the cervix in Case 1, 2, and 3 respectively.We describe a safe and effective approach that employs the preservation of the bladder peritoneum to eliminate the pelvic dead space following laparoscopic PE, with or without partial utilization of the greater omentum.Three patients with gynecologic cancer underwent this operation and developed no intraoperative or postoperative complications.Our experience suggests that laparoscopic PE using the bladder peritoneal barrier to cover the denuded pelvic cavity is a reasonable choice to decrease the risk of empty pelvic syndrome. This ultraradical surgery, involving complete en bloc removal of malignant lesions and pelvic viscera, is associated with a reported morbidity ranging from 32% to 84%. A substantial proportion of the severe postoperative morbidity arises from empty pelvic syndrome, which can be defined as various symptoms due to the pelvic dead space after PE, such as abscess formation, bowel obstruction, hematoma, fistulization, herniation, persistent discharge, and wound dehiscence. Multiple methods and materials have been utilized to avoid these complications including reconstructive procedures involving the utilization of the greater omentum, mammary prosthesis, myocutaneous flaps,–10 dura mater allografts, or using a degradable mesh, to eliminate the pelvic defect. However, so far, the search for an ideal solution continues.Pelvic exenteration (PE) was first described by Alexander Brunschwig in 1948 for the palliative surgical treatment of recurrent gynecologic malignancies,We report 3 cases in which the bladder peritoneum was preserved and used to close the pelvic cavity following laparoscopic PE for gynecologic malignancies. The types of laparoscopic PE performed were total pelvic exenteration (TPE) or anterior pelvic exenteration (APE), depending on the case. All 3 patients underwent operations with our novel technique that uses the bladder peritoneum to act as a biological partition to prevent empty pelvic syndrome by laparoscopy. Meanwhile, a portion of the greater omentum was employed to fill the void following TPE, because of its bulk. The basic characteristics and treatment information of the 3 patients are summarized in Table 22.1This study was approved by the medical ethics committee of West China Second Hospital of Sichuan University . Informed written consents were obtained from the patients for the purpose of publication.2.2,13 except that the peritoneum covering the surface of the bladder was preserved during cystectomy and was used for closure of the pelvic cavity. The dissection began by incising the anterior lobe of the broad ligament and was continued anteriorly in the uterovesical peritoneal reflection  demonstrated urethral and vesical invasion with no distant metastasis. Furthermore, a cystoscopy indicated a cauliflower-like neoplasm about 30 mm in diameter in the bladder neck.The patient underwent an APE via a laparoscopic approach combined with a perineal approach. Radical hysterectomy with bilateral adnexectomy, pelvic lymphadenectomy, cystectomy with bladder peritoneum preservation, as well as resection of the vagina and urethra were performed. Subsequently, the rectouterine peritoneum was pulled out and sutured with the subcutaneous tissue and skin of posterior margin of vulva to form the posterior vaginal wall. The free bladder peritoneum was sutured with the anterior margin of vulva Fig. A, and pa3.2A 50-year-old woman was admitted to our department due to recurrence of stage IIB squamous cell carcinoma of the cervix after radiotherapy (94 Gy in 33 fractions) and 5-cycle of chemotherapy. Physical examination showed a cervical neoplasm measuring 30 × 20 mm with involvement of the upper half of the vaginal wall and parametrium. PET/CT confirmed a hypermetabolic focus at the cervix and absence of distant metastasis.The patient underwent laparoscopic APE. There was tight adhesion between the bladder and the anterior wall of the uterus. Radical hysterectomy with bilateral adnexectomy, pelvic lymphadenectomy, intraperitoneal cystectomy, and partial resection of the urethra were performed. The bladder peritoneum was preserved and sutured to the rectal fascia to achieve closure of the pelvic cavity. Bilateral ureterocutaneostomy was performed with 2 mono-J catheters as ureteral stents, and a pelvic drainage tube was inserted. Samples were taken of enlarged lymph nodes that were suspicious for metastatic disease, and histological evaluation confirmed metastases in the right internal and left external iliac lymph nodes. This procedure lasted 320 minutes, with an EBL of 200 mL. The operative and postoperative courses were uneventful. The final pathological examination indicated poorly differentiated squamous cell carcinoma of the cervix invading the partial vaginal wall and bilateral parametrium. While there were 2 metastatic lymph nodes among the 4 left pelvic lymph nodes sampled, no invasion of the bladder was observed and the resection margins were negative. Finally, the patient was discharged on postoperative day 11. At the 14-month follow-up visit, the patient was doing well.3.3The patient had an adenocarcinoma of the cervix and had undergone radiotherapy (60 Gy in 30 fractions) and 5-cycle of chemotherapy. On physical examination, the top of the vagina presented erosion-like changes and contact bleeding, with involvement of the upper half of the vaginal wall and left parametrium. Magnetic resonance imaging showed a mass measuring 21 × 23 × 28 mm, located predominantly in the anterior lip of the uterine cervix, with questionable invasion into the parametrium and the vagina. PET/CT demonstrated a cervical tumor without distant metastasis.The patient underwent TPE during laparoscopic and transperineal surgeries. There was tight adhesion between the bladder and the anterior wall of the uterus and vagina. Radical hysterectomy, bilateral adnexectomy, pelvic lymphadenectomy, resection of the vagina, rectum, anus, bladder, and urethra were performed with bladder peritoneum preservation Fig. A. The sp4 advances in patient selection, preoperative preparation, surgical technique, and postoperative care have spared many patients from unnecessary complications; however, morbidity following PE still remains a concern.Since Alexander Brunschwig first introduced PE in 1948, Therefore, post-PE management of the pelvic dead space poses a challenge to gynecologists.A substantial proportion of postoperative complications are associated with pelvic dead space after PE. The absence of mechanical support after the removal of the muscles of the pelvic floor and pelvic organs may allow intraabdominal contents to descend, which together with bowel adhesions deep in the denuded pelvis, may lead to intestinal obstruction. When postoperative radiotherapy is needed, the risk of chronic radiation enteritis increases. Moreover, small bowel translocation into the pelvis may also result in the occurrence of a perineal hernia. The void created after radical multivisceral resection leads to pelvic fluid accumulation and may increase the risk of perineal wound infection, abscess formation, and persistent discharge. In addition, the downward pressure of descending intraabdominal contents and the accumulation of fluid in the pelvis may contribute to wound dehiscence. This series of events has been termed the “empty pelvic syndrome” by some authors. prosthetic implants, silicone expanders, and degradable mesh. Other methods employ autologous tissues, including the greater omentum, myocutaneous flaps based on the gracilis, rectus abdominis muscle, gluteus maximus, or the latissimus dorsi muscle.To minimize these unfavorable consequences caused by pelvic voids, a number of methods have been used for pelvic floor reconstruction after extirpation of pelvic organs; however, all these methods have advantages and disadvantages, but none has gained widespread acceptance. Some of these methods include artificial materials, such as polypropylene mesh,,16 The above reconstructive techniques often require additional resources including time, cost, and expertise while negating some benefits of the laparoscopic approach.Pelvic exenteration is traditionally performed with an open approach. However, recent advances in medical technology make it possible to perform complex laparoscopic operations. Laparoscopic PE has been demonstrated to be a feasible procedure with many advantages over open PE, such as less EBL, a shorter duration of hospitalization, and lower postoperative complication rate.This report describes a novel method that employs the preserved bladder peritoneum to close the pelvic void after laparoscopic APE, along with part of the greater omentum after TPE. To the best of our knowledge, this is the first report of the use of this approach. In addition, the use of the bladder peritoneum and the greater omentum has many common advantages. Compared to artificial materials, there is a reduction in allergic and immune reactions, and the risk of displacement is less. Compared to the use of other autologous tissues, the surgical technique presented here is less invasive and easier. There were also no scars on the body due to graft harvesting associated with donor-site morbidity, and it is convenient to peel the peritoneum off the bladder by laparoscopy. The data demonstrate that the newly formed pelvic floor covered the denuded pelvic cavity and prevented the remaining bowel from descending into the lower pelvis effectively.The major strength of this procedure in our cases is that it provides a biological partition between abdominal contents and the pelvic cavity after PE, which can prevent the small bowel entering the lower pelvis. The smooth bladder peritoneum covering the rough pelvic wall can not only decrease the incidence of adhesions, but also absorb exudate present in the wound. The efficacy of using the greater omentum for pelvic floor reconstruction is due to its rich blood supply and lymphatic network, large surface area, and malleable volume, as confirmed by numerous studies.PE has functional, psychological, and psychosexual influence on patients after the surgery; therefore, indications for PE should be carefully determined. The surgical procedures outlined in these cases are not applicable in certain instances, for example: in patients with peritoneal dissemination, distant visceral metastasis, or extrapelvic lymph node involvement. We carefully selected patients in this study, and these operations were performed successfully, achieving clear resection margins (R0). No intraoperative or postoperative complications were observed in any of the 3 patients. All patients were doing well at the most recent follow-up.The main limitations of this study are the small number of cases and the relatively short follow-up period; hence, further investigations are still required. Nonetheless, these results demonstrate the short-term effectiveness and safety of this new approach in selected patients.5In conclusion, it is reasonable to utilize the preserved bladder peritoneum for closure of the pelvic cavity following laparoscopic PE, either with or without partial utilization of the greater omentum. Our method employs a simpler technique than other similar operations, and can reduce the risk of empty pelvic syndrome.Conceptualization: Ping Wang.Data curation: Yiran Wang, Ping Wang.Supervision: Ping Wang.Writing – original draft: Yiran Wang.Writing – review & editing: Yiran Wang, Ping Wang.


