Contained electromechanical morcellation has emerged as a safety approach for laparoscopic myomatous tissue retrieval. This retrospective single-center analysis evaluated the bag deployment practicability and safety of electromechanical in-bag morcellation when used for big surgical benign specimens. The main age of patients was 39.3 years (range 21 to 71); 804 myomectomies, 242 supracervical hysterectomies, 73 total hysterectomies, and 1 retroperitoneal tumor extirpation were performed. A total of 78.7% of specimens weighed more than 250 g (n = 881) and 9% more than 1000 g. The largest specimens, weighing 2933 g, 3183 g, and 4780 g, required two bags for complete morcellation. Neither difficulties nor complications related to bag manipulation were recorded. Small bag puncture was detected in two cases, but peritoneal washing cytology was free of debris. One retroperitoneal angioleiomyomatosis and three malignancies were detected in histology ; therefore, patients underwent radical surgery. All patients were disease-free at 3 years follow-up, but one patient presented multiple abdominal metastases of the leiomyosarcoma in the third year; she refused subsequent surgery and was lost from follow-up. This large series demonstrates that laparoscopic bag morcellation is a safe and comfortable method to remove large and giant uterine tumors. Bag manipulation takes only a few minutes, and perforations rarely occur and are easy to detect intraoperatively. This technique did not result in the spread of debris during myoma surgery, potentially avoiding the additional risk of parasitic fibroma or peritoneal sarcoma. For almost 30 years, endoscopic surgery has been positioned as the standard approach for the management of gynecological tumors in many countries ,2,3,4,5,Due to their recent introduction, it is necessary to evaluate the feasibility, safety, and efficacy of these systems to avoid surgical tissue spreading, as well as to set out guidelines for proper patient and morcellation technique selection . TherefoRetrospective single-center analysis of 1120 patients who underwent laparoscopic uterine surgery and electromechanical IBM for presumed benign pathology, between 2 July 2014 and 14 December 2021, at the Department of Obstetrics and Gynecology, Minimal-Access Surgery and Oncology, Dubai London Clinic and Speciality Hospital, Dubai, UAE. Criteria for inclusion were consented cases in which laparoscopic myomectomy or hysterectomy with in-bag morcellation was performed for presumably benign myomatous pathology. Cases with uterine diseases other than LM or treated outside the proposed time range were excluded. Here we present the results related to type of surgery, bag deployment practicability, intraoperative complications, bag puncture, final peritoneal lavage cytological analysis, and specimen weight and histology. Regarding bag deployment practicability, the following steps during bag handling were described: (a) bag introduction, (b) bag straightening, (c) achieving clear vision, (d) appropriate insufflation and adherence to the port size and sealing, (e) specimen introduction, (f) morcellation, (g) extraction of blood, smaller tissue or fluid debris, and (h) bag extraction.For the analysis, descriptive statistics were used. The bags consist of thermoplastic polyurethane, which is a medical-grade polymer widely used in the medical industry  , and areA total of 1120 patients underwent laparoscopic uterine surgery and electromechanical IBM for presumed benign pathology. The average age of the patients was 39.3 years, ranging between 21 and 71 years, with weight from 41.0 Kg to 127.6 Kg (median 65.5 Kg). In addition, 50.2% were nulliparous, and 71.78% of all cases underwent myomectomy. In total, 71.24% of surgical specimens weighed between 250 g and 999 g, and 7.23% were larger than 1000 g .It was found that, prior to the operation, all patients signed an informed consent for the use of the electromechanical morcellation system MorSafe  as well for the use of surgical bags made of thermoplastic polyurethane. In accordance with their presurgical diagnosis and patient desire, patients underwent hysterectomy or myomectomy. Perioperative intravenous antibiotic prophylaxis  was used in all cases.Regarding the technique used for a big specimen, Ten cases of parasitic myomata were found in patients who had undergone previously uncontained electronic morcellation during laparoscopic myomectomy or hysterectomy a. One reHistological examination revealed the presence of fibroids in most of the cases (78%) . This waThe first case was a 33-year-old nulliparous woman who presented with severe menorrhagia and secondary anemia because of a degenerated 6–7 cm myoma (FIGO type 1–2). She had given a history of a myomectomy in 2011, performed by laparotomy in another country; thus, the details and definitive histology of the resected myoma were preoperatively unknown. For the new clinical situation, the patient underwent laparoscopic myomectomy and in-bag extraction through the umbilicus. Due to high tissue fragility, probably because of the degenerative nature of the myoma, mechanical morcellation was not necessary. Frozen biopsy and definitive histology reported an atypical myoma with bizarre nuclei. One year later, the patient complained of pelvic pain. At this time, the patient brought a copy of the surgical and histological report of the first myomectomy that showed leiomyosarcoma. However, she had not received instructions on close follow-up. The new ultrasonographic and pelvic MRI examination revealed a large mass in the left adnexal area with solid and cystic components. The PET showed no evidence of metastatic disease. Therefore, in accordance with our tumor board recommendations, a total abdominal hysterectomy, left salpingo-oophorectomy, and right and left iliac obturator lymphadenectomy were performed. The 259 g tumor was reported as uterine leiomyosarcoma with negative margins, stage 1b (TIbNOMO). The patient moved to London and continued her treatment there. A few years later, she returned to Dubai and presented with a relapse with multiperitoneal lesions, for which she was referred to a cancer center to continue her treatment.The second case was a 46-year-old nulliparous woman who presented with a large fundal myoma, severe secondary dysmenorrhea, and anemia. Preoperative imaging tests and endometrial biopsy were negative for malignancy. The patient underwent in-bag laparoscopic myomectomy, and due to tissue fragility, the manually morcellated surgical specimen was removed through a suprapubic minilaparotomy. A total of 3183 g of myoma tissue was obtained and reported as leiomyosarcoma. The patient did not consent to the recommended radical hysterectomy and lymphadenectomy and was lost to follow-up.The third case was a 58-year-old parous woman with a history of pelvic pain and postmenopausal bleeding due to myomatosis uteri. No signs of malignancy were present at the preoperative ultrasound examination, PAP cytology, and endometrial biopsy. During the LASH, the frozen biopsy revealed a grade 1 endometrial stromal adenocarcinoma with >50% invasion of myometrium; the tubes and ovaries were reported normal. In addition, enlarged para-aortic lymph nodes were found. Consequently, a radical hysterectomy, bilateral salpingo-oophorectomy, partial vaginectomy, para-aortal lymphadenectomy, and bilateral parametria excision were performed. Postoperatively, six cycles of adjuvant chemotherapy with paclitaxel and carboplatin were given. Follow-up examinations showed the absence of cancer recurrence over 5 years.Regarding the angioleyomiomatosis case, sections of the soft tissue consisted of cellular sheets and irregular fascicles spindled cells with the formation of glomeruloid architecture and epithelioid features. The lesion demonstrated numerous anastomosing structures resembling blood vessels covered by endothelium-like cells embedded in fibrous stroma. There was no evidence of malignancy or tumor necrosis. The tumor was positive for CD31, CD34, D2-40, CD68, Ki-67 (1% to 2% positive nuclear staining) immunomarkers as well for the smooth muscle markers SMA and desmine. The markers HHV-8, S100, synaptophysin, and calretinin were negative. The cytology of peritoneal fluid showed mild subacute inflammation with activated mesothelial cells suggestive of exudative peritoneal effusion . The perRegarding the assessment of the “practicability of in-bag morcellation”, the bags were selected according to the volume of the specimen size in all cases. The mean intraoperative time necessary for the introduction of the endobag was 0:16:52 min, and the mean morcellation time 0:22:51 min . Only inThe large bags enabled the trouble-free morcellation of big species up to 2800 g . TransfeTwo bag punctures occurred. One case was by direct contact with the morcellator blade at the edge of the bag, near the port outlet, which allowed surgeons to exteriorize the section immediately, avoiding any spillage. The other was a microrupture that did not lead to a loss of gas from the bag and was adverted postoperatively at the insufflation and immersion test. This test was routine at the end of each procedure performed . There wAll patients, including those with benign and rare cellular types, had regular annual follow-ups, the longest period being 8 years. No myoma recurrence was observed. One patient with leiomyosarcoma was free of disease at the 5-year follow-up, and the other did not present again at follow-up. There were no hernias at the port sites.Leiomyomata are monoclonal benign tumors that arise from myometrial cells with complex pathobiological origins involving genetic, epigenetic, hormonal, environmental, proinflammatory, angiogenetic, and growth factors . UterineAt our clinic, prior to 2014, morcellation was always performed without a bag, and here we presented the retrospective analysis of 1120 cases of big and giant uterine myomatosis performed between 2014 and 2021 to describe the feasibility and safety of laparoscopic contained morcellation for the retrieval of large surgical specimens from the abdominal cavity. According to our experience, advancements in minimally invasive surgical techniques and tools, such as ultrasonic dissecting devices and in-bag morcellation (IBM), allow women with large fibroids or uteri to undergo safe procedures .After laparoscopic dissection, the surgical specimen (myomata or uteri) could be retrieved from the abdominal cavity through minilaparotomy through the vagina or by means of morcellation (uncontained or in-bag). The latter refers to reducing the surgical specimen into small fragments with a scalpel  or with an electronic power morcellator . In 1973, Kurt Semm introduced the electromechanical power morcellation technique, which was approved by the FDA in 1995 [Nevertheless, uterine sarcomas are rare, and the incidence rate differs across countries, being 0.36/100,000 women in the United States, 0.4/100,000 women across North European countries, and 1.32/100,000 women in Germany ,26. RapiFollowing myoma surgery, a very low rate of unexpected malignancy has been reported. In 2013, Theben et al. found an unexpected malignancy rate  of 0.25% in 1584 patients who underwent LASH . In 2014Regarding sarcoma prognosis, tumor injury during excision and uncontained morcellation, electric or manual, in both laparotomy and laparoscopy ,31,32, pFurther studies have shown that the risk of benign diseases following laparoscopic morcellation is higher than for sarcoma. Tulandi et al.  reviewedThe concern for occult cancer risk and parasitic myomata may compromise the benefits of minimal-access surgery for uterine leiomyoma. However, the standard use of containment systems, as advised by the FDA in 2020, could help to reduce the risk of dissemination by electromechanical morcellation. Recent studies have shown high technical success and a short learning curve in the technique of in-bag morcellation ,45,46,47p = 0.02), which did not vary significantly by the surgeon. There were no differences regarding specimen weight, complication rate, estimated blood loss, or hospital length of stay between the groups (85 cases vs. 49 controls).Our findings are similar to other studies showing that contained morcellation is a feasible and safe method to retrieve large benign surgical specimens and for suspected cases of cancer. There were no intraoperative complications or postsurgical hernias at the port sites ,47,48,49p < 0.001), which was attributed to the higher mean uterine size . According to our experience, in minimally invasive centers, the learning curve to acquire skills in the bag unwrapping technique, as well as the manipulation and morcellation of large tumors, is short, helping to reduce the excess time related to this procedure.Additionally, the analysis of 252 cases of IBM during total laparoscopic hysterectomies performed by Gil-Gimeno et al.  reportedIn summary, the IBM procedure for large and giant myomas proved to be safe and feasible following standard operating procedures for containment systems. The use of the bag added value to the known advantages of laparoscopic myomectomy, which has been associated with fewer complications than open myomectomy in different RCTs and meta-analyses  . HoweverThe strength of this study, the largest ever, is the large weight of the extirpated and morcellated specimens. Similar to other studies, this study has some limitations because of the retrospective setup. Nevertheless, these results add to international knowledge of the clinical outcomes after laparoscopic myoma surgery. Future studies should address the incidence of adhesions related to the use of surgical bags.This study is the largest published until now regarding the role of in-bag morcellation in large and giant myomata surgery. As reported in our first large series on IBM , the preAppropriate bag size selection and the standardization of IBM surgical techniques save operation time, reduce the risk of bag perforation, and maintain the advantages of MIS. A postmorcellation bag insufflation test is useful to confirm a spill-free extirpation. We recommend performing peritoneal washing when bag perforation is detected. Therefore, adequate training in this technique is required to provide the best possible care for patients. Granted, IBM could be proposed as a standard strategy to reduce the risk of occult sarcoma and parasitic myomata while maintaining the significant advantages of minimally invasive approaches.


