Dear Editor,Papillary syringocystadenoma is a rare neoplasm of sweat glands, which is present at birth in 50% of cases.3It usually presents as papule or plaque with a crustal surface that occurs almost exclusively in the head and cervical region.This report details the clinical observations as well as the dermatoscopic and histopathological findings of a case in an unusual location.A 6-year-old male, a native and resident of São José dos Campos, SP, presented a lesion with progressive growth five years ago on the left flank. At the dermatological examination, a papule of pink-erythematous coloration was observed, with a smooth surface and a fibroelastic consistency, measuring 5 mm × 3 mm . DermoscPapillary syringocystadenoma (SCAP) is a rare adnexal tumor most often derived from apocrine cells.In 75% of cases, it is located on the head or cervical region. Some cases in other topographies have already been described . These locations, as well as that of the case described, are even rarer. When located on the scalp, it may be associated with the sebaceous nevus of Jadassohn.Despite variable clinical presentation, a papule plaque is the most commonly found lesion type. In the majority, it is asymptomatic, but it can present pruritus, pain, and/or bleeding; it usually presents progressive growth, as in the case described.4To date, articles on the dermoscopic findings of SCAP are scarce. There is a description of a horseshoe vascular pattern, which was not observed in the present case.5SCAP can infect, bleed, ulcerate, and, in rare cases, it can progress to basal cell carcinoma (9%) or papillary syringo-cystadenocarcinoma. For these reasons, it was decided to perform the excision.None declared.Giovanna de Araujo Horcel: Conception and planning of the study; composition of the manuscript.Juliana Milhomem: Conception and planning of the study.Samuel Henrique Mandelbaum: Critical review of the literature; critical review of the manuscript.Rodrigo Ieiri: Participation in the design of the study.None declared.


Lymphomas are a heterogeneous group of malignant tumours of the haematopoietic system characterized by an aberrant proliferation of mature lymphoid cells or their precursors and mainly represented by non-Hodgkin´s lymphomas (NHL). The aim of this paper was to report two cases of NHLs with mandibular locations by detailing their different clinical, radiological, and histopathological aspects, as well as the approach followed to diagnose these diseases and to provide patients with the appropriate therapeutic management. The first case is about a 72-year-old female patient who was diagnosed with a large B-cell lymphoma while the second one concerns a 16-year-old male patient who was diagnosed with a Burkitt’s lymphoma. These observations represent the two highly aggressive known NHLs according to the WHO classification. The mandibular locations of these diseases are rare and represent only 0.6% of all the reported cases. It is important to note that only a deep and good quality tumour biopsy can provide a diagnosis of certainty. The reference treatment is medical consisting in the introduction of chemo-immunotherapy. As oral surgeons, we have an important role in the early diagnosis of these malignancies and in the patient’s referral to specialized care in order to get the appropriate treatment. Lymphomas are a heterogeneous group of malignant tumours of the haematopoietic system characterized by an aberrant proliferation of mature lymphoid cells or their precursors . LymphomFirst case: a 72-year-old female patient with a history of insulin-dependent diabetes mellitus, hypertension, gout and ischemic heart disease who had undergone coronary angioplasty was referred to the oral medicine and oral surgery department at SAHLOUL Hospital  because of pain and continuous discomfort localized in the right mandibular region. The extra oral clinical examination revealed the presence of one palpable right submandibular lymph node greater than 1 cm and having a firm consistency  and thyroid carcinoma 33%). Thus, they should always be considered in cases of suspicious cervical or oral masses . Most of6% and th3%. Thus,The standard imaging assessment includes a thoracic, abdominal and pelvic computed tomography, with measurement of the lesions that will serve as the initial reference for the treatment response assessment. A PET scanner FDG is also necessary to identify the degree of tumoural extension and to determine the Ann Arbor stage . The twoAs for Burkitt's lymphoma, it is a highly aggressive B-cell non-Hodgkin´s lymphoma that accounts for more than 80% of NHL in the children . ActuallSince our patient was not clinically and biologically diagnosed with a viral infection (EBV or HIV) and histologically there was not a positive staining for EBV, we can consider that we are in front of the sporadic form of BL with a bulky abdominal mass, multiple lymphadenopathy and an extranodal involvement of the oral cavity. The disease staging and the evaluation of the prognostic factors represent a fundamental step in the patient´s management, essential for treatment decisions. The reference treatment is medical consisting in the introduction of chemo-immunotherapy (CI) with R-CHOP because of the high chemosensitivity of the tumour: It combines 6 to 8 cycles of multidrug therapy according to the CHOP protocol containing Cyclophosphamide, Adriamycin, Vincristine and Prednisone with a monoclonal anti-CD20 antibody that is Rituximab . The cycThe complete remission rate of Non-Hodgkin´s lymphomas after chemotherapy is 60 to 80% per year . The patMandibular non-Hodgkin´s lymphomas are rare but they should be included in the differential diagnosis of other jaw lesions. This paper reported two original entities of the highly aggressive grade of these diseases. Diagnosis is always challenging due to the lack of specific symptoms which may lead to misdiagnosis and therefore delay the initiation of the appropriate therapy. Biopsy must always be performed in case of suspicious findings following meticulous clinical examination. As oral surgeons, we have an important role in the early diagnosis of these malignancies and in the patient´s referral to specialized care in order to get the appropriate treatment.


