Perforation of the gallbladder and the spillage of gallstones can be a cause of ongoing morbidity and mortality in patients post cholecystectomy. We report on an unusual case of a 58-year-old male who developed a right upper quadrant lump 8 years after laparoscopic cholecystectomy which was eventually determined to be an abscess from a retained gallstone that had become embedded in the musculature of his abdominal wall. We have also discussed some of the considerations taken in the surgical management of this case. The spillage of gallstones during cholecystectomy can result in long-term morbidity or mortality if they are not completely retrieved. As it is not possible to predict where a retained gallstone may finally lie, so too is not possible to predict when or how a patient may manifest their subsequent complication. Symptoms may range from simple discomfort or pain, to abscess or collections or even potentially devastating fistulae or sepsis. We present a case of an abdominal wall abscess likely due to a retained gallstone dislodging and embedding into the muscle upon removal of the procedural abdominal drain.A well 58-year-old male presented to the surgical clinic with a palpable small tender lump on his right upper quadrant on a background of a previous emergency laparoscopic cholecystectomy for acute cholecystitis 8 years ago. At the time of his emergency procedure, his observations were normal and blood investigations revealed elevated inflammatory markers  and 2. HHis initial cholecystectomy was largely uneventful with a single 6 mm immobile gallstone on pre-operative ultrasound and a normal intra-operative cholangiogram without filling defects or anatomical variant. A 10Fr Blake drain was inserted through the 5 mm medial subcostal port site and removed prior to discharge. Histopathology confirmed acute cholecystitis, but no gallstones were identified. An 18 mm disruption to the distal portion where the wall was incomplete was noted suggestive of an iatrogenic perforation.Eight years later, a computer tomography (CT) scan revealed a thick-walled collection on the deep aspect of the right anterolateral abdominal wall, adjacent to inferior hepatic segment 5 measuring 61 × 24 × 38 mm with a tiny focal calcific density .Given the history and imaging findings, we were suspicious of retained gallstones causing abdominal wall abscess. We scheduled the patient for an operative drainage of the anterior abdominal wall collection. On examination, a RUQ lump was palpable and was in close proximity to the medial subcostal laparoscopic port site scar. A chronic intramuscular abscess cavity was found extending to the peritoneum with adherent omentum. Within this abscess cavity, a small gallstone was identified, and the indurated tissue and abscess cavity were excised to reduce the risk of contamination and ongoing infection . The abdHe had an uneventful post-operative recovery and was discharged the next day on a course of oral antibiotics. He was reviewed in clinic ∼3 weeks later; apart from some intermittent post-operative pain at the surgical site on stretching, he had no other complications and his wounds were healing well.We report on an unusual complication of a retained gallstone resulting in an abdominal wall collection several years after cholecystectomy. It is postulated that the single offending gallstone identified on pre-operative ultrasound was spilt intraoperatively as a result of iatrogenic perforation, eventually became caught in the Blake drain and subsequently dislodged into the muscle layers upon removal, resulting in eventual infection and intramuscular abscess. The incidence of gallbladder perforation has been reported to be within 10–40% with gallstone spillage ranging from 5 to 30% . MultiplWhen faced with calculous cholecystitis, it is ideal to prevent the occurrence of iatrogenic perforation and spillage of gallstones , 6. If sAvoid spillage of gallstones during cholecystectomy.If spillage of gallstones occurs, attempt retrieval of all stones if possible.If there are a low number of gallstones identified on pre-operative imaging, consider confirmation of complete retrieval with ex vivo palpation or examination of the gallbladder specimen.Complications from retained gallstones can present with a variety of signs and symptoms, some of which may be non-specific.Definitive management typically requires surgical retrieval of retained gallstones. In surgically unsuitable patients, source control may be attempted with radiologically guided aspiration or drainage.The patient has provided informed consent which has been retained by the author’s institution.


The cases of splenic hydatidosis are quite rare and giant isolated primary splenic hydatidosis is even rarer. The patients present with vague symptoms or no symptoms at all. Here we report a case of isolated splenic hydatid cyst. So, we should think the differential diagnosis of splenic hydatidosis in any abdominal case of endemic regions.A 27-year-old female presented with left-side abdominal pain for the past 7 months without any particular attraction. Abdominal ultrasound showed a well-defined cystic mass on the upper pole with low-level internal floating debris. Contrast Enhanced CT scan showed well defined cystic lesion measuring about 10.8 × 9.6 × 8.5 cm in the upper pole of the spleen with an exophytic component and minimal homogenous wall enhancement. Laparoscopic Splenectomy was done and albendazole for 3 weeks was prescribed after all the patient was completely normal.In this case, the optimal treatment of giant isolated splenic hydatidosis was splenectomy and prescription of albendazole.We believe in any abdominal case of the endemic region, the hydatid cyst of the spleen should be taken as one of the differential diagnoses and should be managed appropriately before the complication arises. •Splenic HC is often asymptomatic.•Intraperitoneal rupture of splenic HC can cause life-threatening anaphylaxis.•Diagnosis of splenic HC requires a high index of suspicion.•Abdominal ultrasound and computed tomography are modalities of choice for diagnosis.•Standard treatment for splenic HC is splenectomy. The defveolaris . Echinocveolaris . Primaryveolaris . In thisveolaris .2A 27-year-old female recently married with no comorbidities presented to the surgical outpatient department with a complaint of abdominal pain in the left hypochondrium region for 7 months. The pain was gradual in onset, dull aching in character. She has normal bowel and bladder habits with no history of jaundice, fever, vomiting, and significant weight loss. There is no any relevant drug history, family history, genetic information and psychosocial history related with the case. On general examination, she had a GCS score of 15 and her vitals were normal. On per abdominal examination, the abdomen was soft and non-tender in palpation. On auscultation abdominal sounds were present. The laboratory investigations showed hemoglobin, Red Blood Cells (RBCs), White Blood Cells (WBCs), Platelets, Packed Cell Volume (PCV), Prothrombin Time (PT), International normalized ratio (INR), and Partial Thromboplastin Time (PTT), creatinine, electrolytes, and liver function tests were in the normal range. Abdominal Ultrasound showed a well-defined cystic mass with low-level internal floating debris and an echogenic strap measuring 9.5 cm × 9.6 cm was noted at the dependent portion, the mass was noted in the upper pole of the spleen . It was 3E. granules and E. multilocularis are the most popular. These organisms are the etiologies of cystic echinococcosis (CE), also known as cystic hydatid disease, and alveolar echinococcosis (AE), correspondingly. E. vogeli and E. eligarthrus, which generate polycystic echinococcosis, are less commonly seen [Echinococcal infection in humans is often caused by four species, among which nly seen .CE can occur in any organ, among which the liver 75%), lung (15.4%), and spleen (5.1%) were the most dominant . For the5%, lung Meanwhile, concomitant hepatic or pulmonary hydatid cyst (HC) is often reported in 20–30% of cases, representing systemic dissemination. Additionally, ruptured hepatic HC spreads parasites intraperitoneally causing secondary splenic HC .As splenic HC expands slowly with a rate of 0.3 cm–1 cm per year, it is often asymptomatic and detected accidently on physical examination as an abdominal mass in the left hypochondrium (30%). Patients can also present with abdominal pain (25%), discomfort, or compressive symptoms such as dyspepsia, dyspnea, and constipation ,11.Regarding complications, intraperitoneal rupture of splenic HC, which could be spontaneous or trauma-induced, can cause life-threatening anaphylaxis due to slippage of protoscolex mass in the left hypochondrium (30%). Perforation into the colon can manifest as haematochezia, even massive gastrointestinal bleeding. Moreover, a splenothoracic fistula has also been reported. Later, pericystic inflammation is followed by adhesion to a nearby organ. Other complications are secondary infection of splenic HC and splenic atrophy .Diagnosis of splenic HC requires a high index of suspicion, especially in endemic areas. It requires a combination of clinical findings, imaging, and immunological tests. The key point is to distinguish from other splenic cystic formations, including splenic abscesses or hematomas, epidermoid cysts, post-traumatic pseudo cysts and neoplasms .Abdominal ultrasonography and computed tomography are usually the modalities of choice for diagnosis procedures . With a Serology test has a limited role in diagnosis but could be useful for follow-up . The EchStandard treatment for splenic HC is splenectomy, including total splenectomy, partial splenectomy, cyst enucleation and unroofing with omentoplasty, given the low efficacy of the medication. Total splenectomy has a low recurrence rate and should be indicated for large HC in which splenic parenchyma has been drastically reduced and brittle to prevent ruptured complications ,11. At tPerioperative management includes anti-helminthic drugs and vaccinations. Medical regiments are Albendazole 10–15 mg/kg/day for one month or Mebendazole 40–50 m/kg/day for 3–6 months after surgery, combined with Praziquantel 40 mg/kg/wk for 2 weeks during pre and postoperative period. Nevertheless, Albendazole is preferred due to its superior bioavailability. Plus, Hib and meningococcus C conjugate vaccines should be administered at least 2 weeks before or 2 weeks post-splenectomy .Percutaneous aspiration irrigation and reaspiration (PAIR) could be indicated for patients who refuse surgery or have greater anesthetic risk. Still, it is associated with prolonged follow-up and should be avoided in calcified or abscessed cysts .4Despite advances in medical sciences, splenic hydatidosis has been a diagnostic challenge. The various investigation cannot always reach the diagnosis. It is important to consider the hydatid cyst in one of the differential diagnoses in abdominal cysts and must be in splenic cysts if the clinical condition justifies in endemic regions. Standard splenectomy and prophylaxis with albendazole are suggested.The patient was informed, and we have acquired her consent for this publication.None.Writing the paper: Dr. Rajan Kumar Sah and Dr. Chau Thi Minh Dang, Surgical treatment for this patient: Biki Kumar Sah, Study design: Dr. Rajan Kumar Sah, Data interpretation: Vivek Kumar Sah.N/A.Biki Kumar Sah.Not commissioned, externally peer reviewed.None.