Primary seminal vesicle Burkitt lymphoma (PSBL) is rare that is not frequently reported. Burkitt lymphoma is often associated with extranodal organs. The diagnosis of carcinoma in seminal vesical can be difficult. In this report, we present a missed case of PSBL in a male patient who underwent radical prostate and seminal vesicle resection. We retrospectively analyzed the clinical data to explore the diagnosis, pathological features, treatment, and prognosis of this rare disease. The patient visited our hospital for dysuria, and the serum prostate-specific antigen (PSA) was moderately elevated. Pelvic magnetic resonance imaging (MRI) and computed tomography (CT) scans suggested a notable enlargement of the seminal vesicle. The patient then underwent radical surgery and the pathology diagnosis revealed Burkitt lymphoma. The diagnosis of PSBL is difficult, and the prognosis is generally poorer than that of other types of lymphoma. However, earlier diagnosis and treatment may help to improve the survival rate among patients with Burkitt lymphoma. Burkitt lymphoma (BL) is a rare and aggressive type of non-Hodgkin lymphoma (NHL) that accounts for 1–2% of all NHL cases. Additionally, up to 40% of lymphomas in people living with HIV (PLWH) are BL . The firThe patient was a 49-year-old man admitted to our hospital on April 2021 due to dysuria and retention of a catheter for over a month because of urinary retention. His International Prostate symptom score (IPSS) was 23, and he was diagnosed with HIV infection in 2003, for which he was on regular ART . A digital rectal examination (DRE) revealed mild prostate hyperplasia without palpable nodules, and the serum PSA was 7.8 ng/ml. Preoperative MRI and CT scans (Fig. PSBL is a rare disease that can be challenging to differentiate from prostate cancer and other primary seminal vesicle tumors in clinical practice. Primary seminal vesicle carcinoma is relatively uncommon and is often invaded by carcinomas arising in nearby structures, such as the prostate, bladder, and rectum, which can complicate the differential diagnosis . Early dInitially, we suspected prostate cancer in the 49-year-old male patient in this case, who was later diagnosed with BL. DRE suggested primary lymphoma in the prostate invading seminal vesicle. Other studies reported that DRE findings of prostate hyperplasia, hard consistency, and disappearance of the central fissure, or nodules in some patients are similar to primary prostate cancer , 9. In oThe final diagnosis of BL has rarely been reported. In general, HIV-associated lymphomas are characterized by greater extranodal involvement than those found in patients who are negative for HIV. As in endemic and sporadic BL, the translocation of the MYC gene on chromosome 8 is present in almost 100% of BL cases associated with HIV. HIV-associated BL shares many histological features, such as positive CD20, CD10, and Ki-67 [The commonly used treatment for BL is similar to that for other forms of lymphoma, and it mainly includes chemotherapy, radiotherapy, combined chemoradiotherapy, and resection of the tumor to relieve the local symptoms , 2. DunlThe diagnostic and treatment process of this case was not ideal. Firstly, the preliminary diagnosis did not match the expected final pathology, which resulted in a lack of confidence in the clinical diagnosis and treatment process. Our clinical diagnostic and treatment strategies were insufficient, and the patient’s demands and economic ability significantly impacted the diagnostic and treatment processes. We need to learn from this experience and improve future diagnoses and treatments; Secondly, there were some mistakes in the diagnosis and treatment processes. HIV combined with BL in the seminal vesicle is rare condition, and there is a close relationship between seminal vesicle tumors and prostate tumors, which can be easily misdiagnosed. We hope this case can increase clinical physicians’ awareness and provide inspiration for diagnosing rare location tumors in the HIV population. Reported diagnostic and treatment strategies could benefit patients with similar conditions. This report will hopefully serve as cautionary advice to doctors, especially urologists, to change their approach and broaden the thinking model in the clinical diagnosis and treatment process.The diagnosis of primary seminal vesical Burkitt lymphoma is difficult and the prognosis is poorer than other lymphoma types. Earlier diagnosis and treatment may improve the survival of patients with Burkitt lymphoma.


Metastatic disease in the vagina of other origins such as rectal cancer is rare and only very few cases have been reported. A female patient developed an isolated metachronic metastasis located at the lower part of the rectovaginal septum, 8 months after curative resection for proximal rectal cancer. An excision of the tumour was performed with primary closure of the vaginal wall. Histopathological examination confirmed the solid tumour to be metastatic disease from rectal origin with free margins. A year later, the patient received a lobectomy of the left lower lobe, due to distant metastasis of rectal origin 2 years after primary surgery. The patient is currently 4 years postoperatively, alive and shows no sign on recurrent disease. This case illustrates that awareness and early recognition of this rare presentation can lead to adequate treatment plans. Cancer located in the vagina is considered to be a rare entity in the scope of all known cancers. Worldwide, vaginal cancer location encompasses ⁓1–3% of all gynaecological malignancies diagnosed in women . The majWe present the case of a female patient who was referred to us by the gastroenterologist. The patient had been previously evaluated for symptoms of rectal blood loss and changes in passing stools. A colonoscopy revealed a rectal tumour at 14 cm from the anal verge. Histopathological investigation confirmed the diagnosis of a well-defined adenocarcinoma. Rectal magnetic resonance imaging (MRI) showed a clinical T2N2 stage tumour with a length of 4.2 cm, located 14 cm from the anorectal junction. The mesorectal fascia was not threatened, with seven suspected mesorectal lymph nodes. There were no signs of extramural vascular invasion or suspected extramesorectal lymph nodes. There was no sign for disseminated disease.The patient had no relevant previous medical history. She was counselled for neoadjuvant chemoradiation with Xeloda during which her menopause started. The Xeloda was discontinued due to cardiac side-effects. A restaging MRI was performed 8 weeks after ending of the neoadjuvant treatment which showed minimal reduction of tumour size resulting in a clinical T2N2 stage tumour.A laparoscopic anterior resection with a primary end-to-end anastomosis at 10 cm of the anal verge without a diverting ileostomy was performed. Her postoperative recovery was uneventful and she was discharged on day 3 postoperatively. The histopathological examination revealed a radical resected well differentiated adenocarcinoma with a diameter of 1.7 cm, with maximal depth invasion into perirectal fatty tissue and three out of 12 positive lymph nodes, ypT3N1. Tumour regression grading score of three according to the Mandard tumour regression grade .During the regular oncological follow-up 8 months post-surgery she mentioned newly discovered postmenopausal and postcoital vaginal blood loss, local discomfort and mild urinary incontinence. The carcinoembryonic antigen (CEA) level was 3.2 μg/l, and the abdominal ultrasound showed no signs of liver metastasis. Because of these new symptoms, she was referred to a gynaecologist who diagnosed a tumour at a short distance of the introitus on the posterior wall of the vagina. This tumour was of approximately one centimetre in diameter and had a firm, elastic character. Further gynaecological examination showed no abnormalities.Histopathological examination of the biopsy showed an adenocarcinoma metastasis of rectal origin. A full body positron emission tomography (PET)-scan and MRI were made and confirmed the vaginal lesion of 1.7 × 1.4 × 1.5 cm located in the posterior wall of the vagina just above the puborectal sling see  and 2. FAfter 1 month of initial diagnosis, an excision of the tumour was performed through open approach with primary closure of the vaginal wall. Histopathological examination confirmed the solid tumour to be metastatic disease from rectal origin with free margins. The newly discovered symptoms related to the rectovaginal septum metastasis had completely disappeared following the excision. Upon further follow-up at 2 years and 6 months; routine lab results showed a significant increase in the CEA levels at 5.2 ug/l (3 months prior at 4.0 ug/l). Given this significant increase; a PET-scan was again performed. The PET-scan revealed a lesion within the left lower lobe of 5.7 cm in a transverse plane; suspected to be distant metastasis see . CompareThe patient was then electively operated and received a lobectomy of the left lower lobe via the Uniportal Video-Assisted Thoracoscopic Surgery method. This was an uncomplicated procedure and after 2 days, the patient was then discharged. The patient is currently 4 years postoperatively (primary surgery) and in follow-up. The last CT-Thorax/abdomen showed no signs of recurrent malignancy (13.01.2021) and the most recent CEA value was 3.7 ug/l (21.10.2021). The patient is currently alive.Vaginal tumours are rare, compromising ⁓1% of all tumours worldwide. In this group, secondary tumours compromise ⁓80% of all cases, presenting themselves as distant metastases , 6. Mostet al. summarized the most common forms of presentation and symptoms of patients with vaginal tumours. Vaginal bleeding is by far the most common primary symptom in patients presenting either postmenopausal or postcoital [Vaginal tumours could go undetected, as most vaginal tumours are asymptomatic until a later stage. Sadatomo stcoital . Alongsistcoital .Current diagnostic standards of colorectal cancer do not include additional gynaecological examination, nor imaging for the gynaecological tract in asymptomatic patients. Medical professionals should consider a full gynaecological examination with or without extending medical imaging to the gynaecological tract for female patients who present with colorectal cancer and symptoms of the gynaecological tract. Early diagnosis and timely treatment could lead to an increased survival chance as the 5-year survival rate for late stage (III & IV) vaginal cancer is 57% and advanced disease is associated with an elevated risk of mortality HR 1.71) [.71 1, 1. Due to