Cholecystoenteric fistula (CEF) is rare in biliary tract surgery, and cholecystogastric fistula (CGF) is the rarest form of CEF. Although open cholecystectomy with the closure of the fistula is the gold standard treatment for nonobstructing biliary-enteric fistulas, the optimal treatment for CGF has not been established. Laparoscopic and endoscopic cooperative surgery (LECS), a minimally invasive surgery for gastric submucosal tumors, reportedly helps achieve favorable postoperative outcomes. This report presents a case wherein CGF was treated with LECS.An-84-year-old man with a history of chemotherapy for ileocecal diffuse large B cell lymphoma presented with fever and abdominal pain. He was diagnosed with cholangitis, and endoscopic lithotripsy was performed. Abdominal contrast-enhanced computed tomography revealed gastric wall thickening close to the gallbladder, indicating a malignant lymphoma. Esophagogastroduodenoscopy revealed a concavity on the anterior wall of the antrum of the stomach. Direct endoscopic cholangiography, which was performed by the injection of a contrast medium into the concavity, confirmed the concavity to be a CGF. Cholecystectomy with the closure of the fistula using the LECS was performed to check for suture line leakage and the positional relation of the suture line and the pylorus. The resected specimen showed acute and chronic cholecystitis without malignancy. The patient did not experience postoperative complications in the subsequent 3 months.Resection and closure of the fistula using LECS were performed successfully.LECS can be performed for CGF. LECS enables intraoperative observation of the fistula and suture line, and thus reduces postoperative complications. Althoug22 and his body temperature was 38.5 °C. His Eastern Cooperative Oncology Group Performance Status (PS) score was 0. Blood examination showed elevated levels of hepatobiliary enzymes in the serum. Abdominal ultrasonography showed a thickened gallbladder wall with cholelithiasis and pneumobilia of the intrahepatic bile duct. Endoscopic lithotripsy with endoscopic papillary balloon dilation and antibiotic therapy was used for the treatment of acute cholangitis, and this required hospitalization for 2 weeks. Endoscopic sphincterotomy and brush cytology were not performed. A thickened gastric wall close to the gallbladder, which indicated malignant lymphoma, was detected on computed tomography (CT) scan performed during hospitalization. Tumor markers in serum, including carcinoma antigen 19-9, carcinoembryonic antigen, and interleukin-2 receptor, were not elevated. Esophagogastroduodenoscopy showed a concavity on the anterior wall of the gastric antrum  was inserted into the umbilicus via an open technique. Three additional ports (three 5-mm ports [Kii Access System 5 mm]) were inserted into the epigastric, right hypochondriac, and right upper quadrants of the abdomen with visual laparoscopic assistance under 10-mmHg pneumoperitoneum. A thick-walled contracted gallbladder was seen to be tightly attached to the omentum and the anterior wall of the gastric antrum. Because the bed side of the gallbladder wall was embedded in the liver by extensive inflammatory adhesions, partial cholecystectomy was performed, leaving the bed side of the gallbladder wall in place. After separation of the gallbladder from the liver, the cystic duct and cystic artery were cut. Subsequently, it became easy to observe the fistular region. The fistula was observed via endoscopy and marked with the injection of indigo carmine  into the submucosal layer around the fistula. Branches of the right gastroepiploic vessels in the excision area around the fistula were cut using an ultrasonically activated device . Adhesions between the gallbladder and stomach were dissected, and the fistula was exposed a. CompleThe resected specimen showed a thick-walled contracted gallbladder with gallstones measuring about 2 cm in diameter. Histologic examination revealed pseudo-pyloric gland metaplasia and Rokitansky-Aschoff sinuses. Thus, the patient was diagnosed with acute and chronic cholecystitis without malignancy. The patient did not experience postoperative complications in the 3 subsequent months.3CEF is reported in 0.74% of patients undergoing biliary tract surgery  and 0.27A history of right hypochondriac or epigastric pain is the most common presentation besides jaundice, fever, anorexia, nausea, and vomiting ,4. CholaPreoperative examinations include ultrasonography, CT, magnetic resonance imaging, and endoscopic retrograde cholangiopancreatography (ERCP) . Edema oSince there are some reports of conservative treatment ,11,13 orCholecystoduodenal fistula closes spontaneously in many patients . HoweverRegarding the intraoperative findings, dense inflammatory adhesions around the gallbladder, a shrunken and fibrotic gallbladder, which was firmly stuck to the adjoining viscera, and dense omental adhesions indicate the presence of CEF ,4; these4Laparoscopic cholecystectomy and fistula closure for CGF could be performed safely, even with the fistula located near the pylorus. LECS is useful for intraoperative observation of the fistula and suture line, which reduces postoperative complications.The patient was concerned whether the surgery would convert to an open cholecystectomy and fistula closure, which requires a large incision. Therefore, he consented to this laparoscopic and endoscopic cooperative method to avoid the risk of a gastric outlet syndrome. There were no postoperative complications. The patient was informed about the regular medical follow-up.None.This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors.This case report was approved by the Research Ethics Committee of the Ofuna Chuo Hospital (No. 2019-005).Written informed consent was obtained from the patient for publication of this case report and the associated images.Goshi Fujimoto: Responsible for performing the procedure described in the case report, concept and design of the study, acquisition of data, drafting the manuscript, revising the manuscript, and approving the final version of the manuscript.https://www.umin.ac.jp/ctr/) with the unique identifying number UMIN000039775.This study was registered as a case report in the UMIN Clinical Trials Registry (Goshi Fujimoto.Not commissioned, externally peer-reviewed.The datasets supporting the conclusions of this article are included within the article.


