Once temozolomide has failed, no other treatment is recommended for pituitary carcinomas and aggressive pituitary tumors. Recently, the use of immune checkpoint inhibitors (ICIs) has raised hope, but so far, only one corticotroph carcinoma and one aggressive corticotroph tumor treated with immunotherapies have been reported in the literature. Here, we present two cases, one corticotroph carcinoma and one aggressive prolactinoma (the first one reported in the literature) treated with ipilimumab (1 mg/kg) and nivolumab (3 mg/kg) every three weeks, followed by maintenance treatment with nivolumab (3 mg/kg every 2 weeks) in the case of the corticotroph carcinoma, and we compare them with the two previously reported cases. Patient #1 presented a biochemical partial response  and dissociated radiological response to the combined ipilimumab and nivolumab—the pituitary mass decreased from 37 × 32 × 41 to 29 × 23 × 42 mm, and the pre-existing liver metastases decreased in size (the largest one from 45 to 14 mm) or disappeared, while a new 11-mm liver metastasis appeared. The maintenance nivolumab (21 cycles) resulted in a stable disease for the initial liver metastases, and in progressive disease for the newly appeared metastasis (effectively treated with radiofrequency ablation) and the pituitary mass. Patient #2 presented radiological and biochemical progressive disease after two cycles of ICIs—the pituitary mass increased from 38 × 42 × 26 to 53 × 57 × 44 mm, and the prolactin levels increased from 4410 to 9840 ng/mL. In conclusion, ICIs represent a promising therapeutic option for aggressive pituitary tumors and carcinomas. The identification of subgroups of responders will be key. The vast majority of pituitary adenomas, recently renamed pituitary neuroendocrine tumors (PitNETs) ,2, are bHere, we report two cases. First, we present the response of a functioning corticotroph carcinoma to a different dosing regimen of combined immunotherapy with ipilimumab and nivolumab than the one already reported in literature . HYPOCare multidisciplinary reunion conducted by the French Reference Center for Rare Pituitary Diseases HYPO). Clinical, radiological, and pathological data were collected retrospectively from the patients’ medical records. All of the hormonal assays, immunohistochemistry, and methylation analyses were performed with commercially available kits. The study is in accordance with the Ethics Committee of Lille University Hospital and Limoges University Hospital. Informed consent was obtained from each patient after a full explanation of the purpose and nature of all of the procedures used, as well as for publication of the article along with accompanying images.Here, we present a case series describing the clinical management of one corticotroph carcinoma and one aggressive prolactinoma, with an emphasis on the effect of immunotherapy with ipilimumab (1 mg/kg every 3 weeks) and nivolumab (3 mg/kg every 3 weeks), followed by maintenance treatment with nivolumab alone in the case of the corticotroph carcinoma (3 mg/kg every 2 weeks). The cases were treated in two different French centers, Lille and Limoges, where they had been followed since 2001 and 2012, respectively. For both cases, the indication of immunotherapy was validated after a tumor board multidisciplinary discussion conducted at the national level  level of 212 µg/24 h (N 20-110), elevated morning plasma adrenocorticotropic hormone (ACTH) of 115 pg/mL (N < 46), and invasive pituitary tumor measuring 25 × 23 × 20 mm. The first transsphenoidal surgery (TSS), which was subtotal as a result of left cavernous sinus invasion, revealed a tumor with a Ki-67 index of 2%, absence of mitosis, and negative p53; therefore, it was classified as a grade 2a corticotroph tumor.The regrowth of the tumor residue required multiple additional treatments between 2003 and 2017, as shown in 18F-fluorodeoxyglucose (FDG) positron emission tomography (PET)/CT showed an intense uptake by the pituitary mass, the skull , and three hepatic lesions; there was also a suspected T2 vertebral lesion. The abdominopelvic CT showed five hepatic lesions, with maximal diameters of 31, 22, 12, 7, and 5 mm. A liver biopsy was performed and showed the presence of a corticotroph tumor metastasis  expression), confirming the diagnosis of pituitary carcinoma. The pituitary and thoraco-abdominopelvic CT repeated in February 2020 showed the progression of the pituitary mass (anteroposterior diameter: 37 mm vs. 32 mm) and of the two largest liver metastases . The evolution of the pituitary mass and of the liver metastases is presented in In November 2018, the patient was admitted for asthenia, weight loss (10 kg), accentuation of melanoderma, right ptosis, and diplopia due to right third and sixth cranial nerve palsy. The plasma ACTH varied between 3957 and 11,191 pg/mL, compared with 398 pg/mL in May 2018. The pituitary computed tomography (CT) showed the global stability of the pituitary mass compared with the pituitary magnetic resonance imaging (MRI) performed in February 2018. The 18F-FDG-PET/CT showed a very positive metabolic response for the known metastases and the disappearance of the suspect T2 vertebral lesion, but the appearance of a new focal liver uptake was noted, corresponding to a new 11 mm lesion on the CT (described only afterwards on the CT). A concomitant improvement of melanoderma and right ptosis were observed, the diplopia disappeared, and the body weight stabilized. The patient did not report any side effects from the combined immunotherapy other than asthenia on the day of administration.Immunotherapy with ipilimumab (1 mg/kg) and nivolumab (3 mg/kg) every 3 weeks was initiated in February 2019, and was administered for five cycles. A very rapid and significant decrease of plasma ACTH was observed after the first cycle, from 13,813 to 1036 pg/mL. The plasma ACTH continued to further decrease (nadir of 549 pg/mL after three cycles) and remained < 1000 pg/mL during the five cycles of combined immunotherapy. Regarding the radiological response, after three and four cycles, the pituitary mass decreased in size from 37 × 32 × 41 mm to 29 × 23 × 42 mm, while the five known liver metastases decreased in size from 45 to 14 mm, from 30 to 13 mm, from 12 to 5 mm, or were not visible anymore . The 18F-FDG-PET/CT, and a decrease of plasma ACTH were observed. The three other liver metastases remained stable, measuring 12, 8, and 4 mm. At the last follow-up in April 2020, the plasma ACTH was stable (1530 pg/mL) in comparison with March, but higher than in February (1250 pg/mL). The patient died four days later of an unknown cause. As potential side-effects of nivolumab, the patient reported asthenia, anorexia, and progressive weight loss (5 kg).Since May 2019, the patient received maintenance treatment with nivolumab alone (3 mg/kg every 2 weeks). After the fourth cycle of nivolumab, the right ptosis worsened again, concomitantly with a progressive increase of plasma ACTH . The increase in the pituitary mass (from 29 × 23 × 42 to 29 × 25 × 45 mm in July 2019) was initially considered to be non-significant, however the pituitary CT performed in February 2020 confirmed the local progression (38 × 28 × 54 mm). Regarding the liver metastases, in November 2019, the thoraco-abdominopelvic CT showed the progression of the liver metastasis that became visible after the start of the immunotherapy (22 mm vs. 14 mm in July and 11 mm in May), whereas the other liver metastases were stable, measuring 12, 11, and 4 mm. Radiofrequency ablation of this liver metastasis was performed in January 2020, and 1-2 months later, the concurrent stigma of the radiofrequency ablation on the CT scan, a complete metabolic response of the hepatic lesion on the Of note, for the hypercortisolism, the patient also received ketoconazole and mitotane , and after relapse, metyrapone 1000-3000 mg/day from August 2015 to December 2018 , and mitotane up to 2000 mg/day since February 2017 (side-effects: anorexia and vertigo).A 60-year-old male presented in August 2012 with visual disturbances and bitemporal hemianopsia, and was diagnosed with a prolactinoma based on the presence of an invasive pituitary tumor measuring 36 × 31 × 26 mm, and a prolactin level of 5130 ng/mL (N 2.30-14.7). Cabergoline 0.5 mg × 3/week was started, and then increased to 0.5 mg daily from January 2013, enabling a decrease of prolactin to 1330 ng/mL, concurrent with a 50% decrease in tumor volume in March 2013. Cabergoline was further increased and maintained at 1 mg daily from June 2013. Despite an initial further decrease in the tumor volume and recovery of visual signs (nadir prolactin level of 787 ng/mL), the patient presented with the recurrence of the left temporal hemianopsia in December 2015. The pituitary MRI showed rapid tumor regrowth between July and December 2015 (from 7 × 19 × 17 mm to 30 × 25 × 25 mm). The TSS performed in January 2016 was subtotal as a result of left cavernous sinus invasion, and revealed a proliferative tumor , and was therefore classified as a grade 2b prolactinoma. Adjuvant fractionated RT (50.4 Gy in 30 fractions) was performed in 2016. 