Malignant epithelioid neoplasm with ACTB-GLI1 fusion are considered different from the more common pericytic lesions, such myopericytoma, because they have a spectrum of different genetic abnormalities. They appear to pursue a benign clinical course in young adults, although in sporadic cases lymph node metastasis were described.The categorization of this new type of tumor may also lead to new therapeutic strategies, because they might be sensitive to SHH pathway inhibitors.The case involves a 72-years-old multiparous woman who accessed our department after an incidental finding of a right adnexal mass of 43 mm with contrast-enhancement on a control computed tomography scan made for suspected diverticulitis. Our intervention was a detailed ultrasound description of the suspected neoplasm;a diagnostic laparoscopy and the contextual laparotomic removal of abdominal mass; its histological and immunohistochemical analysis. Our main outcome measure is the definition and future recognition of new pathologic entity called malignant epithelioid neoplasm with ACTB-GLI1 fusion.We described for the first time the ultrasound characteristic of this type of lesion using standardized terminology and we believe that it may be the first step to improve the acknowledgement of this novel pathologic entity defined as malignant epithelioid neoplasm with GLI-1 fusions.The online version contains supplementary material available at 10.1186/s12905-022-01679-0. GLI (glioma-associated oncogene homologue) expression in adult tissues is restricted to the fallopian tube, myometrium, and testis. GLI functions as an effector of the sonic hedgehog (SHH) pathway, inducing upregulation or downregulation of multiple downstream targets . Shh sigACTB (beta-acting gene-glioma)-GLI1 fusions, resulting from a translocation   have been reported as the pathognomonic genetic abnormality described in rare characteristic mesenchymal neoplasm with a pericytic phenotype formed by monomorphic spindle cells and immunoreactivity for smooth muscle actin and laminin. These lesions described for the first time as “pericytoma with the t  translocation” are known as perivascular myoid tumors . They arThe activation of GLI1 is made by a promoter change by the ubiquitously expressed ACTB, leading a deregulation of GL1-1 expression and its downstream targets. A combined approach including RNA-sequencing, target exome sequencing and FISH analysis were used to identify these new genetic abnormalities –7.Most of these tumors were found in the soft tissue and in the tongue, with rare cases detected in the stomach and bone. Given the small number of cases. it is difficult to determine the average age of onset; however, most patient were younger than 40 years at diagnosis . They apIn accordance with precision medicine principles, a more and more tailored approach is advisable to improve survival in oncological patients, highlighting the need for a precise diagnosis for each lesion . The catWe present a rare case of malignant epithelioid neoplasm of the ileum with ACTB-GLI1 fusion mimicking an adnexal mass.We present a case of a 72-years-old multiparous woman who accessed our Gynecology and Human Reproduction Physiopathology Unit in September 2020 after an incidental finding of a right adnexal mass of 43 mm with contrast-enhancement on a control computed tomography (CT) scan made for diverticulitis in the department of Gastroenterology of a first-level hospital. Tumoral markers, including CEA, CA 19-9, CA 15-3 and CA 125 were negative.The patient had a history of arterial hypertension, glaucoma and diverticulitis. Moreover, in her 40s she underwent laparotomic cholecystectomy surgery.The diagnostic work-up and the surgical management are shown in Additional file At the admission in our department, she was asymptomatic and a transvaginal (TV) and a transabdominal (TA) ultrasound (US) were carried out by an expert sonographer with at least 5 years of experience in onco-gynecological ultrasound. US showed a normal anteverted uterus with normal left adnexa. A solid tumor that measured 44 × 22 × 43 mm was identified adjacent to the right ovary. According to International Ovarian Tumor Analysis (IOTA) terminology  it was aThe patient was then scheduled for a diagnostic laparoscopy that showed normal uterus and normal adnexa. An exophytic cerebriform whitish tumor of about 4.5 cm, rising form an ileal loop was observed Fig. . LaparotThe post-operative course was regular, and the patient was discharged from our ward without any complications.After some difficulties to identify the histological nature of the tumor, the samples from the ileal lesion were sent to a referral center for further analysis. They confirmed the nature of a malignant mural-based neoplasm. The slides showed a nested epithelioid malignancy that was located within the muscularis propria and the submucosa. The neoplastic cells were uniform and had a clear, vacuolated cytoplasm, small round nucleoli with fine chromatin, and focally prominent nucleoli. ln some areas, the tumor exhibited a moderate amount of hyalinized stroma with a capillary network. This stroma was minimal at the base of the tumor.Immunohistochemical stains showed expression of vimentin, CD10, p63, CD56, and cyclin D1, but did not express CK7l18/8, EMA, BERP4, synaptophysin, chromogranin, calretinin, inhibin, PAX8, WT1, ER, PR, SMA, desmin, caldesmin, CD34, Ckit, DOG1, HMB45, and MART1. Additional immunohistochemical stains showed that the neoplastic cells express S100 in the areas with decreased stroma but did not express SOX10. An immunohistochemical stain for TFE3 was equivocal, the differential diagnosis would include several mesenchymal neoplasms, including clear cell sarcoma-like tumor of the gastrointestinal (GI) tract, which can share histologic features along with S100 labeling; however, the negative staining for SOX10 argued against that possibility. They also considered the recently described entity of soft tissue sarcoma with GLI1 fusions. GLI1 fusion analysis by both FISH and ARCHER were conducted. Both tests founded an in-frame ACTB-GLI1 fusion involving exon 2 of ACTB and exon 6 of GLI1. Given the histomorphology and the fusion identified the final diagnosis was malignant epithelioid neoplasm with GLI1 fusion  allows a better characterization of malignant tumors  and may In conclusion we believe that ultrasound may help to characterize a new pathologic entity, defined as malignant epithelioid neoplasm with GLI-1 fusions even better than CT. The IOTA terminology may be useful to suspect malignant lesions even in the case of this rare tumor.Additional file 1: Video. Detailed ultrasound description of the suspected neoplasm. Diagnostic laparoscopy and laparotomic removal of abdominal mass. Histological and immunohistochemical examination.


Granular cell odontogenic tumor (GCOT) is a rare neoplasm with about 45 cases reported in the literature. It usually occurs in the posterior mandible of middle-aged women.We report a case of asymptomatic GCOT in the posterior mandible of a 28 years old female and provide a literature review of GCOT cases. Some unusual features such as root resorption, displacement of inferior tooth canal, and multilocular appearance were considerable in this case.Complete surgical excision of the lesion was beneficial for the patient. Granular cell odontogenic tumor (GCOT) is a rare benign neoplasm of jaws  which waThe prognosis of GCOT is good and it responds well to curettage . Only onWe have read the Helsinki Declaration and have followed the guidelines in this investigation.A 28 years old woman with a painless carious wisdom tooth in her right mandible attended a dental clinic. A general dentist visited her and Orthopantomography (OPG) radiographs Fig.  were takThe review of the literature shows, to date, 46 cases of GCOT have been reported including the current case. The information presented in Table In the presented case, the lesion was located in the posterior region of the mandible which is the most common site of GCOT with a mandible to maxilla ratio of 3.4:1 , granular cell tumor (GCT), and congenital epulis of the newborn . ImmunorAll previous cases were treated through excision and/or curettage . In the In conclusion, GCOT is a rare tumor that can be completely asymptomatic. Hence, it is important to consider GCOT as a possible diagnosis and not to miss it.