Conjunctival papilloma is a benign neoplastic lesion of epithelial origin, with a minimal tendency toward malignancy and an exophytic growth type. Etiological factors that contribute to the appearance of papilloma are UV exposure, HPV infection, smoking, and immunodeficiency. A case report of limbal conjunctival papilloma treatment with topical interferon alpha-2b eye drops applied to the conjunctival sac.A 49-year-old female patient treated in the Clinical Department of Ophthalmology for conjunctival lesion located in the temporal quadrant near the limbus of the right eye. Ocular examination of the patient's eye did not show any abnormalities: visual acuity—20/20, intraocular pressure—16 mmHg.During physical examination, a broad-based pink lesion of size 4 mm × 6 mm was found in interpalpebral bulbar conjunctiva with prominent feeder vessels and soft consistency. Lesion in the biomicroscopic examination did not show any sign of malignancy. In Swept Source Optical Coherence Tomography (SS-OCT) there was no sign of infiltration into sclera or cornea. In vivo confocal microscopy (IVCM) examination, revealed loss of the normal conjunctival epithelium - hyper-reflective cells, variation of cell size. A clinical diagnosis of limbal conjunctival papilloma was made.Interferon alpha-2b 1 million IU/ml eye drops were applied 4 times a day for 5 months. The treatment lasted 5 months without changing the dosing regimen. Three months after the start of the interferon treatment, a complete regression of the lesion was achieved. Treatment was continued for the following 2 months. Controlled IVCM after 6 months showed only few hyper-reflective cells and fibrotic tissue.Treatment of conjunctival papilloma with topical interferon alpha-2b led to the complete regression of the lesion. Although after the therapy the conjunctiva appears normal both in slit lamp and in the OCT examination, there is a noticeable fibrosis of the tissue in confocal microscopy. During the 14-month follow-up period, there were no recurrent lesions.In this study, topical interferon alpha-2b has been shown to be an effective and safe therapy for small-to-medium-size conjunctival papilloma without any sign of malignancy. Based on the location, age of occurrence, tendency to regrow, and histopathology, conjunctival papilloma can be categorized into the following types: squamous, limbal, and inverted. Most of these lesions were located medially (89%) and inferiorly (71%). To be more precise about the most common location, authors are not unanimous—bulbar conjunctiva (42%—Ash), palpebral conjunctiva , and caruncle  Excision is commonly used method to treat this type of lesions. However it does not always produce the expected result due to frequent recurrences. In cases of small or recurrent lesions pharmacotherapy can be used as the first choice. Regression of the lesion can be achieved with topical interferon alpha-2b.Conjunctival papilloma is a benign neoplastic lesion of epithelial origin, with a minimal tendency toward malignancy and an exophytic growth type.2A 49-year-old female patient was treated in the Clinical Department of Ophthalmology for conjunctival lesion located in the temporal quadrant near the limbus of the right eye. During physical examination, a broad-based pink lesion of size 4 mm × 6 mm was found in interpalpebral bulbar conjunctiva with prominent feeder vessels and soft consistency Fig. . Lesion Treatment of conjunctival papilloma with topical interferon alpha-2b led to the complete regression of the lesion. Although after the therapy the conjunctiva appears normal both in slit lamp and in the OCT examination, there is a noticeable fibrosis of the tissue in confocal microscopy. During the 14-month follow-up period, there were no recurrent lesions.3 (mostly type HPV11).,7 Other types of virus have also been found: type 6  and type 16, 18, or 45 .,7,9,10 It occurs mainly in children and adults under 20 years of age. This type of papilloma has a high recurrence rate.Squamous cell papilloma appears as a grayish red, fleshy, soft, pedunculated, or raised mass with an irregular surface located in the inferior fornix, caruncle, and palpebral regions. It is strongly associated with HPV This type of lesions arises from UV radiation exposure rather than due to infectious factors. In comparison to squamous papilloma, it has a lower recurrence rate. The former usually does not cause deterioration or loss of vision, while the latter may contribute to loss of vision when it extends into the visual axis.Limbal conjunctival papillomas are sessile, gelatinous lesions with epithelium dysplasia and feeder vessels.—signs of which include local inflammation, keratosis, adhesions between the tarsal and bulbar conjunctiva and characteristic localization. Majority of the conjunctival squamous cell carcinomas are in the interpalpebral zone near to the limbus. The papilloma should be differentiated from ichthyosis, sebaceous gland cancer, conjunctival lymphoma, or squamous cell neoplasia, including conjunctival squamous cell carcinoma. Unfortunately, there are only contradicting reports about the use of IVCM to distinguish between malignant and benign conjunctival lesions.–13These lesions rarely become malignant2 laser therapy, and pharmacotherapy along with the use of cytotoxic and immunomodulating medication. Small papillomas may regress spontaneously. Excision of the lesion does not always produce the expected result due to frequent recurrences (from 6% to 27%) and possible seeding, which may lead to multiple new papillomas. Recurrences are more common in children and adolescents than in adults. Cryotherapy, however, is characterized by lower recurrence rate and reduced scarring compared with conventional excision. In the case of lesions without malignant features, recurrence tendency, pharmacotherapy appears to be a better alternative compared with invasive methods. In the literature, there are several reports on the effective use of topical H2 receptor antagonists (cimetidine),,15 dinitrochlorobenzene, interferon alpha-2b,,17–22 mitomycin C,,24 and bevacizumab for the treatment of such types of disorders. According to some authors, surgical excision of the lesion and cryotherapy to the adjacent conjunctiva are the treatment of choice among patients. Adjuvant oral cimetidine and/or topical interferon alpha-2b reduce the recurrence rate, which seems to be the proper approach considering large lesions. Taking into consideration the cytotoxicity of mitomycin C  and the relatively long period for interferon to achieve the clinical effect, despite there is only one report, it is worth to look at bevacizumab as adjuvant therapy for the effective treatment of recurrent lesions. In our patient, complete regression of the lesion is similar result to other reports regarding topical interferon alpha-2b treatment in conjunctival papilloma.,19,21,22 In cases of small lesions or in recurrent papillomas, pharmacotherapy can be used as the first choice. Currently, there are no available reports in the literature comparing the effectiveness of aforementioned methods.Currently, there are several methods available for the effective treatment of conjunctival papilloma, including excision, cryotherapy, COConceptualization: Sławomir Teper, Edward Wylęgała.Data curation: Bartlomiej Bolek, Edward Wylęgała.Formal analysis: Bartlomiej Bolek, Adam Wylęgała.Investigation: Bartlomiej Bolek, Sławomir Teper, Joanna Kokot.Methodology: Bartlomiej Bolek, Adam Wylęgała, Sławomir Teper, Joanna Kokot.Project administration: Bartlomiej Bolek, Adam Wylęgała, Sławomir Teper, Edward Wylęgała.Resources: Sławomir Teper.Software: Bartlomiej Bolek, Joanna Kokot.Supervision: Edward Wylęgała.Validation: Edward Wylęgała.Visualization: Bartlomiej Bolek, Joanna Kokot.Writing–original draft: Bartlomiej Bolek, Joanna Kokot.Writing–review & editing: Bartlomiej Bolek, Adam Wylęgała, Sławomir Teper, Joanna Kokot, Edward Wylęgała.