Pediatric diffuse midline gliomas (DMG), H3 K27- altered, are the most aggressive pediatric central nervous system (CNS) malignancies. Disease outcome is dismal with a median survival of less than one year. Extra-neural metastases are an unusual occurrence in DMG and have been rarely described.Here, we report on two pediatric patients affected by DMG with extra-neural dissemination. Their clinical, imaging, and molecular characteristics are reported here. An 11-year-old male 5 months after the diagnosis of diffuse intrinsic pontine glioma (DIPG) developed metastatic osseous lesions confirmed with computed tomography (CT) guided biopsy of the left iliac bone. The patient died one month after the evidence of metastatic progression. Another 11-year-old female was diagnosed with a cerebellar H3K27- altered DMG. After six months, she developed diffuse sclerotic osseous lesions. A CT-guided biopsy of the right iliac bone was non-diagnostic. She further developed multifocal chest and abdominal lymphadenopathy and pleural effusions. Droplet digital polymerase chain reaction (ddPCR) on pleural effusion revealed the presence of H3.3A mutation . The patient died 24 months after the diagnosis of DMG and 3 months after the evidence of metastatic pleural effusion.Extra-neural metastasis of DMG is a rare event and no standard therapy exists. An accurate and early diagnosis is necessary in order to develop a personalized plan of treatment. Further research is needed to gain further insights into the molecular pathology of DMG, H3K27- altered and improve the quality of life and the final outcome of patients with this deadly disease. EGFR-mutant subtype , H3 K27-mutant, are a rare group of malignancies, first introduced in the 2016 World Health Organization (WHO) Classification of Tumors of the Central Nervous System (CNS) with loss of H3p.K28me3 (K27me3) and usually an H3 c.83A>T p.K28M (K27M) substitution in one of the histone H3 isoforms (CNS WHO grade 4). In the 2021 WHO Classification of Tumors of the CNS, the DMGs have been renamed to “diffuse midline glioma, H3 K27- altered” to include additional molecular changes  that also result in H3 K27 alterations  deficit, headache, and asthenia. Brain computed tomography (CT) scan revealed an enlarged pons characterized by a diffuse hypodense alteration . Subsequ18F-FDG PET/TC) showed mild diffuse bone hypercaptation; however, multiple biopsies of the lesions were non-diagnostic . Primary DMGs were mostly in the brain  and, more rarely, in the spinal cord   and chemotherapy  , multiple extra-neural locations were detected involving bones, lymph nodes, lungs, soft tissue, peritoneum, lungs, and pleura  aged 4–36 years of extra-neural metastases have been reported in the literature . We presP = 0.04) and less local progression , and minor(s)' legal guardian/next of kin, for the publication of any potentially identifiable images or data included in this article.LD and LQ: conceptualization and methodology. LD, MP, AC, GS, GSC, and PS: data curation. MM and AM: formal analysis. LQ, LD, SP, GS, GC, ME, MM, AM, and NN: investigation. GC: resources. MM and LQ: supervision. LD, ME, GSC, AC, and CR: writing—original draft. SP, AM, NN, GC, and MM: writing—review and editing. All authors have read and agreed to the published version of the manuscript.


Plasmacytoma is a rare plasma cell dyscrasia that grows within the axial skeleton or soft tissue structures as solitary or multiple masses. The primary types are solitary plasmacytoma, including solitary bone plasmacytoma (SBP) and solitary extramedullary plasmacytoma, and multiple solitary plasmacytomas. SBP is characterized by localized proliferation of monoclonal plasma cells and is rare. However, SBP with chronic osteomyelitis is even rarer.A 47-year-old man previously diagnosed with chronic osteomyelitis presented with repeated discharge and ulceration in the front of his right tibia.Lower extremity magnetic resonance imaging (MRI) and computed tomography (CT) examinations showed dead bone formation and surrounding inflammatory edema. Thus, the patient underwent dead bone excision and fenestration of the bone marrow cavity. The histopathologic examination results indicated plasmacytoma. Therefore, we administered radiotherapy with satisfactory results.Physicians should pay close attention to chronic osteomyelitis because it may be accompanied by plasmacytoma. Postoperative pathological and immunohistochemical examinations are crucial, and surgical resection of the lesion and local radiotherapy are effective treatment methods. SBP represents early-stage multiple myeloma (MM), and patients with distinct solitary lesions may have occult MM.Solitary bone plasmacytoma (SBP) is rare, accounting for 2% to 5% of all plasmacytic malignancies. These lesions, which are more common in men than in women , are characterized by local proliferation of monoclonal plasma cells, primarily in the axial skeleton, usually the vertebrae and skull. Distal adnexal bone involvement below the elbow and knee is rare, and there is no generalized involvement of myeloma-induced manifestations. The current guidelines provided by the International Myeloma Working Group define SBP as biopsy-confirmed plasmacytosis in bone or soft tissue in the context of normal bone marrow and skeletal examinations (except for primary isolated lesions). Chronic osteomyelitis is associated with avascular bone necrosis and sequestrum (dead bone) formation. However, osteomyelitis complicated with plasmacytoma is extremely rare.Given the low incidence of SBP, diagnostic criteria have been difficult to establish.We report the case of a 47-year-old man with SBP in the tibia that developed after 32 years of chronic osteomyelitis. To our knowledge, this is only the seventh case report of SBP associated with chronic osteomyelitis.Thirty-two years prior at age 15, our male patient developed swelling of the medial side of his right calf with high fever but without obvious indications; he was diagnosed with acute osteomyelitis and underwent symptomatic treatment. Nearly 20 years later, the patient developed intermittent pain in the right calf, which was subsequently broken for debridement and drainage. During this time, multiple old scars were seen in the right calf. However, the patient reported no ulceration or fever during this period.Staphylococcus aureus . The patient incision healed well, and they were discharged without incident. The patient had no pain or walking limitations and no sequelae or radiological signs of recurrence at the last follow-up (12 months postoperatively). SBP diagnoses require strict and comprehensive imaging evaluations and can only be diagnosed after excluding lesions in other body parts, which is common in patients with suspected SBP. Therefore, to differentiate SBP from MM, more relevant tests are necessary, including comprehensive clinical examinations, routine blood, renal function, serum calcium, and immune protein exams, as well as a bone marrow biopsy for histopathological examination. Changes in renal function and the blood calcium level, multiple bone damage sites, abnormal immune protein indexes, and an abnormal Kappa-light chain/Lambda-light chain ratio are features that help diagnose MM. In our case, the patient had a bone marrow lesion in the middle and upper segment of the right tibial trunk, <2% of plasma cells, and normal renal function and serum calcium distribution.SP is a plasma cell disorder characterized by a localized accumulation of neoplastic monoclonal plasma cells in bone or soft tissues  without evidence of systemic involvement . There are 2 types of SP: SBP and solitary extramedullary plasmacytoma.,10 The 2017 International Myeloma Working Group guidelines also acknowledged that PET/CT is necessary to confirm an SP diagnosis. Therefore, PET/CT is a valuable screening tool for myeloma lesions in SP since it examines the entire body in a single study and provides additional information to support inconclusive CT or MRI findings after treatment. Additionally, PET/CT detects disease changes faster than MRI. Therefore, the International Lymphoma Radiation Oncology Group recommends PET/CT as standard testing for SP. However, PET/CT exams are limited; they cannot detect very small lytic bone lesions, especially those in the skull, and they have less sensitivity for detecting diffuse changes in early bone marrow infiltration than MRI. In our case, the lesion was in the tibia, identified by CT, MRI, and emission computed tomography examinations.Plain and enhanced MRI scans are also helpful for differentiating SBP from MM. However, for patients with an SP diagnosis by MRI, it can reveal approximately 30% additional lesions. Positron emission tomography (PET)/CT is highly sensitive for detecting myeloma. in a 37-year-old man with a sinus tract in the femur for 27 years and plasmacytoma. Furthermore, Heilmann reported a case of myeloma complicating osteomyelitis. However, in both cases, the disease was widely disseminated. Next, Baitz and Kyle described a patient with a 40-year history of chronic osteomyelitis who also had a sinus tract in the femur and underwent several operations. The fourth case was presented by Wohlenberg, who further discussed SBP combined with osteomyelitis; he believed that chronic osteomyelitis, lasting 30 to 40 years, contributed to lymphoplasma cellular tissue and bone marrow becoming cancerous. Moreover, in 1984, Parsons described the case of a 68-year-old man with chronic osteomyelitis (40 years) diagnosed with solitary myeloma presenting as a lower femoral fracture. Finally, Roger reported a 42-year-old man with chronic osteomyelitis presenting with a pathological fracture in the femur; this patient suffered trauma from an accident 20 years prior, and postoperative histopathology results from the tissue around the fracture were used to diagnose SBP. To our knowledge, we are reporting the seventh case of SBP with chronic osteomyelitis . Additionally, the high differentiation and angiogenesis of plasmacytomas have been associated with disease progression. Interleukin-6 is a major growth factor, promoting B cell proliferation during bone marrow stimulation, and trauma can trigger inflammatory cytokine secretion by injured cells. Moreover, a considerable number of patients with SBP have a reported history of trauma. Some studies have investigated the association between previous trauma and SBP in younger patients. For example, 1 study investigated the association between tibial involvement and SBP in younger patients, but clear and reasonable evidence of an association was lacking. Trauma can lead to increased cytokine release, resulting in increased proliferation of plasma cells and stromal cells in bone, suggesting that trauma may be 1 disease trigger. However, since there are so few patients, the etiology of SBP in osteomyelitis requires further exploration.The pathogenesis of SBP, especially SBP combined with chronic osteomyelitis, remains unclear, but SBP appears to be a very rare complication of chronic osteomyelitis. The current literature and our case demonstrate that most patients have a long history of osteomyelitis, the longest of which was 40 years. Long-term inflammation may cause pathological changes in the surrounding tissues or cells, eventually leading to plasmacytoma. Wohlenberg also discussed these unusual occurrences, suggesting a similar view. Surgery, however, is not limited to surgical debridement; it also includes 3 ancillary aspects that contribute to successful treatment: adequate dead space management, bone stabilization, and skin graft coverage of the wound. Polymethyl methacrylate beads loaded with antibiotics have been used in the clinical treatment of chronic osteomyelitis and should be adjusted based on the culture results and other patient-specific factors. We identified S aureus in this case, which was sensitive to vancomycin. Therefore, we surgically removed the necrotic tissue and initiated drainage. Then, the medullary cavity was filled with vancomycin bone cement and treated with antibiotics for 6 weeks.Currently, clear and unified guidelines for treating SBP with chronic osteomyelitis do not exist. The general management of chronic osteomyelitis requires aggressive and adequate surgical debridement and long-term antibiotic therapy because local and conservative debridement has been associated with a high failure rate. Ozsahin et al retrospectively assessed the outcomes of 206 patients with SBP and 52 patients with extramedullary plasmacytoma treated with radiotherapy alone (214 patients), radiotherapy combined with chemotherapy (34 patients), or surgery alone (8 patients). They found that the 5-year overall survival rate was 74%, the disease progression-free survival rate was 50%, and the local control rate was 85%. Furthermore, the local recurrence rate for patients receiving radiotherapy was 12%. In contrast, the rate was 60% for those not receiving radiotherapy. The optimal radiation dose for solitary osteo-plasmacytoma is unclear. Still, a previous report indicated that a radiation dose of 30 to 60 Gy was suitable, and the 2022 National Comprehensive Cancer Network guidelines recommended that solitary osteo-plasmacytoma be treated with radiation of the involved field at a 40 to 50 Gy/1.8 to 2.0 Gy dose.Local radiotherapy is the first choice for diagnosing solitary osteo-plasmacytoma, providing effective control. According to worldwide guidelines and clinical studies, local radiotherapy was indicated for treating SBP in our patient. Thus, we administered local radiotherapy after 4 courses of treatment (40 Gy/1.8–2.0 Gy daily). As a result, the patient incision healed well, and they were released from the hospital without incident. In addition, the patient had no pain or walking limitations, nor were there sequelae and radiological signs of recurrence at the last follow-up visit 12 months postoperatively.Surgical treatment is not the first choice for SBP; it is only recommended for unstable bone structures or nerve compression. However, radiotherapy after surgery is recommended.In conclusion, SBP with chronic osteomyelitis is extremely rare, but physicians should pay close attention to patients with chronic osteomyelitis. SBP with osteomyelitis is treatable with surgical resection of the lesion, antibiotic therapy, and local radiotherapy.Formal analysis: Gang Chen.Investigation: Fei-Peng Gong.Resources: Gui-Chao Zhou, Zhi-Sheng Long, Gang Chen.Visualization: Zhi-Sheng Long.Writing – original draft: Xiao-Wei Yang, Gui-Chao Zhou.Writing – review & editing: Zhi-Sheng Long.