Situs inversus totalis is the mirror image transposition of the abdominal-thoracic viscera. Approximately one in every 5,000 to 20,000 live births has situs inversus totalis. Most commonly, it is found incidentally and is asymptomatic. A number of malformations, including cardiac, splenic, and gastrointestinal, have been associated with this condition. Coexistence with duodenal atresia is extremely rare, reported in fewer than 30 cases worldwide and one case in Saudi Arabia. We report a preterm neonate who presented with bilious vomiting. Diagnosis of situs inversus totalis with duodenal atresia type III was established and other anomalies were ruled out. The patient was managed surgically by duodenal-duodenostomy and Ladd’s procedure. The report emphasizes the importance of identifying this condition and recognizing the "mirror anatomy" before carrying out an operation. Once the diagnosis is confirmed, surgical intervention must be performed as soon as possible to prevent complications. Situs inversus disorder is described as a spectrum of transposition of the internal body organs which can be complete  where both the thoracic and abdominal organs are reversed, resulting in a mirror image of the normal body anatomical organs or might be partial  where either the thoracic or abdominal organs are reversed .Other associated congenital defects, such as congenital heart disease, splenic malformation, and intestinal and biliary atresia, can worsen situs inversus totalis ,3. SitusIn Saudi Arabia, only one neonatal case was reported as situs inversus totalis and duodenal atresia . We are This is a case of a baby girl, delivered at a gestational age of 34 weeks via an emergent cesarean section on account of fetal distress. The mother is a Saudi woman aged 37, who was a gravida 4, para 2, with two abortions. The pregnancy was described as uneventful. The infant was delivered through thin meconium-stained amniotic fluid, cried immediately, and was given an Apgar score of 7 and 7 at one and five minutes, respectively. She had a birth weight of 1660 grams, lies at 10th percentile for age and gestation, length of 39.5 cm at 4th percentile, and head circumference of 30 cm at 33rd percentile. The vital signs were within normal ranges for age. She was active, pink in color, and not dysmorphic. An examination of the respiratory system revealed increased respiratory effort, subcostal retraction, and nasal flaring, for which she was placed on non-invasive ventilation and 35% fractionated oxygen (FiO2). Additionally, a cardiac exam revealed heart sounds and palpable impulses over the right precordium. Another systemic examination was within normal limits for age. The patient was shifted to a level three neonatal intensive care unit (NICU) after stabilization.On her first day of life, she developed persistent green gastric aspirates, for which she had serial radiographs taken Figure . RadioloSide-to-side duodenal-duodenostomy and Ladd's procedure were performed on her third day of life. An intraoperative examination revealed right-sided stomach and spleen, left-sided liver, and type III complete duodenal atresia, where the two ends of the duodenum separate distal to the ampulla of Vater, along with intestinal malrotation Figure . The patThe infant made an uneventful recovery and was discharged home to care of parents after 40 days, at corrected age of 40 weeks and 5 days. During outpatient follow-ups, the patient demonstrated good overall health, nutrition, bowel habits, growth catch-up, and normal neurodevelopmental milestones. Currently, she is seven months old, feeding breast milk and high-energy milk formula. In light of the inheritance pattern, she was referred to a higher center for further genetic testing.A situs inversus is an inverted position of abdominal and thoracic organs. A situs inversus totalis occurs when the visceral organs are completely transposed  as in our case. It is called situs inversus partialis when the inversion occurred solely in the abdominal organs. Normal positioning of the organs as observed in most individuals is called situs solitus . It was Ciliary abnormalities are the most common association with situs inversus totalis (the primary ciliary dyskinesia disorders). Patients with primary ciliary dyskinesia (PCD)/Kartagener syndrome have situs inversus totalis in about 50% of the cases . The assIt is estimated that situs inversus totalis patients have a congenital heart disease incidence of 5% to 10%, the majority of which is a ventricular septal defect, transposition of the great vessels, and valvular disease . AnnularWhen fetal situs inversus is detected via prenatal ultrasound, additional testing such as fetal magnetic resonance imaging (MRI) or fetal echocardiography can be performed to help understand the fetus' developmental stage. This could affect management and prognosis. Following birth, additional imaging tests are also recommended to determine whether there are any associated abnormalities. In some studies, ultrasonography is suggested as a radiographic modality of choice to avoid incorrect labeling on radiographs and to assess duodenal atresia . In our Identifying situs inversus in the body is important for diagnosing medical problems and preventing surgical errors that can occur when reversed anatomy goes unnoticed. Duodenal atresia (DA) and malrotation require surgical treatment while the patient is in a stable clinical state rather than an emergency situation. DA is treated exactly the same whether it is associated with small intestinal atresia (SIA) or without. Duodenoduodenostomy remains the preferred management option for the treatment of duodenal atresia as well as Ladd's procedure for midgut malrotation. Our patient had been treated surgically with the aforementioned procedures. The prognosis for isolated situs inversus with an uneventful prenatal and postnatal course is excellent. However, when it is associated with other diseases, the prognosis can be impacted by that condition. Survival rates and long-term prognosis for DA are very good, too, at approximately 90% .The majority of the cases of situs inversus totalis are asymptomatic and are diagnosed incidentally during laparotomy or autopsy. When situs inversus totalis (SIT) is associated with DA, it presents early in the neonatal period. This association is extremely rare with a limited number of reported cases. Plain baby gram showing a reversed double bubble sign and reversed heart shadow is suggestive of DA with situs inversus. The diagnosis can be confirmed with a contrast meal and follow-through series. Screening of other possible associated anomalies is essential. Similar principles should be applied to surgical treatment as they are to classical cases and the mirror anatomy must be considered when performing the surgery. It is important for clinicians to have a high index of suspicion for malrotation with midgut volvulus or intestinal atresia in neonates of situs inversus presenting with bilious vomiting. Whether there are other anomalies or postoperative complications will determine the prognosis. The key to a favorable outcome is an early diagnosis and prompt referral. Considering the high significance of recognizing a rare condition, this case is noteworthy in our opinion.


Large canine pelvic masses, even though benign, can seriously affect dogs’ lives, causing problems regarding defecation and urination, and sometimes complete rectal and/or urethral obstruction. Many of these patients are euthanized owing to their poor clinical condition as a result of chronic compression or because their disease is erroneously considered untreatable. The aim of this study was to evaluate the clinical data of dogs with intra-pelvic benign or well-differentiated malignant masses referred to the Torino Veterinary Teaching Hospital, and treated surgically. Clinical signs, diagnostic approach, surgical procedures and outcome were evaluated. The majority of the dogs recovered uneventfully from surgery in a couple of days, with rapid resolution of the severe preoperative clinical signs. All the dogs experienced long survival with no disease recurrence or progression, even in the well-differentiated malignant tumors. Clinical findings, especially those obtained using digital rectal and vaginal exploration are mandatory for assessing the presence of the tumor and its relationship to the other pelvic structures. Currently, in the authors’ opinion, preoperative computed tomography (CT) is also highly recommended, even if this procedure was not performed in 4 out of the 11 dogs.Dogs with benign intra-pelvic rectal or vaginal masses show symptoms indicating compression on the adjacent organs. Clinical signs usually develop late when the lesion is large enough to interfere functionally. The dogs were referred for severe fecal and/or urinary tenesmus. The data collected included signalment, clinical signs, results of physical examination, pre-surgical diagnostic tests, surgical technique used, surgical complications and histological findings. Digital rectal and vaginal examination allowed the detection of a mass occupying space in the pelvic cavity in all patients. Abdominal ultrasonography and/or total body computed tomography (CT) were used to better characterize the lesion and to exclude a metastatic spread of the tumor in case of malignancy. A dorsal approach to the rectum, a dorsal episiotomy, a midline celiotomy, and a combined perineal and abdominal approach were performed to remove the mass. No postoperative complications were observed. Benign and well-differentiated malignant mesenchymal neoplasms were histologically diagnosed. As a consequence of the chronic urethral compression caused by the mass, urinary incontinence and/or urinary retention were observed for a few postoperative days. Fecal tenesmus resolved in all cases in the immediate postoperative period. The dogs’ quality of life quickly improved after surgery, especially considering the serious and life-threatening pre-surgical clinical conditions. Both the recovery time after surgery and overall survival were also evaluated. Pelvic structures, such as the distal part of the descending colon, rectum, vagina, sacral lymph nodes and prostate can be affected by tumors which, as a result of their increased size, may cause a reduction in the pelvic space, thus determining fecal and urinary problems. The behavior of these tumors can be either benign or malignant. Treatment choice and prognosis vary depending on tumor histotype as well as on the oncologic outcome. Cytology or histology from bioptic samples, imaging techniques for staging purposes and/or preoperative planning are usually recommended.Benign or low-grade malignant intra-pelvic tumors are rare in clinical practice  and, forPatients with benign or low-grade malignant intra-pelvic masses may show signs resulting from the compression of the adjacent organs, such as the rectum and the urethra. Due to the slow growing, non-invasive and non-metastatic behavior of these masses, clinical signs usually develop late in the course of the disease, i.e., when the lesions are large enough to interfere functionally. The dogs are thus presented for tenesmus  and constipation.In some instances, the lesion may cause complete occlusion of the urethra and, less frequently, of the colon-rectum, thus creating a clinical situation not compatible with life .Older dogs are more commonly affected, and poor clinical conditions may sometimes be evident. This, along with the presence of a large pelvic mass, believed to be unresectable, may lead to poor prognostic judgment and to euthanasia.Due to the non-invasive behavior of these tumors, a marginal excision is often feasible and curative. Episiotomy or a dorsal approach to the rectum are the most reported surgical techniques ,7. The rOf the diagnostic examinations which help to differentiate between benign and malignant intra-pelvic tumors, digital rectal examination (DRE) should be utilized. It is a simple examination which allows discovering the presence of a pelvic mass and defining its characteristics, such as consistency, mobility and localization.In the case of a well-defined, compact mass, dorsal or ventral to the rectum at DRE, a benign rectal or vaginal mass can be suspected.The aim of this retrospective study was to support the hypothesis that a simple DRE  may provide important information regarding the non-invasive nature of the lesion. Nevertheless, a preoperative diagnosis should always be attempted. The cytology in case of consistent mesenchymal lesions dorsal or ventral to the rectum is often not diagnostic but helps in differentiating these lesions from an epithelial or round cell lesion, more prone to exfoliate . A biopsThe data of the dogs referred to the Torino Veterinary Teaching Hospital (VTH) for intra-pelvic histologically benign or low-grade malignant masses of rectal or vaginal origin responsible for severe functional problems in defecation and/or urination were reviewed. Diagnostic procedures, type of treatment, postoperative complications and recovery time were evaluated.The medical records of dogs referred to the Torino VTH from January 2002 to December 2020 for fecal and urinary partial or complete obstruction were identified and reviewed. The inclusion criteria were the presence of a well-defined compact mass, dorsal or ventral to the rectum, clinically evaluated at DRE and surgically excised. Only benign or low-grade/well-differentiated malignant tumors were included.Tumors localized in different positions than those dorsal or ventral to the rectum at DRE and arising from anatomical structures other than rectum and vagina were excluded.Written consent was obtained from the owners for the anesthetic, diagnostic, histological and surgical procedures before proceeding. They signed an informed consent for the procedure as a standard procedure for the surgical treatment of AGASACA tumor. No specific informed consent statement was signed for the inclusion in this retrospective study. Perioperative standard-of-care management, including analgesia, was assured for all the dogs. The study did not fall within the application areas of Italian Legislative Decree 26/2014 which governs the protection of animals used for scientific or educational purposes; therefore, ethical approval was waived for this study. The animals were not treated as part of an experimental study; however, out of necessity, only the data regarding the study were later selected and included in this study.The data collected included signalment, clinical signs, results of physical examination including digital rectal and vaginal exploration, pre-surgical diagnostic tests, surgical technique used, surgical complications and postoperative histological findings. The disease-free interval (DFI), defined as the time between surgery and local recurrence or metastatic spread for malignant tumors, and the survival time (ST), considered to be the time between surgery and the dog’s death for any reason, were calculated. Dogs with a follow-up time shorter than 365 days