Neuroendocrine tumors (NETs), which are a rare type of tumor, are defined as epithelial cells with predominantly neuroendocrine differentiation and consist of a spectrum of tumors emerging from stem cells throughout the body and can occur anywhere in the body. While they are rare, the incidence over the past few decades has increased. Here we present a case of a 64-year-old female who was incidentally found to have a duodenal neuroendocrine tumor. The patient initially presented to the emergency department secondary to syncope and collapse. During her trauma evaluation, an incidental lobulated soft tissue mass inferior to the distal stomach was seen on complete computed tomography (CT) scans. The surgery team was consulted for resection of the mass and an octreotide scan was performed prior to resection to further evaluate the mass and to check for any signs of metastatic disease. The octreotide scan demonstrated intense radiotracer accumulation within the duodenal mass consistent with a neuroendocrine tumor and no areas of abnormal radiotracer accumulation suspicious for metastatic disease. Pathology of the resected mass was positive for a well-differentiated neuroendocrine tumor with an organoid pattern and homogenous oval-round neoplastic cells with a salt-pepper nuclear and pseudo glandular arrangement that was well-circumscribed and partially encapsulated with negative margins. Immunohistochemistry was positive for AE ⅓, CD56, Synaptophysin, and chromogranin and negative for CD117, DOG-1, CD34, and CD45. The prevalence of NETs has increased over the years due to the improvement in diagnostic tools, such as upper gastrointestinal endoscopy. In addition to the fact that the duodenum is a rare location for such tumors, neuroendocrine tumors are also typically found in those under 50 years old. However, our patient was found to have both a duodenal mass and was over the age of 50 at the time of presentation and diagnosis. To date, a consensus on a conclusive treatment of duodenal NETs (D-NETs) has not been reached. This case brings to light the importance of further research in diagnosing and treating neuroendocrine tumors and also raises awareness for clinicians to have this in their differential. Neuroendocrine tumors (NETs) are rare types of tumors, only contributing to 0.5% of all malignancies, and are found predominantly in females under the age of 50 . NETs arWhile most NETs are asymptomatic and are incidental findings on imaging, treatment plans are decided based on tumor size, location, grade, stage, and tumor type . In 2005A 64-year-old female with a past medical history of hypertension, glaucoma, and alcohol abuse was initially admitted to the cardiac service for evaluation of syncope and collapse with head trauma and loss of consciousness. Admission labs are shown in Table The surgery team was consulted for resection of the mass. Prior to the resection of the mass, an octreotide scan was performed to further evaluate the mass and to check for any signs of metastatic disease. The octreotide scan demonstrated intense radiotracer accumulation within the duodenal mass consistent with a neuroendocrine tumor and no areas of abnormal radiotracer accumulation suspicious for metastatic disease. She was subsequently scheduled for elective resection of the tumor. Intraoperatively, a horizontal incision was made about 3 cm superior to the umbilicus with a skin knife, which was carried through subcutaneous tissue with electrocautery and the peritoneal cavity was entered. Some omental adhesions to the anterior abdominal wall were encountered and divided. The mass was immediately palpable in the duodenum. The duodenum was separated from its lateral attachments and the tumor was found to be attached to a segment of D2 on the anterior wall. It appeared adherent through the wall with a fairly narrow stalk. The right gastroepiploic vessels were divided and the mass and segment of the anterior wall of D2 was excised. This segment came back negative for gastrointestinal stromal tumor (GIST) tumor. The duodenal defect was closed, the abdomen was then irrigated, Jackson-Pratt (JP) drains were placed, and the skin was closed. Occlusive dressings were placed, the patient was extubated in the operating room, and she was taken to the post anesthesia care unit (PACU) in stable condition. The estimated blood loss was 200 mL. Pathology was positive for a well-differentiated neuroendocrine tumor with an organoid pattern and homogenous oval-round neoplastic cells with salt-pepper nuclear and pseudoglandular arrangement that was well-circumscribed and partially encapsulated with negative margins. Immunohistochemistry was positive for AE ⅓, CD56, Synaptophysin, and chromogranin and negative for CD117, DOG-1, CD34 and CD45 Figure , 1D. UpoNeuroendocrine neoplasms consist of a spectrum of tumors emerging from stem cells throughout the body and can occur anywhere in the body. While they are rare, the incidence over the past few decades as well as the survival rate has increased, indicating that they are more prevalent than previously thought and better diagnostic tools are being implemented to detect them . The cliIn 2015, Fitzgerald et al. stated that there has been an increase in the incidence of D-NETs as well as gastroenteropancreatic neuroendocrine tumors (GEP-NETs) . In theiBecause almost 90% of the D-NETs are non-functional, the majority of them are incidentally detected on imaging or endoscopy . Tissue D-NETs are classified into three grades: G1, G2, and G3, depending on Ki-67 index and the number of mitotic figures. G1 corresponds to a Ki-67 index of less than 2% and/or a number of mitotic figures less than two. G2 corresponds to a Ki-67 index between 3 and 20% and/or a number of mitotic figures between two and 20. G3 corresponds to a Ki-67 index above 20% and/or a number of mitotic figures above 20 . The oveTo date, a consensus on a conclusive treatment of D-NETs has not been reached. However, the management is based on tumor type, stage, location, size, and histological grade. Today, resection of the tumor appears to be the most radical treatment. However, whether it is surgical or endoscopic removal depends on its size. Because the pathogenesis of the disease is yet to be understood, this case brings to light the importance of further research in diagnosing and treating neuroendocrine tumors and also raises awareness for clinicians to have this in their differential.NETs are rare types of tumors that arise from stem cells and can occur anywhere in the body. While the majority of neuroendocrine tumors are asymptomatic, their clinical presentation is very similar to other GI tumors. Thus, it is imperative for clinicians to include this rare malignancy in their differential in patients presenting with broad symptoms. With its increased incidence over the past few decades due to improved diagnostic studies, more research is needed to further investigate the risk factors associated with it, the prognosis, as well as treatment options other than surgical removal.