18F-FDG PET/CT performed in June 2019 nor the medullary MRI performed in August 2019 found any metastasis.Following the first TSS, the patient was kept on 1 mg of cabergoline daily. At the end of 2018, the patient presented again with rapid tumor progression, with optic chiasm compression, for which a second TSS was performed in January 2019 . After surgery, cabergoline was further increased to a maximum of 10.5 mg/week and pasireotide 0.6–0.9 mg twice daily was administered for two months . The administration of temozolomide was decided, but due to the rapid tumor progression with a worsening of the visual field, decreasing visual acuity, and involvement of the left third cranial nerve, before starting temozolomide, a third TSS was performed in June 2019 . Neither the The cabergoline was interrupted in July 2019, being considered ineffective given the rapid progression despite very high doses, and six cycles of 220–290 mg of temozolomide per day for 5 out of 28 days were administered starting at the end of July 2019. The tumor progressed under temozolomide, with worsening of the left cavernous sinus syndrome , a significant increase in the tumor size seen on the MRI performed in September 2019 (39 × 43 × 27 mm compared with 20 × 20 × 23 in June 2019), and a concurrent significant increase in prolactin levels. Then, 1 mg of cabergoline daily was reintroduced, enabling a decrease in prolactin levels, which, nevertheless, remained higher compared with the moment when the temozolomide was started. An improvement in the left cavernous sinus syndrome was also noted (possibly due to the methylprednisolone as well), but with the MRI performed in December 2019 not showing any regression in the pituitary mass (38 × 42 × 26 mm).Given the inefficacy of temozolomide, the administration of immunotherapy with ipilimumab (1 mg/kg) and nivolumab (3 mg/kg) every three weeks was decided. The administration of methylprednisolone was progressively decreased, and stopped at the end of December 2019 in order to start the combined immunotherapy, while cabergoline was maintained at 1 mg daily. Unfortunately, the combined immunotherapy resulted in rapid progression, with the prolactin levels increasing from 4410 ng/mL before initiation to 9840 ng/mL after the first two cycles, and with the size of the pituitary tumor increasing from 38 × 42 × 26 mm in December 2019 to 53 × 57 × 44 mm in March 2020. Left ptosis also reoccurred. After the two cycles, the patient also presented severe side-effects , and therefore the combined immunotherapy was stopped. Starting at the end of March, 15 mg/kg of bevacizumab was administered every three weeks, with, so far, a stabilization of prolactin levels (~8830 ng/mL in April and May 2020), a stable disease shown in the MRI, and good tolerance .HGF gene, deletion of the CDKN2A/B gene, mutation (p.Ser514*) of the BCORL1 gene, mutation (p.Asn2fs*52) of the FLCN gene, and mutation (p.Lys666Glu) of the SF3B1 gene), and (2) next-generation RNA-sequencing in March 2020—Archer® FusionPlex® CTL Panel —revealing the absence of mutations or of fusion transcripts.Of note, the somatostatin receptor type 5 (SST5) immunohistochemistry performed in January 2020 on the tumor block from January 2019 showed the absence of staining, which may partly explain the lack of an effect of pasireotide . At the ICIs block the inhibitory signals of T lymphocyte function and/or activation, and, more precisely, they block CTLA-4 or PD-1, which are found on T lymphocytes or its ligand, programmed death ligand 1 (PD-L1), which is found on antigen-presenting cells and on tumor cells. These inhibitory signals would otherwise enable tumors to evade immune response. By blocking them, ICIs enhance T lymphocyte function and reactivate antitumor immune responses ,14,15. Recent studies on the tumor microenvironment of PitNETs  have demSo far, only two cases of corticotroph tumors have been treated with ICIs ,8. TableAs limited experience is available on the use of ICIs in pituitary tumors, we think that response to treatment should be evaluated on a case-by-case basis. Given the regression of the pituitary mass and of all five pre-existing liver metastases, we consider patient #1 to be a responder to combined ipilimumab and nivolumab, instead of classifying the response as progressive disease based on the new liver metastasis that appeared after the four cycles of combined immunotherapy, as we could have done based on the iRECIST guidelines . Althougp = 0.01, of which only one patient was <55 years old [Moreover, besides a complete/partial response, stable disease, and progressive disease, one should be aware of two additional patterns of tumor response when using immunotherapy, namely: pseudoprogression  ,21 and hears old . PatientRegarding the side-effects of ICIs, combined immunotherapy, besides being more effective, also increases treatment-related adverse events . In the So far, there is not enough data to enable any formal conclusion on the efficacy of ICIs in pituitary carcinomas and aggressive PitNETs, or on which subgroups of patients will prove to respond, but ICIs appear to be a promising therapeutic option. At the moment, there are two clinical trials running on combined ipilimumab and nivolumab, namely: NCT04042753, a clinical trial dedicated to pituitary carcinomas and aggressive PitNETs; and NCT02834013, a basket trial that also accepts pituitary carcinomas. Hopefully, their results will provide valuable information on the efficacy of ICIs in these tumors. At the same time, potential leads for the improvement of ICI efficacy emerging from studies on other cancers, such as the combination of ICIs with drugs targeting angiogenesis ,25 or wi


Angiolipoma, distinguishable from other lipomas by its excessive degree of vascular vessels, are rare in the head and neck and require unique management. A slow growing mass, located underneath the inferior border of the right mandibular angle of a 51-year-old female, was excised under general anesthesia. Unexpected excessive bleeding during the excision was observed and the histological specimen was diagnosed as angiolipoma. As shown in this case report, pre-operative imaging modalities have a crucial influence and are sufficient to diagnose and manage angiolipomas. The “Gold standard” treatment is excision with clear margins and bleeding management should be taken into account according to appropriate differential diagnosis. Lipomas are the most common benign mesenchymal tumors, composed of mature adipocytes. They are defined as a well-defined mass, surrounded by thin fibrous capsule, usually asymptomatic, slow-growing and soft . LipomasUnlike the majority of lipomas, which are encapsulated, angiolipomas may be either encapsulated or non-encapsulated. Moreover, they can be differ from other lipomas by the excessive degree of vascular proliferation mixed with mature adipocytes, as well as their ability to infiltrate the surrounding tissues.The present article reports clinical, radiological, and histological features of an angiolipoma involving posterolateral aspect of the neck of a 51 years old female.In May 2019, a healthy 51-year-old female was referred to the Rabin Medical Centre for the evaluation of a tender mass located 2 cm underneath the inferior border of the right mandibular area, in the posterolateral aspect of the right neck. The patient denied tobacco, alcohol, and illicit drug use. Thirteen years previously, she noticed a small mass in the same area that had progressively slowly increased in size and presented an aesthetic disturbance. Over the weeks prior to her referral, the mass had become tenderer to palpation and raised concern. Clinical examination revealed well-circumscribed, firm and mobile mass, of 4 cm diameter, located in the posterolateral aspect of the right neck. The mass was separated from the surrounding tissues and the overlying skin. There was no audible bruit or liquid flow that suggested a vascular nature. Facial nerve function and cervical lymph nodes were examined and found to be normal. Intra-oral examination revealed normal salivary flow from the orifices of the glands and normal soft tissue appearance. The preoperative clinical findings suggested a benign tumor, favoring lipoma.Neck ultrasound (US) examination, conducted in August 2006 and May 2007, revealed a well-defined solid hypo-echogenic subcutaneous mass located in the right mandibular angle, 0.93 × 2.52 cm in size. Fine-needle aspiration (FNA), performed in March 2007, exposed blood cells but did not yield diagnostic material or a definitive diagnosis. Following nine additional years without adequate medical follow up, the patient was referred to additional neck US in March 2016, which revealed increase in lesion size to 1.45 × 4 cm, with 1.85 cm depth. Moreover, the mass was defined by the radiologist as well defined; heterogenic; cystic and hypo echogenic which evolve blood flow inside and around the mass.Pre-operative contrast-enhanced Computed Tomography (CT) scanning performed around the referral time, revealed a 4 cm diameter, well-demarcated, middle-intensity (between liquid and soft tissue) homogeneous mass adjacent to the parotid gland and the posterior edge of masseter muscle . The radUnder general anesthesia surgery, performed 3 months following her referral, the mass was excised, performing a sub-platysma dissection and managing excessive bleeding during the surgery. The mass was located underneath the posterior edge of the masseter muscle, in contact with the right posterior digastric muscle and with the superficial parotid lobe. Clinical findings revealed non-encapsulated, reddish and lobulated mass . Post-opHistopathologic examination of non-encapsulated, most probably infiltrating intramuscular angiolipoma. Microscopically, the tumor was composed of convolutes thin-walled blood vessels, with clusters of mature adipocytes, and a prominent perivascular arrangement of the adipose tissue .Lipomas, the most common mesenchymal tumors of soft tissue, are uncommon in the head and neck region . RepreseAngiolipoma are benign mesenchymal tumors composed of mature lipocytes and vessels representing 5% to 17% of lipomas ,13. AngiPhysical examinations of the neck that reveal soft, mobile, tender, and slow growing masses, should be considered by the physician under the following differential diagnosis: hemangioma; lipoma; Kaposi’s sarcoma; angiosarcoma; leiomyoma; neurilemma ,20. VariUse of imaging modalities aid the surgeon to localize and make a first differential diagnosis although definitive diagnosis is achieved by histopathology and can differ from clinical diagnosis, which occurred in the case presented. Following the histopathology diagnosis, wide local excision with free margins is the suitable treatment for infiltrating angiolipoma. Surgical excision is curative for noninfiltrating lesions, as the recurrence rate is very low if excision is adequate. However, infiltrating angiolipomas have up to a 50% rate of recurrence . CarefulUsing imaging modalities, such as US, FNA, contrast-enhanced CT and MRI, aids the surgeon to localize and make a first differential diagnosis of neck masses. Careful dissection must be achieved to avoid damage to surrounding structures and achieve proper hemostasis. Surgical excision with free margins is the “Gold standard” treatment and definitive diagnosis is achieved by histopathology.