Intracholecystic papillary neoplasm (ICPN) is a grossly visible, mass-forming, noninvasive epithelial neoplasm arising from the mucosa and projecting into the lumen of the gallbladder. ICPN is a lesser-known tumor of the gallbladder lining, which although has a better prognosis compared to gallbladder adenocarcinoma carries the potential for metastatic transformation with spread to other organs. ICPN is found incidentally on imaging or during postop histological evaluation. However, we present a unique case of ICPN that was incidentally diagnosed in a 72-year-old Eastern European woman following cholecystectomy for acute cholecystitis and was missed on preoperative imaging. Follow-up protocols of ICPN are poorly understood and vastly underreported. We discuss this patient’s findings in light of current literary evidence available on ICPN and outline future directions for better clinical understanding. We also highlight the need for screening guidelines in light of known risk factors to better understand the natural history of the disease to prevent malignant transformation into invasive gallbladder carcinoma. Intracholecystic papillary neoplasm (ICPN) is a premalignant lesion arising from the gallbladder lining . ICPN isA 72-year-old Eastern European woman presented to the emergency department (ED) complaining of mid-epigastric and RUQ abdominal pain radiating to the back for the past three days. The pain was not associated with the consumption of meals and was diffuse on presentation. The patient also reported nausea and vomiting for the past three days and constipation for five days. Her past medical history was remarkable for diabetes mellitus and hypertension. The RUQ pain had gradual onset, was intermittent in nature, and was rated at an 8 out of 10 in severity.Hemodynamic parameters on admission included a temperature of 98.6°F, a pulse of 104 breaths per minute (bpm), respiratory rate of 18 breaths per minute (bpm), blood pressure of 142/82 mmHg, and oxygen saturation of 96% on room air. A review of the systems was positive for diffuse abdominal pain, nausea, vomiting, and constipation. On physical exam, the abdomen was soft but tender to palpation in both mid-epigastric and RUQ regions. Bowel sounds were normal, and there was no distension, masses, rigidity, guarding, or costovertebral angle (CVA) tenderness. Murphy’s sign was positive.Laboratory results revealed a normal white blood cell count (WBC) of 7.5 K/uL (reference range: 5-10 K/uL), an elevated red blood cell count (RBC) of 5.31 x 1012/L (reference range: 3.8-5.2 x 1012/L), a normal hemoglobin (Hb) of 16.0 g/dL (reference range: 12-16 g/dL), a normal hematocrit (Hct) of 46.4% (reference range: 36%-48%), elevated serum glucose of 377 mg/dL (reference range: 70-100 mg/dL), a decreased serum sodium level of 130 mmol/L (136-145 mmol/L), a decreased serum chloride of 92 mmol/L (reference range: 96-106 mmol/L), and a normal prothrombin time (PT) of 12.6 seconds (11-13.5 seconds). Liver function testing demonstrated a decreased aspartate aminotransferase (AST) level of 5.0 U/L (reference range: 8-33 U/L), a normal alanine transaminase (ALT) level of 29.0 U/L (reference range: 4-36 U/L), and a normal alkaline phosphatase level of 56 IU/L (44-147 U/L).A CT of the abdomen and pelvis with intravenous (IV) contrast revealed a distended gallbladder with numerous gallstones, consistent with acute cholecystitis Figure .There was also fatty infiltration of the liver. Imaging was negative for intra- or extra-hepatic ductal dilation. A RUQ U/S revealed gallbladder distension, cholelithiasis Figure , Panel AA hepatobiliary scan confirmed absent scintigraphic activity in the gallbladder with 6.1 mebrofenin choletec injection (mCi) technetium-99m mebrofenin, which was consistent with cholecystitis Figure .The patient was diagnosed with acute cholecystitis and underwent laparoscopic cholecystectomy. The procedure was tolerated well. On postoperative day one, the patient was tachycardic with a rate of 112 bpm but did not have any complaints otherwise. She was discharged on postoperative day three in stable condition.The patient was diagnosed with acute cholecystitis and underwent laparoscopic cholecystectomy. The procedure was tolerated well, and the resected gallbladder specimen was sent to pathology. On histopathologic exam, the gallbladder was filled with thick dark green bile and measured at 9.0 x 4.0 x 3.5 cm and was 0.3 cm in thickness. Further evaluation revealed a rough, polypoid cheese-like lesion at the fundus, measuring 3.0 x 2.0 cm. The cystic duct margin was free of the lesion and was negative for high-grade dysplasia and malignancy. The histopathological evaluation confirmed that the lesion was an intracholecystic papillary neoplasm  on conventional imaging such as U/S or contrast CT. Up to 50% of ICPN cases are detected incidentally, and approximately 10% are missed in imaging studies . Due to The diagnostic protocol of ICPN involves a combination of in-depth clinical evaluation, imaging studies, and histopathological analysis. Diagnostic steps involve clinical evaluation where a thorough medical history and physical examination are conducted, and an assessment of the patients’ symptoms, risk factors, and other medical comorbidities is conducted. U/S, CT, and MRI are the current standards of imaging for detecting ICPN lesions. U/S detects the presence of gallbladder polyps/masses and assesses the size, location, and appearance. CT reveals information about the extent of the tumor invasion into nearby structures and distant or lymph node metastasis. MRI, on the other hand, provides further information about tumor invasion and differentiates a benign lesion from a malignant one . ContrasICPN, along with its counterparts , follow the adenoma-carcinoma sequence with mutations such as KRAS, STK11, CTNNB1, and APC (adenomatous polyposis coli) being commonplace ,9. ICPN Although most cases of ICPN are asymptomatic, it is a premalignant lesion with the propensity to evolve into invasive carcinoma in 6.4% of the cases [ICPN is a rare neoplasm that can be difficult to detect on conventional imaging modalities. Although rare, a small fraction can evolve into invasive carcinoma. Our case illustrates how often these rare lesions can go undiagnosed due to the lack of surveillance and imaging protocols. This unique incidental case of ICPN adds valuable information to the existing literature as the disease onset and progression are unexpected and confirms recent findings, which highlight the use of CEUS and MRI to be superior in detecting ICPN compared to the traditional conventional U/S and CT imaging. The lack of surveillance and diagnostic protocols can lead to delayed diagnosis at advanced stages of malignancy with a poor prognosis compared to patients diagnosed earlier and treated with prompt surgical removal. We hope that our contribution to the existing database will aid in raising clinician awareness of ICPN and related neoplasms as a diagnosis to screen for in patients who may be at the risk of developing it.