Adrenocortical carcinoma (ACC) is a rare, highly malignant endocrine tumor, often associated with a poor prognosis. Most patients who develop ACC are either children of ages 1-6, or adults in their fourth to fifth decade of life. Individuals with a functional cortisol-secreting ACC frequently present with Cushing syndrome. We report a case of an 18-year-old male who was found to have a large ACC tumor, with thrombus extension into the inferior vena cava (IVC), after presenting with Cushing syndrome. ACC presents a challenging scenario for physicians as surgical resection remains the only form of curative therapy, however, despite such treatment many patients quickly develop metastases. Adrenocortical carcinoma (ACC) is a rare, highly malignant endocrine tumor arising from the adrenal cortex, with an annual incidence of one per million per year . ACC canAn 18-year-old male was referred to our hospital for further evaluation of lower extremity swelling. He had also developed new acneic skin issues, hypertension, truncal obesity, temporal fullness, thickening of facial hair, and skin striae involving the axillae, groin, and lower abdomen. A diagnosis of Cushing syndrome was made after serum cortisol came back elevated with adrenocorticotropic hormone (ACTH) being decreased. In Figure An open left radical adrenalectomy and nephrectomy with IVC thrombectomy were performed. Final pathology was consistent with ACC of the left adrenal gland demonstrated by a Weiss score of 6. The specimen included vascular invasion, caval thrombus, and 11 interaortal caval lymph nodes negative for malignancy.The patient recovered well from surgery. He subsequently received adjuvant radiation. While being considered for mitotane therapy, the patient underwent repeat CT imaging three months postoperatively and was found to have developed liver and distant metastases. After thorough consideration, he declined palliative treatment and succumbed to his disease shortly thereafter.ACCs are devastating tumors that continue to burden patients with a very poor prognosis. Physicians currently lack a strong method for early detection of these tumors, which may partially explain why most ACC tumors are identified in advanced stages . The majACC can present as either a functional or non-functional tumor, with each accounting for approximately half of all cases . IsolateSurgical resection remains the only curative treatment option for ACC . AdjuvanThis case demonstrates a rare case of cortisol-secreting ACC with invasion into the IVC. ACC is an aggressively malignant tumor with surgical resection as the only curative option. A substantial subset of patients who develop ACC present with Cushing syndrome. Due to the aggressive nature of ACC, it is important for physicians to be aware of such presentations so that early recognition and treatment can begin. Despite resection, the large majority of patients go on to develop metastatic disease. These tumors present a challenging scenario for physicians as current literature does not offer great insight into options for the prevention or prediction of metastasis.


Ganglion cysts are fluid filled sacs which develop near joints and tendons and are usually asymptomatic. Lower limb ganglion cysts are rare occurrences especially those situated around joint spaces causing nerve compression. We present the case of a 68 year-old female with history of progressive swelling in the left antero-lateral leg, associated with pain, and neurological symptoms of peroneal nerve compression. Magnetic resonance imaging (MRI) revealed a large proximal tibiofibular joint ganglion cyst causing peroneal nerve compression. One year following the left sided presentation, the patient presented with similar but less severe symptoms in her right antero-lateral leg. MRI revealed a small juxta-articular ganglion cyst in the right proximal tibiofibular joint space. We discuss etiology, symptoms, and management of lower limb ganglion cysts. Ganglion cysts are fluid filled structures which develop near joints and tendons. They are usually asymptomatic and rarely require any treatment A 68-year-old female presented to the orthopedic outpatient clinic with a several-month insidious onset of a lump over the lateral aspect of her left knee, with associated lateral based knee pain symptoms. These were initially severe however subsided into a more chronic state with associated nocturnal issues. Additionally, the patient began to experience localized sensory symptoms over the dorsal aspect of the left 3rd, 4th and 5th toes, with sparing of the web spaces and the rest of the dorsum foot. She also reported being prone to tripping after prolonged walking as well as a mild foot drop. The patient has no prior history of knee surgery or trauma, and the only past medical history of note is long term hypertension, for which she has been managed with antihypertensive medication.On clinical examination, the patient had full range of motion in her knee. There were no signs of joint effusion and the knee was stable on both collateral and cruciate ligament testing. There was some mild medial joint line tenderness but no lateral joint line tenderness. She had a large palpable mass over the proximal lateral aspect of her lower leg as well as pain around the proximal tibiofibular joint. Lower limb neurological examination revealed 4 of 5 power in her ankle dorsiflexion and 5 of 5 power in her great toe dorsiflexion, using the Medical Research Council's scale  of muscle power Magnetic resonance imaging (MRI) of the left knee without contrast was performed using a protocol of T1-weighted and Proton Density Fat Suppressed weighted images, in the sagittal, coronal, and axial planes.Images revealed a large multi-loculated ganglion cyst anterolateral to the proximal fibula, measuring 3 cm (anteroposterior) by 2.8 cm (transverse) by 7 cm , causing peroneal nerve compression . AdditioNerve conduction studies were done using electromyography (EMG) on the left leg which displayed present but reduced left common peroneal and left sural sensory responses. In addition, left common peroneal motor response to extensor digitorum brevis was absent when stimulated at the ankle, fibula head and popliteal fossa. No voluntary motor units could be recruited in the left extensor digitorum brevis.Following the EMG studies and MRI, the results were discussed with the patient, and it was agreed that the patient would not undergo surgical excision. The patient decided this based on the associated risks of surgery ie, infection, pain, ongoing disability, common peroneal palsy and permanent nerve damage. She was recommended for conservative management with regular follow-up at the outpatient clinic.A year following the left-sided presentation, the patient presented with new symptoms of pain over the antero-lateral aspect of her right leg and paresthesia in the lateral border of the right foot. On examination, there was a mild degree of swelling in the anterolateral aspect of her right proximal tibia, with altered sensation in the lateral three toes but normal motor power. The symptoms in her left leg remained unchanged with slight increase in size of swelling.MRI without contrast of the right knee was completed, using a protocol of T1-weighted in sagittal plane and Proton Density Fat Suppressed weighted images in the sagittal, coronal and axial planes. This revealed a well-defined lesion measuring 1.1 cm (anteroposterior) by 1.7 cm (transverse) by 1.8 cm  in the interosseous space immediately inferior and in communication with proximal tibiofibular joint space . The appThe patient was conservatively managed with pain relief medication for neuropathic pain and is being followed up in clinic. To this date no surgical excision or decompression has been performed in either leg.The proximal tibiofibular joint, also known as the superior tibiofibular joint, is a plane type joint which consists of the intersection between the head of the fibula and the lateral condyle of the tibia. There is some gliding motion able to take place at this joint however, the main function of the joint is to assist in stability of the lower limb while weight bearing ,7. The jGanglion cysts are non-malignant, fluid-filled sacs that commonly present in the hand and wrist ,10. ThosThe cause of ganglion cysts remains unclear, but is believed to be a result of degenerative changes within the connective tissue associated with joint capsules and tendon sheaths Involvement of the common peroneal nerve due to compression leading to neuropathy is a rare event with very few cases reported in the literature . One casMRI is the preferred diagnostic test for ganglion cysts. It provides accurate localization and delineation of the lesion and distribution of the muscles supplying the peroneal nerve, due to the excellent soft tissue contrast. This is important for surgical planning. Lesions appear as unilocular or multilocular, lobular fluid collections and present with low signal intensity on T1-weighted sequences and high signal intensity on T2-weighted sequences Ganglion cysts do not usually require treatment, and many will resolve on their own. For painful cysts, aspiration or surgery may be recommended ,18. SurgWhile ganglion cysts arising in the upper limb are common and well documented in the literature, a lower limb ganglionic cyst arising in the tibiofibular joint space is a rare finding that should be noted on routine imaging, especially when causing symptomatic peroneal nerve compression. These can be debilitating, and surgical excision is the preferred treatment. This however carries the risk of nerve damage, and risk of recurrence.Written, informed consent for publication of the case was obtained from the patient.