Composite meshes are used for incisional hernia repair because they enable intraperitoneal mesh placement due to their dorsal surface, which is made of inert material. We report, for the first time, to our knowledge, a case of composite mesh migration detected incidentally during a laparotomy for colon cancer in an asymptomatic patient.Our patient was a 71-year-old South Asian man who underwent ventral mesh repair following a postoperative complication after right hemicolectomy for colon cancer. The patient was diagnosed with a metachronous sigmoid cancer 5 years later, for which he underwent laparotomy. During laparotomy, a migrated mesh was incidentally found and extracted from his proximal ileum without any evidence of abscess or fistula formation.To our knowledge, this is the first report of an incidentally found migrated composite mesh from a bowel lumen in an asymptomatic patient. Use of mesh has become standard in a majority of incisional hernia repair to achieve a tension-free repair and reduce recurrence . The comA 71-year-old South Asian man who had under gone a right hemicolectomy for an adenocarcinoma of the colon was found to have a metachronous cancer in the sigmoid colon during surveillance colonoscopy. Following his primary surgery at another institution, he has developed an anastomotic leak during the immediate postoperative period, for which a second laparotomy had been performed on day 5 with a revision of the anastomosis. During the first postoperative week, he had developed an enterocutaneous fistula with primary wound failure. His case was then taken over by the authors for the management of the fistula, which was done nonoperatively. Spontaneous healing of the fistula took 10 months, after which the patient underwent an abdominal wall reconstruction with an inlay polypropylene/polyglactin 910 composite mesh. The reconstruction was done 16 months after the primary surgery, and the mesh was fixed using polypropylene sutures. The patient remained asymptomatic until he was diagnosed with a sigmoid cancer on the basis of surveillance colonoscopy 5 years after the primary event. Following histological confirmation and staging with contrast-enhanced computed tomography, he was scheduled for laparotomy with subtotal colectomy and ileorectal anastomosis. During surgery, several loops of bowel were found to be tightly adherent to the anterior abdominal wall. On palpation, a hard, elongated mass was felt inside one of the bowel loops in the proximal ileum. After separating the loops from the posterior rectus sheath, an enterotomy was made to find the mesh inside the bowel lumen Fig. . There wet al. reported the first case of mesh migration following incisional hernia repair [et al. reported one case of composite mesh migration into the small bowel under experimental conditions in a murine study [There have been few cases of composite mesh migration reported in the literature, although the patients have been symptomatic in every instance, with a complication such as enterocutaneous fistula, bowel obstruction, or intra-abdominal mass formation , 5, 6. Ta repair . A foreia repair . The miga repair . It is pa repair . A slow ne study . Mesh mine study , 11, 12.ne study .Incisional hernia repair involves the use of novel mesh devices designed to reduce complications. Composite meshes are designed to prevent bowel adhesion by using an inert material to the dorsal surface, although several cases of mesh migration have been reported. Reports of composite mesh migration are all associated with patients presenting with either infective or obstructive symptoms. This case report, for the first time, to our knowledge, raises the possibility of complete migration of a composite mesh without causing symptoms or signs.


Recurrent forms of gastrointestinal stromal tumor (GIST) include liver metastases and peritoneal dissemination. Recurrence often occurs within 2 years. We report a case of liver metastasis, which was detected 30 years after resection of a primary lesion in the small intestine and was resected 32 years later.The patient was a 72-year-old woman and was being followed up for ureteral stones at the department of urology of our hospital. Computed tomography (CT) showed a small mass in segment 7 of the liver, 2 years ago. As the tumor gradually increased, a biopsy was performed, and a mesenchymal tumor was diagnosed. The tumor continued to increase in size and partial hepatectomy was performed. GIST was suspected from the sample extracted during hepatectomy. The patient had undergone a resection of the small intestine for a tumor 32 years ago. On tracing her medical records, it was confirmed that resection of the small intestine was performed for the diagnosis of leiomyosarcoma.Based on the block specimen from 32 years ago, the tumor of the intestine was confirmed to be GIST, and the liver mass was finally diagnosed as liver metastasis of the GIST that had occurred 32 years ago.We experienced a case of liver metastasis 32 years after surgery for the first small intestinal GIST. To the best of our knowledge, this case had the longest disease-free interval before metastasis to the liver. We also discuss other cases of delayed liver metastasis of GIST.This paper has been reported in line with the SCARE criteria .2A 72-year-old-woman with no symptoms was referred to our department for a tumor in the liver detected on CT-scan during the follow up for urinary stone. She had a history of resection of the small intestine 32 years ago for a tumor (Details of the disease were unknown). A slightly smaller lesion was seen in the previous CT scans taken at our hospital. Hemato-biochemical findings showed no abnormalities. There was no increase in the tumor markers for gastrointestinal cancer or hepatocellular carcinoma. Abdominal ultrasonography showed a tumor in segment 7 of the liver, which was hypoechoic but was difficult to diagnose on radiology. Abdominal contrast-enhanced CT showed a mass 2.4 cm in size with slight enhancement in segment 7 of the liver . There wAlthough HCC and metastatic liver cancer were suspected based on the above findings, there were no lesions suggestive of a primary lesion on gastroscopy and colonoscopy. Hence, a needle biopsy of the liver was performed, which revealed a mesenchymal lesion that is not usually seen in the liver.Therefore, a surgery was planned. The tumor was located in segment 7 of the liver, and partial resection of segment 7 of the liver was performed . The posBased on the above results, her past medical records were obtained from the institute where she was operated 32 years ago, and it was found that the small intestine was resected based on the diagnosis of a leiomyosarcoma of the small intestine, and the block specimen from that time was available. The results of the block specimen were compared with those of the resected lesion at our institute, and the results were almost identical. Since primary GIST in the liver is extremely rare and PET CT scan showed no other GIST lesion that could have caused liver metastasis, the lesion was finally diagnosed as liver metastasis of small intestinal GIST that occurred 32 years ago. Adjuvant therapy was also considered, but it was not administered at the patient's request, and the patient has been free from recurrence for 5 years.3GISTs account for 0.2–0.5% of all tumors of the digestive tract and have the highest incidence (approximately 80%) among mesenchymal tumors ,7. TheseIn this case, the possibility of GIST was suspected from the pathological analysis of the specimen obtained on biopsy of the liver lesion. However, primary GIST of liver has been rarely reported in the past ,11. An iCases of liver metastases from gastrointestinal GIST more than 10 years later have sometimes been reported in the Japanese and other literature  [[12], . AlthougConsidering the long-interval before recurrence, the period for follow up of GIST after surgery needs to be determined. Japanese guideline  of GIST In this case, adjuvant chemotherapy after resection of the hepatic metastasis was not performed because of the patient's reluctance. Nunobe et al.  reported4We experienced a case of liver metastasis 32 years after surgery for the first small intestinal GIST. Recurrence of late liver metastasis can occur sometimes, and it is necessary for the attending physician to be aware of it and to educate the patient accordingly.The authors report no declarations of interest.No source of funding.We have reported a single case, not a clinical study, with no requirement for ethical approval.Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal on request.Dr Masahiro Ishizaki: Investigation, Writing-original draft, Writing-Review and Editing, Visualization.Dr Futoshi Uno: Review and Editing.Dr Ryosuke Yoshida: Review and Editing.Dr Shunsaku Miyauchi: Collecting datas, Review and Editing.Dr Osamu Honda: Review and Editing about radiological materials.Not applicable.Dr Masahiro Ishizaki.Not commissioned, externally peer-reviewed.


Appendiceal mucocele (AM) is a very rare neoplasm with an incidence of 0.2–0.3% of all appendectomy specimens. AM usually defined as an intraluminal dilation of the appendix in response to mucin accumulation. The preoperative diagnosis is difficult and can be misdiagnosed to any of the other differential diagnosis especially in female patients. If it is suspected, imaging modalities and colonoscopy can be helpful as a guide for the right surgical approach.A 41-year-old lady who found to have an appendicular tumor discovered incidentally on magnetic resonance imaging (MRI) of the abdomen during a follow-up in the referral hospital for persistent right lower quadrant abdominal pain with enlarging right ovarian cyst for 2 years. MRI revealed a sizeable cyst measuring 7 × 4 × 3 cm in the right iliac fossa (RIF) region with high suspicion of an AM. The patient was managed with open right limited hemicolectomy. The patient was diagnosed as a well-differentiated low-grade AM neoplasm with stage 0  based on the 8th edition of the American Joint Committee on Cancer (AJCC) Staging System. The patient had an uneventful recovery and advised for regular follow-up of at least 5 years.AM is a very rare neoplasm. The imaging modalities that can be diagnostic methods are the abdominal ultrasonography (US), transvaginal US, abdominal computed tomography (CT) and abdominal MRI. Fine needle aspiration must be avoided as the risk of perforation is high that will lead to dissemination of the mucinous material causing a serious complication called pseudomyxoma peritonei. Laparotomy is superior on laparoscopy as the risk of perforation is lower in laparotomy.The presentation of AM is usually not specific, because of its anatomic position. Physicians should consider it in the differential diagnosis of persistent enlarging ovarian cyst or adnexal mass. This condition overall is very rare with a 0.2–0.3% incidence of all appendectomy specimens . It’s mo29/L, haematocrit: 35.3%, and platelet count: 280 × 109/L. The liver function test, renal function test, and the coagulation profile were all within normal ranges. Cancer antigen markers including carcinoembryonic antigen and carbohydrate antigen 19.9 were all within the normal range. Colonoscopy was done as a preoperative workup and was unremarkable. The patient underwent laparoscopic exploration which confirmed the presence of AM involving the base of the appendix, inseparable from the caecum. We did not find any synchronous neoplasms in the colon. The case was then converted to open exploration through a lower midline laparotomy incision which showed a cystic mass measuring 9 × 3 × 3 cm involving most of the appendix including the base inseparable from the caecum, sparing 2 cm tip with multiple palpable ileocecal mesenteric lymph nodes. The cystic lesion seems about to rupture at the center. A Limited right hemicolectomy with side to side ileocolic stapler anastomosis was performed. Gross examination revealed a dilated appendix with smooth shiny serosa and thin wall, and it is filled with thick mucus material  based on the 8th edition of the AJCC Staging System  and in a chronic setting 15–29%) .5% and i. Usually% .5% andPreviously, the AM was classified histologically into: simple mucocele, hyperplastic mucocele, mucinous cystadenoma and mucinous cystadenocarcinoma ,9,10.Nowadays, it is classified based on histopathological examination according to the degree of atypia into low-grade appendiceal mucinous neoplasms or high-grade appendiceal mucinous neoplasms ,11.The management of AM is a comprehensive process starting from suspicion of AM, proper workup, right diagnosis, choosing the proper treatment then follow up. The only definitive and curative treatment of AM is surgical resection. In the literature, there is no total agreement on the best surgical approach that can be done to treat the AM. Many circumstances and factors determine the proper surgical technique. The aim of surgery is to resect the mucocele properly and not to cause perforation and spillage of the content causing pseudomyxoma peritonei, a life-threatening complication with less than 45% of 10-years survival and less than 20% of 5-years survival . Laparot4AM is a rare neoplasm in abdominal surgery. The abdominal US, abdominal CT, and abdominal MRI represent useful tools for diagnosis. However, the preoperative diagnosis is difficult and can be misdiagnosed. Appropriate diagnosis and management of AM prevent complications such as pseudomyxoma peritonei. All cases identified to have AM should be assessed for synchronous colonic neoplasms. Colonoscopic surveillance of patients for synchronous colonic neoplasms is warranted.The authors report no conflicts of interest.This study did not receive any funding from governmental or private organizations.This is a case report and it didn’t require ethical approval from ethics committee according to our institution.Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal on request.Study concept or design – AOS, MYD, SB.Data collection – AOS, MYD, MS, SB.Data interpretation – AOS, MYD, MS.Literature review – AOS, MYD, MS, SB.Drafting of the paper – AOS, MYD, MS.Editing of the paper – AOS, MYD, SB.Not required.Mohammed Yousef Aldossary.Not commissioned, externally peer-reviewed.