were excluded from the DFI and the ST evaluations. Recovery time, considered to be the return to normal defecation and/or urination, was also evaluated.The work-up generally included blood cell count, serum chemistry profile, 3-view thoracic radiography and abdominal ultrasonography, or a total body CT scan.Digital rectal examination  was carried out, thus allowing the detection of a mass occupying space in the pelvic cavity and a useful characterization of the mass, i.e., tumor localization, adhesion to the underlying structures and consistency.A cytologic (fine needle biopsy) or a histologic (tru-cut needle) sampling was attempted using a transcutaneous perineal, transrectal or an transabdominal approach, ultrasound guided, as opportune.Due to the long study period, different analgesic and anesthetic protocols were used; antibiotic prophylaxis was administered at anesthesia induction and then every 90 min until completion of surgery.For a dorsal rectal approach, the dogs were placed in sternal recumbency with the tail bandaged, reflected and secured cranially; soft padding was placed between the table edge and the cranial thigh in order to avoid any injury.Anal sacs were manually emptied during a DRE and an anal purse string suture was then placed. After standard aseptic preparation of the area, an inverted U-shape incision was performed between the base of the tail and the anus, starting from the ischiatic tuberosity up to the contralateral tuberosity.The subcutaneous fat and perineal fascia were dissected to visualize the external anal sphincter, the rectococcygeal muscles and the dorsal rectum. The paired rectococcygeal and levator-ani muscles were transected, if necessary .The mass was excised using a combination of sharp and blunt  dissection. Hemostasis was reached by a combination of vessel electrocautery and temporary gauze packing. If transected, the muscles were sutured with 2-0 or 3-0 absorbable monofilament. The subcutis and the skin were closed routinely .Dorsal episiotomy was performed for vaginal masses. The dogs were placed in sternal recumbency  and prepFor those tumors protruding in the abdomen or dropped cranial to the pelvis inlet during the perineal dissection, a standard midline caudal celiotomy was performed. Therefore, the dog was positioned in dorsal recumbency and the entire abdomen, previously clipped, was aseptically prepared. A median skin incision was performed from the umbilicus to the pubis ,12. The After tumor removal, the descending colon, ureters and regional lymph nodes were inspected for abnormalities. The abdomen was closed routinely .Intravenous fluid was usually continued for 24–48 h postoperatively according to the animal’s initial hydration status or until spontaneous eating and drinking. Analgesia consisted of opioids and non-steroidal anti-inflammatory drugs (NSAIDs).Patients were generally discharged from the hospital 2–5 days after surgery with an Elizabethan collar and NSAID therapy. Antibiotic therapy was administrated 24 h postoperatively only if needed .Postoperative complications and wound status were monitored until skin suture removal . Long-term follow-up was obtained for all patients by telephone contact with the owners or referring veterinarians.Eleven dogs were eligible for inclusion in this study; each dog was identified by a number assigned at the beginning of the study.Mixed breeds were the most represented (64%). The breeds affected as well as the clinical signs at presentation are reported in n 2, 9–11). Three (27%) dogs also had urinary problems , the urinary occlusion being complete in 2 cases .Median age was 10 years  and median weight was 20 kg . Eight dogs (72%) were females (6 intact and 2 spayed). There were 3 males (18%) (2 intact and 1 neutered). All the dogs suffered from fecal tenesmus. Four (36%) dogs were referred from the Critical Care Unit of the VTH for complete fecal obstruction . At presentation, 3 patients (27%) had perineal swelling .All the patients also had appetite loss, lethargy and abdominal pain. Other recorded clinical signs were weight loss and poor health conditions . Signalment, clinical signs and localization of the mass are reported in n 1–6, 10, 11) and ventral to it in 3 dogs (18%) (n 7–9)  was performed in all patients. A well-defined, compact, more or less mobile, mass was detected in all dogs. The mass was dorsal  to the rectum in 8 dogs (72%) ( (n 7–9) . In all n 3, 9–11) or transrectal  fine needle aspiration (FNA) biopsy. An ultrasound-guided trans-abdominal tru-cut needle biopsy was performed in 1 case (9%) (n 8).A preoperative diagnosis was attempted in all cases. Ten patients (91%) underwent transcutaneous perineal  and 1 (9%) case (n 8), respectively. In the former case, the lesion was cytologically compatible with a soft tissue sarcoma (n 1) and a mesenchymal tumor (n 7). In the latter case, a sarcoma (n 8) was diagnosed by histology. Cytology was inconclusive in all the other cases (80%) .  cases . .n 1–8). In 3 cases (n 6–8) only the cranial part of the mass was visualized. In the remaining 5 dogs, the mass could not be seen.Abdominal ultrasonography was performed in 8 dogs (72%)  underwent total body CT with intravenous (IV) contrast injection (Seven patients (63%)  had 3-view thoracic radiographs performed for staging purposes.Four patients (36%)  or distant metastases.n 3–5, 9–11) and 1 out of 7 cases (14%) (n 8), respectively. The masses appeared well defined and homogeneous, with mild contrast enhancement in all cases. Colonic and rectal displacement, and urethral compression were always present  ( present .n 6). The patient also had abdominal effusion (modified trasudate-exudate with 0.025 g/L protein concentration). Before proceeding with surgery, a Foley catheter was positioned and, after 2 days of IV hydration, the above values normalized, although they remained in the high range.High creatinine (1308 µmol/L [range 44–159]), blood urea nitrogen (214 mmol/L [range 6.1–21.1]) and phosphate (7.36 mmol/L [0.7–2.1]) serum levels due to a five-day urinary obstruction were present in one dog    .n 6–8, 11). An episiotomy was performed in 1 dog (9%) (n 9). A double approach  was used in 1 case (9%) (n 4) in which the mass, after being bluntly dissected using a dorsal approach to the rectum, slipped cranially into the abdominal cavity  (l cavity .n 7–9).The three patients (27%) having a vaginal mass underwent contextual ovariohysterectomy (n 9), in which the tumor was adherent to the urethra. The latter was damaged and subsequently repaired by means of an anastomosis at the level of the urethral papilla.The tumor was easily removed principally using digital blunt dissection in all patients (91%) with the exception of one , 1 (9%) (n 6), 3 (27%)  and 2 (18%) dogs , respectively.A well-differentiated leiomyosarcoma, a fibroleiomyoma, a fibroma and a leiomyoma were observed in 2 (18%) ; the histological findings were compatible with a fibroma or a leiomyoma; however, it was not possible to differentiate between them. Immunohistochemical investigations were proposed but were not accepted by the owners.A benign mesenchymal neoplasm was histologically diagnosed in 3 (27%) cases , even though a blood transfusion was not needed in any case, and the bleeding was managed by combining both hemostasis with a electrocautery and gauze packing. During the surgical excision of the mass, the lumen of the rectum or the vagina was never penetrated.Bleeding during the dissection of the mass occurred in all cases and was copious in some of them . Two of these dogs  developed urinary incontinence which spontaneously improved within two postoperative months, without, however, regaining complete functionality.Bladder atony due to prolonged over-distension was observed in 3 (27%) patients (n 9). A Foley catheter was placed and left in situ with a closed system collection for 5 days. Normal urination was restored within two postoperative weeks.Immediately after surgery, functional urinary retention with anuria developed in 1 dog  which was hospitalized until Foley catheter removal.The patients were discharged two days after surgery in all cases except for one  were lost to follow-up approximately 90  and 1330  days after surgery. The latter dog was included in the ST evaluation because its follow-up time was longer than 1 year. Dogs , lost in follow-up at approximately 90 days after surgery, were not included in the ST evaluation.Follow-up was available for 7 patients (64%); four (36%)  with malignant disease.Owners, questioned about their dog’s quality of life after surgery stated that it was highly improved. No patient, at least for those which had a follow-up available, had a local recurrence, despite marginal excision of the tumor. Distant metastases were not observed in the 2 dogs . Median ST was 950 days  with overlapping of the DFI and the ST as no patient died from recurrence or metastasis.n 6, 11), 3 out of the 7 (43%) dogs died from causes unrelated to the tumor , and 2 out of the 7 (28%) dogs developed other tumors .At the time of writing, 2 out of the 7 (28%) dogs are still alive  attached to the surrounding structures, dorsal or ventral to the rectum, has an elevated possibility of being benign or behaving in a benign manner.All the dogs in this retrospective study showed the findings described above at DRE. They were easily surgically removed, for the most part by digital blunt dissection, in all the patients, also including the two cases postoperatively histologically defined as well-differentiated malignant tumors. In the case of masses characterized by greater malignancy it probably would not have been possible to remove them so easily without leaving macroscopic portions of the tumor in place or without penetrating the lumen of the rectum or the vagina. Post-surgical histology confirmed the benign nature of the mass in 10 dogs and a low-grade leiomyosarcoma in 2 cases.According to the literature, the dogs in this series were middle-aged ,14. PatiIn all cases, a preoperative diagnosis was attempted. Although a larger cellular specimen is expected from smooth muscle tumors as compared to other mesenchymal tumors, preoperative cytology was diagnostic in only 2 out of the 10 cases which underwent the procedure, likely due to the poor tendency of these tumors to exfoliate, . HoweverA needle core biopsy via a tru-cut needle was attempted in 1 case and the histologic diagnosis of sarcoma was obtained. This procedure is more likely to yield a diagnosis than a fine needle biopsy; however, it has limitations due to its major invasiveness and the need for anesthesia. The waiting times for a histological report may also represent a limitation for those patients in which the urethra and the rectal mass compression could be so severe as to cause complete urinary and/or fecal obstruction, leading the animal to a life-threatening status.Diagnostic imaging should always be carried out for staging purposes and for pre-surgical planning. Metastatic spread to the abdominal structures, dilation of the ureters and the renal pelvis, and abdominal effusion can be detected by abdominal ultrasound; the latter may not be very useful for visualizing intra-pelvic tumors. Osseous pelvic structures act as a barrier to a complete sonographic evaluation, and only the abdominal portion of the mass can be investigated. Nevertheless, in the case of a well-defined, firm, dorsally or ventrally located mass at DRE, abdominal ultrasound should be used as a first line diagnostic approach for investigating the urinary apparatus  and the distal digestive tract, and for excluding ileo-sacral lymphadenopathy. Abdominal radiographs do not usually provide sufficient information regarding intrapelvic masses arising from soft tissues. Indirect signs due to the compression of the mass on the adjacent organs can be observed Abdominal radiographs can be useful for excluding pelvic bone involvement. Thoracic radiographs can be used to exclude lung metastases.Computed tomography is currently the most indicated diagnostic tool in such cases since it is now easily accessible at relatively low cost. Compared with ultrasound and radiographic examination, CT provides information regarding the presence, location, size and extent of the disease, and its relation with the surrounding organs, thus providing information for surgical planning. Moreover, contrast enhancement better delineates the vessels into or in proximity of the mass. A previous study reported that benign masses, such as fibromas and leiomyomas, are more likely to have a homogeneous architecture after contrast administration due to less tissue necrosis and vascularization as compared with malignancies . A CT scMarginal surgery was performed on all the dogs in this series. Different approaches were chosen according to the localization of the mass. The perineal approach  is less invasive and has low morbidity; however, if the mass lies in the most cranial part of the pelvic canal or in the caudal abdomen, the perineal approach may not be sufficient for removal of the mass. In those cases, a median caudal celiotomy, or a combined perineal and abdominal approach may be required.The dorsal approach to the rectum provides good surgical exposition of the caudal part of the rectum; since these tumors are often well encapsulated, a blunt dissection is generally enough to remove them entirely, preserving the structures involved in the fecal continence mechanism, i.e., the pelvic nerve plexus ,19.n 9) the urethra was iatrogenically damaged during the dissection; however, it was promptly replanted at the level of the vaginal urethral papilla. No other complications, such as infection and suture dehiscence, in the short-term follow-up, or fecal or urinary incontinence in the long-term follow-up were observed.Intraoperative complications, including hemorrhage, are rare. In one dog  suggesting a local recurrence were absent in all the dogs having an available follow-up. The 18-year-long study period also represented a limitation. The diagnostic procedures were different among the dogs included in the study.Even if rare, intra-pelvic masses can be benign or behave in a benign manner. Clinical signs, referring to rectal and urethral compression and DRE can help the clinician in supporting this diagnostic hypothesis. Cytology is often inconclusive but helps to exclude epithelial or round cell malignant intra-pelvic masses. Total body CT represents the most useful diagnostic exam in such cases. All the dogs included in this study were referred to the VTH with the owners’ conviction of a poor prognosis since an unresectable malignancy was originally suspected by the referring veterinarians. In some cases, euthanasia had also been proposed. Despite this, after a thorough clinical examination, including a DRE combined with appropriate imaging exams, usually represented by a CT scan, a marginal excision was curative in all the dogs with an available follow-up, including those with a well-differentiated malignant tumor. Improvement in the quality of life was rapid in all cases.