Soft tissue sarcomas (STS) represent a small group of adult solid malignancies, with risk factors such as environmental factors, genetic predisposition, and prior radiotherapy. In STS patients with a novel swelling, differential diagnoses include recurrence, second primary cancer, metastasis from unknown primary cancer, and radiation-associated STS, the latter usually occurring approximately 10 years after radiotherapy. We present the case of a 64-year-old male patient with pleomorphic rhabdomyosarcoma, who underwent resection and radiotherapy. The patient presented again 5 years later with painful swelling in the area of the prior sarcoma, raising suspicion of recurrence. Resection was performed and a diagnosis of pleomorphic sarcoma (not otherwise specified [NOS]) was made. The patient was treated with radiotherapy and remained sarcoma-free for the following 7 years. A molecular analysis of both neoplasms, using RNA next-generation sequencing, did not detect any specific fusions. Due to the lack of rhabdomyoblastic differentiation in the second sarcoma and the low likelihood of a second primary in the same previously irradiated location, the diagnosis of a radiation-associated STS was suggested. This short report illustrates the difficult diagnostic work-up of a presumably radiation-associated STS, as these neoplasms lack characteristic morphological and immunohistochemical features. In our case, the suggested diagnosis may have pointed against another course of radiotherapy in an already irradiation-harmed region. Therefore, a relatively low latency period between surgery, radiotherapy, and diagnosis of another STS should not automatically point towards recurrence and may prompt further in-depth investigation. Sarcomas in general account for less than 1% of all adult solid malignancies and of all sarcomas the vast majority are soft tissue sarcomas (STS) with an estimated incidence of 3–4 per 100,000 patients per year .Due to the low incidence and more common differential diagnoses, STS are difficult to diagnose. Differential diagnoses in patients presenting with soft tissue swelling include preceding trauma with concurrent hematoma as well as a metastasis from unknown primary cancer. The STS patients presenting years after initial diagnosis with a novel swelling are highly suspicious for recurrence; however, second primaries or soft tissue metastasis from other cancers should be considered. Another differential diagnosis to keep in mind is radiation-associated STS, usually occurring approximately 10 years after radiotherapy , 4.In the following short report, the history, management, and later diagnostic work-up of a patient with two STS in the same location, occurring over a prolonged period are presented.In 2008, a 64-year-old male patient presented to our outpatient clinic after a tumor excision located on his right forearm at a different hospital. Histological analysis revealed a malignant mesenchymal neoplasm composed of haphazardly arranged spindle-shaped and pleomorphic cells with prominent nucleoli and prominent eosinophilic cytoplasm Fig. . ImmunohDuring the patient’s stay at our center, a melanoma located in the neck was excised. The melanoma was not ulcerated and infiltrated 0.75 mm into the dermis (AJCC 2018: pT1a). Due to positive resection margins of the first excision, a re-resection with safety margins of 1cm was performed. Histological analysis revealed a melanocytic nevus without further evidence of melanoma.In 2013, 5 years after the first sarcoma resection, the patient presented with painful swelling of his right forearm, which he had first noticed 1 month previously, without preceding trauma. Since 2008, multiple nevi and another melanoma, located in the thoracic region, had been excised, but the patient could not provide further information or medical records regarding this topic. Magnetic resonance imaging (MRI) showed a 2 cm large expansion in the subcutis arising at the same location as the pleomorphic rhabdomyosarcoma resected in 2008. The lesion showed contrast enhancement and was directly attached to the muscular fascia. These findings suggested recurrence.An incisional biopsy was performed and showed a malignant mesenchymal neoplasm composed of pleomorphic cells with enlarged irregular nuclei, focally prominent nucleoli, and abundant eosinophilic cytoplasm. Numerous mitotic figures were present Fig. . ImmunohA wide resection was performed but histological analysis revealed positive resection margins. A subsequent re-resection resulted in negative margins. Negative pressure wound therapy and split-skin graft were subsequently applied to support wound healing. Subsequently, the patient underwent radiotherapy of the right forearm, with a dose of 25 Gy applied in 25 fractions. He remained disease-free for the following 7 years up to this date with regular follow-ups consisting of thoracic CT scans and MRI scans of the right forearm.ALK, BCOR, BRAF, CAMTA1, CCNB3, CHMP2a, CIC, EPC1, EWSR1, FOS, FOSB, FOXO1, FUS, GLI1, HMGA2, JAZF1, KMT2a, MEAF6, MGEA5, MKL2, NCOA2, NTRK1, NTRK2, NTRK3, PAX3, PDGFB, PLAG1, RAB7a, RET, ROS1, SS18 (SYT), STAT6, TAF15, TCF12, TFE3, TFG, TGFBR3, USP6, VCP, and YWHAE. In both specimens, no specific fusions were detected. A pathological re-evaluation confirmed the diagnoses of a pleomorphic rhabdomyosarcoma and a pleomorphic sarcoma (NOS). Due to the low likelihood of a second primary in the same previously irradiated location, the diagnosis of a radiation-associated STS was suggested.In 2020, the molecular analysis  using next-generation sequencing-based anchored multiplex PCR technique, which had not been available at our institution when the patient underwent treatment, was performed on both the pleomorphic rhabdomyosarcoma and the pleomorphic sarcoma (NOS). The panel searched for fusion in the following genes: In the current case a patient presented with painful swelling of his right forearm and history of pleomorphic rhabdomyosarcoma with subsequent radiotherapy in the same location as well as two melanomas located elsewhere. Pathological analysis of the specimen showed pleomorphic sarcoma (NOS). Although neither histomorphological nor immunohistochemical analysis proved it to be a recurrence of the known pleomorphic rhabdomyosarcoma or a metastasis of the melanoma, recurrence was the most likely differential diagnosis. This assumption was supported by the short latency period of 5 years between radiotherapy and diagnosis of pleomorphic sarcoma (NOS), together with the low probability of a different sarcoma in the same location. The postulated recurrence was treated with wide resection and radiotherapy.A fusion panel, performed on both neoplasms (primary specimen and suspected recurrence) to gain more knowledge of the neoplasm’s biology, showed no specific fusions in both specimens. The immunohistochemical evaluation confirmed the lack of rhabdomyoblastic components in the second neoplasm, thus arguing against local recurrence but favoring the second neoplasm. Due to preceding radiotherapy exposure, it was concluded that the STS may have been triggered by radiotherapy of the first sarcoma, thus possibly constituting a radiation-associated STS.According to the literature the median time between radiotherapy and the occurrence of radiation-associated STS ranges between 9.3 years , with soThe problem in our case was the short latency period between radiotherapy and the occurrence of the second neoplasm, pointing away from radiation-associated STS, and the relatively obvious differential diagnosis of a local recurrence due to the location of the second neoplasm; however, considering that radiation-associated STS can occur with a latency period considerably below the median time range reported in the literature and that the second neoplasm did not show rhabdomyoblastic differentiation, radiation-associated STS rather than local recurrence seems reasonable.The differentiation between a recurrent and a secondary radiation-associated malignancy is crucial for patients’ treatment. The subsequent radiotherapy in a possibly radiation-associated STS may have been avoided in this patient. Furthermore, radiation-associated sarcomas have worse disease-specific survival than sporadic sarcomas , 4, as wLimitations of our diagnostic work-up include the absence of typical morphology and lack of robust markers for the diagnosis of radiation-associated STS. Prieto-Granada et al. reported the usefulness of loss of H3K27me3 expression as a reliable ancillary marker in the context of radiation-associated or sporadic malignant peripheral nerve sheath neoplasm [This case illustrates the difficult diagnostic work-up of a presumably radiation-associated STS, as these neoplasms lack characteristic morphological and immunohistochemical features. In our case, this diagnosis may have pointed against another course of radiotherapy in an already irradiation-harmed region. Therefore, a thorough medical history with examination and radiology should be combined with histopathology for a diagnosis that is as accurate as possible. Furthermore, a relatively low latency period between surgery, radiotherapy, and diagnosis of another STS should not automatically point towards recurrence and may prompt further in-depth investigation.