Primary lymphomas of the colon account for 0.5% of all primary colon malignancies. Burkitt´s lymphoma is a B-cell lymphoma with aggressive clinical behavior. Herein, we describe a case of a male patient who presented with signs of large bowel obstruction, underwent surgery and found to suffer from Burkitt´s lymphoma of the ileocecal region. The histopathological examination was indicative for Burkitt´s lymphoma. To the best of our insight this is one of the few reported cases of such type of lymphoma in an adult patient presenting with bowel obstruction. Burkitt´s lymphoma is a rare malignancy in adults affecting gastrointestinal tract. It has a high proliferation potential and can rapidly progress to advanced disease. Early diagnosis is necessary to prevent complications and improve overall prognosis. Primary lymphomas of the colon account for 10-20% of all gastrointestinal lymphomas and approximately 0.5% of primary colon malignancies. Most gastrointestinal lymphomas are of B-cell origin that also applies to Burkitt´s lymphoma (BL), an aggressive type of malignancy which usually is diagnosed in children and immunocompromised patients, with few cases reported in middle-aged or elderly people . Herein,A previously healthy 63-year-old Caucasian male patient, with no significant past medical history, presented to the emergency department suffering from abdominal distension, nausea and vomiting. The aforementioned symptoms started abruptly approximately six hours before his admission to the hospital. He denied taking any prescription or over-the-counter medications. Two years prior he had undergone lower gastrointestinal endoscopy, which showed no mucosal abnormalities. Clinical examination revealed a well-nourished male patient with no previous abdominal surgical scars. The patient was diaphoretic and his vital signs were as follows: blood pressure 100/75mmHg, 115bpm, SaO2 95%, temperature 37.4°C. Blood counts were within normal limits, except for creatinine levels reaching 1.7mg/dl. The rest of biochemical profile showed nothing remarkable. Significant abdominal dissension was noted along with hyperactive bowel sounds on auscultation. X-rays of the chest and abdomen were indicative for large bowel obstruction, a finding that was confirmed by computed tomography (CT) scan , which aThe patient was taken to the operating room after written consent was obtained. He underwent open right hemicolectomy with en-mass resection of a small part of abdominal wall with primary anastomosis of terminal ileum and transverse colon. His postoperative course was uneventful and he was discharged five days after the surgical operation. Histopathological examination was indicative for extensive malignant infiltration of the colon and terminal ileum from Burkitt’s lymphoma with small atypical lymphocytes with round to irregular nuclei, prominent nucleoli and scant cytoplasm consistent with the pathognomonic “starry sky” appearance of BL. Immunochemistry reported positive results for CD10, CD20, Pax-5, bcl-6 and negative for markers like CD5, CD23, cyclin-D1 and CD30. The cytogenetical analysis revealed proto-oncogene (c-myc) rearrangement (>80%), BCL-2 (-), Ki-67 100% . The speBurkitt´s lymphoma is a highly aggressive malignancy usually affecting children and young adults. In more elderly population its incidence is low, with patients 20-30 years of age showing a figure of 0.6 per million . The firBL is notable for remarkable uniformity of nuclear size and contour along with chromosomal 8: 14) translocation and overexpression of c-myc, adopting a pattern of characteristic 'starry-sky' pattern with sheets of monomorphic neoplastic lymphoid cells and interspersed histiocytes. Burkitt´s lymphoma typically expresses pan-B-cell antigens like CD19, CD20, CD22, and CD79a and co-expresses CD10, Bcl-6, CD43 and p53. On the other hand, BL shows negativity for CD5, CD23, Bcl-2, CD138, or TdT. The proliferation fraction is nearly 100% [: 14 tranIn conclusion, BL is a rare malignancy in adults affecting gastrointestinal tract, mainly in ileocecal region. Thanks to the better understanding of the biology of BL, there has been significant improvement in chemotherapeutic drugs. In the era of modern chemotherapy, BL may readily respond to treatment, provided that it is diagnosed before extensive dissemination of disease occurs. Therefore, a high clinical suspicion along with prompt diagnosis and treatment based on multidisciplinary approach is required. Surgical therapy may play a role in the management of advanced complicated cases such as bowel obstruction or perforation, but chemotherapy remains the mainstay of treatment.


Occasionally, acute ischemic stroke can be difficult to differentiate from acute intracranial infection. We describe a patient who presented with sudden onset of right hemiparesis and fever. Magnetic resonance imaging (MRI) was consistent with an acute stroke, showing multiple lesions with restricted diffusion in the left middle cerebral artery territory. These lesions were not enhancing and were not associated with vasogenic edema. A diagnosis of acute stroke was made based on the clinical and radiographic data. Follow-up MRI obtained eleven days later showed interval development of ring enhancement and vasogenic edema surrounding the previously noted core of restricted diffusion. Based on these findings, the diagnosis was revised to cerebral abscesses and the patient was treated successfully with antibiotics. In retrospect, the largest diffusion-weighted lesion on baseline MRI demonstrated two characteristics that were atypical for stroke: it had an ovoid shape and a subtle T2 hypointense core. This case demonstrates that acute clinical and radiographic presentation of cerebral abscess and ischemic stroke can be strikingly similar. Follow-up imaging can be instrumental in arriving at an accurate diagnosis. Brain abscesses typically present with progressive headache, an altered level of consciousness, progressive focal neurologic deficits, and/or seizure . OccasioA 65-year-old man with a past medical history of diabetes mellitus developed sudden weakness of the right arm and leg. He presented the following day to our hospital; on physical examination, he was found to have a fever of 38.7 °C, mild right hemiparesis , right facial palsy, and dysarthria. He scored four points on the National Institutes of Health Stroke Scale. His cardiac examination findings, including echocardiography (EKG) and transthoracic echocardiography, were unremarkable. MRI of the brain was performed and multiple high signal intensity lesions were seen in the left frontal and parietal lobes on T2-weighted MRI and on diffusion-weighted imaging (DWI) with an associated low signal on the apparent diffusion coefficient (ADC) map . We subsOver the next several days, the patient did well and was presumed to have experienced an embolic stroke as well as bronchitis complicated by septicemia. On the eleventh day after symptom onset, the patient experienced a focal motor seizure involving his right hand and follow-up MRI of the brain was performed. The T2-weighted MRI was notable for interval development of extensive vasogenic edema surrounding the left-sided brain lesions and the post-gadolinium T1-weighted MRI images demonstrated the ring enhancement of these lesions . Based oThis case illustrates that the clinical and radiographic presentation of a patient with evolving cerebral abscesses can be confused with cerebral infarction. Cerebral abscesses can present with acute focal “stroke-like” symptoms and the MRI may demonstrate cerebral lesions with restricted diffusion and no contrast enhancement, mimicking acute infarcts. Although cerebral abscesses are rare compared with ischemic infarcts, knowledge of the potential similarities in both the clinical and imaging presentation of these disorders can facilitate accurate diagnosis and prompt initiation of appropriate treatment.The typical symptoms of brain abscess include slowly progressive headache with an alteration in level of consciousness and neurological deficits. This case illustrates that some patients, however, present with acute stroke-like symptomatology. This is consistent with previously reported cases of an acute stroke-like presentation of cerebral abscesses ,3,4,5. ADespite the striking similarities that can exist between evolving cerebral abscesses and acute cerebral infarcts, as illustrated by our case, there are a few subtle radiographic findings that might, in retrospect, have helped to establish the correct diagnosis. First, the dominant left parietal lesion (see magnified view in the last column of


Gastric hamartomatous inverted polyp (GHIP) is a pathological condition where enlarged gastric glands with cystic dilatation grow in the submucosa. It is difficult to excise the tissue due to its location. In addition, even if the tissue is taken correctly, making an accurate diagnosis is difficult due to foveolar epithelium in the tissue, which can be misdiagnosed as gastric mucosal epithelium. Thus, an accurate diagnosis of GHIP is rarely established from a biopsy alone preoperatively. We here report a case of GHIP with a central dimple, which was diagnosed and treated using a modified combination of laparoscopic and endoscopic approaches to neoplasia with a non-exposure technique (modified CLEAN-NET).A 60-year-old man with a submucosal tumor (SMT) in the stomach was referred to our hospital by a primary care doctor. On examination, a gastrointestinal stromal tumor was suspected. Modified CLEAN-NET was performed for diagnostic and therapeutic purposes. The histopathological examination of the resected specimen showed an enlarged gland duct in the submucosal layer. This finding, along with immunostaining results, led to the diagnosis of GHIP. The postoperative course was uneventful without any symptoms.GHIP should be considered among the differential diagnoses of SMT of the stomach. Modified CLEAN-NET may be beneficial in the removal of SMTs such as GHIP with a central dimple because it can avoid stomach deformation of the stomach and tumor dissemination. Gastric hamartomatous inverted polyp (GHIP) is characterized by marked submucosal glandular proliferation associated with cystic dilation . It is dAn asymptomatic 60-year-old man was referred to our hospital because of a submucosal tumor (SMT) of his stomach found by an upper gastrointestinal endoscopy at a regular health check. The patient had been on medications for hypertension and old cerebral infarction. The upper gastrointestinal endoscopic examination revealed a gastric SMT with a central dimple at the posterior wall of the upper gastric body Fig. a. EndoscA camera port was inserted through an umbilical incision, then four additional ports (three 5-mm ports and one 12-mm port) were inserted into the upper abdomen in the shape of an inverted trapezoid under a pneumoperitoneum of 10 mmHg. The location of the SMT was not detected from the abdominal cavity. Therefore, endoscopy was used to identify the tumor location. The tumor was located at the posterior wall near the cardia of the stomach. We opened the omental bursa and used anchor sutures around the tumor to secure the surgical field. We then marked the margin line around the SMT from outside the stomach by pushing the margin with the endoscope , the pyloric ring, or the lesser curvature is sometimes technically demanding, because the likelihood of the occurrence of postoperative stenosis caused by deformity of the stomach is higher in these locations. In addition, a tumor located on the posterior wall was also reported to be difficult to remove by CLEAN-NET, due to its inaccessibility . FujishiModified CLEAN-NET is one of the potential treatment modalities for GHIP, in terms of conducting en bloc resection because it avoids dissemination, is minimally invasive, and has a reduced chance in resulting in deformity of the stomach.In this case, we found modified CLEAN-NET to be a feasible procedure with a non-exposure technique for GHIP with a central dimple that was not able to be removed orally. The clinical application of modified CLEAN-NET will be expanding to various types of tumors by taking account of its advantages and limitations.