Papillary fibroelastoma (PFE) and nonbacterial thrombotic endocarditis (NBTE) account for <1% of all cardioembolic strokes. When there is no evidence of infection, and an exophytic valve lesion is seen on echocardiography, PFE may be an initial imaging diagnosis. NBTE, or Libman-Sacks endocarditis, is a rare entity and can present with varied imaging findings. This report presents a case of embolic stroke and NBTE mimicking a PFE. We discuss a 49-year-old female with a past medical history of diabetes mellitus who presented with headache and right-hand numbness. The initial CT head was negative and the MRI brain showed multiple infarcts in the watershed areas where anterior and posterior brain circulation meet and overlap. A transesophageal echocardiogram (TEE) showed a left ventricle (LV) mass initially diagnosed as PFE. The patient was started on aspirin only with no anticoagulation since we thought the stroke was related to an embolus from a tumor, not a thrombus. The patient underwent surgery but the pathology report revealed a diagnosis of organizing thrombus with abundant neutrophilic infiltration and no neoplastic proliferation. This case report highlights the importance of a comprehensive evaluation of valvular masses and the diagnostic approaches currently available to help clinicians differentiate between various causes of embolic stroke like PFE, bacterial endocarditis, and NBTE. Early differentiation is critical because it can affect the treatment and outcome. This report shows that echocardiography of endocardial and valvular lesions may provide a differential diagnosis, but a definitive diagnosis requires microbiology and histopathology. Advanced imaging techniques such as cardiac CT or cardiac MRI may assist in identifying select cases that are at lower risk for subsequent embolic events, in which surgical intervention may safely be avoided. Papillary fibroelastoma (PFE) and nonbacterial thrombotic endocarditis (NBTE) are rare entities and account for <1% of all cardioembolic strokes . NBTE isA 49-year-old female with a past medical history significant for obesity, asthma, and type 2 diabetes mellitus presented to the emergency department with headache, numbness in the lateral three digits of the right hand, nausea, vomiting, and chills of a day's duration. She had no prior history of migraine or other recurrent headaches. Her past history was negative for cardiac or neurological conditions, and she denied any chest pain, palpitations, shortness of breath, or other neurological symptoms.Her labs on admission were significant for an elevated cardiac troponin of 0.46 ng/mL  that went up to 1.14 ng/mL on the next day. CT head obtained at the time of admission was unremarkable. However, due to her persistent headache and continued numbness of the right-hand digits, we obtained a CT angiogram of the head on the following day, which revealed an acute left parietal white matter infarct. Subsequent MRI of the brain showed multiple bi-hemispheric ischemic infarcts, highly suggestive of thromboembolic events.The workup for embolic stroke included a transthoracic echocardiogram (TTE) that revealed mildly impaired left ventricular (LV) systolic function with an ejection fraction (EF) of 45-50%, and a medium to large-sized mass attached to the mitral valve in the left ventricle, which was thought to represent a tumor, vegetation, or, less likely, a thrombus Figure . In collA TEE was obtained on hospital day three to better characterize the mass, and it revealed a 1.7 x 1.3 cm mass on the anterolateral papillary muscle, with an appearance consistent with PFE, and cardiothoracic surgery was consulted for resection Figure . No antiOn hospital day seven, the patient underwent surgery, and a friable, pedunculated mass appearing to originate from the chordae of the papillary muscles was resected. In contrast with our anticipated finding of a fibroelastoma, the pathology report revealed a 1.7 x 1.4 x 0.2 cm organizing thrombus with abundant neutrophilic infiltration and no neoplastic proliferation Figures , 4. She NBTE is a rare disease and echocardiography is the initial diagnostic modality in case of clinical suspicion for NBTE; however, to establish a definitive diagnosis, microbiology and histopathology may be required. Around 13.7 to 17 million people experience a stroke each year globally ,6. CardiPFEs are the second most common primary cardiac tumor in adults ,11. PFE'NBTE is a rare condition that has been reported in individuals of all age groups but is most common between the fourth and eighth decades of life and is often only discovered on postmortem examinations . NBTE moTwo-dimensional TTE is typically recommended as part of the initial evaluation of patients with suspicion of intracardiac masses. In the case of NBTE, vegetations are frequently left-sided and involve the mitral valve in 66% of cases . One impWhile this case presented a high-risk lesion with a clear indication for surgical resection, NBTE and PFE can present with lesions of widely varying size and degrees of risk for future events, and in such lesions, additional early diagnostic workup may serve to spare select patients from surgical intervention. Both CMR and CCT can provide high-resolution, noninvasive images of the heart. CMR is often preferred, as T1- and T2-weighted images can provide additional insights into the microenvironment of a tumor, which may be helpful in better characterizing masses of uncertain etiology ,20.While echocardiography may aid in the differentiation of endocardial and valvular lesions, a conclusive diagnosis can only be reached through the utilization of microbiology and histopathology. Early utilization of advanced imaging techniques such as CCT or CMR may assist in identifying select cases that are at lower risk for subsequent embolic events, in which surgical intervention may safely be avoided.


Liposarcoma often occurs in the extremities and retroperitoneum. Primary mediastinal liposarcoma is uncommon, and there is no settled opinion regarding adjuvant therapy after surgery. We have recently experienced a relatively rare case of primary dedifferentiated liposarcoma of the posterior mediastinum. The patient was a 76-year-old woman. An abnormal shadow was noted in the posterior mediastinum. Esophageal submucosal tumor and gastrointestinal stromal tumor were suspected; endoscopic ultrasound-guided fine needle aspiration was performed, but a definitive diagnosis could not be obtained. As the tumor tended to slowly grow, surgical resection was performed. Based on histopathological findings, the patient was diagnosed with primary dedifferentiated liposarcoma of the posterior mediastinum. Owing to the presence of a positive surgical margin, postoperative radiotherapy (60 Gy/24 fr/6 w) was administered. No recurrence was observed after three and a half years of follow-up. The prognosis of primary dedifferentiated liposarcoma of the posterior mediastinum with a positive surgical margin is poor, but postoperative radiotherapy may be useful. Liposarcoma often occurs in the extremities and retroperitoneum. Primary mediastinal liposarcoma is a relatively rare type of disease and accounts for less than 1% of all types of mediastinal tumors . The preThe patient was a 76-year-old woman. A chest X-ray during a health checkup revealed an abnormal shadow in the right lower lung field Figure . A closeChest magnetic resonance imaging (MRI) revealed a lobulated mass with a clear margin in the right posterior mediastinum. A heterogeneous mild hyperintensity was observed on T2-weighted imaging Figure  and a miIn accordance with the intraoperative findings, thoracotomy was performed by creating a 13-cm incision at the fifth intercostal space with the thoracoscope placed at the left lateral position. The tumor adhered to the right lower lobe, posterior mediastinal pleura, and right lower pulmonary vein. The adhesions were dissected circumferentially. Since the adhesion between the tumor and the right lower lobe was relatively strong and infiltration was suspected, partial combined resection was performed. By contrast, the esophagus and carina, which were located at the deepest part, were easily dissected, and the tumor was finally resected. The adhesiolysis site of the esophagus was covered with pericardial fat pad. The surgery lasted for one hour and 53 minutes, and the amount of blood loss was 20 mL. The resected specimen appeared as an encapsulated yellow-to-grayish white lobulated mass, with calcification and ossification, and measured 11 cm × 7 cm × 4 cm  and cyclin-dependent kinase 4 (CDK4). However, the patient tested negative for h-caldesmon, desmin, cluster of differentiation 34 (CD34), S-100, and signal transducer and activator of transcription 6 Figures .The chest tube was removed on postoperative day two, and the patient was eventually discharged on postoperative day eight. After surgery, radiotherapy (60 Gy/24 fr/6 w) was performed by a respiratory physician. Since the patient was undergoing radiation therapy for a posterior mediastinal tumor, periodic echocardiography was performed to monitor for cardiotoxicity. No decline in cardiac function was observed after the completion of radiotherapy. Every six months, the patient underwent a chest CT as a routine examination. No recurrence was observed after three and a half years of follow-up.The incidence of soft tissue sarcomas (STS) is approximately 40 cases per one million population per year, accounting for approximately 1.5% of all types of malignant tumors . The comPrimary liposarcoma of the mediastinum is commonly found after tumor growth due to the occurrence of symptoms such as dyspnea, chest pain, and tachycardia caused by compression and infiltration of the intrathoracic structures ,6. ChestLiposarcoma is mainly classified into five types: well-differentiated, myxoid, round cell, pleomorphic, and differentiated. Immune markers for specific tumor types, such as MDM2 and CDK4 for dedifferentiated liposarcoma, are often used for diagnosis . Ortega In this patient, a posterior mediastinal tumor was incidentally found in the absence of any symptoms. Through the conduct of imaging examinations, esophageal GIST was listed as a differential diagnosis, but a definitive diagnosis was not made. However, surgical resection was still performed. This was a relatively rare case of primary dedifferentiated liposarcoma of the posterior mediastinum, a tumor with a relatively poor prognosis, diagnosed based on the postoperative histopathological findings.The first-line treatment for primary liposarcoma of the posterior mediastinum is complete surgical resection . SeveralOrtega et al. reported that 11 of 18 patients with primary liposarcoma of the posterior mediastinum underwent complete resection, but only four of them had negative surgical margins . This maBecause the surgical margin was positive, postoperative radiotherapy (60 Gy/24 fr/6 w) was administered in this patient, owing to the increased risk of recurrence. Only a few patients received radiotherapy after surgery . The patThis was a case of a patient with primary dedifferentiated liposarcoma of the posterior mediastinum, a tumor with a poor prognosis, in whom posterior mediastinal tumor was incidentally found in the absence of any symptoms. Our findings suggest that postoperative radiotherapy may be effective in patients with positive surgical margins. Further studies are warranted to accumulate relevant data related to this case in order to generalize the use of adjuvant therapy in patients with primary dedifferentiated liposarcoma of the posterior mediastinum.