Intraventricular schwannomas are rare with only 31 cases reported.2 The wide differential of intraventricular lesions combined with the rarity of intraventricular schwannomas makes diagnosis a challenge, both radiographically and histopathologically. We report on a case of an intraventricular schwannoma and demonstrate how DNA methylation can be used to confirm the unusual diagnosis.Schwannomas are benign nerve sheath tumors arising from peripheral, cranial, or autonomic nerves.A 44-year-old otherwise healthy Caucasian male with a history of migraines presented with a 1-year history of progressive headaches with occasional nausea and gait instability. Neurological exam including cranial nerve exam was unremarkable. Fundoscopy did not show any evidence of papilledema. Gait was grossly normal. There was no family history of any neoplastic disease or known heritable genetic conditions.Magnetic resonance imaging (MRI) with gadolinium showed a 1.7 × 1.4 × 1.2 cm enhancing tumor centered in the lateral ependymal surface of the right lateral trigone with associated vasogenic edema in the periventricular white matter of the right parietal lobe . The medDifferential diagnosis for this lesion was primarily neoplastic in nature and included high-grade glioma, ependymoma, subependymoma, meningioma, choroid plexus tumors, metastases, or schwannoma. We trialed a period of surveillance with radiographic and clinical follow-up over several months, without any documented change in lesion character or size. However, given the patient’s escalating symptoms, lack of diagnosis, and concerning vasogenic edema, surgery was ultimately offered.We performed a right-sided parietal mini-craniotomy and inserted an external ventricular drain (EVD) into the atrium of the right lateral ventricle under stereotactic guidance. The EVD was followed microsurgically into the atrium of the ventricle where the choroid plexus was visualized, and posteriorly, a firm, tan-red-colored mass somewhat adherent to the choroid plexus but not obviously originating from it. We microsurgically devascularized the lesion using bipolar electrocautery and resected it in a piecemeal fashion to achieve a gross total excision. There were no intraoperative complications, and the patient’s postoperative course was unremarkable with a 2-day hospital stay. Postoperative MRI showed near total resection with one small focus of enhancement possibly representing residual tumor. This was stable at 1-year follow-up .Histopathology showed a spindle cell neoplasm with alternating dense interweaving fascicles and looser hypocellular areas . There w3 The top-ranked diagnosis was schwannoma  as per the manufacturer’s protocol. Raw methylation data were subjected to classification using a recently developed and validated methylome-based CNS tumor classification model.hwannoma . Chromoshwannoma . Loss ofhwannoma  and D. T2 The majority are located in the lateral ventricles (63.6%) with the rest in the fourth (27.3%) and the third (9.1%) ventricles. There have been 2 reported cases of “malignant schwannomas” that recurred early after subtotal resection with intracranial metastasis despite adjuvant radiotherapy.5 For benign intraventricular schwannomas, total resection was curative and was achieved in 25 of the 31 cases. Even those with a subtotal resection did not demonstrate any recurrence at 10 years, suggesting close radiographic surveillance may be appropriate if the pathology does not demonstrate malignancy.6Intraventricular schwannomas are rare entities with only 31 prior cases reported worldwide.7 described the association of Schwann cells with peripheral or autonomic nerve fibers located within the choroid plexus of the ventricle. These Schwann cells can undergo a neoplastic transformation and become benign or malignant schwannomas. Another theory suggests that ectopic debris from neural crest cells that migrate into the ventricular system during disorganized or abnormal embryogenesis can also undergo neoplastic transformation.8 These theories may explain the close anatomic association of these tumors with choroid plexus in several previous reports, as well as in our own case. A final theory suggests that pluripotent mesenchymal stem cells from the subventricular zone may differentiate into Schwann cells following tissue injury, such as in multiple sclerosis, post-stroke, and other pathologies.11There are several theories regarding the pathogenesis of intraventricular schwannomas. Benedikt12–14 In our unsupervised analysis, we included known cases of choroid plexus tumors (papillomas and carcinomas), gliomas (IDH-mutant and wildtype), meningiomas, and schwannomas  to determine the similarity of this case’s methylation profile to known entities. Overall, although our analysis included relatively few cases of cranial versus peripheral schwannomas compared to previous studies, we found this tumor most closely resembled tumors of vestibular origin. However, future studies are needed to determine if intraventricular schwannomas represent a new methylation subgroup with different tumor biology and mutational landscape.All previous diagnoses of intraventricular schwannomas have relied entirely on histology and immunohistochemistry. Recent revisions of the WHO classification in 2016 integrate molecular alterations with histopathological changes for specific tumors. For schwannomas, the diagnosis remains entirely histopathological. We previously established the molecular landscape of schwannomas and have shown that DNA methylation profiling identifies schwannoma subgroups based on anatomical location .15Our case demonstrates the clinical utility of DNA methylation profiling in brain tumor classification and highlights how epigenome-wide DNA methylation analysis can be leveraged as an additional tool to improve brain tumor classification using modeling approaches, particularly for challenging brain tumors in rare locations, with discriminative capabilities reaching 99%.No funding was allocated for this work.J.Z.W., N.P., N.I., C.V., S.S., A.G., P.D., and F.N. prepared the text and figures. F.N., S.K., and M.Y. provided the methylation data. J.Z.W., F.N., S.S., N.P., N.I., and G.Z. edited the clinical portions.