EWSR1-CREB family gene fusions have been reported in young patients with histologic features reminiscent of the myxoid variant of angiomatoid fibrous histiocytoma. Here, we report 2 cases of adult males with midline intracranial EWSR1 myxoid neoplasms. Patient 1 (age 37) presented with headaches and MRI demonstrated an enhancing right thalamic mass extending into the quadrigeminal cistern. Near-total resection was achieved, and residual disease was treated with stereotactic radiosurgery (SRS). Five-year follow-up is negative for recurrence. Patient 2 (age 28) had a past medical history significant for high-grade B-cell lymphoma of his femur and kidney for which he received systemic and intrathecal treatment. He presented to our institution with headaches, visual changes, nausea, and vomiting, and MRI demonstrated a homogenously enhancing intraventricular tumor. Gross total resection was achieved, yet despite postoperative radiation therapy, he progressed 11 months later. He was then treated with radiosurgery and crizotinib, leading to stable disease 23 months after start of targeted therapy. In both cases, EWSR1 gene rearrangement was identified by fluorescence in-situ hybridization (FISH). Reverse transcription polymerase chain reaction (rt-PCR) showed a CREB1 gene fusion partner in both cases. Our cases expand the clinicopathologic features of these newly recognized tumors and include an older age at presentation and a relatively elevated proliferation index. We also present a durable response to monotherapy with a MET inhibitor in response to this gene fusion.EWSR1 fusions have been identified in multiple neoplasms involving bone and soft tissue. Recently, cases of intracranial myxoid mesenchymal neoplasms harboring EWSR1-CREB1 family gene fusions have recently been identified in myxoid mesenchymal neoplasms in the CNS. Gene fusions between the Ewing sarcoma breakpoint region 1 gene (EWSR1) and members of the cAMP response element binding (CREB) family, which include CREB1, CREM, and ATF1, are not unique to this neoplasm as they have been identified in multiple other tumors including clear cell sarcoma of the gastrointestinal tract, hyalinizing clear cell sarcoma of the salivary gland, primary pulmonary myxoid sarcoma, and angiomatoid fibrous histiocytoma. These neoplasms have different clinical presentations, histologic morphology, and patient outcomes, despite sharing a common molecular alteration. Likewise, EWSR1-rearranged myxoid mesenchymal neoplasms of the CNS have distinct histologic features and have a reported propensity for the supratentorial region of adolescents and young adults. The upcoming World Health Organization (WHO) classification of tumors for CNS may have a different nomenclature for these tumors, possibly angiomatoid fibrous histiocytoma/intracranial myxoid mesenchymal tumor. Here, we present 2 cases of intracranial myxoid mesenchymal tumor with EWSR1-CREB1 fusions that expand the clinical presentation and provide therapeutic outcomes.A 37-year-old male presented with a 2-month history of new onset migraines, neck pain, and right eye pressure. As a part of his ophthalmologic work-up, he underwent brain MRI demonstrating a 1.7 × 1.6 × 1.5 cm well-circumscribed homogenously enhancing mass centered in the pulvinar of the right thalamus extending into the adjacent quadrigeminal cistern and abutting the pineal gland with associated ventriculomegaly . He undePathology of the lesion demonstrated small atypical, focally cohesive spindled and stellate cells embedded within a myxoid matrix with focal microvascular proliferation and non-specific perivascular inflammation. Few mitoses were identified within the atypical cells and endothelial cells ,b. The KAfter discussion of his case at our institution’s tumor board, surgical resection was recommended based on the pathologic findings. He then underwent a right retrosigmoid craniotomy for the lesion. He received dexamethasone and hyperosmolar therapy to treat his edema and underwent MRI demonstrating near-total resection . He had At his 3-month follow-up, he had improved neurologically to the point where his tracheostomy and gastrostomy had been removed. Unfortunately, MRI revealed progression of his residual disease  which waA 28-year-old male with a history of high-grade Burkitt lymphoma of his femur and kidney, for which he received intrathecal methotrexate and systemic vincristine and cyclophosphamide ten years prior, presented to an outside hospital with new onset headaches, associated vision changes, nausea, vomiting, and at least one documented seizure episode. MRI demonstrated an avidly enhancing, lobulated, intraventricular mass measuring 5.7 × 4.8 × 2.7 cm  centeredThe final pathology demonstrated a neoplasm composed of epithelioid cells with scant eosinophilic cytoplasm and abundant myxoid stroma arranged in nests, cords, and focally sheet-like growth patterns. Prominent small- and medium-sized blood vessels were seen. Mitoses were readily identified on routine hematoxylin and eosin (H&E) stains .2 In contrast to AFH, intracranial EWSR1 myxoid mesenchymal tumors tend to lack lymphocytic cuffing and a prominent vascular component histologically. Also, in the initial series of myxoid AFH, none of the tumors were located intracranially.3 In addition, myxoid AFH is reported to have rare local recurrences, whereas 25% (3 of the now 12) reported intracranial neoplasm have recurrences.5 And, as our repertoire of molecularly defined tumor types expands, we know that the shared gene fusion, such as that identified on our tumor type, AFH, CCS, GICSS, and PPMS, does not equate to a shared tumor family.Intracranial myxoid mesenchymal tumor with EWSR1 fusion is a relatively new entity first described in 2017.1–11Fifteen cases of intracranial myxoid mesenchymal tumors have been reported. The age range in the literature is 12–67 years, and the majority are supratentorial. With the addition of our 2 cases, this neoplasm demonstrates an overall 1:1 predominance. All reported cases are multinodular, lack lymphocyte cuffing, and have myxoid backgrounds. Only 5 reported cases have an increased proliferative index, yet none display necrosis. A majority of reported cases lack pseudoangiomatoid spaces, a pseudocapsule, and have a collagen component. Most reported cases have EMA and desmin positivity and EWSR1 fusion partners include CREB1, CREM, and ATF1 with CREB1 being the most common .1–112 Lastly, our cases demonstrate successful disease control following surgical resection and radiotherapy in one patient, as well as stable disease in a patient who received crizotinib monotherapy, also after surgical resection and radiotherapy. Crizotinib is a tyrosine kinase receptor inhibitor with multitargeted action at ALK (anaplastic lymphoma kinase), c-MET , and ROS1 (c-ros oncogene 1). Indications for the drug are based on clinical trials for ALK-positive non-small cell lung cancer (NSCLC). However, case reports have discussed responses with crizotinib in ALK-positive anaplastic large cell lymphoma (ALCL) as well as ALK-translocated inflammatory myofibroblastic tumor (IMT). There are clinical trials being performed with Crizotinib in neuroblastoma given the suspected role ALK mutations play in the malignancy of this disease.12 Of interest to our case report, recent work was published highlighting ALK mutations being over expressed in angiomatoid fibrous histiocytoma (AFH) raising questions of therapeutic potential. Since EWSR1 rearrangement is present in majority of AFH cases, further research is needed to determine the relationship between ALK and EWSR1 fusion proteins and whether the former may act as downstream target for the latter.13 There is also a report of using crizotinib to achieve long-term durable response in EWSR1-CREB1 fusion in a patient with gastrointestinal neuroectodermal tumor.14 The presumed mechanism is that this fusion product results in upregulation of the MET pathway, sensitizing it to a receptor tyrosine kinase inhibitor such as crizotinib. Our goal for precision medicine treatment with crizotinib was 24 months but discontinued after 23 months because the patient had been experiencing fatigue, a known adverse effect with this drug.12 Although the patient was still working, there was a desire on the patient’s part to return to more normalcy after completing nearly 2 years of treatment. Ultimately, decision was made to continue off treatment with surveillance monitoring. Nonetheless, this second case highlights a durable response to personalized and targeted treatment paradigm and lends credence to the hypothesis that MET inhibitors can be leveraged effectively in the setting of EWSR1-CREB1 fusion.Our cases have similar characteristics to the previously described cases such as shared histopathology, an intracranial location, and having EWSR1-CREB1 gene fusion. Both of our cases were midline tumors, and both our cases occurred in slightly older adults than most reported cases. Our cases also differ from those previously reported cases in that they have somewhat higher proliferative indices and mitotic activity. Interestingly, case 2 occurred in a patient with a remote history of Burkitt lymphoma treated with intrathecal and systemic chemotherapy. This association to childhood malignancy was also reported in another case with the development of an intracranial EWSR1 tumor 5 years after treatment for neuroblastoma.We present 2 cases of intracranial myxoid mesenchymal tumor with EWSR1-CREB gene fusion who have long-term follow-up currently demonstrating an absence of progression in both, one of which in response to targeted crizotinib monotherapy.Conflict of interest statement. D.T. has received grant funding from Celldex, NWBiotech, Novocure, and Merck; he has received personal fees from Novocure and prIME Oncology. A.G. has received personal fees from Novocure and Monteris Medical. He has also received research funding support from Orbus Therapeutics. The other authors declared no conflict of interest.