We present the case of a 29-year-old patient whose pain began with the interscapular region, progressing to paresthesia and loss of muscle strength in the lower extremities. MRI of the spine was done, a lesion was found in T2 to T6, ependymoma was suspected and was taken to subtotal resection with laminectomy, the histopathological report, as well as the immunohistochemistry, was compatible with glioblastoma type not otherwise specified (NOS). He received adjuvant with radiotherapy and concomitant chemotherapy, but he progressed to the cervical and lumbar spine, the patient died 16 months after diagnosis. A review of the literature is made and the clinical and radiological characteristics and treatment protocols that have been used in this entity are reported. Primary spinal cord glioblastoma multiforme (GBM) is a CNS tumor that is clinically, histologically, and genetically heterogeneous, and is a rare tumor type accounting for 1% to 5% of all glioblastomas and only 1.5% of all tumors of the spinal cord .This tumor occurs particularly in younger patients (mean: 26 years old), is a little more common in men ,4, and cDue to its low incidence, the literature is limited to reports of cases or small series, so it is not surprising that little is known about the clinical characteristics and the treatment.A 29-year-old male patient, with no relevant medical or family history, experienced mild dorsal pain, paresthesia, and loss of muscle strength in the right leg, and three months later presented the same symptomatology in the contralateral leg, as well as urinary incontinence. Two months later he had numbness in both lower limbs and could not walk as well as constipation; non-contrast and contrast-enhanced MRI of the spine revealed a hypointense and hyperintense lesion from T2 to T6 vertebrae on T1 to T2 images, respectively  positive, so, the diagnosis of glioblastoma type not otherwise specified (NOS) was confirmed.We offered adjuvant concurrent chemoradiation (CCRT). A total radiation dose of 5400 cGy in 30 fractions at 180 cGy per fraction per day was given to the tumor bed along from T2 to T6) with concomitant temozolomide at a dose of 75 mg/m2  with seven cycles of carboplatin and irinotecan as a protocol due to a shortage of temozolomide in our institution.Three months after the completion of maintenance QT, the patient complained of pain in the lower back, and we repeated MRI of the craniospinal axis. Many implants were observed from C4 to T8 and in the lumbar region, nodular images were found next to the nerve roots Figure .No lesions were found in the brain parenchyma, but we considered leptomeningeal spread, so, a total radiation dose of 3000 cGy in 10 fractions at 200 cGy per fraction per day was given to the whole brain and total spine with volumetric modulated arc therapy (VMAT) technique, as palliative treatment, unfortunately, he presented Collet-Sicard syndrome and died a week later.Primary tumors of the spinal cord represent a heterogeneous group of diseases in the CNS, their treatment is very complex owing to their low incidence and morbidity associated with treatment. These tumors are divided into three anatomical categories: intradural intramedullary ; intradural extramedullary ; and extradural , hematopoietic malignancies .In adults, 80% of neoplastic lesions located in the spinal cord are extramedullary tumors. Low-grade gliomas account for 50-88% of intramedullary tumors . Until 2Despite aggressive treatment, the prognosis of patients with primary spinal cord GBM remains poor, with an overall survival of 10-14 months , in the Therapeutic options for primary spinal cord GBM remain controversial because there are few reported cases, but usually include surgery, radiation therapy (RT), and QT similar to intracranial GBM. Surgical options include gross total resection (GTR), subtotal resection (STR), and biopsy. Complete resection with the greatest preservation of neurological function is the most recommended surgical approach, however, this pathology tends to be infiltrative and it is difficult to distinguish between the margin of the tumor and the adjacent normal tissue, the association with survival according to the type of resection has not yet been established, there are studies that do not find benefit with a GTR in terms of overall survival and even associate it with higher mortality , 11, 16.2 similar to intracranial glioblastoma are reported [2 is continued, but the results in the primary spinal cord GBM are still not clear and it is important to mention that we have dose-limiting toxicity in spinal cord because of its radiosensibility [Adjuvant radiotherapy is recommended because there is a high probability of spread and recurrence, the ideal dose has not been established, in the literature, doses of 50.4 Gy with concomitant temozolomide at a dose of 75 mg/mreported , after csibility . In thisAfter four months without neurological deterioration, our patient presented leptomeningeal dissemination because MRI of the whole brain and spine revealed new lesions in the cervical and lumbar spine. This probability of leptomeningeal dissemination has already been reported in the literature, the craniospinal irradiation has even been proposed, but, its effectiveness remains unclear . In thisAll current therapeutic measures have produced disappointing results and little data is available on their true value.The initial presentation and evolution of this case were like the cases reported in the literature, for example, the age of 26 years, thoracic location; the diagnosis was made with histopathological examination, even a second evaluation was needed because of the rarity of the pathology. It had clinical progression despite aggressive management , unfortunately in our institution, there was a shortage of temozolomide, so treatment with carboplatin and irinotecan was chosen, but the patient presented leptomeningeal dissemination and was treated with craniospinal irradiation. The patient had a survival of 16 months (a little longer than that reported in the literature). All this shows that spinal GBM is an aggressive pathology, with an unfavorable prognosis despite treatments.There are no clinical studies that regulate the therapeutic conduct to be followed, for this reason, and we consider that it is important to improve the registration and cooperation of many centers to understand this pathology.


Pyogenic liver abscess (PLA) is an uncommon yet potentially fatal disease. The disease most commonly arises from biliary infection but may also result from hematogenous seeding and portal spread secondary to bowel contamination, direct seeding, or penetrating trauma. The diagnosis is suspected when there is a hepatic lesion on imaging. Confirmation of diagnosis requires purulent aspirate or bacterial growth on Gram stain/culture of the abscess or blood. The mainstay of treatment is antimicrobials in conjunction with either percutaneous abscess drainage or aspiration. Surgical drainage is reserved for cases of ruptured abscess, peritonitis, or in the presence of an underlying surgical cause. PLA typically presents with fever and abdominal symptoms. We report a case of an unusual manifestation of PLA, presenting as hiccups, which led to a significant delay in the diagnosis and treatment. Pyogenic liver abscess (PLA) is an uncommon yet life‐threatening condition that typically presents with fever, jaundice, and abdominal pain. We report an unusual case of PLA manifesting as hiccups. This case highlights the importance of thoroughly investigating persistent or intractable hiccups for an underlying occult cause including the possibility of a PLA to prevent a delay in the diagnosis and treatment of this highly fatal disease. Pyogenic liver abscess (PLA) is a life‐threatening condition that carries a mortality rate of 2–12%.3, segmented neutrophils 85%, hemoglobin 13.6 g/dL, mean corpuscular volume 84.2 fL, creatinine 1.12 mg/dL, aspartate aminotransferase 17 U/L, alanine aminotransferase 37 U/L, alkaline phosphatase 111 U/L, and total bilirubin 0.7 mg/dL. Contrast‐enhanced, abdominopelvic computed tomography (CT) scan revealed acute sigmoid diverticulitis and diffuse, multiloculated hypodense lesions consistent with liver abscesses, with the largest lesions measuring 8.3 × 5.6 cm and 3.9 × 3.5 cm in size  of 23 400 mmHiccups, also known as singultus, are a common occurrence caused by involuntary, repetitive myoclonic contractions of the diaphragm and intercostal muscles. Hiccups are classified according to their duration: acute (<48 h), persistent (>48 h), and intractable (>1 month). Acute hiccups tend to be self‐limited, transient, and typically do not warrant extensive work‐up. Persistent or intractable hiccups require further investigation for pathology involving the diaphragm, gastrointestinal, cardiopulmonary, or central nervous systems. Medication‐induced and psychogenic causes should also be explored. The most common causes of hiccups include gastroesophageal reflux disease, Parkinson's disease, anxiety, and gastric distension secondary to irritants, i.e. alcohol, tobacco. Serious diseases have been reported to present with hiccups such as pulmonary embolism, myocardial infarction, cerebrovascular accident, subdural hematoma, brain tumor, and intracranial aneurysm.Liver abscesses may pose as an irritant to the diaphragm and be a rare cause of persistent hiccups. There are only two published reports in the literature to our knowledge that describe hiccups as an initial presentation for a PLA.Klebsiella pneumoniae, have been associated with an increased risk of gastrointestinal malignancy.The diagnosis of PLA is established when there is a hepatic lesion on imaging (i.e. ultrasound and CT) in addition to purulent aspirate or bacterial growth on Gram stain/culture of abscess or blood. The mortality of PLA has declined in recent years likely due to improved imaging and diagnostic techniques.Symptomatic treatment of persistent hiccups alone may result in delayed or missed diagnosis, which could be life‐threatening. This case highlights the importance of thoroughly investigating persistent or intractable hiccups for an underlying occult cause including the possibility of a PLA to prevent a delay in the diagnosis and treatment of this highly fatal disease.Written informed consent has been obtained.