We present a difficult case of huge squamous cell carcinoma (SCC) of lower lip that was successfully treated by intra-arterial infusion with methotrexate (MTX).This 42-year-old female patient present with a fungating lower lip SCC of approximately 10 × 5 cm in size. MTX 25 mg was infused continuously to each side of external carotid artery every 24 h using two portable pumps. Totally, MTX 300 mg was given over 6 days. After treatment initiation, the tumor shrank dramatically and disappeared completely 2 months after the therapy. The patient was now recurrence-free at the recent follow-up 4 and half years after therapy.Intra-arterial infusion chemotherapy has the advantage of delivering a high concentration of anticancer drug to the lesion to induce a rapid shrinkage of the tumor and the side effects are limited. Intra-arterial infusion with MTX achieves good tumor response to lower lip cancer with excellent anatomical and functional preservation.This therapy may be a treatment option in lower lip cancers with unresectable lesions, or in those patients who are unwilling to undergo resection. Early surgical excision is the treatment of choice for lower lip squamous cell carcinoma (SCC). We present a difficult case of huge SCC of the lower lip that was successfully treated by intra-arterial infusion with methotrexate. The lesion was extensive, which made complete resection difficult. This case report is reported in line with the SCARE criteria .This 42-year-old female patient presented with a fungating lower lip mass of approximately 10 × 5 cm in size in May 2015. Two years before admission, a bean-sized tumor on her lower lip was noted and had been growing slowly for 2 years. Histopathologic examination showed grade 1 SCC. The patient declined surgical resection because of the extensive cosmetic and functional disability likely to result. She was admitted for intra-arterial infusion chemotherapy. There was no regional lymphadenopathy on admission. Systemic image survey including computed tomography (CT) and positron emission tomography (PET) showed no evidence of distant metastasis .Fig. 1LoThe implantable port-catheter system  was used for catheterization. Under general anesthesia, the catheter was inserted through the right superficial temporal artery into the external carotid artery with the tip located proximal to the branching of the facial artery. The distal end was embedded subcutaneously along the lateral neck and connected to the port, which was implanted subcutaneously near the infra-clavicular region ,3. The sIntra-arterial infusion chemotherapy has the advantage of delivering a high concentration of anticancer drug to the lesion to induce a rapid shrinkage of the tumor. Intra-arterial infusion with MTX achieves good tumor response to early lower lip cancer with excellent anatomical and functional preservation . The uniWritten 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.No conflicts of interest.No funding of this research.This study is exempt from ethical approval.Fully informed patient consent was obtained and provided.Study concept and design of study: M.C. Sheen.Data collection: Y.T. Sheen, Y.Y. Chen.Data analysis and interpretation: Y. T. Sheen, M.C. Sheen.Writing the paper: Y.T. Sheen.Revising the manuscript critically for important intellectual content: M.C. Sheen, Y.T. Sheen.This study don’t involve the human participants.Y.T. Sheen.Y.Y. Chen.M.C. Sheen.Not commissioned, externally peer-reviewed.


This paper reports an uncommon location and complication of a cystic lymphangioma. Few cases of infected colonic cystic lymphangioma were described in literature. Symptoms are non-specific and setting the diagnosis on radiological features remain challenging. Urgent open surgery is generally required for therapeutic and diagnostic purposes.We describe a case of a young man who presented with an acute abdomen mimicking bile peritonitis, infected tumor of the colon or even a complicated hydatid disease of the liver, which is endemic in our country. CT-scan was compulsory to suspect the diagnosis of infected cystic lymphangioma but remained insufficient to rule out other more frequent diagnoses. The patient underwent an emergency open surgery consisting in a bowel resection, in whose case an infected cystic lymphangioma was barely suspected preoperatively but confirmed by the pathological examination of the specimen. No postoperative complications were noticed.Setting the diagnosis of an infected cystic lymphangioma of the colon is tough. Many differential diagnoses are more frequently suspected and radiological examinations can be helpful. However, an emergency surgery is mandatory in order to avoid septic shock and resect the lesion, sometimes at the cost of bowel resection. Laparoscopic or endoscopic treatments are feasible but are not the standard in emergency cases.Acute presentation of cystic lymphangioma of the colon is very scarce and can be life-threatening leading to urgent open surgery, although endoscopic or laparoscopic treatment remain feasible. Further studies are needed to select which technique is suitable for this disorder. •Cystic lymphangioma is a benign lesion.•Colonic location of cystic lymphangiomas is scarce.•Infected cystic lymphangiomas of the colon is rarely described in literature.•Open Surgery is the standard treatment of cystic lymphangiomas especially in case of emergency. Lymphangiomas of the colon are scarce, representing less than 5% of all lymphangiomas. Although most lymphangiomas remain asymptomatic, complicated ones may lead to an emergency surgery. The final diagnosis is obtained through histological examination of the specimen. We report herein an uncommon case of a 25-year-old man who underwent urgent open surgery for an acute abdomen due to an infected cystic mass of the ascending colon related to an infected lymphangioma.The case report has been reported in line with the SCARE criteria .2A 25-year-old man with no past medical or drug history, presented to the emergency room with an acute pain of the right upper quadrant and flank evolving for three days associated with fever. The patient reported no chronic abdominal pain or transit disorders.Physical examination showed that the patient had fever 39 °c. The abdominal examination revealed the presence of a 10 cm - regularly shaped, soft, and fixed mass in the right hypochondria and flank. The rest of the abdomen revealed no remarkable abnormalities.Laboratory tests showed a high c-reactive protein level (173.5mg/l) but a normal white blood cell count (7553/μl). No anemia nor hemostasis disorders were noticed. Furthermore, urine culture was normal.Firstly, we decided to perform an abdominal ultrasound that showed a cystic formation of the right pericolic gutter with a thick wall and several septa within. The mass had close relationships with the sixth liver segment and the ascending colon. At first, we suspected a hydatid cyst of the liver because Tunisia is an hydatid endemic area. However, a malignant tumor of the colon could not be ruled out.Therefore, we indicated an abdominal CT scan to characterize the mass and determine its exact location. It revealed a regular 10-cm- intraperitoneal cystic mass independent