Background: Horner’s syndrome (HS) classically consists of the symptom triad of miosis, ptosis, and anhidrosis. It is caused by impairment of a certain pathway in the sympathetic nervous system. It may also appear as part of the clinical signs of other diseases and syndromes, including Pancoast tumors, intradural and/or epidural tumors, thoracic outlet syndrome, syringomyelia, brachial plexus injury, and aortic dissection. Here, we report a very rare complication of vertebral column resection in children, and we present the clinical findings of a case of Horner’s syndrome with a current literature review. Case presentation: A five-year-old child with severe congenital kyphoscoliosis qualified for surgical treatment of the spinal deformity via a posterior approach, with three-column osteotomy and fusion. Results: After successful surgery, the patient presented with HS due to distraction of the sympathetic nerve trunk and, thus, innervation to the left eye. At the 4-year follow-up, the child had fully recovered. Conclusions: Pediatric HS after posterior instrumented scoliosis correction surgery with posterior vertebral column resection of the thoracic spine is very rare. This is the first reported case of HS after posterior vertebral column resection and spinal fusion for congenital kyphoscoliosis without the use of epidural analgesia. Symptom resolution may be variable and, in some cases, delayed. Horner’s syndrome (HS) classically consists of the symptom triad of miosis, ptosis, and anhidrosis . It is aBased on scientific reports, the incidence of HS as a complication of cervical spine surgery is quite high after anterior cervical discectomy, ranging from 0.06% to 3.8%. Some researchers have noted a higher rate (as high as 57%) of HS with oblique corpectomy after an anterolateral approach to the cervical spine and after revision surgery ,5. In coThe purpose of this work was to present a case report of a very rare complication after posterior spinal surgery in a pediatric patient with a severe congenital spinal deformity.A five-year-old child was referred to our spine unit for surgical treatment of a severe congenital spinal deformity of malformed vertebrae and failure of segmentation at the cervical and thoracic levels. A congenital spinal defect was diagnosed before the age of one by a pediatrician who referred the child for outpatient treatment to a pediatric orthopedist. Initially, the child was treated conservatively, with observation and rehabilitation. The curvature of the spine progressed as the child grew. At around age four, the curvature progressed most rapidly. Attempts to treat with a corset were ineffective, and the only option for further treatment was surgery.After a thorough orthopedic examination and additional tests , and consultations with other specialists, including a cardiologist, pulmonologist, neurologist, and neurosurgeon, the five-year-old female pediatric patient with congenital scoliosis and severe kyphoscoliosis qualified for surgical treatment of the spinal deformity via a posterior approach with three-column osteotomy and fusion .Neurological examination before the surgical treatment indicated neurological function was intact. MRI and CT performed before the surgery showed multilevel mixed congenital spinal anomalies in the cervical and thoracic spine, without spinal cord pathologies  using the costotransversectomy approach  with posCorrection of the deformity included right-sided compression and left-sided distraction. The pleura remained intact during the surgery, and no chest tube was needed .After successful surgery, on the same day, the child presented with worsening left-sided ptosis, miosis, and anhidrosis. No other symptoms were noted in the physical examination and neurological status examination. No blunt trauma occurred to her neck during surgery. No other symptoms or neurological deficits were noted, nor any headaches. Other than the triad of signs presented, all examinations were normal. The child was diagnosed with Horner’s syndrome, and we then sought to intensively diagnose the cause of the disease . We carefully reviewed the family’s medical history and found no cancer, neurological disease, stroke, or vascular disease. The imaging tests performed before the operation showed no changes in the lungs or the nervous system. We also noted no vascular anomalies. Given the lack of any clear pathologies, we began to consider intraoperative trauma. We suspected a hematoma in the brain could cause similar symptoms or a dissection of the carotid artery. We performed a Doppler ultrasound of the carotid arteries, computed tomography of the head and cervical spine, and magnetic resonance imaging of the head and cervical spine. These studies did not show pathologies that could explain the cause of the disease. The only possible cause of the symptoms of Horner’s syndrome was spinal correction of the deformity. The operation consisted of resection of the fused T3–T4 vertebrae, and then compression on the right side and distraction on the left side of the spine. Although we did not observe any changes in the monitoring of the spinal cord intraoperatively, the symptoms that occurred on the left side fit the clinical status of the patient. The child presented with HS due to distraction of the sympathetic chain at the high thoracic area and thus to the left eye. Since we did not find any other confirmation of Horner’s syndrome, the patient was discharged home on an elective basis, as after every elective spinal surgery. After six months of follow-up, the HS had fully resolved. At the four-year follow-up, the child was fully intact . No specThe treatment of pediatric spinal deformities depends on their etiology. The gold standard in the treatment of idiopathic scoliosis is rehabilitation and corset treatment, and in the case of curvature progression despite the treatment above of over 50 degrees, Cobb-surgical treatment ,16. In tThe etiology of HS in children and adolescents is not yet fully understood. Often, it is congenital or acquired . In the Perioperative etiological factors must also be considered. The study by Nasser et al.  showed tOn the other hand, Mueller et al. reported HS after scoliosis correction, which was not caused by epidural anesthesia but was due to a mechanical cause, the placement of the implant and a hook located around the T4 vertebral epiphysis . There aIt should also be noted that there are some studies where pediatric HS cases were found to be idiopathic. George et al. noted no etiology in 70% of infants with HS diagnosed in the first year of life , and SmiPrevention of acquired HS may be possible through familiarization with the procedures that may cause this complication. HS persistence may be observed on cranial, cervical, and vascular imaging. Most studies analyzing HS propose a systematic approach to lesion localization by means of accompanying signs and symptoms, followed by anatomical imaging with focused magnetic resonance imaging or computed tomography with angiography . Some auIn our case, HS might have been due to injury to the sympathetic chain when vertebral column resection was performed, the osteotomy was closed without any anterior wall support by cage or mesh and the left-sided osteotomy underwent gentle distraction. We presume that the cause of HS was the stretch effect of surgery near the level T3–T4 bone block of the spine, although the complete preganglionic lesion should have occurred at T1–T2. In addition, the anatomy of the cervico-thoracic section has a complicated course of vertebral arteries, which may increase the risk of vascular damage. Therefore, we suggest that the course of vessels be properly diagnosed by CT angiography before planned surgical treatment. It is necessary to confirm the course of the vertebral artery in congenital defects of the spine, as in careful preparation in the case of preservation of the transverse opening at the level of the hemivertebral vertebra in CT images. The absence of intraoperative complications and cardiorespiratory impairment, combined with the delayed onset of blockage symptoms, are likely to support these explanations. Hered et al.  showed tPediatric HS after posterior instrumented scoliosis correction surgery with posterior vertebral column resection of the thoracic spine is rare. The diagnosis of HS immediately after surgery may not be easy, particularly when the patient is sedated. The possible causes of HS should always be considered. Avoidance of high chest tube insertion, professional and mindful anesthesia care during anesthesia administration, and minimization of surgical dissection in the oculosympathetic nerve pathway may help minimize the potential risks of iatrogenic HS in children. Special care should be taken during corrective procedures using the compression-distraction technique in the high thoracic spine, which may lead to a direct injury. Symptom resolution is variable and may be delayed.


Background: There is a broad spectrum of vulvar pigmented lesions that differ based on their histopathological and clinical features. Chronic vulvar purpura is a rare entity, associated with a broad morphological spectrum, from lichen aureus, Zoon’s vulvitis, pigmented purpuric dermatosis and with lichen planus as in our case. Case presentation: In this article we discuss a case of an 86-year-old white woman with hyperpigmentation on her upper vulva, next to the introitus, with complaints of urine incontinence. Biopsy revealed subepithelial stromal lichenoid inflammatory infiltrate containing plasma cells, lymphocytes and some neutrophilic granulocytes as well as dilated and congested vessels. Hemosiderin deposits and erythrocyte extravasation were found. There was evidence of hyperkeratosis with hyper granulosis and erosions. Spongiosis was also noted. Few melanocytes were identified with no sign of malignancy. These findings correlate with the diagnosis of vulvar lichen planus. Conclusions: Chronic vulvar purpura is a clinical term used for different chronic inflammatory dermatoses presenting as red bluish or violaceous discolorations on the vulva, often associated with cayenne-pepper-like speckling. Considering a great overlap of possible diseases, the final diagnosis could be challenging. It is important to exclude a melanocytic tumour in these cases.  Chronic vulvar purpura is a clinical term used for different chronic inflammatory dermatoses presenting as red, bluish or violaceous discoloration on the vulva. The most common diseases associated with chronic vulvar purpura include Zoon’s vulvitis, lichen aureus, pigmented purpuric dermatosis and vulvar lichen planus .Morphological changes in Zoon’s vulvitis have generally been attributed to a combination of irritation/moisture and mechanical/friction trauma and are as such non-specific. Zoon’s vulvitis can either develop as an isolated condition or represents an unrelated phenomenon in the background of a more specific inflammatory process. Clinical and morphological overlap between vulvar pigmented purpuric dermatosis and Zoon vulvitis suggests a site-specific mucosal reaction to an erosive process, either inflammatory (hypersensitivity reaction) or traumatic. Genital lichen planus can present clinically as chronic vulvar purpura, but is distinguished from potential mimickers by the presence of interface/lichenoid tissue reaction associated with vacuolar degeneration of basal keratinocytes and necrotic basal keratinocytes ,2.Pigmented purpuric dermatoses presenting at genital sites are exceedingly rare, with only a few cases reported in the literature ,3. No meWe are reporting a case of an 86-year-old woman presented with a hyperpigmented patch and papule on the vulva.An 86-year-old Caucasian woman was admitted to the Division of Gynaecology and Obstetrics, University Medical Centre in Ljubljana, Slovenia due to darkly pigmented irregular patch on her vulva, of six-month duration. She did not complain about the presence of flares of pruritus, burning or pain, which are otherwise typical symptoms of vulvar lichen planus. Physical examination revealed a well-demarcated brownish to blackish macule with irregular borders measuring 3 × 2 cm in diameter located at the vestibule and over the clitoral frenulum , asterixThere were no lesions on the rest of the vagina or vulva, or on general skin examination. Nevertheless, she had been suffering from urinary incontinence for a considerable period of time. Since the possibility of melanoma could not be ruled out on clinical grounds, three punch biopsies were performed, two from the irregular patch and one from the satellite lesion. The patient received no previous local treatment.The first punch biopsy from the hyperpigmented patch revealed mild spongiosis and exocytosis of lymphocytes associated with a mild to moderate lichenoid infiltrate in the subepithelial stroma, composed of lymphocytes, plasma cells and neutrophils A. AbundaThe third punch biopsy, from the satellite lesion, revealed a prominent lichenoid tissue reaction A associaThe diagnosis of vulvar lichen planus presenting as chronic vulvar purpura was set based on a review of histopathological samples. After the biopsy, the patient was scheduled for a follow-up in 3 months and received no treatment due to lack of clinical symptoms relating to her condition.Chronic vulvar purpura is a clinical term used for different chronic inflammatory dermatoses presenting as red bluish or violaceous discolorations on the vulva, often associated with cayenne pepper-like speckling . The mosZoon vulvitis, also designated vulvitis plasmacellularis, presents clinically as a well-demarcated erythematous or ecchymotic patch(s) most commonly localized on the inner aspects of the labia minora, vestibule/introitus, urethral meatus and the clitoris . The hisPurpuric lesions are characterized by red or purple discoloration caused by bleeding into the skin, whereas pigmented vulvar lesions are characterized by a change in color or pigmentation of the skin, caused by an increase in the production of melanin or the accumulation of other pigments. Pigmented vulvar lesions can be benign or malignant and can be caused by a variety of conditions such as freckles, age spots, or melanoma. They can be dark or light and can appear in various sizes and shapes ,8.Pigmented purpuric dermatoses presenting at genital sites are extremely rare ,3. They Genital lichen planus can develop as an isolated condition in the absence of cutaneous lesions. Three clinical variants are generally recognized, including erosive, classic/papular and hypertrophic lichen planus . In contThe treatment for vulvar lichen planus can vary depending on the severity and presentation of the condition. Treatment may be topical with the use of corticosteroids, calcineurin inhibitors or oral with the use of corticosteroids, azathioprine and mycophenolate mofetil. Thus, conservative or non-pharmacologic management may be considered in the case of asymptomatic patients as in our case .Although clinical presentations and the histopathological findings of chronic pigmented vulvar lesions are well described in other case reports, there is still a great overlap of findings among possible diagnoses in our case report. Therefore, physicians must consider all of the differential diagnoses and possible underlying diseases before reaching a conclusion based on clinical and histopathological findings. The final diagnosis that is needed for proper treatment is challenging because of the resemblance and the morphological spectrum of purpuric vulvar diseases. A proper diagnosis can only be made by a healthcare professional after a physical examination and possibly a biopsy.