Case report. A 68-year-old female presented with symptomatic hypoglycemia, weight loss, and abdominal mass identified on CT scan of the abdomen. Blood work during symptomatic hypoglycemia was consistent with an IGF-2-producing tumor. The abdominal mass pathology was consistent with solitary fibrous tumor surrounding the adrenal gland, and resection resulted in complete resolution of hypoglycemia. Discussion. Understanding the biochemical mechanisms behind glucose regulation is necessary to diagnose and adequately treat Doege–Potter syndrome, a paraneoplastic syndrome observed in patients with solitary fibrous tumors. Solitary fibrous tumors can be characterized by specific histologic and immunohistochemical studies.  We present a case of refractory hypoglycemia, weight loss, and retroperitoneal solitary fibrous tumor.  This report describes the clinical workup of a patient presenting with hypoglycemia and a retroperitoneal tumor. This case is unique because of its presentation with severe, refractory hypoglycemia and the tumor's location in the retroperitoneum, given the majority of solitary fibrous tumors are found in the lungs originating from the pleura. A 68-year-old female patient presented to the emergency department with refractory neuroglycopenic symptoms. In the emergency room, her glucose level was noted to be <50 mg/dL on multiple occasions despite repeated administrations of IV dextrose and PO intake. She described a 3-week history of episodes characterized by confusion, diaphoresis, and severe weakness which were improved with ingestion of food. She also described a weight loss of 30 pounds over the last 4-5 months despite normal appetite. She had no history of type 1 or type 2 diabetes mellitus and did not use insulin or antidiabetic medications. Medical history included hypertension and gastroesophageal reflux, and she did not endorse a history of smoking, alcohol, or recreational drug use. No pertinent family history was identified. On physical examination, she was found to have a palpable, mildly tender right upper quadrant mass and palpable hepatomegaly. A CT scan demonstrated evidence of an 18.6 cm mass projecting inferior to the liver and superior to the right kidney, in the approximate location of the right adrenal gland . The masClinical workup of her hypoglycemia proceeded as follows . HypoglyScreening for use of oral hypoglycemic agents to rule out factitious hypoglycemia was negative. Antibodies to insulin were also undetectable. Our surgical colleagues recommended ruling out adrenal hypersecretion, as elevated precursor hormones are often seen in rapidly dividing adrenocortical carcinoma. Serum renin, aldosterone, 11-deoxycorticosterone, and 17-hydroxyprogesterone were all within normal limits. 5-HIAA and urine metanephrine levels were normal as well, which lessened suspicion for pheochromocytoma or neuroendocrine tumor. Although these conditions are not classically associated with hypoglycemia, we felt it would be useful to exclude additional causes of a large retroperitoneal mass.Given the in-depth negative workup other than the abdominal mass, the decision was made to pursue resection. The gross pathology specimen  showed aUnfortunately, IGF-1 level, which was drawn at the time of a hypoglycemic episode, could not be resulted because the sample was hemolyzed en route to the laboratory. By the time we were alerted, the patient was in pre-op for surgical resection. It would have been ideal to have this value, but this case highlights that clinical suspicion in the setting of appropriate imaging, low insulin, C-peptide, and beta-hydroxybutyrate, and an increase in glucose following glucagon injection point to an IGF-2-producing tumor. Finally, in the setting of an acutely ill patient, we anticipate a low/suppressed IGF-1 level.A follow-up CT scan one month after surgery demonstrated interval resection of the previously noted large, lobulated right suprarenal mass. There were no findings to suggest residual or recurrent disease.Solitary fibrous tumors (SFTs) arise from mesenchymal cells and account for less than 2% of all soft tissue tumors . SFTs usThe effectiveness of the normal physiological reactions to falling blood glucose concentrations makes hypoglycemia an uncommon clinical event. The first response to falling serum glucose concentration is a decrease in the secretion of endogenous insulin. Once glucose concentrations fall below the normal physiological range, counter-regulatory hormones are released in response simulating glucagon, cortisol, catecholamine, and growth hormone secretion . Togetheβ-hydroxybutyrate, as an alternative energy source for the brain [Although hypoglycemia from non-islet cell tumors is very rare, when present it often results from overproduction of pro-insulin-like growth factor-2 (IGF-2) which is an incompletely processed high molecular weight protein. Due to its poor affinity to serum IGF-2 binding proteins, IGF-2 freely enters tissue eliciting its insulin-like effects and therefore leads to hypoglycemia , 8. Whenhe brain , 10. Howhe brain . Just lihe brain . Continuhe brain , 12. Suphe brain . Althoughe brain . We woulFrom a pathology perspective, the gross appearance of a typical or conventional SFT is a well-circumscribed mass with tan-white homogeneous cut surfaces that can be cystic or hemorrhagic with sizes ranging from 1 cm to 20 cm . HistoloImmunohistochemically, CD34, Bcl-2, and CD99 SFT usually show a strong diffuse pattern of staining; however, in 5–10% of SFT, CD34 can be negative. These markers can also show positivity in other neoplasms of mesenchymal differentiation, limiting the utility of these markers, particularly CD34, in the workup of the differential diagnosis . The mosβ cells. This resolved with decreasing levels of IGF-2 and a re-equilibration in glucose homeostasis. A study by Le Jeune et al. [The only definitive treatment for NICTH is complete tumor resection. As in previously reported cases, our patient's symptoms completely resolved once the tumor was resected. Normalization of plasma glucose levels occurred shortly after tumor resection. Postoperatively, during the recovery phase, she experienced episodes of postprandial hyperglycemia. This was presumably secondary to residually suppressed insulin secretion from pancreatic e et al.  showed te et al. .In cases of incomplete resection or tumors that cannot be surgically removed, there are medical management options. Han et al.  describeSFTs have been described in extrapleural sites such as the upper respiratory tract and the orbits but are most commonly found in the lungs originating from the pleura. If hypoglycemia is demonstrated in connection with a solitary fibrous tumor, it is referred to as the Doege–Potter syndrome. This case is unique because of its presentation with severe hypoglycemia and its location “outside the thoracic cavity.”