Anal cancer is a rare cancer accounting for ∼2% of all gastrointestinal tract malignancies . Among aHerein, we present the case of a 63-year-old man who was diagnosed with primary anal canal lymphoma that was completely resolved after six cycles of chemotherapy. This report focuses on anal canal lymphoma and reviews the relevant literature.A 63-year-old man presented having had anal pain and hematochezia for 1 month. He also reported frequent defecation (five times/day), tenesmus, and weight loss of 4 kg during the same period. The patient had no relevant medical history apart from dyslipidemia and no significant surgical, social, or family history. Physical examination findings were normal except for an anorectal mass palpable on digital rectal examination. No superficial lymph nodes (LNs) were palpable.in situ hybridization results for MYC and EBV with in situ hybridization were negative. Chest computed tomography (CT) revealed no evidence of lymphoma, and enhanced abdominal/pelvic CT and magnetic resonance imaging showed a mass encircling the rectum and enlarged regional LNs  and other routine hematological and biochemical investigations, including tumor markers (carcinoembryonic antigen and carbohydrate antigen 19–9) and lactate dehydrogenase, were within the normal range. Human immunodeficiency virus antibody test and Epstein-Barr virus (EBV) DNA results were negative. Colonoscopy revealed an ulcerative fungating mass with a soft margin that extended from the anal rim to the rectum, measuring ∼5 cm on the anal canal . Histoloonal LNs . PositroBased on the clinical, histopathologic, and immunohistologic features, the patient was diagnosed with primary anal canal DLBCL and regional LN involvement . A rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisolone (R-CHOP) chemotherapy regimen was performed for six cycles. Subsequent CT and colonoscopy with a biopsy of the lesion revealed that the mass had completely disappeared . The patCancers of the anal canal represent ∼2.4% of all gastrointestinal neoplasms. Anal lymphoma represents 0.2% of anorectal tumors, most of which correspond to NHLs . The comThe symptoms of gastrointestinal lymphoma vary, including abdominal pain, weight loss, mass, and hematochezia, in addition to the features of obstruction, such as nausea, vomiting, and changes in bowel habits . In our The clinical presentation of anal canal lymphoma usually mimics those of other anal cancers, such as anal SCC. Lymphomas are malignant neoplasms of lymphocyte cell lines that originate from the deep mucosa or submucosa; therefore, lymphomas can appear as sub-epithelial lesions. In this case, colonoscopy revealed a mass with central ulceration and a soft margin, which could be seen in the sub-epithelial lesion.In these cases, colonoscopy with biopsy is the most useful diagnostic test. Obtaining adequate samples that allow histopathological diagnosis is vital for establishing appropriate treatment. If lymphoma is suspected based on the initial hematoxylin–eosin staining, immunohistochemical assessment is conducted on fixed, paraffin-embedded tissue specimens using antibodies specific for differential diagnosis. Chest and abdominal/pelvic CT is recommended, and bone marrow biopsy and positron emission tomography are recommended for proper staging. In our patient, the final diagnosis was primary DLBCL of the anal canal with Ann Arbor stage IIE 1, which is defined as a tumor with regional LN involvement and an international prognostic index of 1.The treatment for anal canal lymphomas remains uncertain. While surgical treatment may be indicated for some localized tumors, medical management is considered the primary treatment . RapidlyIn conclusion, malignant anal canal lymphomas are not easy to distinguish from other anal cancers based on endoscopic features without histopathologic examination. However, when lesions with a relatively soft margin exhibit a rapid growth pattern, the occurrence of primary anal canal lymphomas is possible. Accurate diagnosis using a multimodality approach and proper treatment can improve the patient’s prognosis.S.B.P. and Y.K.C.: acquisition of data, drafting of the manuscript, critical revision of the manuscript for important intellectual content, and study supervision. All authors read and approved the final manuscript.


The term "encephalocele" refers to the herniation of brain tissue caused by a cranial bone defect. It could be congenital, traumatic, neoplastic, or arise spontaneously. The possibility of traumatic fronto-ethmoidal encephalocele should be considered in patients who have experienced trauma. We report a case of a 16-year-old male with a recent history of a bike accident presented with sudden unilateral rhinorrhea. Non-contrast computed tomography (NCCT) brain was done, which showed findings of left fronto-ethmoidal encephalocele. The patient was managed with single-staged surgery without any complications. An encephalocele is defined as the herniation of brain tissue through a skullbone defect, which can be congenital, traumatic, neoplastic, or spontaneous in origin. Traumatic encephalocele usually results secondary to head trauma or can be iatrogenic . Almost A 16-year-old male presented to the casualty with an episode of loss of consciousness for five minutes with spontaneous recovery and no similar previous episodes. He had no significant past medical history, and he was not on any medications. His family history was unremarkable. He had a past history of road traffic accidents in the form of a bike accident a month ago. He was traveling at a medium speed (40-50km/hour) without wearing a helmet when he met with an accident with a car and fell on the road with mild trauma to the frontal area in the form of bruises and mild swelling over the forehead which was managed at home. The patient did not seek any medical assistance for the same. During the present visit, apart from the loss of consciousness for five minutes in the morning as described above, the patient only complained of a unilateral running nose, which he noticed two days ago and was sudden in onset, whitish in color, non-foul smelling, and not associated with blood stain. There was no history of fever, neck pain, weakness in both limbs, or sensory symptoms in the form of tingling or numbness.On physical examination, the patient was conscious and alert, with a Glasgow Coma Scale (GCS) score of 15/15. Neurological examination shows the power of 5/5 in all four limbs and no neuro-deficit. Other systemic examinations were unremarkable. Routine blood investigations like complete blood count, liver function test, and kidney function test were done, which were within normal limits. The patient underwent non-contrast CT brain, which showed a linear displaced fracture of the posterior wall of the left frontal sinus and the vertical portion of the left cribriform plate along with herniation of left frontal lobe parenchyma through this defect, as shown in Figure Anatomically, the ethmoid sinus is surrounded by thin walls and hence can be easily damaged by trauma and can result in encephalocele . CerebroEncephaloceles are classified according to the anatomical area of the bony defect, which is important from a surgical point of view. Recently, a revised classification by Gerhardt includes head dome, occipital, fronto-ethmoidal, and basal encephalocele . In our If this is left untreated, it may lead to permanent CSF fistulas, meningitis, and intractable seizures ,10.In a case of post-traumatic unilateral rhinorrhoea, fronto- ethmoidal encephalocele should be considered as a differential. Prompt diagnosis of this condition is beneficial as it could prevent several life-threatening complications like CSF leak and meningitis. It is possible to diagnose encephalocele and CSF leak using radiological modalities like CT and MRI along with clinical symptoms and hence should be screened if history is suggestive.


Intestinal duplication and ectopic pancreas are two rare independent congenital anomalies. Few reports describe cases of patients with ectopic pancreas in an intestinal duplication causing acute peritonitis.A 31-year-old man was admitted to the hospital for epigastric pain. The patient was diagnosed with acute peritonitis caused by the acute pancreatitis of an ectopic pancreas in a jejunal duplication, with intestinal malrotation. The patient underwent the partial resection of the jejunum and Ladd’s procedure. The histopathological findings indicated ectopic pancreatitis in the jejunal duplication.We presented the case of acute peritonitis caused by the acute pancreatitis of an ectopic pancreas in a jejunal duplication in an adult with intestinal malrotation. Surgery is the primary treatment and is necessary for a definitive diagnosis. Intestinal duplication is a rare congenital anomaly, which can be localized anywhere from the mouth to the anus . EctopicA 31-year-old man with no related medical history visited his general practitioner because of epigastric pain. Acute cholecystitis was suspected, and the patient was referred to the surgical department of our hospital. On admission, the vital signs were as follows: blood pressure, 124/70 mmHg; pulse, 70 beats/min; and temperature, 36.5 °C. The physical findings included right upper quadrant pain, negative Murphy sign, and no rebound pain. Blood tests revealed the following elevated inflammatory markers; white blood cell count, 9300/μL (3100–8400/μL); C-reactive protein level, 12.1 mg/dL (< 0.3 mg/dL); total bilirubin level, 2.2 mg/dL (0.4–1.5 mg/dL), and gamma glutaryl transferase level, 87 U/L (13–64 U/L). Contrast-enhanced abdominopelvic computed tomography (CT) revealed a 30-mm oval soft tissue mass with microlobulated margins below the gallbladder. The density of the mass was similar to the pancreas , followed by the ileocecal valve (30.2%), duodenum (9.6%), stomach 8.2%), jejunum (8.2%), colon (6.8%), and rectum (5.5%) 1.5%, fol–7. Most .2%, jejuTable The preoperative diagnosis of intestinal duplication and ectopic pancreas may be difficult owing the rarity and absence of specific findings. CT may provide some informative findings, and endoscopic ultrasonography (EUS) may be useful for the intestinal segments that can be reached by an endoscope. CT typically shows a spherical duplication cyst as a thick-walled cystic lesion with enhancement of the inner lining and tubular duplication as an intestinal structure with a blind end . The ectIn the reported cases, the ectopic pancreas in the intestinal duplication was resected using endoscopic submucosal dissection (ESD), apart from l the duplication muscle layer in one case. ESD is a treatment option if the lesion is amenable to endoscopic resection and is adequately deep to be safely removed. In other cases, including the present case, small bowel resection or partial gastrectomy, including the intestinal duplication and the cyst, were performed. In general, complete resection is the treatment of choice for symptomatic, possibly malignant, and incidental cases of ectopic pancreas to prevent future complications.Our patient also had intestinal malrotation. The intestinal malrotation was most likely not associated with the peritonitis in this case. The coexistence of intestinal malrotation with ectopic pancreas and intestinal duplication is extremely rare, and this was the first case to be reported to the best of our knowledge.We present a case of acute peritonitis caused by the acute pancreatitis of an ectopic pancreas in a jejunal duplication in an adult with intestinal malrotation. CT and MRI may be useful for the preoperative diagnosis of ectopic pancreas. Surgery is the primary treatment and is necessary for a definitive diagnosis. This report aimed to raise awareness and provide information regarding this rare case to help clinical management.