from the liver but repressing the ascending colon with heterogonous internal density. Moreover, the mass was multilocular edged by thick walls showing low contrast enhancement after intravenous injection. Peritoneal effusion was detected on CT scan in the pericolic gutter and douglas pouch, to boot , Fig. 2.An infected cystic lymphangioma was then suspected besides the diagnosis of intraperitoneal hydatid disease or infected malignant tumor of the colon. We decided then to perform open surgery for diagnostic and therapeutic purpose.We found a 10-cm-cystic-mass containing a serous bloody fluid in the right pericolic gutter depending from the ascending colon. The colon was not distended. No liver metastasis nor peritoneal carcinosis were noticed. An ileocolic en bloc -resection was performed with an immediate ileocolic anastomosis. .Fig. 3MaMacroscopically, the 10 –cm colonic cystic mass had many septas and contained serous fluid. It was obviously arising from the ascending colon. In addition, histopathological examination revealed dilated lymphatic and vascular components lined with attenuated endothelial cells. These findings were consistent with the diagnosis of a cystic lymphangioma. The patient's post-operative course was uneventful. He was discharged on the 5th post-operative day. A 6-months clinical and radiological follow-up showed no symptoms or recurrence with good tolerance to regular diet.3Lymphangioma of the colon is a rare condition firstly described by Chisholm and Hillkowitz in1932 . LymphanNowadays, with the widespread use of endoscopy for preventive, diagnostic and therapeutic goals, this disorder was reported more frequently. Matsuda et al. reported Diagnosis is impossible on clinical and biological examinations alone making Imaging compulsory. The differential diagnosis includes a wide range of cystic intraabdominal lesions. The particularity of our case is that Tunisia remains a hydatid endemic area and the first diagnosis suspected for cystic mass is the intraperitoneal hydatid cyst, infected one in this case. In emergency situations US examination is the first level of imaging investigation because of its non-invasiveness, low cost, and non-use of radiation, in order to identify the lesion and to define its structural type characteristics as well as the size. However, CT scan is essential in order to obtain additional informations such as structural feature, internal and peripheral contrast enhancement, as well as loco-regional relationships and a fortiori to rule out differential diagnosis such as hydatid disease and colonic tumors. On CT, these masses show densitometry characteristics of the fluid type, regular margins, and only a peripheral contrast enhancement. For non-urgent presentations, the literature also suggests a role of MRI  in charaTreatment of cystic Lymphangiomas is nonconsensual and depends on its various presentations. Colonic lymphangioma can be managed by endoscopic polypectomy for lesions ranging to a maximum measurement of 2–3.5 cm . RecentlIn our case, the choice of laparotomy was mainly driven by the fact that the patient presented with an acute abdomen and consequently, infected tumor of the colon or a hydatid cyst of the colon couldn't be excluded.Complete resection is the standard radical treatment aiming to prevent recurrence. In fact, incomplete resection results in a 10% post-operative recurrence rate. Complete resection is essential for lymphangiomas of the intestinal tract, and usually requires bowel resection. Pathologic study is mandatory to retain the diagnosis.4Infected Lymphangiomas of the colon are exceptional. The diagnosis can be challenging. The management is various. An open surgery with complete resection is the standard treatment for complicated ones. Laparoscopic and endoscopic treatment hence must be considered.There is no ethical committee in our country (Not applicable for this manuscript).No sources of funding.All authors contributed to the study design. Wael Rebei MD (The operator), performed the surgery. Ahmed Ben Mahmoud, Yacine Ouadi and Atri Souhaieb wrote the manuscript draft. Montasser Kacem, Wael Rebei and Anis Haddad read and corrected the manuscript. All authors read and approved the final manuscript.It is a case report.Ahmed Ben Mahmoud.Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal on request.Not commissioned, externally peer reviewed.All authors declare that they have no any conflicts of interest.


Esophageal subepithelial lesions (SELs) are rare, and the majority of them are benign. SELs are often covered with normal mucosa, thereby resulting in some malignant SELs to be easily missed or misdiagnosed. We report 2 cases of esophageal intramural squamous cell carcinomas (SCCs) that presented as SELs and were endoscopically removed.Case 1 is a 63-year-old man with abdominal distension; case 2 is a 65-year-old man with increasing dysphagia for 2 months.In case 1, endoscopy showed a 1.5-cm mucosal eminence with normal overlying mucosa. Endoscopic ultrasound (EUS) revealed that it might be derived from the muscularis mucosa or submucosa. In case 2, endoscopy revealed a 1.2-cm hemispherical lesion covered with smooth mucosa. Furthermore, EUS revealed that this lesion might be derived from the submucosa.In both cases, the lesions were removed by endoscopic submucosal dissection (ESD). Pathological examination revealed esophageal SCC nests with intramural growth patterns.The first patient underwent postoperative radiotherapy, whereas the second patient did not receive any additional treatment. Both patients agreed to regular follow-up, and no tumor recurrence or metastasis was observed.First, not all esophageal SELs are benign, and a small number of SELs can be malignant. Second, these cases illustrate the value of newer endoscopic techniques, especially ESD. Thus, it is important to be alert when visualizing the esophagus for the possibility of a subtle SEL so that further evaluation and treatment, if necessary, can be undertaken, ideally with a less invasive approach afforded by ESD. They usually do not produce obvious clinical symptoms and are thus often incidentally found. A significant proportion of esophageal SELs are leiomyomas.,4,5 Because only 1% of esophageal SELs are malignant, they are often overlooked or misdiagnosed. Therefore, the accurate and timely diagnosis of SELs is very important. Some published studies reported that endoscopic ultrasound (EUS) can be used to assess the layer of the tumor origin and to obtain tissue specimens for pathological diagnosis to determine benign and malignant tumors via EUS-guided fine-needle aspiration (FNA). However, EUS has some limitations in terms of lack of reliable visualization of the muscularis mucosa. In addition, EUS-FNA can destroy the tumor capsule, which might lead to the increase in the risk of tumor rupture and seeding. During recent years, with the development of endoscopic technology, endoscopic submucosal dissection (ESD) and submucosal tunnel endoscopic resection can achieve complete resection. This has led to the correct diagnosis of several malignant SELs that lacked specific endoscopic presentations.,8Esophageal subepithelial lesion (SEL) is rare, accounting for <1% of all