Eccrine spiradenoma (ES) is a rare benign skin adnexal tumor originating from eccrine sweat glands. The features of ES on ultrasonography (US) have received little attention. Therefore, we report the sonographic findings in a case of an ES that originated from the abdominal wall and discuss the previously reported cases.A 53-year-old woman was admitted to our hospital with a complaint of a painful nodule on the right side of her abdominal wall of 1-year duration.The mass on the right side of abdominal wall was diagnosed as ES by histopathological examination.The patient subsequently underwent total excision of the mass.The patient recovered well and had no complications during the 1-year follow-up.As eccrine spiradenoma (ES) is rare and most of the tumors are excised without prior imaging studies. Little is known regarding the features of ES on ultrasonography (US). Familiarizing with the clinical and US features of this rare tumor may increase awareness of the disease among sonographers and clinicians. Because of its rare incidence and usually excised without prior imaging studies, few cases describing the ultrasonography (US) imaging findings of ES have been published. Therefore, we report the sonographic findings in a case of an ES that originated from the abdominal wall and discuss the previously reports.22.1A 53-year-old woman inadvertently found a painful nodule on the right side of her abdominal wall 1 year before. The size of the nodule was like a soybean, and the nodule has recently increased in size. The patient had intermittent pain that had recently worsened and hence was admitted to our hospital. Physical examination revealed a mass on the right side of the abdominal wall, which was approximately 1 cm in diameter. It was blue to purple in color, with a smooth contour, protruding from the surface of the skin, accompanied by obvious tenderness and no swelling or ulceration of the skin. She did not have any history of longstanding illness and malignancy. There was no history of any abdominal surgeries or trauma. Laboratory tests revealed normal findings.2.2Longitudinal and transverse US was performed using a SuperSonic US device equipped with a SL15–4 linear probe. The sonographic images revealed several mass lesions in the dermis and superficial subcutaneous fat layer. They showed no extension into muscle layer. The larger 1 measured 1.8cm × 1.6cm × 0.8 cm, with well-defined margin, heterogeneous echo texture, and posterior acoustic enhancement. The lesions were in close proximity and showed similar sonographic structure , CK8/18 (+), EMA , S100 (+), CEA (-), Ki-67 (approximately 15%+), p53 (++), LCA(lymphocyte+), ER(scatter+). Special staining showed: PAS (+) (Fig. 3 ES is a rare and benign tumor of the sweat glands, located in the dermis and subcutaneous tissue. This tumor may occur at any age, but it is most frequently seen in 11 to 40 years old people and without a predilection for either gender.,3 Congenital cases are occasionally reported. There is a special clinical manifestation in ES. It often presents as a single nodule ranging in size from 0.3 to 5 cm in diameter. Patients most often present with complaint of a painful nodule. The mass is mostly blue or skin color, may be seen as pink, red, purple, brown nodule on skin as well. Congenital cases are occasionally reported. ES is frequently solitary while multiple cases are often hereditary, autosomal dominant genetic diseases and have family history.ES was first reported by King in 1930. In 1956, the clinical and pathological manifestations were described in detail by Kersting and Helwing. 2015, Balaban M first reported the sonographic and sonoelastographic findings of a benign ES. Then, Hwang presented a case analysis of its ultrasound performance in 2018 with the clinical data of 8 patients who underwent preoperative US at 4 different medical institutions and analyzed the US features. A comparative analysis of this case with the cases described in the literature was performed. In this study the ES was seen on the right side of abdominal wall compared with the literature where the location was thought to occur more commonly in the head and neck, and upper trunk by Balaban M and more commonly in the upper extremities by Hwang. This study was concordant with the first one. The ES located in dermis and the subcutaneous fat layer, without extension into the muscle layer was similar to those previously published reports. Although the sizes of ES in this case varied, all of these tumors measured only 1 or 2 cm while the literature showed that ES can range in size from 1 to 10 cm. Furthermore, compared with the imaging findings of previously reported cases, this case showed the same lobular shape and well-defined margin. However, it is worth noting that this lobular appearance was misdiagnosed as tumors by US at first which deserves more attention for diagnosis of this lesion. Moreover, this case showed hypoechoic, heterogeneous echotexture, posterior acoustic enhancement and blood flow in the central and peripheral region. These findings were also concordant with the previous reports. Ultrasonography plays a very important role in the location and differential diagnosis of ES, but the definitive diagnosis is usually based on histopathological diagnosis since the clinical and sonographic findings of the tumor are not typical. In addition, pathological diagnosis showed that the pain accompanying this tumor may be attributed to the vast nervous and capillaries plexus that surrounds the connective tissue capsule. Surgical resection is the curative treatment option in ES and has favorable prognosis. Although ES is a benign tumor and rarely shows malignant transformation, its early diagnosis and complete surgical removal are still important due to the risk of malignant transformation. Rapid growth, bleeding and ulceration in the short term indicate the possibility of malignant transformation and surgery should be performed as soon as possible.Sonographic characteristics of ES have been reported in only a few reports. In 2008, Jin first reported the US findings of an ES in the arm. A hemangioma may be hypoechoic or hyperechoic relative to the surrounding tissue and have high vessel density that help in differentiating hemangiomas from ES. Angioleiomyoma appears as an oval mass with well-defined margins, homogeneous hypoechogenicity, and mainly located in the calves or ankles, parallel to the skin. A glomus tumor shows an oval appearance, inner hypoechogenicity, color ball sign. Most of the glomus tumors are seen at the ends of the limbs, especially under the fingernails. Skin endometriosis are usually ill-defined, irregular in shape with heterogeneous echo texture. The size and symptoms of this tumor are always related to the menstrual cycle.However, ES also needs to be differentiated from other clinically painful nodules, such as schwannomas, hemangioma, angioleiomyoma, glomus tumors, skin endometriosis. Most of the schwannomas are oval and solid, and have a clear boundary and less blood flow signal. The rat tail sign, target sign are the characteristic on US.In this study, we reported a case of an ES that originated from the abdominal wall. ES is a rare benign tumor and usually excised without prior imaging studies. Therefore, its US characteristics has received little attention. Although the definitive diagnosis is usually based on histopathological diagnosis, US is useful in localization and differential diagnosis. We have learned that ES is usually located in the subcutaneous fat layer, with well-defined margin, lobulated appearance, heterogeneous echo texture, and posterior acoustic enhancement. The color Doppler flow is present in the central and peripheral region. To summarize, familiarizing with the clinical and US features of this rare tumor may increase awareness of the disease among sonographers and clinicians.Conceptualization: Yuanyuan Xing, Hongyuan Xue, Yuquan Ye.Formal analysis: Yuanyuan Xing.Investigation: Yuanyuan Xing, Xiaojing Wu, Caina Xu.Resources: Yuanyuan Xing.Writing – original draft: Yuanyuan Xing.Writing – review & editing: Yuanyuan Xing, Li Sun, Huiying Li, Yafei Zhang.


An 82‐year‐old woman with precordial pain at rest was admitted to the Emergency Department for possible cardiac heart disease; electrocardiogram excluded ischemia and high‐sensitive troponin was normal. Echocardiogram revealed a hyperechoic mass adjacent to the mitral annulus. Electrocardiography‐gated computed tomography (CT) angiography exam confirmed the presence of the mass protruding into the atrioventricular groove, adjacent to the posterior mitral. On the precontrast images the lesion was hyperdense with some scattered central calcific spots. CT findings are typical of a giant caseous calcification of the mitral annulus and excluded the diagnoses of pseudoaneurysm (it does not show any communication with the left ventricular cavity), neoplasm/abscess  or infected/abscessified mitral calcification . This is a benign condition that can be easily misdiagnosed as ventricular aneurysm or pseudoaneurysm on the contrast‐enhanced images, when the caseous content is isodense to the iodinated blood pool. Informed Consent for the publication was obtained by the patient.