Osteoid osteomas are benign bone tumors that are commonly found in the cortical segments of long bone but can occasionally occur in the talus of the foot. They typically present in younger males and are characterized by lesions with a vascularized nidus surrounded by sclerotic bone. Plain radiographs can often miss the diagnosis, requiring further imaging with computed tomography (CT) or magnetic resonance imaging (MRI). Lesions often lead to a significant inflammatory response resulting in an impaired range of motion and nocturnal pain. Conservative management with non-steroidal anti-inflammatory medications and a walking boot is considered first-line therapy, with failure to respond being an indication for surgical intervention. Surgical treatment traditionally consisted of en bloc resection but has been replaced by CT-guided radio-frequency ablation (RFA) when conservative management has failed. Four cases of osteoid osteoma of the talus are presented which all went on to RFA after conservative management failed. The patients’ non-specific symptomatology and unremarkable findings on plain radiographs led to further evaluation using MRI or CT, which aided in the diagnosis. Following imaging, RFA was performed which resulted in 100% relief of pain and symptoms in all four patients and a return to full activity without limitations. Osteoid osteomas of the talus present unique challenges due to the non-specific symptoms and complex surrounding anatomy that accompanies this condition. Management should include the use of CT for localization and RFA of the lesion, which we have shown leads to complete resolution of symptoms and return to normal daily activities. Originally characterized by Jaffe in 1935 . AffectePatients with osteoid osteomas often present with edema, decreased range of motion, and most characteristically, nocturnal pain. The severe pain associated with osteoid osteomas has been linked to excess prostaglandin production, reaching levels 30x higher than normal bone. This increase in prostaglandin production from within the nidus leads to extreme inflammation and pain ,6. MedicThe most common finding on plain radiographs is a radiolucent nidus within a zone of sclerotic bone. In certain cases, such as intra-articular osteoid osteomas, the complexity of the surrounding anatomy can conceal the nidus on plain radiographs, revealing only encircling sclerosis. Therefore, the preferred method of diagnosis and localization is via computed tomography (CT) or magnetic resonance imaging (MRI), in which a well-defined nidus, occasionally with calcifications, can be seen surrounded by a region of sclerotic bone . Bone scSurgical treatment of osteoid osteomas historically consisted of en bloc resection or curettage. These techniques allow for the effective removal of the nidus and sclerotic bone, as well as the ability to perform histology to confirm the diagnosis and complete resection. However, the removal of large portions of bone is unfavorable due to increased fracture risk, the need for bone grafting, and longer recovery times. Starting in the 1990s, surgical treatment of osteoid osteomas was accomplished via radio-frequency ablation (RFA) or thermo-coagulation guided by CT. The benefits of these methods include reduced bone loss as well as faster recovery times but can be complicated when complex neurovascular anatomy is situated nearby or when there is a recurrence of osteoid osteomas after primary treatment .Osteoid osteomas of the talus occur in less than 3.4% of cases and present unique challenges due to the complex anatomy of the area including the partial intra-articular nature of the bone . DiffereCase one2. He denied a prior history of injury to the right foot or ankle. He was focally tender to palpation over the medial aspect of the talonavicular joint with minimal edema. Passive manipulation of the talonavicular joint elicited mild discomfort. The remaining portions of his foot and ankle were non-tender and he was neurovascularly intact. Imaging with plain radiographs was unremarkable and further imaging with MRI was obtained which aided in the diagnosis of osteoid osteoma of the talus  of 24.4 kg/ms Figure .The patient failed conservative management, which consisted of immobilization in a walking boot and oral NSAIDs. Therefore, he was scheduled for an image-guided RFA. After the ablation, his symptoms resolved within 24-48 hours after the procedure. He returned to full activity and reported no limitations. Overall, he was very satisfied with his outcome and was doing well six months post-procedure.Case twoA 21-year-old female presented with a four-year history of pain along the medial aspect of her right hindfoot. There was no history or trauma or inciting event. She had seen several specialists prior to presenting to our institution and had been diagnosed with a stress reaction within the talus. Physical therapy improved the mobility and strength of her ankle but did not alleviate her pain. She took oral NSAIDs before bedtime, which gave her excellent relief and allowed her to sleep. She presented to our institution for an additional opinion.2. Upon examination, her skin was intact, and she was focally tender about the medial aspect of her talus. Her ankle and hindfoot were flexible, but hindfoot inversion elicited mild pain in the medial aspect of her hindfoot. She had full strength, normal sensation, and palpable pedal pulses in the lower extremity. Since the patient had persistent hindfoot symptoms, an MRI was also performed at our institution, and she was diagnosed with an osteoid osteoma of the talus (Figure The patient was otherwise healthy and had a body mass index of 20.4 kg/mThe patient failed conservative management, which consisted of immobilization in a walking boot and oral NSAIDs. Therefore, she was scheduled for image-guided RFA Figure . Post-prCase threeA 20-year-old female presented with a three-month history of right hindfoot pain. Her initial symptoms occurred when she jumped off a desk and landed awkwardly on her right foot. She was seen initially by an outside health care provider and X-rays at that time were unremarkable. She completed a course of physical therapy without notable improvement. An MRI was obtained that showed some bony edema within the talus and she was placed into a walking boot. She presented to our institution for a second opinion as she was still experiencing almost exclusively nocturnal medial hindfoot pain exacerbated by side-to-side hindfoot motion. Her pain was relieved when she took oral NSAIDs.2. Upon examination, her skin was intact, and she was focally tender about the medial aspect of her talonavicular joint. Her ankle and hindfoot were flexible, but hindfoot inversion elicited mild pain in the medial aspect of her hindfoot. Her neurovascular examination was normal. Since the patient had persistent hindfoot symptoms, an MRI was repeated at our institution, and she was diagnosed with an osteoid osteoma of the dorsomedial talus (Figure The patient was otherwise healthy, non-smoker, with a BMI of 21.6 kg/ms Figure .The patient failed conservative management, which consisted of physical therapy, immobilization in a walking boot, and oral anti-inflammatory medications. Therefore, she underwent an image-guided biopsy and RFA of the lesion. After the ablation was performed, she reported 100% relief of her symptoms within 48 hours of the procedure. She returned to her normal activities and reported no complications after the ablation procedure.Case fourA 12-year-old otherwise healthy male presented with several months of right foot pain. The family attributed the pain to a minor injury from sports; the patient had been immobilized briefly. When he continued to have pain, an MRI was obtained, and the outside healthcare provider believed the patient had sustained a nondisplaced talus fracture. He was immobilized again and presented to our institution for a second opinion. On examination, he had mild swelling and pain over the dorsal aspect of the talus but was otherwise asymptomatic and neurovascularly intact. He did have nocturnal pain that was relieved with NSAIDs. A CT was obtained that demonstrated an osteoid osteoma in the neck of the talus Figure .The patient underwent image-guided RFA of the lesion. He was seen two weeks post-procedure and both the patient and his mother reported that he had complete improvement of his pain 24 hours after the procedure. He was able to wean out of his CAM walker boot into regular shoes. Physical therapy was initiated, and he returned to physical education and activities at four weeks post-procedure.Pathology reports available for review showed bone-forming lesions composed of interlacing trabeculae and sheets of woven bone in a loose fibrovascular stroma with conspicuous osteoblastic lining and osteoclast-type giant cells, which is consistent with osteoid osteomas Figure .We present four cases of osteoid osteoma within the talus that presented with hindfoot pain around the talonavicular joint. NSAIDs paired with immobilization elicited inconsistent symptom reduction and all patients were subsequently treated with image-guided RFA following the failure of conservative management. Specimens were collected during the procedure and post-operative pathology showed findings consistent with the diagnosis of osteoid osteoma. All four patients achieved full relief of symptoms within 24 to 48 hours and were able to return to full activities without limitations.Due to the often nonspecific nature of the history and physical examination findings in these patients as well as the normal plain radiographic imaging findings, further evaluation with CT or MRI is often necessary. Therefore, a high degree of clinical suspicion is important when patients present with ankle pain that does not subside with rest and lack diagnostic findings on plain radiographs. This is even true when advanced imaging like MRI is obtained, as demonstrated in the fourth case when the initial diagnosis was thought to be a fracture of the talus.Osteoid osteomas have been previously reported within the talus by several groups -14. FindIn our four cases of osteoid osteoma of the talus described above, we opted to perform image-guided RFA due to the intricate nature of the ankle joint and the complex surrounding neurovascular anatomy. In one of these cases, even with advanced imaging the original diagnosis was thought to be a fracture of the talus, thus demonstrating the difficult nature of identifying these lesions. These imaging-guided minimally invasive procedures resulted in complete remission of symptoms within a day of the operation with a return to normal daily activities without complications in our four cases, therefore we recommend the use of imaging-guided RFA for the management of osteoid osteomas of the talus and other anatomically complex areas of the body.


A 3-year-old castrated male, American Pit Bull Terrier presented to Texas A&M University due to a 3-week mixed cerebellar and general proprioceptive ataxia, circling, head tilt, and dull mentation. Neurologic examination revealed signs of vestibular and mesencephalic dysfunction. Postmortem examination revealed a 1.1 × 1 × 0.8-cm, soft, dark red, well-circumscribed, left-sided mass, extending from the crus cerebri of the midbrain caudally to the pons. Microscopically, the neoplasm was composed of a spindle-shaped interstitial population of cells interspersed between a prominent capillary network, consistent with the reticular pattern of hemangioblastoma. Interstitial cells had strong, diffuse, intracytoplasmic immunolabeling for neuron-specific enolase (NSE) and were variably positive for intracytoplasmic glial fibrillary acidic protein (GFAP). Vascular endothelial cells had strong diffuse, intracytoplasmic immunolabeling for von Willebrand factor (VWF) glycoprotein. To date, only six cases of hemangioblastoma have been reported in canines, five in the spinal cord, and one in the rostral cerebrum. Our case may represent the first canine hemangioblastoma localized to the brainstem. Hemangioblastomas are rare, low-grade, highly vascular tumors of uncertain histogenesis . They haA 3-year-old castrated male, American Pit Bull Terrier dog presented to the Texas A&M Veterinary hospital for the evaluation of a 3-week, progressive course of mixed cerebellar and proprioceptive ataxia, left-sided circling, transient left head tilt, and dull mentation. Neurologic examination revealed delayed postural reactions in the right limbs, delayed oculocephalic reflexes bilaterally, and positional nystagmus, all consistent with central vestibular and mesencephalic dysfunction.*-weighted (T2*W) transverse plane; fluid-attenuated inversion recovery (FLAIR) transverse plane; and T1-weighted (T1W) transverse, sagittal, and dorsal plane images of the brain were obtained. Additional T1-weighted (T1W) transverse, sagittal, and dorsal plane images were acquired following intravenous administration of gadobutrol. MRI revealed a strongly contrast-enhancing intra-axial mass expanding the brainstem, which was heterogeneously T2W and FLAIR isointense to hyperintense and T1W isointense to hypointense compared to white matter  of the head was performed with the patient under general anesthesia, using a knee coil and 3T magnet . T2-weighted (T2W) transverse, sagittal, and dorsal plane; T2e matter . Multiplmorrhage . Surrounmorrhage . This mamorrhage . PrioritDirofilaria immitis) in the pulmonary artery and right ventricle. Following necropsy, the brain was fixed in 10% neutral buffered formalin for 72 h prior to sectioning. A cut section revealed a 1.1 × 1.0 × 0.8-cm gelatinous, soft, well-circumscribed, left-sided mass extended from the crus cerebri of the midbrain caudally to the pons  or, in humans, part of the autosomal dominant disorder von Hippel–Lindau disease, which leads to hemangioblastoma of the brain, retina, and other organs . PreviouThe original contributions presented in the study are included in the article/supplementary material, further inquiries can be directed to the corresponding author.Ethical review and approval were not required for the animal study because the case report is a description of a clinical case. Written informed consent was obtained from the patients/participants for the publication of this case report.All authors listed have made a substantial, direct, and intellectual contribution to the work and approved it for publication.