Primary hepatic neuroendocrine carcinoma (PHNEC) manifests as a rare type of liver tumor. PHNEC is not specifically clinical or radiographical and is often misdiagnosed and mistreated. Here, we present a case report of PHNEC in a 50-year-old woman who was admitted to our department with concealed pain in the right upper abdomen. The initial diagnosis was a probable hepatic space-occupying lesion with tumor bleeding. The patient was subjected to a partial right hemihepatectomy, cholecystectomy, partial resection of the lower lobe of the right lung, partial resection of the diaphragm, and resection of the right perirenal fat sac to alleviate her symptoms. After surgery, gene sequencing was performed to determine the possible cause of the condition. However, five months after discharge, the patient was hospitalized again because of retroperitoneal and peritoneal multiple metastases. Nine months after surgery, the patient died. This case is likely to aid in furthering our understanding of PHNEC to improve the future diagnosis and treatment of this disease. Most patients are treated for tumor growth and complications. In this case, the patient was diagnosed with a hepatic space-occupying lesion at admission and was later diagnosed with PHNEC via pathology after an operation.Neuroendocrine tumor (NET) refers to a tumor that originates from neuroendocrine cells  and is aA 50-year-old woman was admitted to the hospital on March 22, 2021, because of dull pain in the right upper abdomen for more than 1 month, and the pain was concentrated in the region of the liver for more than 10 days. Before admission, computed tomography (CT) scans acquired in another hospital revealed the presence of multiple space-occupying lesions in the right lobe of the liver. A physical examination was performed after admission. The abdomen was flat and soft with no tenderness or rebound tenderness over the entire region. There was a palpable area situated 8 cm below the liver costal, which was soft with no percussion pain in the liver area. Palpations were not observed under the spleen costal, her bowel movements were normal, bowel sounds normal, and there was no edema in both her lower extremities. Routine hospital tests were performed: C-reactive protein 35.37 mg/L. Liver function tests: Child A grade; ICG 15R = 1.7%, HBsAg (−). Carcinoembryonic antigen and alpha-fetoprotein were normal. Past surgical history: The patient had undergone a total isthmusectomy and a bilateral subtotal thyroidectomy in April 2019.A CT scan showed a cystic-solid mass (16.2 × 10 × 10.7 cm) on the right lobe of the liver, but no abnormalities were found in the intrahepatic and extrahepatic bile ducts and gall bladder. Furthermore, there was a malignant tumor on the right lobe of the liver, with ruptured hemorrhage and hepatic adenoma . MRI + MAfter general anesthesia, a reverse L-shaped incision was used. The abdominal cavity was explored, the gallbladder was removed, and intraoperative ultrasound and intraoperative fluorescence imaging devices were used to detect the number and location of the tumor. The tumor was located in the right lobe of the liver. The capsule of tumor was incomplete, the boundary was unclear, there was ischemic necrosis and bleeding in the center of tumor. The tumor invaded the diaphragm, the size was about 15 cm × 15 cm. An anterior approach hepatectomy was used, a vascular occlusion device was placed at the first porta hepatis, and the liver parenchyma was transected by harmonic ace and bipolar coagulation. The Glissonean pedicle was clipped and cut off, combined with the right caudate lobe resection, the right deltoid ligament, and the right coronary ligament was separated, right semihepatectomy was completed. Because the tumor invaded the diaphragm, and the diaphragm was partially excised 10 cm × 10 cm . Part ofThe operation time was 217 min, and the blood loss was 300 ml. No autologous blood was transfused during the operation. The postoperative CT scan confirmed that the tumor had been resected . The pat1. Neuroendocrine carcinoma of the right lobe of the liver  2. Right renal capsule fibro-fatty tissue infiltration/metastatic neuroendocrine carcinoma 3. Invasion of diaphragmatic striated surface connective tissue/metastatic neuroendocrine carcinoma . ImmunohGene sequencing was performed with a sample of the patient's tissue after the surgery. Totally, 14 gene variants were detected, among which 2 variants with potential clinical significance were in the CCND1 and TP53 genes.The patient returned to the hospital for re-examination a month after discharge. A CT scan showed the presence of multiple masses occupying the posterior and upper parts of the pancreatic head and the right abdominal and retroabdominal cavities, with a maximum size of 3.1 × 2.7 × 5.2 cm, which was indicative of metastases. A 5-month follow-up ultrasound after discharge showed a solid mass in the right renal first hepatic hilum areas and in the upper segment of the abdominal aorta, which was considered evidence of a malignant tumor. The mass had increased in size in comparison with the previous scan. The patient was readmitted to the hospital because of the presence of an abdominal mass. A CT examination showed that there were multiple metastases in the retroperitoneum and abdominal cavity, the largest being 9.0 × 8.5 cm, and a portal vein tumor thrombus had formed. After mass resection of the right hepatic lobe, the patient was found to have multiple metastases in the peritoneum and retroabdominal cavity. After admission, symptomatic and supportive treatments were given. After completing the relevant examinations, the patient was advised to obtain comprehensive treatment in the Medical Oncology Department, where she was evaluated for surgery and other possible regimens. The patient and her family were informed about the condition and the related risks; they expressed their understanding of the risks but asked to be discharged from the hospital. After 9 months of follow-up, the patient died.NET is a type of rare tumor that originates from the neuroendocrine cells. According to the World Health Organization classification of digestive system tumors in 2019 (5th edition), NETs can be distinguished into well-differentiated and poorly differentiated tumors (NECs) based on genetic evidence and differences in clinical, epidemiological, and histological features and prognosis. In addition, NET can be divided into G1, G2, and G3, which refer to low-, moderate-, and high-grade malignancy, respectively, based on its proliferative activity . NETs caPrevious case reports on PHNEC show that it is more common in middle-aged people and women –10. PHNEPHNEC is difficult to diagnose clinically. The main reason for this difficulty is that the clinical manifestations in the patients are nonspecific. The results from imaging techniques, such as ultrasound, CT, and MRI, can easily be confused with other types of liver tumors, although they are indicative of this disease. The imaging studies usually reveal hypoechoic, hyperechoic, or mixed-echo lesions, with rings around the tumors. Color echoes can be misdiagnosed as hemangioma based on the blood flow echo signals . In someCommon serological tumor markers, such as AFP, CEA, and CA 19–9, have shown no diagnostic significance for patients with PHNEC. However, when 17 patients with PHNEC were analyzed, it was found that CA 19–9 and ferritin possessed a certain diagnostic value for the disease . The diaSurgery is currently the primary treatment for PHNEC . Knox etThe concept of individualized tumor therapy has gradually emerged with advancements in medical science. In the past, individualized tumor therapy was narrowly referred to as targeted therapy. This concept has now been extended to the combination of multidisciplinary comprehensive treatment cooperation groups based on various factors, such as tumor stage and differentiation, performance status, and clinical course of the patients . PatientThe use of genetic sequencing was explored for this patient, and the genetic report suggested two meaningful mutations in the CCND1 and TP53 genes. CCND1 encodes a protein that transmits extracellular growth signals to the cell cycle by activating cyclin-dependent kinase (CDK)4 and CDK6, also called cyclin D1. The amplification and overexpression of CCND1 in human tumors lead to carcinogenicity. The CCND1 gene has been amplified and overexpressed in breast cancer tissue. The gene interacts with estrogen and hormone receptors in various forms to promote tumor growth. Its presence suggests that a patient is at a high risk for breast cancer. Studies on the relationship between CCND1 and genetic susceptibility to digestive system tumors are still in progress. TP53 encodes p53 protein, which is a tumor suppressor as well as a transcription factor that stimulates the cells to respond to carcinogens, such as DNA repair and apoptosis. TP53 is the most common mutant gene in human cancers, and its absence is closely related to the development of most cancers. Mutations in TP53 are mostly missense mutations that occur in the conserved regions of exons 5, 6, 7, and 8, and these are often seen in various hematological tumors and skin cancers. At present, gene mutations and the pathogenesis of NETs are being studied.According to the genetic variations seen in patients, the gene sequencing suggests that the targeted drug that may benefit patients is piperacillin and the recommended chemotherapeutic drugs are platinum and vinblastine. According to the NMPA/FDA guidelines, based on the type of cancer  that the patient currently suffers from, certain drugs such as sunitinib and everolimus are recommended. After discharge, the patient has been taking sunitinib for targeted drug therapy.According to the consensus guidelines for diagnosing NETs proposed by the North American Neuroendocrine Tumor Society, platinum-based drugs (including cisplatin and carboplatin) and etoposide, have shown good results. Hence, they are listed as standard drugs for PHNEC . It has In this case, the patient's clinical manifestations were not particularly indicative and there was no diagnosis of hepatitis or liver cirrhosis. Additionally, the tumor markers were normal, and the imaging results suggested the presence of solid liver space-occupying lesions. Therefore the possibility of hepatic NETs was considered. The diagnosis of patients with PHNEC needs to be supported more effectively with a tissue biopsy and immunohistochemistry. Surgical treatment would be the first choice, but a more comprehensive treatment is also a good choice for those without surgical indications or complex treatment methodology. The increased use of advanced gene detection technology should be encouraged in all patients to widen our knowledge of PHNEC. Therefore, in the future, a combination of gene sequencing and imaging and immunohistochemical techniques can aid in achieving a better diagnosis in patients with PHNEC. Such a diagnosis would, in turn, refine the therapeutic options available to the physician for managing the patients with this condition.