Malignant melanoma of the vulva is a rare and aggressive tumour with dismal prognosis. It tends to recur and metastasize early. Surgical excision with or without regional lymph node dissection is still the treatment of choice with adjuvant therapy decided on a case by case. Furthermore, HIV infection has been associated with more aggressive disease. Herein we present a 45-year-old HIV-infected female patient on antiretroviral therapy who presented with vulval ulcer for one year. On examination, she had ulcerated nodule on the labia majora. Radiology showed vulvovaginal tumour without involvement of the adjacent organs. Malignant melanoma was confirmed on both the incisional biopsy and vulvectomy. She responded poorly to radiotherapy. Furthermore, she presented with recurrence and metastatic disease a month after surgery. She was lost to follow-up clinic. Malignant melanoma originating from the female genital tract is rare . It arisA 45-year-old female patient who presented with a vulvar ulcer for one year without other symptoms. She was HIV infected with CD4+ of 291 cells/μL on highly active retroviral treatment. She was previously treated for pulmonary tuberculosis for six months. She had no other co-morbidities or family history of malignancy. On examination, she had good performance score. The abdomen was soft with right inguinal lymphadenopathy. Per vaginal examination revealed an ulcerated nodule on the right labia majora extending from the right labia minora into the right lower 2/3 of the vagina. This lesion was close to the anal sphincter, but did not involve the pelvic bone. It was draining foul smelling dark discharge. She did not have pigmented lesion/nevus on any part of the body. A biopsy of the ulcer showed malignant melanoma. The patient was counselled about the disease and the prognosis thereof. She was further investigated for extent of the disease and possible metastasis. The chest X-ray (CXR) was unremarkable . The MagAn ulcerated, multinodular and rubbery tumour measuring 90x50x25mm was received  with pelPost-surgically, she presented with recto-vaginal fistula after 23 days. A diverting colostomy was performed and discharged to home. She came back after a month to the gynaecology clinic with shortness of breath, weakness and pain of the lower limbs. Per vaginal examination revealed bleeding and non-healing operation side and a well colostomy. Although the biopsy was not done at this stage, a clinical diagnosis of local recurrence was made. A CXR at this point showed upper lobes nodules and left pleural effusion in keeping with lung metastasis . She wasIn general, melanomas are some of the most aggressive cancers in human, despite their location . Vulvar NRAS and BRAF point mutations. They occur early and remain throughout the course of disease including metastasis [BRAF mutation in this case was also used to exclude clear cell sarcoma of soft part which was a morphological differential diagnosis. BRAF mutation is also associated with more aggressive behaviour. Whilst surgery still remains the treatment of choice, radical surgery has not proven to increase patient´s survival [en bloc resection for deeper lesions, with a safety margin of 2-3cm and regional lymphadenectomy [Histologically, the common cell types are epithelioid, followed by spindle and mixed type. Confirmatory immunohistochemical stains include positive S-100 protein, melan A and HMB-45 . Carcinotastasis . They casurvival . Wide loenectomy -9.in-situ melanoma, a 1cm margin for melanomas with thickness less than 1mm, 1-2cm margins for a thickness of 1-2mm, and 2cm margins for melanomas with thickness greater than 2.01mm. Adjuvant therapy can be directed by lymph node status [There are no differences in recurrence or survival between women treated with excision or hemivulvectomy compared to those who underwent radical vulvectomy and lymphadenectomy . Currente status , 9.The FIGO staging system seemed to be a suboptimal predictor of recurrence and survival while the AJCC staging was found to be more appropriate to determine prognosis and therapy in these patients . The SocThe accurate and early diagnosis is important in vulvovaginal melanoma because of its unfavourable and unpredictable prognosis; with the tendency to recur and metastasize. HIV infection is associated with more aggressive behaviour and poorer prognosis compared to non-infected patients. Surgical treatment constitutes the most fundamental therapeutic management as adjuvant treatment has not shown to improve prognosis/survival. Molecular investigation has a role to play in management of this tumour as mutation of certain genes may have a role in targeted therapy to improve survival and outcome.


Individuals with an incongruence of their chromosomal sex and genital appearance are classified as having a disorder of sexual development (DSD), and they often present with ambiguous genitalia. The diagnosis and management of DSD patients are usually challenging and require a multidisciplinary approach. Gender assignment should not be based solely on physical exam and imaging but also on the genotype and hormonal function of the gonads. We present an infant born with ambiguous genitalia; the parents were told they were having a male infant during the prenatal ultrasound but at birth, the infant was found to have female-appearing external genitalia with no palpable gonads. MRI of the abdomen was inconclusive, but further workup, including karyotype, hormonal function, and intraoperative evaluation, was consistent with a male infant. He was, therefore, subsequently assigned to the male sex. Individuals with an incongruence of their chromosomal sex and genital appearance are classified as having a disorder of sexual development (DSD). The incidence is estimated at 1:2500-5000 live births -4.Gonadal development starts at fertilization when chromosomal sex is established. It was at first assumed that gonadal differentiation is solely governed by the absence or presence of the sex-determining region Y (SRY) gene located on the Y chromosome, which transforms a bipotential gonad to testes. It is now believed that gonadal determination involves a complex of gene expressions and transcription factors causing the activation of the testicular pathway while at the same time inhibiting the ovarian conduit (or vice versa). As the testes are established, Leydig cells and Sertoli cells are formed and produce testosterone and anti-Mullerian hormone (AMH), respectively, around the eighth week of gestation. This leads to the regression of the paramesonephric ducts and differentiation of the Wolffian duct into male reproductive organs. The absence of AMH and low levels of testosterone leads to the persistence and differentiation of the paramesonephric ducts into fallopian tubes, uterus, and superior portion of the vagina . Given tInfants born with DSD usually present with ambiguous genitalia, in which the external genitals cannot be clearly identified as male or female. Once life-threatening disorders have been ruled out, evaluation of an infant with ambiguous genitalia requires a multidisciplinary team to assign an appropriate sex and gender assignment.We present a case of an infant born with ambiguous genitalia due to severe hypospadias, requiring complex medical investigation and decision-making for proper gender reassignment.An infant was born at 34 weeks gestational age via C-section secondary to preeclampsia with oligohydramnios. The patient was noted to have intrauterine growth restriction and was small for gestational age. At the 17-week prenatal ultrasound, parents were told they were having a male infant. At birth, however, external genitalia appeared female with no palpable gonads. The newborn screen was normal. An ultrasound was performed and noted a uterine-appearing structure and a possible right ovary. Parents were thus advised to name and raise the patient as a female at discharge from the neonatal intensive care unit. However, the karyotype later came back 46, XY.The patient was seen by pediatric endocrinology at four weeks of age. At this time, the infant was starting to gain some weight. The genital exam was similar to that at the time of birth but with marked clitoromegaly. Because the initial ultrasound showed a uterine structure and possible ovaries with no mention of male structures on an XY infant, the SRY gene was sent and came back positive. The infant had a follow-up at eight weeks of age, at which time, the exam showed a more phallic structure. The hormonal workup revealed normal testosterone and AMH levels of 82.42 ng/dL and 96 ng/mL, respectively, with an undetectable estradiol level, consistent with an infant with intact testicular function. LH and FSH were also normal at 2.56 mIU/mL and 4.26 mIU/mL, respectively. An MRI of the abdomen and pelvis and was read as (1) Gonadal tissue within the bilateral inguinal canals with mixed testicular and ovarian MR characteristics; (2) Clitoromegaly; (3) Soft tissue structure interposed between the rectum and bladder likely representing the vagina; (4) No uterus or abdominopelvic gonadal tissue identified.The patient was subsequently referred to pediatric urology and seen at five months of age. The physical exam noted severe penoscrotal hypospadias with bifid scrotum Figure ; incomplThe diagnosis and management of DSD patients can often be challenging and requires a multidisciplinary approach, usually including an endocrinologist, urologist, and psychologist.DSD evaluation is warranted for grossly atypical genitalia, but there are less conspicuous presentations for which it is also recommended. With predominantly male genitalia, further investigation is indicated for bilateral undescended testes, severe hypospadias, or hypospadias with undescended testes and/or micropenis. With predominantly female genitalia, further evaluation should be done for significant clitoromegaly, posterior labial fusion, or palpable gonads inguinally or in the labioscrotal folds.Infants born with ambiguous genitalia are most commonly categorized as either undervirilized males or virilized females. Therefore, careful physical examination and family history of consanguinity or prenatal androgen exposure is important. It is also important to get a rapid karyotype and/or SRY gene probe, allowing for categorization as XX DSD, XY DSD, or sex chromosome DSD .Initial testing should be to rule out congenital adrenal hyperplasia (CAH) due to the potential for a life-threatening adrenal crisis. This includes a 17-hydroxyprogesterone level (17-OHP) to test for the most common form of CAH (21-hydroxylase deficiency), as well as electrolytes to check for hyponatremia and hyperkalemia. Female infants usually present with virilization and hence are typically diagnosed early, whereas male infants are usually missed and are either seen at five to 10 days of life with an adrenal crisis or when the newborn screen comes back abnormal. Our patient was initially thought to be a virilized female due to what appeared to be an enlarged clitoris, absence of inguinal structures or testes, and the initial US showing a uterus and an ovary. The newborn screen came back normal, as did 17-OHP.Gonadal activation is ideally investigated when testosterone peaks at one to three months of life, also referred to as mini-puberty. Gonadal function is investigated via levels of luteinizing hormone (LH), follicle-stimulating hormone (FSH), testosterone, dihydrotestosterone (DHT), and AMH. LH is a good indicator of the hypothalamic-pituitary-gonadal axis (HPG); low levels of LH could indicate hypogonadotropic hypogonadism while elevated LH levels represent inadequate androgen activity/synthesis such as in complete androgen insensitivity syndrome (CAIS)/partial androgen insensitivity syndrome (PAIS) or gonadal dysgenesis. The serum AMH level is valuable for assessing gonadal function and helps explicate the level of defect in an XY DSD patient. A low level of AMH in an XY DSD suggests gonadal dysgenesis -7.First-line imaging is an ultrasound to evaluate the presence or absence of a uterus, as well as to attempt the visualization of gonads. However, ultrasound results are user-dependent and can be misleading, as seen in this case, and thus an MRI of the abdomen/pelvis is typically more reliable. It is therefore important to emphasize that gender assignment should not be based solely on imaging but also on the genotype and hormonal function of the gonads. In this case, the initial ultrasound stated the presence of a uterus. The differential for an XY patient that does have a uterus would include XY gonadal dysgenesis or Swyer syndrome, persistent Müllerian duct syndrome, and ovotesticular DSD. The failure of the primitive gonad to differentiate into testes in XY gonadal dysgenesis would result in decreased or absent AMH production and thus the development of a uterus and other paramesonephric structures. The most common etiologies are mutations in SRY, NR5A1, and WT1 . PatientAfter presenting initially with ambiguous genitalia, our patient’s physical exam at two months of age appeared more virilized, likely due to the surge of male hormones or mini-puberty. A complete hormonal and genetic workup revealed an XY infant with intact testicular function. It was more evident at that time that the infant had severe penoscrotal hypospadias. Hypospadias is a congenital malformation in males where the urethra does not open at its usual position at the tip of the glans, instead found anywhere in the ventral midline as far back as the perineum. In severe cases of hypospadias, especially with a concomitant penoscrotal transposition, the atypical anatomy may be mistaken for clitoromegaly with a vestibular groove and interpreted as female genitalia.There are several non-medical aspects of the diagnosis and management of DSD patients that may be overlooked but are of particular importance. Setting expectations for the timeframe of an extended workup, as well as awareness and acknowledgment of the stress of a DSD workup and diagnosis, are key starting points for family involvement in the process . It is aIt is recommended that families delay typical rites like birth announcement and completion of the birth certificate until a diagnosis is finalized. Giving a gender-neutral name is usually advised as well. Parental access to counseling and psychosocial support is essential to help families handle the unique situation of raising an infant with DSD . DecisioInfants born with ambiguous genitalia require a multidisciplinary approach. Proper evaluation includes a thorough physical exam, karyotype, hormonal workup, imaging, and in complex situations such as this case, surgical exploration. Parental counseling is key to helping families cope with the situation and provides the support they need to make informed decisions about their child's future.