A bacterial mass in the urinary tract is a very rare entity. We report the first case of a bacterial ball within the urinary tract of a patient with diabetic cystopathy on long term urinary indwelling catheter. She presented with fever and gross haematuria. CT scan of abdomen and pelvis revealed a gas containing hyperdense mass within the bladder suspicious of bladder stone. The lesion was resected, and histopathology revealed a matrix of acellular materials with bacteria colony. An 82-year-old Chinese Female presented with fever, gross haematuria, and lower abdominal pain for 1 day. Her past medical history includes diabetes mellitus, hypertension, and chronic kidney disease. Functionally, she is non-ambulant, and bed bound due to previous right hip fracture. She is under follow up with urology for diabetic cystopathy and 6 weekly change of long-term indwelling urinary catheter. Her urinary catheter was changed 1 month prior to admission. She has no previous surgical intervention to the urinary tract.3/µL. Her glycaemic control is poor as evidenced by HBA1c of 10.3%, fasting blood glucose levels of 11.0 mmol/L and post-prandial blood glucose levels of 21.0 mmol/L. Her calcium, phosphate and uric acid levels were within normal limits. She has mild acute kidney injury with a serum creatinine was elevated at 148 μmol/L. Urinalysis showed haemopyuria, positive leukocyte esterase, and negative for nitrite test. Urine culture revealed mixed bacterial growth of more than three different species including Enterococcus faecalis. Blood culture had no bacterial growth. Urine cytology revealed no malignant cells. A plain KUB X-ray performed showed an opacity in the left central side of the pelvis consistent with the ‘’popcorn’’ calcification of an involuted fibroid. The colon and rectum were markedly faecal loaded. She was empirically treated with intravenous Ceftriaxone. A non-contrasted CT abdomen and pelvis revealed a large hyperdense mass with pockets of gas measuring 3.8 × 3.7 x 1.7 cm (Hounsfield unit of 350–422). Foleys catheter was seen in situ. Bladder wall was diffusely thickened. There is a small 6 mm calculus in the right kidney. The upper urinary tract was otherwise unremarkable.Physical examination revealed lower suprapubic tenderness. Renal punch was negative on both sides. Blood test results showed: haemoglobin 9.7 g/dL, white blood cell count 12.8 × 10Enterococcus faecalis and mixed Gram-negative bacilli enteric organisms. Histology of tissue sent showed bacteria, small amounts of necrotic debris and some fibrinous exudates. No hyphae or fungal elements were seen. Bladder biopsy showed acute on chronic inflammatory picture. She was discharged back to nursing home with a silicone indwelling urinary catheter.Manual bladder washout performed under ultrasound guidance with a size 24 Fr catheter evacuated 100 cc of clots. She was placed on continuous bladder irrigation. She responded well to antibiotics and was afebrile after 2 days of antibiotics. Her inflammatory markers were down trending and serum creatinine return to baseline. The bladder irrigation was stopped, and her urine remained clear. She was discharged back to nursing home and planned for an elective cystolithotripsy. However, she was re-admitted again a week later recurrent gross haematuria. Manual bladder washout was done and she was placed on continuous bladder irrigation. Repeat urine culture was negative. She was planned for cystolithotripsy in the same admission. However, intraoperative rigid cystoscopy revealed a soft cream coloured material densely adherent to the bladder mucosa. Bladder mucosa was severely trabeculated with multiple small diverticula. Initial attempts to aspirate and washout the mass with Toomey syringe was unsuccessful. A 26 Fr continuous flow resectoscope was used to dislodge the bacterial ball from the mucosa. It is then resected to smaller pieces with Bipolar energy and washed out. On resection, we noted certain areas of the bacterial ball exhibit laminated appearance. A random biopsy of posterior bladder wall was taken at the end of the procedure. Her urine was subsequently clear and post-operative stay was uneventful. Intraoperative tissue sent for aerobic culture revealed 1 Intraoperatively, the authors noted it is green and brown in colour, similar to fungal ball and difficult to differentiate from one another. Histologically, the matrix consists of thick acellular materials with bacterial coloniesBacterial ball has been reported by Kim et al in patients with chronic rhinosinusitis.2 Presence of air within the fungal ball gives rise to laminated appearance of fungus balls. There have been more than 20 cases been reported in the literature since then. Fungal urinary tract infections are most commonly caused by Candida species, but may also be caused by Cryptococcus neoformans, Aspergillus species and endemic mycoses.3Chrisholm and Hutch has first reported fungal balls within the urinary tract in 1961.3 Other associated conditions include prolonged antibiotic therapy, use of corticosteroids or immunosuppressive agents, urinary stasis, and indwelling intravenous or urinary catheters.4 There are multiple mechanism which explains why diabetic contributing to increased risk of urinary tract infection. Immune system impairment and high tissue glucose levels in poorly controlled diabetic patients create a favourable environment for the growth and multiplication of microorganisms.5,6,7The most common predisposing factor for fungal urinary tract infection is diabetes mellitus.5 This causes marked inflammatory reaction and necrotizing papillitis. These pseudohyphae eventually forms obstructing fungal within the collecting system. In our case, we hypothesize bacterial ball forms from agglutination of necrotic tissue material, mucous and foreign debris, which is similar to fungal ball. The risk factor for formation of bacterial ball in our patient is likely multifactorial; diabetes mellitus, neurogenic bladder and long-term indwelling urinary catheter.Michigan postulated that in patient with renal candidiasis, pseudohyphae are seen growing into and within tubules.8Fungal and bacterial balls may prove to be a diagnostic challenge on conventional imaging. Experience on CT to diagnose such lesions are limited in view of rarity of the disease. Takemura et al has reported a case of fungal ball mimicking urothelial carcinoma on MRI.9,10 Clinically, they present with intermittent fever and suprapubic pain. CT scan is almost diagnostic and reveals non-enhancing hypoattenuating content with enhancing peripheral rim.9,10 Cystoscopy often shows an elevated mass with inflamed overlying mucosa.9,10 Definitive treatment of bladder wall abscess requires drainage of the collection, which can be performed through transurethral route or percutaeneously under CT guidance.9,10Another possible differential diagnosis in our case is bacterial wall abscess. This condition is rare and has been associated with chronic urinary tract infection, history of permanent or intermittent catheterisation.In our case, the bacterial ball was initially misdiagnosed as a gas containing bladder stone on CT scan. Conservative management will likely fail leading to recurrent haematuria and urinary tract infection. It is difficult to evacuate the bacteria ball by bedside manual bladder washout due to its consistency, size, and adherent nature. We propose direct visualisation, resection, and evacuation of the lesion. Microbiology laboratory findings and histopathology will help in confirming diagnosis of bacterial ball.The further characterisation of MRI ﬁndings on fungal urinary tract infection is desired in order to assess the diagnostic power of this sophisticated device, as the diﬀerential diagnosis of fungus balls and neoplasms is sometimes challenging and can signiﬁcantly inﬂuence the clinical outcomesBacterial ball in the bladder is a very rare entity. We report the first case in the English literature. Evacuation of the bacterial ball together with aggressive antibiotics therapy is effective and can lead to excellent outcomes.Bacterial ball in bladder is a very rare cause of lower urinary tract infectionIn rare cases, bacterial ball may develop in patients with neurogenic bladder and on long term indwelling urinary catheter placementBacterial ball may mimic a bladder stone on CT scan as it is hyperdense with Hounsfield unit of 420 unitsIntraoperative findings of bacterial ball includes a soft mass with laminated appearance.Preferred treatment will be evacuation of the bacterial ball, which may necessitate the use of a continuous flow resectoscope together with aggressive antibiotics therapy.