The imperforate hymen and transverse vaginal septum are female reproductive tract abnormalities. Their concurrent occurrence is rare. So far, no manifestation of both anomalies within the same family has been reported in the literature. We report the first familial case of two sisters having both mentioned abnormalities. Our patient was the youngest sister of a 14-year-old, who presented with cyclic lower abdominal pain. Clinical and imaging examinations showed an imperforate hymen with hematometrocolpos. In addition, the surgery confirmed a low transverse vaginal septum, which was resected using a virginity-preserving procedure. In conclusion, the presence of both mentioned abnormalities between siblings reflects a potential genetic etiology, also in order to avoid late diagnostics of such anomalies, the imperforate hymen must be screened in the birth room. Female reproductive tract anomalies are often discovered by the evaluation of menstrual complications or fertility disorders. These anomalies are common. They present an incidence rate of 2% to 3% . The incPatient information: a 14-year-old girl with no personal medical or surgical history presented with primary amenorrhea and abdominal pain. For family history, the oldest sister was diagnosed with an imperforate hymen with transverse vaginal septum four years ago.Clinical findings: during the examination, it was observed that the patient had tanner stage IV breast and pubic hair, as well as, normal-appearing labia majora and minora. Also, while examining the external genitalia, an absence of a hymenal opening without a bulge or bluish discoloration of hymen was revealed, as it can be seen in  was kept for virginity preservation. After the incision, no drainage of blood or fluid was noticed. This led to performing a vaginoscopy that confirmed a complete transverse vaginal septum at the lower third of the vagina. This vaginal septum was excised by a bipolar electrode introduced through the operative channel of the hysteroscope, and about 800 ml of brown menstrual efflux was drained. Realizing, the hysteroscopy confirmed a normal cervix, an ostia bilaterally, and no intrauterine defects. The final stage of the surgery consisted of suturing the hymen´s edges and keeping a Foley catheter in the vagina above the septum level inflated with a saline, that was kept for 15 days.Follow-up and outcome of interventions: the patient was followed for six months after the surgery. All along this period of time, we observed a complete resolution of symptoms as well as the appearance of regular cycles. Furthermore, the examination did not show stenosis of the hymen. Checking the medical records of the older sister showed that both sisters were subject to the same surgery and follow-up. In order to compare closely both cases, we summoned the older sister. Her check-up revealed that her hymen was still permeable.Patient perspective: the patient was satisfied with the diagnostic and the proposed care.Informed consent: patient gave his consent for his images and other clinical information to be reported in the journal.By referring to female embryology, the lower part of the vagina and the hymen have the same embryological derivations. They are both derived from the urogenital sinus . The faiTo sum up, our case demonstrates that the occurrence of both imperforate hymen and transverse vaginal septum can happen between siblings. This suggests the existence of a genetic etiology and most likely a recessive transmission. Routine postnatal screening of girls with a family history of these two abnormalities should be employed as a preventive measure against late diagnosis. Unless a large number of familial cases is studied, it remains difficult to prove surely the mode of transmission of these two abnormalities. Accordingly, further experience with a larger number of patients is necessary for analyzing genes and determining which one might be responsible for the manifestation of this phenomenon.


In patients with history of malignancy, new-onset liver lesions often present diagnostic challenges. We present the case of a patient with history of neuroendocrine tumor and new-onset echo-rich hepatic lesion, in whom attenuation imaging helped to make the diagnosis. Attenuation imaging is an ultrasound-based technique that allows for the quantification of hepatic fat content on the basis of a measurement of sound attenuation.We present the case of a 62-year-old Caucasian female patient who underwent pylorus-preserving pancreaticoduodenectomy Whipple surgery in 2004 for histologically well-differentiated neuroendocrine tumor with a proliferation rate of 3% of the pancreatic head. During the course, single liver metastases were resected in 2009, 2010, and 2013. In 2019, hemihepatectomy was performed when two liver metastases recurred. The liver metastases each showed a proliferation rate of 10% with vigorous expression of chromogranin A, synaptophysin, and somatostatin. The most recent follow-up examinations showed a normal chromogranin A value and the patient reported a good general condition. However, sonography revealed a blurred, echoic lesion in the liver. On contrast-enhanced sonography, the lesion showed identical behavior to the surrounding liver parenchyma. In the asymptomatic patient, liver biopsy did not seem to be indicated at the current time. Measurement of the attenuation coefficient by attenuation imaging showed a significantly higher measurement in the area of the echo-rich lesion than in the rest of the liver. The overall findings are consistent with focal fat deposition.Attenuation imaging appears to be useful in the evaluation of unclear echo-rich liver lesions. In particular, primary non-malignant-appearing liver lesions that are unremarkable on abdominal contrast-enhanced ultrasound can be more accurately assessed. New-onset hepatic lesions present a diagnostic challenge in patients with previously diagnosed neuroendocrine tumors. In particular, atypical lesions that are not primarily malignancy-suspicious on B-scan ultrasound, such as cystic or echo-rich changes, often remain unexplained without liver biopsy. Due to good availability and risk-free and cost-effective procedures as well as sufficient sensitivity for metastasis detection in the liver, transabdominal ultrasound is considered a suitable method to document marker lesions in neuroendocrine tumors (NET) during the course according to the national S2K guideline. To achieve an increase in accuracy, contrast-enhanced ultrasound can be performed. Attenuation imaging is a relatively new ultrasound-based technique that allows for conclusions to be drawn about hepatic fat content by measuring the attenuation coefficient. Attenuation imaging is currently used to diagnose diffuse fatty liver, but the measurement box can be freely placed by the examiner, which allows placement even on a (sufficiently large) focal lesion.In this report, we present the case of a 62-year-old Caucasian female patient who underwent pylorus-preserving pancreaticoduodenectomy Whipple (pp-Whipple) surgery in 2004 for histologically well-differentiated neuroendocrine tumor with a proliferation rate of 3% of the pancreatic head. Subsequently, multiple liver metastasis resections of segment VI/VII in 2009, an abscessed liver metastasis recurrence with diaphragmatic involvement in 2010, an atypical liver segment resection in segment VI in 2013, and finally a right hemihepatectomy in 2019 were required when two new liver metastases were discovered. Initial histology showed pancreatic neuroendocrine tumor (pNET) with a proliferation rate of 3%, and a proliferation rate of 10% with vigorous expression of chromogranin A, synaptophysin, and somatostatin was seen at each of the metastatic resections. The patient regularly presented for follow-up examinations since the initial diagnosis. Chromogranin A levels were normal since disease onset, and the patient most recently showed no B-symptomatology or symptoms of hormone-responsive neuroendocrine tumor and good exercise tolerance (ECOG 0).R2 > 0.90 was sought. The average AC is displayed directly after the measurement.Since pp-Whipple surgery, recurrent cholangitis occurred, which was well treatable with antibiotic therapy. For the first time, an echo-rich blurred lesion was detected in the liver 9 months ago. The lesion did not show increased vascularization and did not show signs of NET recurrence on B-mode ultrasound. At the time of the onset of the lesion, an elevated C-reactive protein (CRP) value of 95 mg/l was detectable. Therefore, antibiotic therapy was initiated if cholangitis was suspected, and cholestasis values were also slightly elevated. In the short-term control, the CRP value was again completely normal, however, the hepatic lesion progressed sonographically in size, so that a correlate of the cholangitis undergone was not assumed. Subjectively, the patient showed wellbeing. Contrast-enhanced sonography was performed for further clarification of the lesion. Here, the echo-rich lesion behaved like the surrounding liver tissue. No perfusion defects could be visualized on contrast-enhanced ultrasonography. Another possible hypothesis discussed was local iron accumulation or focal/zonal fat deposition. For medical and ethical reasons as well as the long and sometimes complicated postoperative severe disease course, the patient did not consent to a new invasive liver biopsy. For further clarification, attenuation imaging, which provides a quantitative measurement of the fat content of the liver, was performed. Attenuation imaging (ATI) is integrated into the Aplio i800 device from Canon Medical Systems and can be performed with the conventional i8CX1 transducer. ATI quantifies the sound attenuation of the ultrasound signal and takes advantage of the fact that ultrasound waves are attenuated by the tissue properties of the liver parenchyma with increasing penetration depth . The attHere, it was found that in the area of the echo-rich altered liver, significantly increased values of 0.75 dB/cm/MHz could be measured. In areas of the B-Mode sonographically