Struma ovarii are rare ovarian tumors, of monodermal germ cell origin, containing predominantly thyroid tissue. They are typically benign unilateral pelvic masses. Among the rare cases of malignancy, the most common histological type is that of the papillary carcinoma. A definite preoperative diagnosis of these tumors is difficult to achieve since most imaging findings are generally nonspecific. A rare exception is the case of synchronous abnormal thyroid blood tests where an ovarian teratoma should be highly suspected, especially when no pathology of the thyroid gland coexists. Surgical excision is the mainstay of treatment. Taking into account that struma ovarii are mainly encountered in patients of a childbearing age, a conservative surgical approach is a reasonable option. We present the case of a 56-year-old woman who was diagnosed with both a primary and recurrent struma ovarii while investigating the incidental finding of elevated blood laboratory levels of the thyroglobulin (Tg). However, synchronous abnormal blood laboratory levels of the thyroid function may raise the suspicion of an ovarian goiter, especially when no pathology of the thyroid gland is described.3, T4), the thyroid-stimulating hormone (TSH), and the antithyroid antibodies were within normal range values. A neck ultrasonography (US) had already been performed describing a heterogeneous thyroid gland and bilateral small nodules with benign radiological patterns.We present the rare case of an otherwise healthy 56-year-old woman who was initially referred to our department due to incidentally found high levels of serum thyroglobulin (Tg) . The levels of the thyroid hormones (TA magnetic resonance imaging (MRI) of the neck was not diagnostic demonstrating no remarkable pathological findings of the thyroid gland.Further investigation was followed to exclude an ectopic origin of Tg. A US of the abdomen followed by a computed tomography scan (CT) of the upper and lower abdomen was performed. A heterogeneous mass sized 11 × 9 × 9.9 cm was detected in the pelvis. The lesion had irregular cystic and solid components, findings highly suggestive of an ovarian teratoma. The tumor was contiguous with the left ovary with no signs of invasion to the neighbor organs. The right ovary showed no pathology, and the uterus had features of fibromatosis. No other intraabdominal pathology was described .Taking into consideration these findings, surgical excision of the adnexal mass was the treatment of choice. Provided that Tg levels remained high postoperatively, the option of a complementary total thyroidectomy was also discussed with the patient. Given the patient's postmenopausal age, a laparoscopic total hysterectomy with bilateral salpingo-oophorectomy was initially proposed. However, taking into account the patient's preference as well as the fact that no pathology of the right ovary was detected, a more conservative approach was finally decided. A laparoscopic left salpingo-oophorectomy en bloc with the irregular mass was performed. The tumor was removed intact, preserving its capsule and preventing any content spillage . The patMacroscopic examination of the tumor showed an irregular grey-yellow cystic neoplasm, filled with hair and fat-like tissue. Histopathological report set the diagnosis of a left ovarian mature cystic teratoma composed predominantly of thyroid tissue. Thyroid follicles were revealed in fibrous septa, and the immunohistochemistry result was positive for thyroglobulin. No remarkable findings were observed in the left fallopian tube.During a three-month follow-up period, the Tg blood levels were within the normal range. An additional US of the thyroid gland confirmed no remarkable findings. In accordance with the patient's desire, we recommended an annual follow-up including laboratory and imaging examinations avoiding any further surgical procedure.Six months later, the patient readmitted in our department due to a new remarkable increase in the Tg blood levels far above the normal (>1500 ng/ml). A CT scan of the lower abdomen demonstrated a 11 × 10.8 × 11.5 cm pelvic mass. The lesion was located centrally in the pelvis and consisted of solid and cystic compartments. Additionally, a limited fluid collection was also revealed in the right pelvis. No other pathology was detected .An early recurrence of the struma ovarii was suspected. Following written informed consent from the patient, an open total hysterectomy with right salpingo-oophorectomy was successfully performed. The pelvic mass was removed en bloc with the remaining genital organs. Neither rupture of the tumor nor intraperitoneal seeding was encountered. The patient had an uncomplicated postoperative period.The pathologic report described a grey-yellow mass sized 12 × 8.5 × 7.4 cm totally occupying the right ovary. A nodular lesion with a maximal diameter of 0.4 cm was additionally detected at the uterine wall. The microscopic examination reported typical findings of an ovarian teratoma. Immunohistochemical markers of the thyroid tissue were found positive, and the definitive diagnosis of a benign struma ovarii was set. The right fallopian tube had a normal configuration and histology. Lastly, the nodular lesion of the uterus was found to be a small leiomyoma.During a one-year follow-up period, the patient is in a good clinical condition. Tg levels remain within the normal range, and consecutive US examination of the thyroid gland as well as of the lower abdomen has not revealed any pathology.Stroma ovarii are rare tumors diagnosed typically in women between the ages of 40 and 60 years old. They account for nearly 5% of all the ovarian tumors . These tMost stroma ovarii are asymptomatic and are detected incidentally during a routine medical examination. In some reports, these tumors are characterized as slowly enlarging pelvic masses associated with abdominal pain or other nonspecific symptoms . As regaIn our case, the high levels of Tg in combination with a grossly normal thyroid gland raised the suspicion of an ectopic origin of the Tg.Generally, mature teratomas have characteristic features in the US and CT imaging. A typical image in the US shows a heterogenous adnexal mass with smooth contours and acoustic shadowing due to sebaceous material or/and thin echogenic bands due to hair follicles inside the cystic cavity . A charaRegardless of the accuracy of the imaging modalities, no definite preoperative diagnosis can be made. Therefore, it is the histopathological report of the tumor that sets a definite diagnosis .The necessity of a second pathologoanatomical opinion is highlighted in the literature, given the rarity of these cases . ImmunohSufficient evidence regarding the management of stroma ovarii supports that surgical excision is the treatment of choice. There are very few data available comparing radical versus conservative surgical approach and no established guidelines regarding the extensiveness of the surgical procedure. The option of a more conservative approach is supported in most case series, especially when childbearing women are involved and taking into consideration that struma ovarii arise mostly unilaterally, usually from the left ovary .A similar approach is as well recommended even when there is a high index of suspicion for malignancy, mainly due to the low metastatic potential of the tumor and low risk of recurrence. In contrast, total hysterectomy with bilateral salpingo-oophorectomy or/and omentectomy can be a reasonable choice for postmenopausal women or those without maternity plans . MetastaIn this regard, lymph node dissection in the para-aortic lymph nodes is mostly advocated when a mucinous variant of the struma ovarii is diagnosed due to their lymphatic pattern of spread . In caseIn the presented case, our initial approach was oriented towards a more conservative surgical procedure, in accordance with the patient's decision. However, in the event of a recurrent tumor, a more extensive surgical procedure was performed in an attempt to eliminate the risk of recurrence. The option of a complementary total thyroidectomy was finally avoided since the laboratory levels of Tg remained low and no remarkable pathology of the thyroid gland was detected during consecutive diagnostic imaging.Struma ovarii are rare and mainly asymptomatic ovarian tumors making preoperative diagnosis quite challenging. Physicians should maintain an appropriately high index of suspicion for an ovarian goiter, mostly in cases of female patients presented with elevated laboratory levels of the Tg and no synchronous pathology of the thyroid gland. Additionally, postoperative increase in Tg levels can be a useful indicator of recurrence during the follow-up period.