esophageal tumors.We report 2 cases of esophageal SELs removed by ESD and diagnosed as intramural squamous cell carcinoma (SCC).22.1A 63-year-old man was referred to our department on May 02, 2017 owing to a 2-month history of abdominal distension. He had no remarkable medical history, and physical examination indicated no positive symptoms.Upper gastrointestinal endoscopy revealed a mucosal eminence of 1.5 cm (greatest dimension) with normal overlying mucosa seen in the esophagus located at 25 cm from the central incisor  because of the vertical cutting margin was positive. Regular follow-up endoscopy, chest and abdominal CT, and serum tumor marker for 2 years did not reveal any tumor recurrence or metastasis.2.2A 65-year-old man with a 2-month history of increasing dysphagia was admitted to our hospital on June 06, 2017. The patient's medical history was unremarkable, and physical examination revealed no characteristic features.White light endoscopy revealed a hemispherical lesion covered with smooth mucosa located at 30 cm from the central incisor Fig. A. No obvThe multidisciplinary team suggested that the patient should receive regular follow-up, and postoperative treatment should be provided if any signs of recurrence or metastasis were observed. Presently, the patient's dysphagia has been considerably relieved, and no signs of recurrence and metastasis have been observed 1 year after surgery.3 In a 58-year-old man who underwent transhiatal esophagectomy, Von Rahden et al reported that postoperative pathological examination suggested SCC with an intramural growth pattern. Schmitz et al reported a case of intramural SCC located at the gastroesophageal junction with features of a benign lesion. The patient eventually underwent surgery to remove the lower third of the esophagus and the entire stomach. Additionally, Sonthalia et al reported a case of intramural SCC diagnosed by EUS-FNA. Subsequently, the patient was found to have a distant metastasis in the right iliac crest identified by 18-flourodeoxy glucose–positron emission tomography. Kishino et al reported a case of esophageal cancer with features of SET, which had already intramurally metastasized to the stomach.Intramural growth is an extremely rare presentation of esophageal SCC, and only 5 cases have been published in the literature. McGregor et al reported the first case of esophageal intramural SCC in 1976, and the patient was treated with chemoradiotherapy instead of surgery and died 7 weeks after the final course of radiation. Autopsy revealed carcinoma cells that might have arisen from an esophageal intramural squamous epithelial cyst.Our cases differ from these previously published reports in that the tumors were removed in both our patients by ESD. Therefore, this is the first report of esophageal intramural SCCs found and entirely resected by endoscopy. Furthermore, the 2 cases differed in the pathological findings. In case 1, the nests of SCC cells were scattered under a normal, intact mucosa and the infiltration range was from the lamina propria mucosa to the submucosa. In case 2, the nests of cancer cells were massive, and the margin of the cancer tissue was at a certain distance from the vertical margin. The first hypothesis is that the tumor might have originated from the squamous epithelium in an esophageal cyst, small diverticulum, or squamous cell metaplasia in the esophageal glands. The other possibility is that cells from squamous intraepithelial neoplasia might have spread to the submucosal layers by the ducts of the submucosal glands. These cells might then have the capability to grow and infiltrate under an intact epithelium, forming a malignant carcinoma.According to the literature, there are 2 hypotheses about the pathogenesis of esophageal intramural SCC. Kim et al reported a BSCCE resected by endoscopic mucosal resection. Eventually, pathological examination revealed the involvement of vertical margin. Di et al described a case of BSCCE with special endoscopic features, including a gently rising slope on white light endoscopy, little or abnormal staining on iodine chromoendoscopy, and intrapapillary capillary loops with a small-sized avascular area on magnifying endoscopy with narrow-band imaging. This lesion was completely removed by ESD, which meant that the ability to perform en bloc resection made ESD a superior procedure for histopathological evaluation.With the development of endoscopy and particularly of ESD, endoscopists are able to manage a disease in terms of diagnosis and resection, and this enables endoscopists to identify more lesions with caution. Although esophageal malignant SELs are very rare, some cases of malignant lesions that presented as esophageal SELs and were removed by endoscopy have been reported. Basaloid squamous cell carcinoma of the esophagus (BSCCE) is mainly visualized as an elevated lesion with a normal epithelium. first reported an intraluminal polypoid form of esophageal leiomyosarcoma treated with endoscopic resection with an electric snare in 2008. Yamamoto et al performed en bloc resection by ESD for the therapeutic diagnosis of leiomyosarcoma in cases where several biopsy specimens could not confirm the pathology results and the tumor had rapidly grown. Additionally, esophageal GISTs can present as SELs and some of them have the potential to become malignant. Although esophageal GISTs are extremely rare, accounting for about 1% of all GISTs, the prognosis is far worse than that of gastric GISTs.,20 Furthermore, several studies revealed that endoscopy could achieve en bloc resection in case of esophageal GISTs, and endoscopic resection is safe, effective, and minimally invasive.,8,21,22 Moreover, several lymphomas presented as SELs and treated with endoscopic resection have been reported.–27 In addition, according to the literature review, some other rare cases were treated with endoscopy, similar to follicular dendritic cell sarcoma and adenoid cystic carcinoma.,29In addition to the abovementioned epithelial-derived esophageal malignancies, mesenchymal malignancies can occur in the esophagus. Tatsushi et alIn conclusion, esophageal intramural SCC is a rare disease, which may present with the features of SEL. It is important to be alert when visualizing the esophagus for the possibility of a subtle SEL so that further evaluation and treatment, if necessary, can be undertaken, ideally with a less invasive approach afforded by ESD.The authors are grateful to the 2 patients who gave their informed consent for publication.HZ and HX designed and wrote the manuscript. HZ and FL reviewed literature data. BS performed histological assessment and evaluations. HX performed the upper endoscopy and provided the endoscopic photos. HZ and FL collected the medical history of the patient. All authors critically revised the manuscript, approved the final version to be published, and agree to be accountable for all aspects of the work.Conceptualization: He Zhu.Data curation: He Zhu, Bing Shi, Fudong Li.Formal analysis: He Zhu, Bing Shi, Fudong Li.Investigation: Fudong Li.Methodology: He Zhu, Bing Shi, Fudong Li.Resources: He Zhu.Writing – original draft: He Zhu.Writing – review & editing: He Zhu, Bing Shi, Fudong Li.