Associated midgut malrotation and colon cancer in adult patients is extremely rare and associated with delayed diagnosis and increased morbidity. We present the case of a patient with a three-week history of weakness, diarrhea, and abdominal pain with invagination of the distal ileum. Exploratory laparotomy with ileocecal resection revealed invagination, malrotation, and cecal adenocarcinoma. Intestinal malrotation is an in-utero developmental disorder, characterized by an improper fixation of the midgut and failure of its normal embryonic rotation between the fifth and 12th week of gestation . ConcomiAn 86-year-old woman with Alzheimer’s disease was admitted to our emergency department after an approximately three-week history of weakness, diarrhea, and abdominal pain. She had a medical history of open appendectomy in adolescence, endoscopic colonic polypectomy, and no previous abdominal pain. Malrotation was not known beforehand. A physical examination revealed conspicuous abdominal tenderness and abdominal distension. No lump was palpable. Laboratory values showed an elevated C-reactive protein (93 mg/L), normal lactate, and anemia (67 g/L) requiring blood transfusion. Abdominal computed tomography (CT) with intravenous contrast Figure  showed aIntestinal malrotation is a congenital abnormality resulting from a less-than-normal 270° anti-clockwise rotation of the midgut along its vascular pedicle during embryologic development. More than 90% of patients with intestinal malrotation present symptomatically within the first year of life, although malrotation can remain asymptomatic even in adults ,13.TherWe report the case of a patient with cecum cancer who also had intestinal malrotation and distal ileum invagination. Open surgical resection and lymphadenectomy were performed. Anatomical malrotations can lead to challenging approaches for surgical resection. For safety, surgeons should always consider a laparotomy if malrotation is suspected on imaging beforehand. The causal relationship between intestinal malformation and carcinogenesis is not clearly established and remains to be further investigated.


Magnetic Resonance Imaging (MRI) is the best approach to investigate the hypothalamic-pituitary region in children with central precocious puberty (CPP). Routine scanning is controversial in girls aged 6-8 year, due to the overwhelming prevalence of idiopathic forms and unrelated incidentalomas. Cerebral lipomas are rare and accidental findings, not usually expected in CPP. We report a girl with CPP and an unusually shaped posterior pituitary gland on SE-T1w sequences.A 7.3-year-old female was referred for breast development started at age 7. Her past medical history and physical examination were unremarkable, apart from the Tanner stage 2 breast. X-ray of the left-hand revealed a bone age 2-years ahead of her chronological age, projecting her adult height prognosis below the mid parental height. LHRH test and pelvic ultrasound were suggestive for CPP. Routine brain MRI sequences, SE T1w and TSE T2w, showed the posterior pituitary bright spot increased in size and stretched upward. The finding was considered as an anatomical variant, in an otherwise normal brain imaging. Patient was started on treatment with GnRH analogue. At a thorough revaluation, imaging overlap with adipose tissue was suspected and a new MRI scan with 3D-fat-suppression T1w-VIBE sequences demonstrated a lipoma of the tuber cinereum, bordering a perfectly normal neurohypophysis. 3D-T2w-SPACE sequences, acquired at first MRI scan, would have provided a more correct interpretation if rightly considered.This is the first evidence, to our knowledge, of a cerebral lipoma mimicking pituitary gland abnormalities. Our experience highlights the importance of considering suprasellar lipomas in the MRI investigation of children with CPP, despite their rarity, should the T1w sequences show an unexpected pituitary shape. 3D-T2w SPACE sequences could be integrated into standard ones, especially when performing MRI routinely, to avoid potential misinterpretations. Magnetic resonance imaging (MRI) is the gold standard for investigating the pituitary gland and adjacent structures in children with pituitary related disorders , includiA 7.3-year-old female was referred to the pediatric endocrine outpatient clinic for assessment due to breast development that started at age 7. The patient was born at term by spontaneous delivery without complication, and her past medical history was unremarkable. In the family history paternal grandmother was reported to have menarche at 9 years. Father’s height was normal , and reliable information about his puberty onset was not available, making firm conclusion on potential CPP in the father impossible. No history of CPP was reported in the maternal branch. After initial examination, which was unremarkable apart from the Tanner stage 2 breast see Table 1,Brain MRI is used in CPP work-up to exclude rare neoplasia  underlying the precocious activation of the hypothalamic-pituitary-gonadal axis . MRI is Brain lipomas are, classically, accidental findings during brain imaging (CT or MRI scans) originally arranged for other reasons. Since 1987 many theories have been postulated to explain the pathogenesis of intracranial lipomas. Nowadays the most accepted considers them as congenital malformations resulting from abnormal persistence and abnormal differentiation of the meninx primitiva during the development of the subarachnoid cisterns , 23. LipIntracranial lipomas can be tubulonodular or curvilinear in shape , and theOn review of the first scan images, 3D-T2w SPACE sequences had been acquired but not enough considered to evaluate the unusual finding in the SE-T1w sequences. 3D-T2w SPACE sequences showed the lipoma conserved a moderately hyperintense signal, differing from the neurohypophysis, which appeared normally hypointense Figure 3Our report is not the first describing a cerebral lipoma in CPP. Osteolipomas of the tuber cinereum have been described in females in both 2002 and 2012 , 10, andOur experience highlights the importance of considering suprasellar lipomas in the MRI investigation of children with CPP, despite their rarity. Furthermore, we suggest the (routinely) use of 3D-T2w SPACE sequences to avoid potential misinterpretations should the T1w sequences show an unexpected pituitary bright spot shape, in way to promptly complete the examination with 3D-FAT suppression T1w VIBE sequences.The repetition of an MRI, in general, may generate in patients, and their family, further anxiety about own health, and uncertainty in the information received by the medical team. Therefore, every single brain MRI, especially in children, should carefully performed to be conclusive on the findings reported, to the best of moderns imaging techniques. The implementation of 3D-T2w SPACE sequences in a standard MRI scan, as demonstrated in this case, allow to point to the correct interpretation of abnormal neurohypophysis shapes from the first scan. Its routinely use in patients with central precocious puberty positively impacts on the medical management, avoiding potential confounding messages to the families, due to a significant percentage of cerebral incidentalomas, even in those as rare as suprasellar lipomas.The original contributions presented in the study are included in the article/Written informed consent was obtained from the minor(s)’ legal guardian/next of kin for the publication of any potentially identifiable images or data included in this article.CG managed the endocrine investigation of the patient, drafted the manuscript, and supervised the entire work. LN, CS, and EP performed the MRI scans and revised the manuscript for the MRI imaging details. MM postulated the presence of a cerebral lipoma and revised the entire manuscript. All authors contributed to the article and approved the submitted version.The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.All claims expressed in this article are solely those of the authors and do not necessarily represent those of their affiliated organizations, or those of the publisher, the editors and the reviewers. Any product that may be evaluated in this article, or claim that may be made by its manufacturer, is not guaranteed or endorsed by the publisher.


Thymic cysts are rare lesions  and, most of the times, are incidental findings. The coexistence of a lymphoma and a thymic cyst is rare. In the case reported herein, microscopic foci of a diffuse large B-cell lymphoma were identified in the wall of a resected thymic cyst. This case report adds to the current knowledge of this rare entity and highlights the necessity of early surgical resection of mediastinal cysts over watchful waiting. Thymic cysts are rare lesions  and, most of the times, are incidental findings . The coe18fluorodeoxyglucose PET-CT scan showed hypermetabolic activity in the periphery of the mass  with no other local or distant hyperfixation  with cystic features situated in the anterior mediastinum. There were no signs of invasion of the adjacent structures . An 18flfixation . The lesfixation . The posThe histopathological analysis of the surgical specimen was compatible with a thymic cyst. However, in its wall, there were foci of neoplastic cells, which were bigger than adjacent lymphocytes. Some of the cells were even bigger; they had bigger and multiloculated nuclei that were similar to those of Hodgkin cells. Immunohistochemical analysis was negative for cytoceratin AE1/AE3, placental alkaline phosphatase, and Octamer-binding transcription factor 3/4, and positive for CD45, CD20, CD79a, and CD30. Many neoplastic cells were partially positive for p63 . In concTaking into account the aggressive potential of the disease, the multidisciplinary tumor board decided to administrate adjuvant treatment, even though resection margins were free from tumor invasion and thymic cyst involvement by the neoplastic tissue was microscopic. Consequently, 6 cycles of an R-CHOP regimen  were administered.Thymic cysts are rare lesions and can be unilocular or multilocular –9. Most The coexistence of a lymphoma and a thymic cyst is a rare entity, with few cases reported in the literature –9. The eNeoplastic cells can be organized in nodules, and they are bigger than normal lymphocytes, as in the case reported by Haque et al. . The cysThe coexistence of a thymic cyst and a lymphoma is rare and, most of the time, is discovered during histopathological examination of the resected specimen. The exact pathogenetic mechanism is unknown. This case report adds to the current knowledge of this rare entity and highlights the necessity of early surgical resection of mediastinal cysts over watchful waiting.The original contributions presented in the study are included in the article/supplementary material, further inquiries can be directed to the corresponding author/s.Written informed consent was obtained from the patient for the publication of any potentially identifiable images or data included in this article.AA: data analysis and manuscript writing. IP: literature research and manuscript writing. KG: synthesis of data, editing, and manuscript writing. SM: manuscript writing and final approval. All authors contributed to the article and approved the submitted version.The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.All claims expressed in this article are solely those of the authors and do not necessarily represent those of their affiliated organizations, or those of the publisher, the editors and the reviewers. Any product that may be evaluated in this article, or claim that may be made by its manufacturer, is not guaranteed or endorsed by the publisher.