We report an unfortunate case of rectal perforation and subsequent peritonitis in a 74-year-old lady who underwent haemorrhoidal artery ligation in order to treat complex large external and internal haemorrhoids. Serious complications following haemorrhoid surgery are rare and this is the first documented case of delayed intra-abdominal rectal perforation following a haemorrhoid artery ligation operation and converted Ligasure open haemorrhoidectomy. Haemorrhoids are described as enlarged anal vascular cushions that typically cause discomfort and rectal bleeding on defecation. Management is conservative or surgical, depending on the severity of symptoms and degree of prolapse. Surgical options include traditional haemorrhoidectomy (open or closed) with or without energy devices, stapled haemorrhoidopexy or Doppler-guided artery ligation such as the haemorrhoid artery ligation operation (HALO). Complications post-haemorrhoid surgery are rare, with serious complications such as rectal perforation and subsequent peritonitis scarce in published cases. We present a case of rectal perforation to the intra-abdominal cavity in a 74-year-old lady, identified 7 days after HALO and subsequent converted open Ligasure haemorrhoidectomy.A 74-year-old lady was referred to our department complaining of fresh rectal bleeding on defecation for many years. Her past medical history included primary biliary cirrhosis and oesophageal varices, for which she was medicated with rifaximin, ursodeoxycholic acid, spironolactone and omeprazole. Her operative history included a laparoscopic cholecystectomy and a hysterectomy. On examination, her abdomen was non-tender with no palpable masses. Proctoscopy and rigid sigmoidoscopy revealed the presence of two large external haemorrhoids with three internal haemorrhoids in the 3, 7 and 11 o’clock position. The rectal mucosa looked normal at 20 cm of visualization.It is of note that, in the past, she had presented to another surgeon with symptomatic haemorrhoids. At this time, it was felt that her risk for operative treatment was too high due to cirrhosis. On this occasion, however, operative treatment was deemed necessary and the patient subsequently underwent HALO. Examination under anaesthesia and sigmoidoscopy was performed intra-operatively. Large fourth degree prolapsing haemorrhoids with a large intra-rectal venous component and five large internal haemorrhoids were identified. Two large arteriovenous fistulae were seen at 7 and 11 o’clock. Unfortunately, attempts at suturing caused major bleeding of mixed venous and arterial type. This was not controlled by simple suturing and, therefore, the procedure was converted to open Ligasure haemorrhoidectomy, performed with the aid of a Park’s retractor. A post-operative sigmoidoscopy revealed no damage to surrounding organs or further bleeding. She was discharged after 48 h of inpatient observation.Unfortunately, the patient re-presented to the emergency department at 5 days post-discharge complaining of abdominal pain and constipation. On examination, umbilical tenderness with guarding was elicited and bowel sounds were present. Remarkable admission laboratory tests included an elevated C-reactive protein (234.6), acute kidney injury stage 1 , deranged anticoagulation (INR of 1.6 and patient not on anticoagulation) and hyponatraemia (sodium of 124). A venous blood gas revealed a lactate of 3.5 with a normal pH and bicarbonate level. Haemoglobin and platelet counts were normal. A computerized tomography (CT) abdomen and pelvis scan showed a large amount of pneumoperitoneum and ascites. After initial resuscitation, urgent laparoscopy was performed. After laparoscopic insertion, pus and peritonitis was seen in all quadrants and early conversion to laparotomy was done. Many adhesions and a large faecal collection were seen upon entering the abdominal cavity. A 2-cm rectal perforation with clean edges was discovered at the left anterior side of the rectum at the level of the peritoneal reflection. The sigmoid and upper rectum was mobilized. The bowel was divided close to the perforation. An end colostomy was brought out. Abdominal lavage was done with copious amounts of saline and the distal rectum was washed through the anus with saline until the fluid returned clear. Two Robinson drains were inserted, and the abdomen was closed. The patient was subsequently admitted to the intensive care unit (ICU) for close observation. Initially, her vital readings were stable, and her stoma was functioning normally. She was extubated 48 h post-laparotomy. Unfortunately, whilst in ICU, her inflammatory markers continued to rise, and her renal and respiratory function deteriorated. She was deemed to be in multi-organ failure and sadly passed away 12 days post-laparotomy.Haemorrhoids are defined as a pathological enlargement of the anal vascular cushions and are classified according to extent of prolapse; first degree haemorrhoids are within the rectum, second degree haemorrhoids prolapse externally on defecation while spontaneously reducing, third degree haemorrhoids require manual reduction once prolapsed and fourth degree haemorrhoids cannot be reduced. Conservative management or banding is reserved for lesser degree haemorrhoids, whereas HALO, stapled haemorrhoidectomy or the conventional Milligan Morgan open haemorrhoidectomy procedure are necessary to repair third and fourth degree haemorrhoids. The conventional approach is most often used for third and fourth degree haemorrhoids, while HALO is efficacious for second and third degree haemorrhoids , 2. The Severe post-haemorrhoidectomy complications such as rectal perforation and peritonitis are rare . In publWe cannot explain why rectal perforation occurred in this case. Acknowledging that post-operative sigmoidoscopy was unremarkable, it is likely that this was a delayed perforation. A thermal injury from the Ligasure device is possible with delayed perforation. Delayed perforations have been documented following endoscopic colorectal procedures and this has been termed coagulation syndrome or transmural burn, resulting in localized peritonitis and serosa breakdown . An alteAs far as we are aware, this is the first documented case of delayed intra-abdominal rectal perforation following HALO and converted Ligasure open haemorrhoidectomy. To determine this, PubMed online library and MEDLINE database were searched from 1996 to 10 January 2021 with search terms ‘rectal perforation’ and ‘haemorrhoidectomy’.We encourage surgeons to be cognizant of delayed intra-abdominal perforation as a potential complication of Doppler-guided THD/HALO and Ligasure-assisted open haemorrhoidectomy. Follow-up CT scanning in the post-operative period may be beneficial in patients with intra-operative complications. Moreover, consideration of pre-operative optimization in patients with portal hypertension is pertinent. Pre-operative transjugular intrahepatic porto-systemic shunting or medical management should be regarded .None declared.None.


Prepancreatic portal vein (PPV) is a congenital anatomical variant of the portal vein (PV). PPVs are extremely rare and generally classified into two categories, prepancreatic preduodenal portal vein and prepancreatic postduodenal portal vein (PPPV). Prepancreatic preduodenal portal veins are rare, with approximately 100 reported cases globally; PPPVs are even more atypical, with less than 20 documented cases globally. Despite the extremely low occurrence, PPPV knowledge and recognition are important, especially for hepatobiliary-pancreatic (HBP) surgeries, such as pancreaticoduodenectomy (PD) for patients of a PPPV. Here, we report a case of PPPV and a literature review.A 73-year-old-male with ampullary carcinoma underwent PD at our hospital. Preoperative enhanced CT revealed an abnormal L-shaped PV, identified as a PPPV. Both the PPPV and the postpancreatic “normal” superior mesenteric vein (SMV) divaricated from the SMV at the caudal side of the pancreas. A splenic vein and inferior mesenchymal vein flowed into the postpancreatic “normal” PV, which encircled the common bile duct and potentially flowed into the liver, forming a cavernous transformation at the hilar plate. During surgery, we attempted to isolate the PV from the pancreas and common bile duct. However, it was difficult to isolate from the pancreas. The PPPV was so fragile that bleeding from the PPPV became uncontrollable. To remove the tumor, we resected the PPPV and reconstructed a “normal” PV as an autogenous graft. To maintain intraoperative hepatic blood flow and avoid small bowel congestion, an antithrombogenic bypass catheter was placed between the SMV and umbilical vein during reconstruction. After surgery, several complications occurred, such as PV thrombosis and hyperammonemia. The patient was discharged on postoperative day 45.PPPV is a rare vascular variant but is easily diagnosed preoperatively due to its distinct shape on CT imaging. However, isolating the PPPV from the pancreas and bile duct is incredibly difficult and potentially associated with increased operative risks and postoperative complications. PV resection rather than isolation is a potential solution to reduce the risk of hemorrhage, even in the absence of invasion. Awareness of vascular variants is essential to minimize postoperative risks associated with surgery, especially hepatobiliary-pancreatic (HBP) surgery. Anatomical variants of the branching pattern of the intrahepatic portal vein (PV) have been reported in approximately 20–35% of the population , 2. HoweA 73-year-old Japanese male was transferred to our hospital with high levels of serum AST, ALT, and γ-GTP. He did not complain of any symptoms. His surgical history revealed that subtotal gastrectomy with Billroth II reconstruction was performed due to a gastric ulcer 33 years prior (the operation record was unavailable). His medical history also included multiple cerebral infarctions and a chronic subdural hematoma. There were no other significant findings in his family medical history, social history, or lifestyle history.Contrast-enhanced CT revealed a 16-mm tumor in the ampulla of Vater with low contrast  was administered on postoperative days 3–7 to prevent PV thrombosis. However, enhanced CT on postoperative day 8 showed thrombosis in the reconstructed PV. Fortunately, intrahepatic PV flow was favorable due to cavernous transformation. Then, edoxaban (30 mg/day) was administered starting on postoperative day 8. Several complications occurred but were treatable, such as hyperammonemia (treated by Lactulose and L-arginine/L-citrulline), pancreatic fistula  and delayed gastric emptying . The patient was discharged after 45 days of care.In terms of pathological findings, the ampullary carcinoma was diagnosed as stage IIB . The reA wide range of congenital conditions may affect the portal venous system. A PPPV is an extremely rare congenital vascular variant and has a very complex embryological etiology. At the fourth week of embryonic life, three anastomoses develop around the duodenum and between the paired vitelline veins: cranial-ventral (within the liver), dorsal (posterior to the duodenum) and caudal-ventral (anterior to the pancreas). Under normal conditions, the ventral bud fuses side-by-side with the dorsal bud. The dorsal bud forms the upper head, body, and tail of the pancreas, whereas the ventral bud forms the inferior head and uncinated process , 8. DuriA PPPV is often detected as an L-shaped or inverted L-shaped PV by contrast-enhanced CT , 10. AddThe first presentation of PPPV was reported in 1972 by Brook et al. . A literFor malignant disease such as distal bile duct carcinoma, pancreatic head cancer, and ampullary carcinoma, it is necessary to perform PD . In general, PD with PV resection is related to more surgical complications (Clavien Dindo grade ≥ 3) than PD without PV resection . In factNakao et al. , 23 repoFor PPPV reconstruction, it might be difficult to perform end-to-end anastomosis given the long distance. Shimizu et al.  used theSince PPPV is a rare vascular variant, this study has several limitations. First, owing to the previously reported literature, it was easy to diagnose preoperatively, given the typical L-shaped or inverted L-shaped PV on CT. However, Tanaka et al.  reported