Langerhans cell histiocytosis (LCH) is a rare condition that presents clinically in various ways. The cause and subsequent development of LCH are idiopathic and not fully understood. This disease is mainly seen in childhood. It is rare to have bilateral temporal bone LCH as the initial presentation. LCH can affect many organs. However, the bilateral involvement of the temporal bone is very uncommon. Therefore, we believe documenting cases of this presentation can lead to a better understanding of the epidemiology and prevalence of the disease, which can contribute to its management planning.A one-year-old boy was referred to a tertiary otolaryngology clinic with bilateral postauricular swelling, hearing loss, but no tenderness or ear discharge. During the patient evaluation, a CT scan was requested to further investigate the bilateral swelling, which showed bilateral bony destructive lesions in the temporal bone area. Next, the patient was scheduled for a biopsy of this lesion under general anesthesia. A biopsy of the right mastoid confirmed the diagnosis of LCH. The patient was started on LCH IV protocol for multifocal bone lesions (MFB) with special site induction. A follow-up fluorodeoxyglucose positron emission tomography/CT (FDG PET/CT) was performed on the whole body with the impression of mild interval improvement of the temporal bones’ masses bilaterally with stable bilateral cervical lymphadenopathy.LCH is a rare pathology that requires comprehensive effort from various medical and surgical teams to reach the right diagnoses and start the patient on the best available treatment plan. Langerhans cell histiocytosis (LCH) is a rare condition, and it presents clinically in various ways. The cause and subsequent development of LCH are idiopathic and not fully understood . It is sThe incidence of LCH is approximately 5.4 cases for every one million people annually . This diIt is found that 14-61% of pediatric patients with LCH show involvement of the temporal bone and the ear . HoweverThe presentation of LCH with ear involvement can manifest as any of the following conditions: a polyp or mass in the canal of the external ear, swelling in the postauricular region, chronic inflammation of the external ear or middle ear, conductive hearing loss, vertigo, and sometimes, paralysis of the facial nerve , or, leaA one-year-old boy with no known medical illnesses had bilateral postauricular swelling for five days, which was noticed by his parents in early March 2018. They initially sought medical help at the local hospital. A CT image showed a destructive soft tissue lesion in both the mastoid area and the petrous part of the temporal bone, causing narrowing of the ear canal.The case was referred to a tertiary care center. Upon presentation to a tertiary care ENT clinic, the child's history was taken, revealing a healthy, full-term child of normal vaginal delivery with no health-related issues, except for the postauricular swelling in the absence of any other ear or vestibular symptoms. Perinatal and family histories were unremarkable.Upon examination, he was found to be an alert and active child with no facial dysmorphic features. There was a bilateral swelling in the postauricular area, which was more prominent on the right side, with no tenderness or discharge. The ear canal was almost blocked; however, no discharge or any other swelling was seen. The rest of the ENT examination, including cranial nerves and flexible scope, was unremarkable.The CT scan showed bilateral bony destructive lesions in the temporal bone area, which was larger on the right side, with a soft tissue component and intracranial extensions involving the external and internal ear cavities. An MRI of the temporal bone showed heterogeneous enhancement of the lesion bilaterally Figure . A positAfter confirming the location and characteristics of the mass, the patient was taken for a mastoid lesion incisional biopsy under general anesthesia. A biopsy of the right mastoid showed the proliferation of Langerhans cells in a dense eosinophilic infiltrate Figure . Immunoh2 IV bolus and prednisone 40 mg/m2/day orally as a weekly dose (reduction after the fourth week). Another six-week course of vinblastine and prednisone was given afterward. In June 2019, the last dose of maintenance therapy (vinblastine and prednisone every three weeks) was given, with a total duration of treatment of 52 weeks. Assessment with CT and PET scan was carried at the end of week 52 and showed active residual lesions in bilateral mastoid areas. Hence, in August 2019, a decision was made to start the salvage protocol with vincristine 1 mg/m2 IV over one minute once a week and cytarabine 500 mg/m2 twice daily for five days as a two-hour IV infusion. In January 2020, an assessment of CT of the chest and MRI brain and sinuses was performed, which showed further improvement of residual lesions and less enhancement  with special site induction. In May 2018, the induction therapy was started for a duration of six weeks with vinblastine 6 mg/mt Figure . MoreoveA follow-up FDG PET/CT was performed for the whole body with the impression of mild interval improvement of the temporal bones masses bilaterally with stable bilateral cervical lymphadenopathy.The present case of LCH presented with bilateral postauricular swelling, which was seen on imaging and confirmed by biopsy. The patient was managed by LCH IV protocol and followed up with a PET/CT scan, revealing a mild interval improvement bilaterally with stable cervical lymph nodes.In a study by Coleman et al. , a two-yFurthermore, another case of LCH was reported by Kleinjung et al. . In thisGupta et al.  have repThis disease is associated with an easily relapsing locally invasive growth pattern with the possibility of malignant tumor dissemination ,11. It iLCH is a rare pathology that can affect many organs. A comprehensive effort involving various medical and surgical teams is warranted to reach the right diagnosis and start the patients on the best available treatment plan.


Small intestinal perforation in patients with Burkitt lymphoma is extremely rare. We present the first report of such a case.A 53-year-old woman was admitted with abdominal pain and vomiting. Abdominal examination revealed rigidity and tenderness in the upper abdomen.Computed tomography scan showed thickening of the wall of the jejunum, intra-abdominal free gas, and ascites; the patient was diagnosed with small intestinal perforation, and underwent emergency surgery on the same day. Laparoscopic findings were a 50 mm jejunal perforation and perforation in the transverse mesocolon. A partial jejunal resection of the perforated area, partial transverse colectomy, temporary colostomy, and intra-abdominal drainage were performed. Histological examination showed diffuse infiltration of medium-sized atypical lymphocytes in the perforated area, exhibiting a “starry sky” appearance.Immunostaining results showed that the atypical lymphocytes were CD20 and virtually 100% Ki-67 positive, and CD56, CD30, and EBER negative. The lesion was identified as Burkitt lymphoma (BL). The postoperative course was favorable. On postoperative day 18 the patient began chemotherapy through the hematology department. Currently, the patient is in remission.The majority of the malignant lymphomas occurring in the digestive tract are identified in the stomach; over 90% are B-cell lymphomas and mucosa-associated lymphoid tissue lymphoma Nakamura et al. BL originating from the small intestine accounts for only about 9%.The incidence of BL in the small intestine is low. Pretreatment BL can lead to bowel perforation. Prompt involvement of the hemato-oncologist after definitive diagnosis, and commencing chemotherapy as early as possible after surgery, are thought to improve prognosis. The majWe report a case of acute generalized peritonitis due to BL perforation in the small intestine which required life-saving emergent surgery. The aim of this report is to clarify that Burkitt lymphoma can cause small bowel perforation.The work in this case has been reported in line with the SCARE criteria .2A 53-year-old woman was brought to the hospital in an ambulance with a 1-month history of recurrent abdominal pain and vomiting. The abdominal pain and frequent vomiting usually occurred after eating. She was diagnosed 1 year prior to presentation with left-sided breast cancer. There is no relevant family or medication history. The patient was admitted for medical evaluation at our facility. Physical examination revealed a weight of 46 kg, height 154 cm, blood pressure 110/62 mmHg, temperature 38.5 °C, and a pulse rate of 90/min. She had generalized tenderness with board like rigidity of the abdomen.4/μL, C-reactive protein level of 0.82 mg/dL, and lactate dehydrogenase level of 703 U/L. Abdominal computed tomography scans revealed peripheral wall thickening of the jejunum, small amounts of free gas in the abdominal cavity, and ascites  observed in the jejunum was necrosed. Inflammatory changes were observed in the mesentery of the small intestine and the transverse mesocolon near the perforated area .Fig. 3SuHistological examination of samples from the perforated area of the jejunum, the mesentery of the small intestine, and the transverse mesocolon, revealed a large number of diffuse medium-sized atypical lymphocyte infiltrates and histiocytes with phagocytosed nuclear fragments, and a “starry sky” appearance. Immunostaining showed that the atypical lymphocytes were CD20 positive, and CD56, CD30, and EBER negative. CD3 and CD5 were positive only for a small number of intervening lymphocytes, and CD20 and Ki-67 staining was positive in almost 100% of the cells . A diagnShe had an uneventful postsurgical recovery period and on postoperative day 18 was transferred to the hematology department to commence chemotherapy. To date, the patient has been followed-up for five months and is currently in remission.3BL is a B-cell lymphoma, a form of non-Hodgkin’s lymphoma. It is characterized by aggressive growth and rapid development of clinical features. BL accounts for 1–2% of all malignant lymphomas  and is fBL in the small intestine is often characterized by abdominal pain, nausea, constipation, and an abdominal mass . BL freqA pathognomonic finding is the “starry sky” appearance, characterized by diffuse proliferation of homogeneous cells. These cells are approximately the same size as that of the nuclei of the histiocytes  and exhibit prominent macrophages with phagocytose nuclear fragments. The tendency of these cells to proliferate is extremely high, and Ki-67 staining has been reported to be over 95% positive. Some chromosomal translocations, such as t, t, and t are frequently seen in BL, and most cases exhibit c-MYC gene abnormalities in the eighth chromosome.The diagnostic criteria of malignant lymphomas in the digestive tract have not been established. However, Dawson et al., suggested the following five diagnostic criteria: no superficial lymph node swelling, no distinct swelling of mediastinal lymph nodes, no abnormalities in the peripheral blood results, mainly gastrointestinal lesions with and metastasis limited to the regional lymph nodes, and no metastases in the liver or spleen ,17.AccorMultidrug chemotherapy centered on cyclophosphamide hydrate is reported to have an extremely poor prognosis with an average survival time of 5.6 months; however, recent reports have indicated that, in adults, multidrug treatment has resulted in remission in 75%–90% of patients . There aOur case satisfied all of the five diagnostic criteria for malignant lymphomas of the digestive tract, as proposed by Dawson et al. Histopathological tests revealed the characteristic “starry sky” appearance, and Ki-67 staining was positive for almost 100% of the lymphocytes; therefore a diagnosis of BL was made. Histopathological findings of the excised bowel also showed severe infiltration of diffuse medium-sized atypical lymphocytes in the jejunal wall around the perforated area, and the primary site of the tumor was diagnosed as the jejunum. The postoperative course was favorable, and the patient was transferred to the hematology department, where she continued to receive chemotherapy and remained in remission as of the 5-month follow-up.A search of the terms “Burkitt’s/Burkitt lymphoma”, “perforation”, and “small intestine” in PubMed from 2000 to 2020 showed a number of cases where gastrointestinal perforation occurred during chemotherapy. However, to our knowledge, there are no reports of small intestine perforation due to BL of the digestive tract before treatment, and our report seems to be the first of such a case.In cases of small intestinal perforation in adults, emergency surgery should be conducted as early as possible to avoid life-threatening complications. Remission can be achieved using chemotherapy for a number of gastrointestinal diseases, such as small bowel cancer, gastrointestinal stromal tumor, Crohn's disease, and even aggressive malignancies with an extremely poor natural course, such as BL.4The incidence of BL in the small intestine is low. Prompt involvement of the hemato-oncologist after a definitive diagnosis is made, and commencing chemotherapy as early as possible after surgery, are thought to influence prognosis.All authors declare that there is no conflict of interest.This research did not receive any specific grant from any funding agency in the public, commercial, or not-for-profit sectors.The institutional ethics committee considers that ethical approval is not necessary for a case report.Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal on request.Yuji Takayama, Masaaki Saito: conceptualization, investigation, data curation, writing and reviewing.Kosuke Ichida, Yuta Muto: conceptualization, investigation, data curation, reviewing.Akira Tanaka, Toshiki Rikiyama: conceptualization, data curation, writing, reviewing.Researchregistry6002.Masaaki Saito, the corresponding author of this paper.Not commissioned, externally peer-reviewed.