Odontomas are benign tumors of jaws with mixed tissue, which are the result of proliferation of odontogenic epithelium and mesenchymal cells. They occur almost centrally and seldom peripherally.There is no report of such a lesion externally while attaching a tooth crown. In this case, we present a lesion on the buccal surface of the right maxillary central incisor crown,which is misconstrued with a dental overgrowth. Such cases may confuse diagnosis during clinical examination. Radiographically, such odontomas may be mistaken for various other lesions.Ultimate diagnosis should be relied upon microscopic evaluation and histopathological results. Odontomas are benign lesions with mixed tissue, which are the result of proliferation of odontogenic epithelium and mesenchymal cells. Unlike real neoplasms, odontomas are most likely hamartomas  . Based The accurate etiology of odontomas is still unclear, but infection, local trauma, genetic factors, or family history of some syndromes such as Gardner syndrome and Hermann syndrome can cause odontoma  . The moIn the literature, there is no report of such a lesion externally while attaching a tooth crown. This location is attributed to dental overgrowths such as enamel pearl, bifurcation ridge,and talon cusp, which have an organized structure that includes, enamel, dentin, and sometimes pulp in order that we often see in a normal tooth structure  - 6.In this case, we present a lesion on the buccal surface of the right maxillary central incisor crown, which is not completely similar to the above lesions.A 14-year-old male presented with expressed concern about the abnormal shape of the right maxillary central incisor crown . His maiThe right maxillary central incisor had a lesion that was first looked like calculus. The consult with periodontists revealed that it was not calculus. The lesion was extended tothe cervical part of crown and root.The size of lesion was about 8 mm. The patient had no pain and no clinical symptom. It was not associated with caries and vitality tests revealed normal pulp response.No association with other dental anomalies was established. A periapical radiograph was provided . On radiInitially, it was attempted to remove the lesion with an ultrasonic scaler; but it was not possible. So finally, therapeutic procedure included complete removal of the lesion with finOdontoma is a benign odontogenic lesion. They are probably a hamartomatous malformation of functional ameloblasts and odontoblasts rather than true neoplasms  . They uThe exact etiology of odontomas is unclear, different factors such as local trauma, infection, growth pressure, heredity and developmental influences may be the causes  , 8- 9Odontomas may be revealed at any age, but their highest prevalence is in the first and second decades of life with a slight male tendency  . The caRadiographically, they generally appear as small, solitary, or multiple mixed radiolucent-radiopaque lesions. Complex odontoma appears as an irregular mass of calcified materialsurrounded by a thin radiolucent area with smooth periphery and the compound type shows calcified structures resembling teeth in the center of a well-defined radiolucent lesion  . The iret al. [ Compound odontomas are tooth like structures. Their histopathologic feature looks like pulp tissue in the center, surrounded by a dentin shell and then enamel matrix.Complex odontomas are disorganized combination of dentin, enamel, cementum, and areas of pulp tissue without orientation; the current case had similar structure  . Althouet al.   for theet al.  ; no repet al. [ et al. [ Vengal et al.   indicat et al.   reporteUnlike dental overgrowths, our case did not have a regular structure of enamel and dentin. So based on histopathological results, it evokes an odontoma. Consequently,the case is neither a typical odontoma based on the location, nor a typical overgrowth, so we called it juxtacoronal odontoma. An unusual juxtacoronal odontoma on the right maxillary central incisor region is reported. Such cases may confuse the diagnosis during clinical and radiographical examinationsand they might be mistaken for various other lesions. Therefore, the clinicians might rely on microscopic evaluation.All authors declare no conflict of interests.


Clinically, the extension was approximately 5×5 mm with normal surrounding mucosa. No tooth mobility was noted. No signs of fluid discharge or nasal regurgitation was found. On palpation all inspectory findings were confirmed. No tenderness on palpation was present. Depth of the lesion was through and through from palatal to buccal cortex. On further radiological examination, cone beam computed tomography (CBCT) (B) showed a single round shape hypodense area between left maxillary premolars, approximately 1×1 cm in diameter. Aggressive destruction of buccal and lingual cortical plate with perforation in floor of maxillary sinus in left premolar region was noted (C and D). External root resorption was seen with both left premolars associated with the lesion. Differential diagnosis considering aggressive maxillary lesion was made as lateral periodontal cyst, traumatic injury and malignant tumor. Patient was planned for excisional biopsy with extraction of involved premolars under local anaesthesia. Histopathological examination revealed plump spindle cells arranged in fascicles and myofibroblastic cells with occasional mitosis. Immuno histochemistry (IHC) further confirms the morphological findings, which were inconsistent with the findings of inflammatory myofibroblastic tumor (IMT). Thus, IMT of oral cavity should be included as a differential diagnosis of any aggressive lesions or for any localised palatal perforations.A 19-year young female patient presented with the chief complaint of palatal perforation since 15 days. No significant medical or familial history. No h/o of trauma. On clinical examination (A) a single round perforation was present on hard palate extending antero-posteriorly from distal surface of upper left 1


Spigelian hernia (SH) occurs due to the protrusion through a congenital or acquired defect or weakness in the Spigelian aponeurosis. SH accounts for only 0.1–0.4% of occurrence and a 17–24% risk of strangulation. We hereby report a case of a 34-year-old gentleman presented with concomitant incarceration of the omentum with small intestine and testis in Spigelian hernia sac. We have successfully operated on this patient via a transperitoneal approach with a small incision over the hernia site. This incision could be an alternative to midline laparotomy as a safe and effective method in managing incarcerated SH in an emergency setting. Spigelian-cryptorchidism syndrome is mostly reported in the paediatric age group and they are commonly presented with swelling of the abdominal wall or intestinal obstruction with associated ipsilateral cryptorchidism.A 34-year-old gentleman was presented to the emergency department with a one-day history of painful irreducible swelling over the right lumbar region, in which he was associated with several episodes of vomiting but without flatus. Upon further questioning, the swelling over the right lumbar region appeared intermittently upon straining since childhood. It was associated with mild discomfort and occasionally, the aforementioned patient would notice the pain radiates to the groin when there is swelling. He denies any history of constipation or abdominal distension previously. He was diagnosed with right undescended testes since birth; however, he was unsure of any intervention performed previously. He is, otherwise, not known to have any comorbidity. He works as a manual labourer, denies consuming any alcohol, but smokes one pack a year for the past 14 years. He was born term with no significant birth history.Upon presentation in the emergency department, he was stable and not septic looking. Additionally, he was hemodynamically stable. A firm, tender, non-fluctuant, non-pulsatile, irreducible mass measuring 5 × 10 cm in size was identified . There wHe underwent emergent open hernia repair with an oblique incision over the swelling. Intraoperatively, a defect on the lateral border of the rectus abdominis muscle was found along the arcuate line with the hernia sac measuring 6 × 10 cm containing 25 ml of serous fluid, gangrenous omentum, dusky terminal ileum with cystic atrophic testes A and B. The presentation of SH can be very vague and may vary. Most cases are asymptomatic, which may be detected for an unrelated reason, or they may present pain or a bulge in the lower abdomen in the region of Spigelian aponeurosis. If left untreated, the hernia may become incarcerated and strangulated. In a recent study, approximately 27% of patients with SH required emergency surgery for strangulation or incarceration.Clinical examination is the mainstay of the diagnosis. The vast majority of SH lie in the SH belt, a transverse zone of 6 cm above the interspinal plane, which is at the Spigelian belt. SH may sometimes be misdiagnosed as a direct inguinal hernia given its exact location. In some cases, the hernia may not be apparent, even with positioning or Valsalva manoeuvre; this is especially true in obese patients. Therefore, a physical exam alone may only accurately diagnose this rare hernia in one-half of patients.The mainstay of management of this anomaly includes repair of the hernia and scrotal placement of the undescended testes. One should remember to look for undescended testes within the hernia sac in the case of SH, with no testes found within the scrotal sac or inguinal region. Embryologically, the gubernaculum is a prerequisite for the development of the inguinal canal. For the patient in this study, his testes were found to be atrophied and no gubernaculum was found; hence, it was assumed that there was a lack of an inguinal canal. The inguinal canal was not explored in the emergency setting. There are various techniques that have been reported in the management of the rare SH. Many have embarked on laparoscopic repair over the last three decades, with laparoscopic transabdominal preperitoneal repair with mesh reported to have the lowest recurrence and complication rate. The overall recurrence rate in laparoscopic repair was 2.8% and open repair was higher at 4.2%.It can be concluded that the patient in this work is likely to have a congenital condition consistent with the findings of cryptorchidism with ipsilateral atrophied testes found in the SH sac. There are many ways to manage SH, depending on the expertise available in one centre although it was reported that open repair has a higher complication and recurrence rate. Managing an SH is almost equivocal to other types of hernia. Treating risk factors is of the utmost priority; however, in emergency circumstances, exploration is more trivial.