Epithelioid hemangioendotheliomas (EHEs), low-grade malignant tumors of vascular endothelial cell origin, are characterized by vascular endothelial proliferation. In 2002, the World Health Organization classified EHEs as locally aggressive tumors with the potential to metastasize. Currently, the diagnosis of EHE is based on pathology, histological and immunohistochemical examinations. There are no standard treatment guidelines. We here report a 69-year-old man who presented with left-sided chest and abdominal pain for more than 2 months. Enhanced computed tomography of the thorax and abdomen in another hospital suggested a mass in the left adrenal region that was considered malignant. Positron emission tomography- computed tomography in our hospital suggested a large multi-loculated, hypermetabolic, cystic mass in the left adrenal region that was considered malignant. Accordingly, a puncture biopsy of the mass was performed and the diagnosis of EHE confirmed by pathological examination, including immunohistochemical staining. This patient was treated with the programmed death 1 (PD-1) immune checkpoint inhibitor toripalimab with long-term success. The best response was stable disease (SD) with a progression-free survival (PFS) of more than 13 months. The patient is still alive now. Because the sample size of previous studies was small, further studies are needed to determine the safety and efficacy of toripalimab in the treatment of EHE. EHEs usually occur in middle-aged patients, with the median age of 36 years (range approximately 7 to 83 years) . These l9/L, liver chemistry showed the alanine transaminase (ALT) was 67.8 U/L, and the aspartate aminotransferase (AST) was 126.9 U/L. CT of the chest and abdomen showed a mass in the left adrenal region, which was considered to be a malignant lesion. The mass was adjacent to stomach, kidney, and spleen, resulting in a high risk of puncture. Positron emission tomography-CT (PET-CT) was subsequently performed, which also showed a large, multi-loculated, hypermetabolic, cystic mass in the left adrenal region, and additionally showed hypermetabolic masses in the lung, peritoneum, liver, gallbladder, and multiple lymph nodes, suggesting distant metastasis.  of the chest and abdomen. It was shown a huge mass in the left adrenal area, which was considered a possible malignant tumor, and multiple nodules in both lungs suggestive of metastatic cancer. His symptoms persisted, prompting referral to our hospital for further evaluation. The patient’s medical history included resection of a right retro-auricular meibomian cyst and left retro-auricular cystectomy. Our patient had no history of hypertension, diabetes and coronary heart disease, with no psychosocial abnormalities observed. The patient’s younger brother had a history of oral cancer. Physical examination: superficial lymph nodes were not palpably enlargement. Cardiopulmonary and abdominal examination did not show any significant abnormalities. Coagulation tests, urinalysis, stool analysis, urea, creatinine, uric acid, and an electrocardiogram were all within normal limits. Routine blood tests showed the white blood cell (WBC) was 3.35×10astasis. Figure 1WWTR1-CAMTA1 fusion gene and the other with formation of an uncommon YAP1-TFE3 fusion gene  showed the heart rate dropped gradually, sinus bradycardia was discovered. After 12 cycles of treatment, dynamic ECG was performed to evaluated the cardiac side effect and showed multiple variations: sinus rhythm; supraventricular asystole, occasionally with poor transmission and short paroxysmal supraventricular tachycardia; occasional ventricular asystole; and no significant abnormal dynamic changes in ST-T. The slowest heart rate was 47 beats/min and the fastest was 129 beats/min, with an average of 59 beats/min. No other adverse effects.In conclusion, we presented a case of advanced left suprarenal area EHE patient. The patient was treated with toripalimab and achieved a SD, with a long-term PFS of more than 13 months. To our knowledge, this is the first report of retroperitoneal EHE treated with PD-1 inhibitor. The PD-1 inhibitor toripalimab may be a promising agent for the treatment of advanced EHE. However, further prospective and controlled trials with large number of patients are needed to assess the safety and efficacy of toripalimab in EHE, as well as to find potential efficacy predictive targets.The original contributions presented in the study are included in the article/supplementary material. Further inquiries can be directed to the corresponding author.Written informed consent was obtained from the individual for the publication of any potentially identifiable images or data included in this article.YB drafted the paper. HZ, LP, and XN revised the paper. Pathological images are provided by BL, ML, LH, and FW did literature search. All authors contributed to the article and approved the submitted version.


A 47‐year‐old man with a background history of gastroesophageal reflux disease (GERD) and seasonal asthma underwent a gastroscopy for further investigation. Endoscopy revealed numerous polypoid lesions diffusely distributed in the lower third of the esophagus, with histology revealing squamous papilloma with occasional intraepithelial lymphocytes. The diagnosis was esophageal squamous papillomatosis (ESP), which is a rare condition characterized by exophytic and circumferential projections with friable mucosa diffusely spread through the esophagus with unclear etiology and malignancy risk. Esophageal squamous papillomatosis in an otherwise healthy 47‐year‐old man seen on endoscopy as part of investigation for reflux symptoms. Esophageal squamous papillomatosis (ESP) is a rare condition characterized by well‐defined and exophytic circumferential projections, with friable mucosa diffusely spread through the esophagus. The incidence of singular or limited squamous esophageal papillomas is proposed to be between 0.01% and 0.04% of the population,A 47‐year‐old man with a background history of gastroesophageal reflux disease (GERD) and seasonal asthma underwent a gastroscopy for further investigation. There was no history of regurgitation, dysphagia, weight loss, or melena. There was no relevant past medical history, and laboratory tests were unremarkable. Cross‐sectional imaging with computed tomography of the abdomen and pelvis revealed no significant anatomical findings. Endoscopy revealed numerous polypoid lesions diffusely distributed in the lower third of the esophagus with sparing of the mid and proximal esophagus Fig. . EndoscoESP is a rare condition characterized by well‐defined, exophytic, circumferential projections, with friable mucosa diffusely spread through the esophagus. The pathogenesis of ESP remains unclear. Two major theories are suggested. The first is that mucosal irritation caused by mechanical and chemical irritants results in cellular damage and then hyper‐regeneration through damage‐repair mechanisms. Approximately 70% of singular esophageal papillomas are located in the distal third of the esophagus and have been associated with reflux, esophagitis, prolonged nasogastric intubation, and nitrosamine exposure, supporting this theory.The clinical progression of singular lesions has been shown to be highly variable, ranging from regression to progression to squamous cell carcinoma.Informed consent was obtained from the patient for the use of data and images for this manuscript.


Pyriform sinus fistulas (PSFs) are rare congenital anomalies of the third or fourth brachial pouch. Dyspnea is reportedly secondary to compression by a neck mass. However, hoarseness, as the first symptom of PSF, has not yet been reported.This report describes an 11-year-old girl presenting with hoarseness as the first symptom of PSF. Hoarseness occurred 2 days prior to admission. On admission, she had fever, hoarseness, and an elastic soft mass on her left anterior neck. Contrast-enhanced computed tomography of the cervical region demonstrated an abscess partially infiltrating the thyroid gland and an air pocket near the pyriform sinus. Pharyngoscopy revealed swelling of the left arytenoid region, with purulent retention. The left vocal cord was swollen but not paralyzed. Additionally, the laboratory data indicated thyrotoxicosis. Suspecting a PSF infection, parenteral treatment with cefotaxime and dexamethasone was initiated. On the following day, the hoarseness disappeared, and the fever resolved. Four weeks after onset, the thyroid hormone levels returned to the normal range, and a barium esophagogram revealed residual contrast in the left pyriform sinus, leading to a diagnosis of PSF.PSF presenting with hoarseness as the first symptom in patients should be considered. Pyriform sinus fistulas (PSFs) are rare congenital anomalies caused by incomplete obliteration of the third or fourth brachial pouch . PSFs ofDyspnea may appear secondary to lesion compression due to a neck mass , 4. Howe3  and C-reactive protein level of 3.4 mg/dL; increased free T4 of 1.98 ng/dL [reference 1.02~1.52 ng/dL], decreased free T3 and thyroid-stimulating hormone (TSH) level of 2.71 pg/mL [reference 2.78~4.90 pg/mL] and 0.009 μIU/mL [reference 0.62~3.36 μIU/mL] respectively; and an elevated thyroglobulin level of 308 ng/mL [reference 0~33.7 ng/mL]. Contrast-enhanced computed tomography (CT) of the cervical region revealed an abscess partially infiltrating the thyroid gland and an air pocket near the piriform sinus (Fig. An 11-year-old girl was referred to our hospital with the chief complaint of a swollen left-sided neck mass. Two days prior, hoarseness began in the morning, followed by fever and sore throat in the afternoon. On the next day, neck discomfort with tenderness occurred. On admission, she had a fever of 38.2 °C, hoarseness, and an elastic soft mass with mild tenderness on the left anterior neck (Fig. Three weeks after discharge, a barium esophagogram revealed residual contrast in the left pyriform sinus, and PSF was diagnosed Fig. . HoweverThe initial symptoms of PSF include neck mass with or without abscess, acute thyroiditis, and thyroid lesion –3. In soAcute suppurative thyroiditis is a rare clinical condition in childhood because the thyroid gland is remarkably resistant to infections owing to its high iodine content, rich blood and lymphocyte supply, and protective fibrous capsule . InfectiDiagnosing PSF can be difficult owing to its rarity, and PSF should be considered when examining neck abscesses or acute suppurative thyroiditis. In the acute phase of infection, identifying the fistula tract is difficult because of swelling of the mucosa and surrounding tissues . In our Antibiotics and percutaneous drainage are commonly used to treat PSFs in the acute phase of infection . SurgicaThis study describes a rare case of PSF with acute suppurative thyroiditis with hoarseness arising as the first symptom. Therefore, the possibility of PSF in patients presenting with hoarseness as their first symptom should be considered.