normal liver, the values were in the normal range with an average of 0.63 dB/cm/MHz. This demonstrates that the method is able to quantify and detect zonal hyperlipidemia in unclear liver findings.At the current time, there is no known published comparable case in which attenuation imaging has been studied in patients with focal or zonal fat deposition.Previous studies have shown a statistically significant correlation between histologically diagnosed steatosis content and the attenuation coefficient , 4, 6, 7et al. were able to show in a small patient collective that at an AC of > 0.745 dB/cm/MHz a high-grade steatosis with > 66% fatty hepatocytes can be detected with a sensitivity of 100% and a specificity of 82.4%. The area under the curve was 0.923 [et al. were able to detect fatty degeneration of > 66% of hepatocytes with a sensitivity of 100% and a specificity of 71.4% in n = 38 patients with non-alcoholic fatty liver disease (NAFLD) and a cut-off value of 0.74 dB/cm/MHz [If these values are placed in the context of previously published measurements, the impression arises that there is probably a high degree of fatty degeneration of the liver in the echo-rich zone. Bae et al. chose an AC of > 0.635 dB/cm/MHz as a cut-off value for S1 steatosis (> 5% fatty hepatocytes), with a sensitivity of 74.5% and a specificity of 77.4%. Furthermore, a high negative predictive value for the detection of clinically significant hepatic steatosis of more than 5% and more than 10%, respectively, was demonstrated. The degree of fibrosis and inflammation did not correlate with AC [et al. the mean attenuation coefficient in patients without steatosis hepatis in histopathological findings was 0.63 dB/cm/MHz [In that patient, a mean attenuation coefficient of 0.63 dB/cm/MHz could be measured in the area of the remaining liver. Bae r = 0.91 to r = 0.98, suggest good intra- and interindividual reproducibility and, with r = 0.81, a high correlation of the results of ATI with the results of a magnetic resonance imaging proton density fat fraction (MRI-PDFF) examination as well [Previous results of attenuation imaging, with correlation coefficients ranging from Focal fat deposition is rarer than focal fatty sparing. The cause of fat deposition is not yet fully understood; various hypotheses are discussed, for example, increased storage of fat due to relative ischemia caused by reduced portal vein flow. Increased insulin levels in portal venous blood are also discussed. Focal fat deposition is often localized subcapsular. The localization of focal fat deposition and focal fatty sparing is similar, as both changes are presumably due to portal venous flow .As a limitation, it must be mentioned that the presented case report does not allow for any conclusions about the assessment of the technique. Further prospective pilot studies are necessary to answer these questions.This case report demonstrates that attenuation imaging may help in the evaluation of echo-rich zonal changes in the liver. In particular, in cases where liver biopsy appears to be ethically unacceptable, attenuation imaging could help to establish the diagnosis.


Autoimmune gastritis is a chronic immune-mediated disorder characterized by varied clinical manifestations and that should be endoscopically managed over time, as the gastric atrophy contributes to microenvironmental alterations of the stomach milieu, and an increased cancer risk has been linked to this condition. Here, we report the unusual case of a woman who developed a cardiac high-grade pyloric adenoma in a context of previously undiagnosed autoimmune gastritis with synchronous neuroendocrine cell hyperplastic and dysplastic lesions. Autoimmune gastritis (AIG) is an immune-mediated, chronic, organ-specific disorder characterized by the destruction of oxyntic mucosa, mediated by anti-parietal cell antibodies (PCA) which recognize the subunits of the H+/K+ ATPase, a proton pump that can be found in the parietal cells . The netThe neuroendocrine lineage is not the only one interested by the pro-carcinogenic processes in the context of AIG. In fact, the glandular atrophy that characterizes this autoimmune process is accompanied by metaplastic changes of the oxyntic mucosa with its progressive “antralization” and the development of pseudo-pyloric and intestinal metaplasia. These atrophic and metaplastic changes are associated with an increased risk of development of pre-invasive and invasive neoplasms of epithelial-glandular origin, even if the cell of origin has not been recognized with certainty . Other fPyloric gland adenomas (PGA) are rare mass-forming neoplasms composed of closely packed pyloric glands, lined by columnar or cuboidal cells with pale or eosinophilic cytoplasm and round nuclei without prominent nucleoli, showing a characteristic immunoreactivity for MUC5AC and MUC6, respectively in the superficial and deep glandular components –11. AlthIn this paper we report the case of a 79-year-old woman who underwent a partial gastric resection for a pyloric gland adenoma which arose in an unusual site (cardia) on a background of undiagnosed AIG and was associated with neuroendocrine cell hyperplasia and dysplasia in the adjacent fundic mucosa.The patient has been suffering from essential hypertension since roughly the age of 50, and she has been on an angiotensin converting enzyme inhibitor since then. Also, she has been suffering from autoimmune thyroid disease, namely Hashimoto's thyroiditis. The patient has a first-degree family history of various cancer types, including breast cancer (mother), endometrial cancer (daughter), and lung cancer (father). She has never smoked, nor is she a drinker.Since August 2021, the patient has been complaining of mild and transitory dyspepsia that did not cause nocturnal awakening. These symptoms were considered as non-troublesome and did not prompt an immediate medical evaluation. However, she progressively lost 6 kg and, in late September, dysphagia for both solid food and liquids occurred, with nausea and a few vomiting episodes. For these reasons, an upper gastrointestinal endoscopy was performed, showing, in stomach retroflection, a polypoid sessile, though irregular, lesion (Paris classification 0-Is) of roughly 3 cm, easily bleeding, with a inhomogeneous mucosal pattern, in the fundus of the stomach, close to the cardia. The lesion was also observed with narrow band imaging, showing a deeply altered mucosa, with an irregular pattern, architectural distortion, and irregular vascularization. Consistently, biopsies from the lesion showed high-grade epithelial dysplasia. At this first evaluation, esophageal biopsies were not taken, as the cause of dysphagia was clearly attributed to the lesion found in the cardia region. Additionally, biopsies from normal-appearing gastric mucosa were not taken due to the bleeding secondary to the biopsy sampling of the lesion. Given the endoscopic suspicion of gastric cancer, a multidetector CT scan was prescribed, showing a 3-cm polypoid mass in the stomach fundus, involving the cardia area . No pathFollowing the imaging studies, the patient underwent a proximal gastrectomy, considering that an endoscopic approach was contraindicated due to both the localization of the lesion and the staging. Upon gross examination, the gastric mucosa showed a polypoid, mass-forming 3-cm lesion, centered on the cardia, just below the gastro-esophageal junction. The lesion was composed of closely packed pyloric-like glands, mainly lined by columnar cells with round nuclei and inconspicuous nucleoli, and glassy-to-eosinophilic cytoplasms . Focal avia a process called “paligenosis”   , 25. WheA peculiar feature of the case here described is the PGA location in gastric cardia, which might appear as an unusual site for PGA development. However, it should be recalled that (i) in AIG, anatomic cardia may be involved by the same pseudopyloric and/or intestinal metaplastic mucosal changes of gastric corpus-fundus, as seen in the present case, and (ii) PGAs, despite their name, exhibit phenotypical and immunophenotypical features more similar to mucous neck cell lineage of oxyntic glands rather than to pyloric gland cells . Indeed,Another relevant histologic lesion found in the specimen was the so-called “ECL cell dysplasia”, a poorly known form of neuroendocrine cell proliferation in the oxyntic mucosa, falling short of the diagnosis of gastric NET , 26. ECLTo conclude, we have herein reported an unusual case of two different-and concurrent-gastric cancers involving an atypical gastric region  in a patient with AIG. It is known that AIG is burdened by a substantial diagnostic delay due to its proteiform clinical manifestations , especiaThe raw data supporting the conclusions of this article will be made available by the authors, without undue reservation.The studies involving human participants were reviewed and approved by San Matteo Hospital Foundation. The patients/participants provided their written informed consent to participate in this study. Written informed consent was obtained from the individual(s) for the publication of any potentially identifiable images or data included in this article.CG, ML, and CR conceived and designed the case report, contributed to the clinical and pathologic diagnosis, summarized the case, reviewed the literature, and drafted the manuscript. AP contributed to clinical diagnosis and surgical treatment and provided input in the preparation of the manuscript. AG contributed to radiologic diagnosis and provided the radiologic image. GA contributed to pathology diagnosis and immunohistochemistry. AD and AV supervised the conception, analysis, and manuscript drafting. All authors critically revised the paper for important intellectual content, provided approval of the final version and agreed to be accountable for all aspects of the work, ensuring that questions related to the accuracy or integrity of the work are appropriately investigated and resolved.This work was supported by a Grant of the Italian Ministry of Education, University and Research (MIUR) to the Department of Molecular Medicine of the University of Pavia under the initiative Dipartimenti di Eccellenza (2018–2022).The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.All claims expressed in this article are solely those of the authors and do not necessarily represent those of their affiliated organizations, or those of the publisher, the editors and the reviewers. Any product that may be evaluated in this article, or claim that may be made by its manufacturer, is not guaranteed or endorsed by the publisher.