This case illustrates how molecular imaging findings non-invasively corroborate the correct diagnosis of osteitis fibrosa cystica generalisata with the formation of multiple benign brown tumors.Benign so-called “brown tumors” secondary to hyperparathyroidism are a rare diagnostic pitfall due to their impressively malignant-like character in various imaging modalities. We present the case of a 65-year-old male patient with multiple unclear osteolytic lesions on prior imaging suspicious for metastatic malignant disease. Eventually, findings of


Mediastinal cystic lesions, such as paratracheal air cyst (PTAC) and bronchogenic cyst (BC), are rare anomaly usually found incidentally in thoracic imaging. Special attention is needed in the case of thoracic surgery.All three patients were male, 71, 73, and 76 years old. Preoperative CT showed each had a lobular cystic lesion at the right posterolateral side of trachea in the thoracic outlet 11, 14, and 19 mm in size, respectively, with air density and tracheal communication, leading to a diagnosis of PTACs. An oval cystic lesion, 7 mm in size, was found in one patient at the right lateral side of the upper esophagus with low density and without tracheal communication, leading to a diagnosis of paraesophageal BC. Intraoperative findings of the three PTACs demonstrated a soft bulge from the membranous portion of trachea that was left intact. The BC had an oval elastic structure, mimicking a metastatic lymph node, and was removed with the mediastinal lymph nodes. Histological examination showed ciliated columnar epithelium, confirming a diagnosis of BC.PTACs are associated with increased intraluminal pressure due to chronic lung disease. BCs are congenital anomalies that originate from abnormal budding of the embryonic foregut.PTACs and BCs need to be considered in preoperative image diagnosis in patients with esophageal cancer. PTACs should be left intact to avoid tracheal injury, while removal of isolated BCs is recommended as a diagnostic and therapeutic measure. •Paratracheal air cyst (PTAC) and bronchogenic cyst (BC) are rare anomaly.•Three PTACs and a BC were found in patients who received thoracoscopic esophagectomy for esophageal cancer.•PTACs were carefully left intact to avoid tracheal injury.•BC was removed for diagnostic and therapeutic measure.•PTACs and BCs need to be considered in preoperative image diagnosis in patients with esophageal cancer. He was a heavy smoker with 20 cigarettes per day for 50 years, but without medical history of chronic lung disease, such as chronic obstructive pulmonary disease (COPD) or emphysema. Preoperative computed tomography (CT) detected an oval thin-wall cystic structure between the right posterolateral side of the trachea and the right subclavian artery A, 11 mm The second patient was a 71-year-old male, who was diagnosed with ESCC in lower esophagus, T1bN0M0 Stage I. He was a heavy smoker with 20 cigarettes per day for 45 years, but without medical history of chronic lung disease. CT showed a multilocular cystic structure, 14 mm in size, at the right posterolateral side of trachea at the mid-upper thoracic esophagus A. The cyThe third patient was a 76-year-old male, who was diagnosed with ESCC in mid-lower esophagus, T1bN1M0 StageIIB. He had no smoking history, nor medical history of chronic lung disease. CT showed a multilocular cystic structure in the neck, 19 mm in size, with internal air density A, which All three patients recovered without postoperative complications. No remnant cyst-related complication, no enlargement of the remnant cyst, nor recurrence of the cyst, was observed during 18–115 months follow-up after surgery.3PTACs are air-filled collections adjacent to the trachea and that are sometimes described as tracheal diverticulum peri RRLN lymph node metastasis is frequently seen, preservation of PTACs by careful separation from the esophagus and lymph nodes is necessary to avoid tracheal injury during the esophagectomy Asymptomatic small PTACs usually require no treatment As shown in the above-mentioned reports, many of the PTACs in esophagectomy patients have been reported from Japan. Difference among countries in the incidence of PTACs is unclear. One possible reason is that upper mediastinal lymph node dissection is commonly performed in Japan, where majority of esophageal cancer are squamous cell carcinoma and arise at mid esophagus.BCs are congenital anomalies that originate from abnormal budding of the embryonic foregut and tracheobronchial tree Preoperative diagnosis by CT, intraoperative identification, and careful removal of the BCs under consideration of possible communication with the trachea is recommended. However, in the patients presenting with tracheal injury, primary closure or repair using the pericardial or intercostal muscle flap should be considered There have been reports on PTACs or BCs each, however, to our knowledge, this is the first report that describes PTAC and BC at the same time, suggesting the importance of differential diagnosis and different therapeutic strategies in patients who underwent esophagectomy.This work was reported in line with the SCARE 2020 criteria 4Small mediastinal cystic lesions, such as PTACs and BCs, need to be considered in preoperative image diagnosis in patients with esophageal cancer. PTACs with tracheal communication should be carefully left intact to avoid tracheal injury, while removal of BCs is recommended as a diagnostic and therapeutic measure.This study had a small number of cases. It was not a clinical trial.MEXT KAKENHI Grant Number 21K08729.This work was partly supported by a Grant-in-Aid for Scientific Research (C) This case report was approved by the Ethics Committee of Toyama University Hospital.All patients fully understood condition of their disease and the surgical procedure they were agreeing to undergo, and a written informed consent was obtained from the patients for surgery. Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal on request.Not applied.The Guarantor of this case report is T. Okumura.Not commissioned, externally peer-reviewed.T. Okumura produced the case report conception and design, obtained informed consent, and wrote the main manuscript body.T. Okumura, T. Watanabe, K. Shibuya, T. Akashi, S. Sawada, K. Tazawa, and F. Yamagishi performed surgery.K. Nomoto performed pathological diagnosis.T. Watanabe, T. Miwa, T. Akashi, N. Kimura, N. Takeda, T. Uotani, H. Baba, K. Hirano, K. Shibuya, I. Hashimoto, S. Hojo, K. Matsui, I. Yoshioka, S. Sawada, K. Tazawa, F. Yamagishi, and T. Fujii participated in the patients' care and critically reviewed the manuscript.None of the authors have financial competing interests to disclose.