Vertebral hemangiomas are relatively frequent among tumors of the spine. Most of them are asymptomatic and the diagnosis is usually made based solely on imaging. However, although rare, some hemangiomas with atypical imaging features (aggressive hemangiomas) can pose a diagnostic challenge. Clinically, these patients present with neurological symptoms. In imaging, aggressive hemangiomas appear as lesions with significant osseous expansion or extraosseous extension, mimicking the appearance of other tumors, such as metastasis or plasmacytoma. In such cases, a biopsy is often required to obtain a histopathological diagnosis in order to rule out the differential diagnoses mentioned above. We report on two cases of aggressive hemangiomas whose diagnosis remained uncertain until the pathology analysis. On CT-scan control immediately after biopsy, we have been surprised to observe the formation of gas bubbles inside the biopsied lesion, spreading over almost the whole vertebra. This gas web sign may support its liquid-filled spaces composition and its benign nature. Our goal was to highlight this finding and its usefulness. Vertebral hemangiomas are relatively frequent among tumors of the spine. Most of them are asymptomatic and the diagnosis is usually made based solely on imaging. However, some hemangiomas with extensive osteolysis, cortical expansion or associated with a soft tissue mass (aggressive hemangiomas) can pose a diagnostic challenge, mimicking the appearance of other tumors, such as metastasis or plasmacytoma, therefore requiring a histopathological diagnosis. We report on two cases for which the diagnosis of vertebral hemangioma remained uncertain until the pathology analysis. On CT-scan control immediately after biopsy, we have been surprised to observe the formation of gas bubbles inside the biopsied lesion, supporting its liquid-filled spaces composition and its benign nature. Our goal was to highlight this finding and its usefulness.A 60-year-old female presented with chronic mechanical cervicobrachial neuralgia and paresthesia of the three last fingers of her right hand. There was no trigger factor. The pain was intermittent and moderate, but it was worsening over time in the past few weeks. She had no known medical history and took no drug therapy. The physical examination was unremarkable, in particular, she had no motor deficit. MRI showed no disc herniation but a lesion of the T8 vertebra that was further characterized by a CT-scan. It was an osteolytic lesion of the vertebral body with right and left pedicle and lamina extensions, containing thickened vertical trabeculae ; A–B. TT1 weighted images  and in skull in 10–20% of the cases. They have distinctive histological findings depending on the amount of the fat, interstitial edema and blood vessels. The majority of vertebral hemangiomas are asymptomatic and have common characteristic imaging features. However, approximately 1% of vertebral hemangiomas produce neurological symptoms.6 For example, the presence of fatty component is indicative of inactive form of vertebral hemangioma, whereas lesions with low signal intensity on MRI indicate more active vascular type with potential to compress the spinal cord.7 Typical vertebral hemangioma appears as a “polka dotted” vertebra at CT-scan due to thickened vertebral trabeculae, in reference to a clothing pattern consisting of equally sized and spaced filled circles.8 When seen on sagittal and coronal images, hemangiomas appear as vertically oriented thickened trabeculae, known as the “corduroy sign”. On MRI, vertebral hemangiomas are hyperintense on both T1- and T2 weighted imaging, mildly hyperintense on STIR sequence. Thickened trabeculae appear as low signal areas on both T1- and T2 weighted imaging.8 These lesions show variable enhancement after contrast administration. Some vertebral hemangiomas differ from typical hemangiomas in the form of increased vasculature with reduced or absent fat content, mostly assessed by MRI. They appear as iso- to hypointense on T1 weighted imaging, and hyperintense on T2 weighted and STIR imaging. Aggressive hemangiomas refer to lesions with significant osseous expansion or extraosseous extension. Features such as involvement of the entire vertebral body, extension to the neural arch, expanded cortex with indistinct margins and soft-tissue mass have been described in aggressive hemangiomas.9 On MRI, they appear hypointense on T1 weighted images and hyperintense on T2 weighted and STIR images. Diffusion-weighted imaging has been used to differentiate hemangioma and metastasis in difficult cases, and found it to be a useful tool in the diagnosis, characterization and differentiation of both.10 At angiography, aggressive vertebral hemangiomas show characteristic dilatation of arterioles of vertebral body, multiple blood pools in the capillary phase and intense opacification throughout the vertebral body. Vertebral hemangiomas are typically ametabolic on 18FDG-PET scanning with no uptake on bone scan. However, rarely they may have atypical imaging features and appear hypermetabolic.11Vertebral hemangiomas can demonstrate various imaging patterns. The variations of signal intensity in the MR images correlates with the histological picture, as the composition of the lesion varies between fatty tissue, vasculature and interstitial edema.Although typical hemangioma can be easily diagnosed based solely on imaging, some aggressive hemangiomas can pose a challenge. Indeed, hypervascular metastases, plasmacytomas and lymphomas can share the same expansile osteolytic appearance on imaging. Metastases to the spine are commonly multiple and involve the pedicle and posterior vertebral elements. There is also a history of primary tumor most of the time. Plasmacytomas can show marked erosion, expansion and destruction of the bone cortex. Lymphomas usually appear as lesions with associated epidural, paraspinal soft tissue masses and homogeneous enhancement on contrasted MRI. In some cases, Paget's disease of the spine can have a similar appearance but is usually distinguished by expansion of the vertebral body with peripheral cortical thickening demonstrated on CT-scan.3,12In such cases, where diagnosis remains uncertain after imaging, a pathological diagnosis is required, generally by a CT-guided biopsy, considering that the management differs between hemangioma and the other differential diagnoses mentioned above. Indeed, as majority of vertebral hemangiomas detected are asymptomatic, no specific treatment is required. For the other cases, the treatment options are many, including surgical decompression, percutaneous vertebroplasty with polymethyl methacrylate, intralesional injection of ethanol, intra-arterial embolization and radiotherapy, even though this latter practice remains controversial.We presented the case of two patients with aggressive vertebral hemangioma whose diagnosis was uncertain on imaging but confirmed at histopathology, in order to describe a possible new post-bioptic finding. We describe the formation of gas bubbles inside the biopsied vertebra, notably at distance from the biopsy path. We can only speculate on the origin of the gas inside these lesions. The gas might have penetrated from the outside through the trocar and diffused through the entire lesion between trabeculae. The capacity of the lesion to let enter and dispatch gas bubbles supports the idea that the lesion is mainly composed of liquid-filled spaces. Indeed, only a liquid lesion could let the gas pass through, contrary to solid tumor, such as plasmacytoma. Besides, the presence of vessels organized in cavities interconnected via porous walls seen in pathology could explain the ability of the gas to diffuse through the lesion. We have also noted that the gas was arranged in a linear fashion, as though it was confined to tubular structures (such as vascular channels seen in pathology). Due to these imaging features, and in particular the capacity of the gas to form and to spread over almost the whole vertebra, we find the term of “gas web sign” the most appropriate to depict this finding.13 The authors assumed that the fluid in these lesions may have resorbed spontaneously, creating negative pressure and allowing nitrogen gas from the blood to accumulate in them. Nevertheless, all their patients underwent head and neck surgery before pneumatosis was detected. Similarly, the presence of gas bubbles in vertebrae was described in osteoporotic fracture as the “intravertebral vacuum phenomenon,” and constitutes an argument for benign vertebral fractures as opposed to malignant fractures.14–16 During the biopsy procedure, our patients were placed prone on the CT table. This positioning can be assimilated to a spine hyperextension, point that can be placed in parallel with the vacuum phenomenon appearing during extension of the spine.14,15The presence of gas inside a vascular lesion has been reported in lymphangiomatosis of bone.In clinical practice, this sign can be interesting for two reasons. Firstly, because biopsy of aggressive hemangioma may present a risk of bleeding, with occurrence or worsening of neurological symptoms. It is therefore potentially hazardous to obtain several samples. Observing gas bubbles at the first track prevents from performing additional samples. Secondly, because samples are usually quite small or quality being compromised in case of hemorrhage, negative results at pathology are not rare. The formation of gas bubbles inside the lesion after biopsy, freely diffusing through the whole lesion, could become an additional argument in favor of the diagnosis of hemangioma. However, these results are preliminary and may need further research to establish the usefulness of this diagnostic sign.We presented two cases of aggressive vertebral hemangiomas, whose diagnosis remained uncertain at imaging, thus requiring pathological confirmation. We have noted a post-bioptic finding consisting in the formation of gas bubbles freely diffusing through the whole lesion after the biopsy, in favor of its liquid-filled spaces composition and its benign nature. This gas web sign may be of additional value in non-conclusive cases favoring aggressive vertebral hemangioma, but there will be further studies needed to assess its value and accuracy.Aggressive vertebral hemangioma can present with significant osseous expansion or extraosseous extension on imaging, therefore mimicking other tumors, such as metastases, plasmacytomas or lymphomas.In such cases, a pathological diagnosis by biopsy is required.The formation of gas bubbles inside the biopsied lesion and along the biopsy path (“gas web sign”) may be of additional value in non-conclusive cases favoring aggressive vertebral hemangioma. However, these results are preliminary and may need further research to establish the usefulness of this sign.