Undifferentiated carcinoma is a very rare histologic subtype, representing only 0.8% to 5.7% of all pancreatic exocrine neoplasms. Additionally, spontaneous abdominal hemorrhage is a particularly rare, life-threatening cause.A 68-year-old man was taken by ambulance to our hospital because of sudden-onset abdominal pain. Contrast-enhanced abdominal computed tomography revealed a huge mass measuring 99 × 70 mm in the pancreatic tail with enhanced rim staining in the peripheral area. Imaging also showed extravasation and fluid collection beside the tumor. Hence, spontaneous rupture of the pancreatic tumor and intra-abdominal bleeding were diagnosed. Emergency laparotomy was performed because of acute abdominal pain with peritoneal signs. With an intraoperative diagnosis of rupture of the pancreatic tumor, distal pancreatectomy was successfully performed. Histologically, hematoxylin and eosin staining showed round to spindle-shaped, highly pleomorphic mononuclear cells and multinucleated giant cells as well as a component of ductal adenocarcinoma. Immunohistochemical staining showed that the tumor cells were negative for AE1/AE3, whereas the non-neoplastic osteoclast-like giant cells were positive for CD68. Taken together, these results led to a diagnosis of undifferentiated carcinoma with osteoclast-like giant cells. The patient’s postoperative course was uneventful.We experienced an extremely rare case of spontaneous rupture of an undifferentiated carcinoma with osteoclast-like giant cells presenting as intra-abdominal bleeding. Obtaining a correct preoperative diagnosis is quite difficult at the first evaluation. Undifferentiated carcinoma should be considered as a differential diagnosis in the case with spontaneous rupture of a pancreatic tumor.The online version contains supplementary material available at 10.1186/s40792-022-01437-2. Undifferentiated carcinoma of the pancreas (UCP) is an extremely rare histologic subtype of pancreatic cancer that accounts for 0.8% to 5.7% of all pancreatic exocrine neoplasms –3. UCP iA 68-year-old man with a history of hypertension, diabetes mellitus, and myocardial infarction was taken by ambulance to our tertiary referral hospital’s emergency department because of sudden-onset left upper quadrant pain of 1-h duration accompanied by nausea and sweating. The patient had a treatment history of percutaneous coronary intervention and placement of three stents in his coronary arteries, and he was taking two kinds of anticoagulant drugs, 100 mg of aspirin and 75 mg of thienopiridines.d-dimers (2.9 µg/mL), and hemoglobin A1c (9.0%); his myocardial deviation enzymes were not elevated  and increased concentrations of aspartate aminotransferase (53 U/L), lactate dehydrogenase (492 U/L), Macroscopic examination of the resected specimen revealed that the ruptured tumor was located in the pancreatic tail. It was approximately 80 mm in diameter, multilocular-cystic, and well-defined; hemorrhage was present, and the tumor had a septum Fig. a, b. HisAlthough there are many potential causes of acute abdomen, spontaneous abdominal hemorrhage  is a particularly rare, life-threatening cause . VisceraUCP is an extremely rare tumor. In the Pancreatic Cancer Registry in Japan, in which 28,655 cases of pancreatic neoplasms were registered from 1981 to 2004, UCP represents only 0.1% of all pancreatic exocrine neoplasms . The medCECT is reportedly essential for prompt diagnosis of intra-abdominal bleeding . We alsoIn the resected specimens of our case, the tumor was rubbery and fleshy and exhibited cyst formation, hemorrhage, extensive necrosis, and gross infiltration into the adipose tissue. These findings are consistent with the above-mentioned report of a large series of UCP and were presumably due to rapid tumor growth, intra-tumoral hemorrhage, necrosis, and subsequent cystic degeneration .Because of the extremely low incidence of UCP, survival data are controversial and the survival benefit of surgery for UCP remains uncertain. No therapeutic strategies for UCP have been established. A previous report of 35 patients with UCP demonstrated that the 1-, 2-, and 5-year overall survival rates were 59.1%, 30.7%, and 12.2%, respectively, which are comparable with those of pancreatic ductal adenocarcinoma  as a varWe have herein reported an extremely rare case of spontaneous rupture of an undifferentiated UCP presenting as intra-abdominal bleeding. Obtaining a correct preoperative diagnosis was quite difficult at the first evaluation. As indicated by our case, UCP with osteoclast-like giant cells should be considered as a differential diagnosis of pancreatic tumors.Additional file 1: Table S1. The reported cases of spontaneous rupture of the pancreas.