Sinonasal malignancies are known for their associated poor prognosis and diversity of histologic features. While poor prognosis is largely due to advanced disease at presentation, histologic features also play a significant role. Therefore, accurate pathologic diagnosis is of utmost importance. Here, we describe a 63-year-old male with chronic left-sided nasal obstruction and left-sided epistaxis who was found to have a large mass occupying most of the nasal cavity extending through the nasopharynx to just below the nasopharyngeal surface of the soft palate. During surgical excision, the mass was noted to originate from the floor of the maxillary sinus with erosion of the medial wall of the maxillary sinus. Pathology revealed a diagnosis of INI1-intact poorly differentiated composite carcinoma with rhabdoid phenotype and sarcomatoid and squamous cell carcinoma foci arising within an inverted papilloma. Included in this report is a detailed description of both the patient's medical course and this pathologically novel sinonasal neoplasm. We aim to elucidate this rare tumor's complex features in order to improve future diagnosis and stimulate prospective research on sinonasal malignancies with complex histology. While accounting for less than 0.5% of all malignancies, sinonasal malignancy remains an important disease, as it tends to be associated with a poor prognosis . While tHere, we present what appears to be the first case of a patient with poorly differentiated composite sinonasal carcinoma with rhabdoid phenotype, focal sarcomatoid, and SCC arising within an inverted papilloma.A 63-year-old male nonsmoker with a history of type 2 diabetes, hyperlipidemia, and hypertension presented with left-sided nasal obstruction and intermittent left-sided epistaxis for a duration of several years. He denied history of trauma or dental work, as well as tooth pain, facial numbness, or past sinus surgery. Physical exam was notable for a mass in the oropharynx just inferior to the soft palate extending down from the nasopharynx. Nasal endoscopy revealed a large fleshy and friable mass in the left nasal cavity obstructing the view of the middle turbinate and touching the nasal floor, causing complete nasal obstruction. Computed tomography (CT) of the sinuses was concerning for a mass extending from the left maxillary sinus antrum to the nasopharynx and oropharynx with complete opacification of the adjacent paranasal sinuses .The patient was taken to the operating room for left-sided endoscopic sinus surgery with complete excision of the mass for both diagnosis and treatment. The mass was firm, extremely vascular, and appeared to erode through the medial wall of the maxillary sinus from an attachment site on the floor of the maxillary sinus.Pathologic examination revealed a poorly differentiated composite sinonasal carcinoma with rhabdoid phenotype, foci of sarcomatoid cells, and SCC arising within an inverted papilloma . The tumGiven the histopathologic and immunohistochemical findings described here , this tuSMARCB1, which codes for INI1, is a tumor suppressor gene located on chromosome 22q11.2 . INI1 seThough few, there have been reports of rhabdoid-type tumors and sinonasal carcinomas of other types identified to be INI1-intact , 4. One Lastly, it is important to consider how the composite histology of this tumor impacts the prognosis. This specific tumor is undescribed in the literature, and thus predicting outcomes poses a significant challenge. Perry et al. speculated that rhabdoid cells represent a late-stage neoplasm, which is consistent with the observed poor prognosis . Agaimy While various subtypes of sinonasal tumors are most often identified based on histologic features, poorly differentiated tumors of the sinonasal tract are known to be more difficult to classify, owing to the inherent heterogeneity of their histologic features . The tum


Acute pelvic pain is a common complaint in reproductive age women and has a large differential diagnosis. Decision for conservative vs. surgical management is often dependent on clinical, biochemical and imaging findings. Isolated tubal torsion is a rare cause of pelvic pain that requires prompt diagnosis and surgical management to avoid morbidity. Here, we report non-pregnant women of reproductive age presenting with acute lower abdominal pain. Raised inflammatory markers and ultrasound findings prompted management for Pelvic Inflammatory Disease. Despite some improvement with antibiotics, the patient had ongoing symptoms. At surgery, bilateral para-tubal cysts and a left sided hydrosalpinx were found, along with an isolated left tubal torsion. Isolated tubal torsion most commonly occurs in reproductive aged women, and risk factors include intrinsic tubal pathology and extrinsic lesions. Clinically, biochemically and radiographically, it is often indistinguishable from other pelvic pathology, potentially leading to diagnostic delay, and necrosis of the tube. Acute pelvic pain in women of reproductive age is a common clinical presentation. The differential diagnosis in a non-pregnant woman of reproductive age is broad and includes ovarian torsion, cyst accident (rupture of follicle or cyst), pelvic inflammatory disease (PID), appendicitis, intestinal obstruction or perforation, renal stones or cystitis .Decision for conservative management vs. diagnostic surgery in a woman with suspected pelvic pathology is dependent on a combination of factors, including the patient’s age, severity of pain, associated symptomology, risk factors, inflammation and tumour markers and imaging findings. In many cases, diagnostic laparoscopy is the only definitive method for confirming a suspected diagnosis.Isolated fallopian tubal torsion, characterized by tubal rotation around its own axis without ipsilateral ovarian torsion, is a rare but important cause of acute pelvic pain in this population, with the potential for significant morbidity if surgical diagnosis and correction is delayed .A 44-year-old female presented to the Emergency Department with lower abdominal pain and ‘feeling of pressure in rectum’ during bowel motions associated with an increased urge to defecate. She had not been sexually active for more than 10 years. On examination, she was tender in the lower abdomen bilaterally, and cervical motion tenderness was elicited. Bloodwork demonstrated a raised white cell count and C-reactive protein and negative beta hcg. She was diagnosed with PID and commenced on intravenous antibiotics.CT abdomen showed two cystic structures in the pelvis, and follow-up ultrasound demonstrated a 5.8-cm unilocular simple cyst contained within the right ovary, and a 3-cm left tubo-ovarian complex with associated hydrosalpinx –3.Ovarian tumour markers, blood cultures, vaginal swabs and STI screening were negative. The patient clinically improved and on Day 3 was discharged on oral antibiotics  with outpatient follow-up arranged. Subsequent ultrasound 3 months later suggested a 5-cm simple right ovarian cyst and a left hydrosalpinx, and she had ongoing intermittent pain. She was taken to theatre for a bilateral salpingectomy and right cystectomy.Operation findings consisted of left hydrosalpinx and para-tubal cyst adhered to pouch of douglas, with the left tube torted 3 times  and 5. TIsolated tubal torsion is an uncommon, but important cause of acute pelvic pain in women, with a reported prevalence of 1 in 1.5 million [The aetiology of tubal torsion is poorly understood, however, predisposing factors include hydrosalpinx, para tubal cysts, ectopic pregnancy, ovarian mass or extrinsic lesions  , 2. OtheIn this case however, the torsion was left sided. From an aetiological perspective, there were two risk factors for isolated tubal torsion, the hydrosalpinx and the small para-tubal cyst.Para tubal cysts arise from the broad ligament between the ovary and tube, and most commonly originate from the remnants of the Mullerian or Wolffian ducts. They account for 10–20% of adnexal masses and are most commonly found in the third and fourth decades of life . They arA hydrosalpinx is a distally obstructed fallopian tube filled with serous or clear fluid. While it can occur in children and adolescents associated with tubal agenesis, it most commonly occurs in reproductive aged women, seen as a complication of infection (including PID), surgery or ovarian cysts , 6. PID Trans-vaginal ultrasound is the gold standard modality for characterizing an adnexal mass, though para-tubal cysts and ovarian cysts can be difficult to distinguish , 4. UnfoDiagnostic laparoscopy is the only method of confirming tubal torsion . SurgicaAs isolated tubal torsion is rare and the presentation is non-specific, it is commonly misdiagnosed clinically, as was the case here. When she presented to emergency, this woman was given a presumptive diagnosis of PID and managed accordingly. Presumptive diagnoses of PID in the clinical setting leads to early antibiotic administration which can help to prevent PID complications, but the diagnosis carries negative connotations which can lead to psychological and relationship stress. Diagnosis is usually confirmed by positive cervical swabs for chlamydia/gonorrhoea or positive vaginal swabs, though it can occur in the absence of an STI . Imaging