Chordomas are rare malignant bone neoplasms that are presumed to arise from chordal remnants in the fetal stage and typically occur along the axial skeleton. The extra-skeletal chordomas reported to date include soft tissue of the extremities and nasopharynx. Chordoma arising from the gastrointestinal wall has not been previously described.We report on a 42-year-old man with primary chordoma presenting as a gastroduodenal submucosal tumor centered on the pyloric ring. The patient was consistently asymptomatic, and the tumor was an incidental finding. However, during a follow-up at approximately 1.6 years, an increase in tumor size was identified on computed tomography (CT), and surgical resection was performed without a definite pathologic diagnosis. The patient was successfully treated with distal gastrectomy, and the histological diagnosis was a conventional chordoma. The diagnosis was confirmed via immunohistochemical staining for brachyury, pan-cytokeratin, S-100, and SOX9. Postoperative CT and magnetic resonance imaging revealed no recurrence or metastasis during the 1.5-year follow-up period.Primary chordomas of the digestive tract are rare. Embryologic development of the notochord does not explain the existence of remnants in the gastrointestinal wall. Moreover, notochordal remnants, as precursors of chordoma, were not identified in the current case. The gastroduodenal chordoma may not have originated from embryonic notochordal remnants but through aberrant brachyury activation without a notochordal precursor.We report the first case of primary gastrointestinal chordoma in humans. The tumor was completely removed surgically, without postoperative recurrence. •This is a case of primary gastrointestinal submucosal chordoma in a 42-year-old man•Chordoma is a rare bony tumor that most frequently affects the axial skeleton•Extra-skeletal chordomas are extremely rare in any species•This is the first report of primary chordoma of the digestive tract in humans These usually arise from the axial skeleton of the sacrum, skull base, or mobile spine 2A 42-year-old man was referred to our institution for a pyloric ring submucosal tumor. The patient was asymptomatic and underwent upper gastrointestinal endoscopy as part of a regular check-up. A submucosal tumor lesion was incidentally detected on the posterior wall just before the pyloric ring of the stomach to the duodenal bulb a. ContraGrossly, there was a relatively well-demarcated, solid lesion a located3Chordomas were first described by Virchow in 1857 as tumors with a histological appearance of pleomorphic cells with dark nuclei and vacuolated or granular cytoplasm In the current case, microcystic/reticular schwannoma was the differential diagnosis based on hematoxylin–eosin (H&E) staining characteristics and S100 positivity Chordomas are believed to arise from the vestigial notochordal tissue because they almost always arise in the axial skeleton and resemble the normal notochord Locoregional recurrence, rather than distant metastasis, is a common event following complete resection and represents a major clinical challenge Some limitations of the current report should be mentioned. First, given that a long-term follow-up period of around 5 years is recommended, the 1.5-year follow-up period, in this case, is relatively short to conclude that there is no recurrence. Second, we did not perform comprehensive genomic profiling to detect somatic variants or chromosomal rearrangements specific in the tumor cells. Extended molecular profiling may yield insight into how gastrointestinal chordomas differ from common axial–skeletal chordomas in terms of drug targeting and disease entity classification.4In this report, we describe the first documented case of chordoma arising within the gastrointestinal wall, which is a novel site of development for this rare neoplasm. The tumor was completely removed surgically without postoperative recurrence within the 1.5-year follow-up period. No chemotherapy or radiation therapy was administered. Analysis of H&E characteristics and positive staining properties, including those of brachyury and SOX9, is essential for an accurate diagnosis. Although it is difficult to determine whether the tumor, in this case, arised from the notochord, no notochordal remnants were identified in the location of the tumor. Owing to their easy resection with sufficient margins, gastrointestinal chordomas may have the advantage of avoiding recurrence.Not commissioned, externally peer-reviewed.Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal on request.This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors.This study was exempted from ethical approval.Ryotaro Hashizume: Board-certified pathologist who made the diagnosis, and was involved in the conceptualization, methodology, investigation, formal analysis, and writing of the original draft.Shinsuke Matsuda: Board-certified surgeon performed the operation, methodology, investigation, and formal analysis.Moritaka Nagai: Involved in the methodology and investigation.Kazuki Hirata: Clinical laboratory technician who prepared tissue specimens and helped with the investigation.Hiroshi Imai: Board-certified pathologist who made the diagnosis, conceptualization, methodology, investigation, and formal analysis.Ryoji Kushima: Board-certified pathologist who made the diagnosis, methodology, investigation, formal analysis, and reviewed and edited the manuscript.This study was not a first in man study.Ryotaro Hashizume, MD, PhD.Ryotaro Hashizume accepts full responsibility for the work and/or the conduct of the study article, providing full access to data, and controlling the decisions about the publishing of the paper.The authors have no conflicts of interest to disclose.


Encountering an obturator hernia itself is a rare entity for practicing surgeons globally. Synonymously known as the "old lady's hernia" is usually seen in fragile geriatric multiparous female patients. We share our experience of this rare entity where the patient presented with features of small bowel obstruction. Her diagnosis was delayed in the previously attended other centre as the possibility of an obturator hernia was overlooked. A timely clinical examination of the patient complemented by a computed tomography scan of the abdomen and pelvis helped us to obtain a diagnosis and proceed with immediate surgery. Intraoperatively the rarity was present bilaterally although the obstruction was on one side only. Prompt diagnosis and treatment benefits patients in this potentially lethal condition. However, her symptoms persisted and she presented at the emergency of Nepal Mediciti Hospital with complaints of increasing abdominal pain associated with feculent vomiting, progressively increasing abdominal distension and obstipation. She also gave a history of chronic cough with heavy biofuel exposure. She had a kyphotic posture but she was unaware of the cause of her kyphosis. She didn't have a significant medical family or psycho-social history.She had no previous history of abdominal surgery.The patient weighed 38 kg. She was tachycardiac, hypotensive, slightly short of breath and dehydrated. Her abdomen was grossly distended with diffuse tenderness and hyperactive bowel sounds were heard on auscultation. She didn't have groin swelling and per rectal examination was unremarkable. The patient was resuscitated with two litres of lactated ringer solution and her urine output was monitored with a Foley catheter. A nasogastric tube was inserted which yielded feculent effluent. Her lab reports showed leukocytosis with neutrophilia and elevated urea levels. Other blood parameters were normal. An urgent contrast-enhanced computed tomography (CECT) scan of the abdomen and pelvis was done which showed a rightsided obturator hernia containing a small bowel loop lying between the pectineus and obturator muscles with thickened wall and narrowed lumen causing bowel obstruction .As the patient was discharged from another centre with oral medication, trying to convince them for the surgery was really challenging. The patient party was reluctant to undergo surgery and after a thorough counselling informed consent was taken from the patient's family and an urgent midline laparotomy was done. Our expert team of anaesthesiologists placed an epidural catheter despite her kyphotic posture which played an important role in the non-turbulent pain-free postoperative period .The low midline laparotomy revealed that a nonischemic ileal loop had herniated in the right obturator canal causing upstream dilatation of small bowel loops. Along with that she also had an obturator hernia with an empty sac on the left side .The herniated viable ileal loop in the hernia was reduced into the peritoneal cavity and both the hernial defects were closed bilaterally with a mesh plug .th postoperative day. At the time of discharge, she was tolerating oral feeding and was comfortable with oral analgesics. She followed up in the outpatient clinic a week later, where her recovery was found to be satisfactory.She was kept in the intensive care unit for 2 days and oral feeding was started on the second postoperative day. Her leukocyte count and renal function returned to normal range on the second postoperative day. Post-operative period was uneventful. She came out unscathed and was discharged on the 62 This is the only case encountered by our team since the inception of the hospital in 2017. This also shows the rarity of OH in our population. A study was published on a decade-long review of the emergency presentation of OH in Sydney, Australia which encountered just eighteen cases with a mortality rate of 27.8%. The women with a broader pelvis, greater transverse diameter and high laxity of pelvic floor secondary to their parity are more preponderant (about 6 to 9 times more than men) to develop this rare entity.4 It is less common on the left side due to the protection offered by the sigmoid colon to the obturator canal. However OH can be bilateral in 20% of the cases.5Obturator hernia (OH) is a rare condition encountered by a surgeon and accounts for less than 1% of all intraabdominal hernias.6 Adhering to these findings in the literature our patient was an elderly, emaciated, multiparous female with a symptomatic OH on the right side and occult OH on the left.Depending upon the type of surgery opted by the surgeon the rate of detection of occult contralateral hernia has been reported from 6% to 63%.7 This is an important factor to be considered in the history of the patient to suspect the possibility of OH. The Howship-Romberg sign  or Hannington - Kiff sign (loss of adductor reflex) is considered pathognomonic of irritation of the obturator nerve by the hernia but is rarely elicited in the emergency as the patient is usually sick.8Clinical diagnosis of OH is quite challenging due to the vague symptoms at presentation. In most cases, patients present with non-specific aching or dull abdominal pain associated with nausea and vomiting. Up to 80% of patients with OH usually have symptoms of intermittent partial bowel obstruction due to a high proportion exhibiting Richter's herniation of the bowel into the obturator canal.10 We also opted for the immediate CT scan which aided in prompt diagnosis and appropriate treatment within no time. Various techniques have been advocated for the reduction of OH and repair of defects which may be intraperitoneal or extra peritoneal approaches along with or without mesh hernioplasty. Intraperitoneal approach includes open laparotomy or laparoscopic transabdominal preperitoneal repair. Extraperitoneal approach consists of either trans - inguinal approach or laparoscopic totally extraperitoneal approach.6 The intraperitoneal approach has the advantage of evaluating the bowel and treating a possible ischemic bowel with resection, especially in emergency cases. While the extraperitoneal approach is better for elective cases but the viability of herniated bowel has to be proven preoperatively.6 We opted for the intraperitoneal approach with a low midline laparotomy as our patient presented in the emergency with complete obstruction and a CT scan revealed that the bowel loops were grossly dilated with the possibility of bowel strangulation. We have planned to offer the laparoscopic extraperitoneal approach to the patients whenever we see them again as we have been routinely doing laparoscopic extraperitoneal surgeries for elective groin hernias.Early diagnosis of OH can be made with a computed tomography scan of the abdomen and pelvis and images showing bowel herniating through the obturator foramen and lying between the pectinus and obturator muscles. This has been shown to be the best diagnostic clue.To conclude, although obturator hernia is a rare entity it should always be thought of especially in the emaciated elderly lady without prior abdominal surgery who presents with features of intestinal obstruction. Detailed history and physical examination combined with an early CT scan could be very helpful to establish the preoperative diagnosis. Early diagnosis and prompt surgical treatment are the keys to reduce morbidity and mortality associated with this condition.