Partial splenectomy allows preserving immune function in benign splenic lesions such as epidermoid cysts. Determining the plane of resection and perfusion of the spleen remnant can be difficult, especially in centrally located lesions. We present a 13-year-old girl with a symptomatic splenic cyst of 6 cm in diameter located next to the splenic hilum. Laparoscopic partial splenectomy was performed through a 10-mm umbilical approach and three accessory 5-mm ports. Intraoperative intravenous injection of indocyanine green (ICG) at 0.2 mg/kg guided the careful dissection of the splenic hilum and checked the spleen perfusion once the upper arterial branch was clamped. The subsequent wash-out of the ICG allowed inspection of the peripheral vascular return of the splenic remnant through polar veins. Surgery was uneventful with minimal blood loss. Follow-up ultrasound scan revealed a well-perfused small splenic remnant with no signs of recurrence.Laparoscopic partial splenectomy is feasible in benign splenic tumors, especially in those cases of peripheral location. Fluorescence facilitates the safe dissection of the splenic hilum, the visualization of the transection plane of the spleen and the perfusion of the remnant in cases of anatomically and technically complicated partial splenectomies. Splenic cysts are a rare entity in children, and splenectomy is the first choice. Loss of spleen's immune function is associated with potentially serious complications such as postsplenectomy sepsis.We present an unusual case of a child with a hilar splenic cyst in which a laparoscopic partial splenectomy guided by indocyanine green (ICG) fluorescence was performed.A 13-year-old patient was admitted to our center reporting a 1-year history of diffuse abdominal discomfort and asthenia. She visited the emergency department due to worsening pain and vomiting during the last 24 hours. Abdominal examination and blood tests were normal. An ultrasound scan was performed revealing a 5 × 5.3-cm smooth-edged oval lesion with hypoechoic content, echoes, and thin septa. Magnetic resonance imaging (MRI) and computed tomography (CT)  reveale2pneumoperitoneum with a 4 L/minute flow rate. The surgeon, assistant, and scrub nurse were on the right side of the patient.The surgical procedure was performed with the patient in supine position with the left side elevated 45 degrees. A 10-mm umbilical Hasson trocar was inserted by open technique and three 5-mm accessory trocars were later placed subxiphoid, epigastric, and on the left flank. Laparoscopic equipment from Stryker Corporation  with a led and near-infrared light source (L10 Led Light Source with AIM) and 5 mm 30 degrees optics (1588 AIM) was used. Laparoscopy was performed with a 12 mm Hg COAn orthotope spleen with a large peripheral cyst on its lateral side was found. The spleen had some adhesions to the diaphragm and abdominal wall. Small accessory spleens were found on the medial aspect .The procedure started with bipolar cauterization and section of the short gastric vessels. Then, dissection of the splenic hilum identifying the splenic artery and vein was performed. After ligature of the superior branch of the splenic artery, the fluorescent dye was administered intravenously. ICG-PULSION 25 mg powder for solution  was diluted in 5 mL of bidistilled water to a concentration of 5 mg/mL. From this dilution, a bolus of 0.2 mg/kg was administered. Seventy-two seconds after administration, the contrast was observed in the splenic artery and subsequently fluorescence in the lower-lateral end of the spleen, while the region corresponding to the cyst remained without uptake .After verifying ICG wash-out through the inferior peripheral polar vessels , the suPathological anatomy reported a 96 g specimen. Macroscopically, normal splenic parenchyma surrounded a smooth and thin-walled cyst. Microscopic study showed a cyst covered by a nonkeratinized epithelial lining with an immunohistochemical profile corresponding to an epithelial cyst.The postoperative course was uneventful. The patient did not require blood transfusion and was discharged after 48 hours. Follow-up ultrasound 1 month after surgery revealed a well-perfused small splenic remnant with no signs of recurrence.Splenic cysts are rare entities. In our setting, up to 90% of primary splenic cysts are epidermoid (nonparasitic), excluding neoplastic cysts. Most primary splenic cysts are diagnosed in children or young adults. Symptoms can range from an incidental finding to life-threatening complications such as rupture, bleeding, or infection. Up to 70% of cysts are symptomatic,The confirmatory diagnosis is radiological. The initial imaging test is an ultrasound scan with pulse Doppler. CT or MRI allow better anatomical definition and surgical preparation. It is important to carefully delineate the splenic vascularization, both arterial and venous branches.Less aggressive therapeutic alternatives have been described for splenic cysts. Ultrasound monitoring may be useful in simple cysts under 5 cm. Percutaneous aspiration alone or combined with the injection of sclerosing agents, and other surgical procedures such as marsupialization or unroofing have shown a very high recurrence rate, and are also associated with the creation of adhesions that hinder subsequent surgery. Therefore, their use is nowadays discouraged.Splenectomy as treatment of choice is being replaced by partial resection procedures that preserve part of the splenic parenchyma, thus avoiding infectious complications related to the loss of spleen's immune function. Postsplenectomy sepsis is the most serious complication, having a prevalence of 4% of the cases with a mortality of 1.5%.Partial splenectomy is currently considered to be the ideal procedure. Like the vast majority of splenic surgical procedures, the ideal approach is minimally invasive. The dissection of the splenic vessels and their branches continues to be the critical step in this surgery, specially hindered by the broad anatomical variability. For this reason, preoperative angiography is recommended to define the vascular anatomy.ICGLimitations to the use of ICG are scarce. In patients with abnormal liver function the uptake and especially, the elimination may be altered. It is contraindicated in those allergic to iodinated contrasts or with thyroid disorders.The use of fluorescence in surgery is emerging, but experience in splenic pathology is very limited. Kawasaki et alWe consider that the use of fluorescence provides advantages over conventional laparoscopic partial splenectomy. It facilitates vascular dissection by defining the anatomy in real time, it demonstrates adequate arterial perfusion of the remaining tissue, and it increases the safety of a technically complicated procedure, without increasing the surgical time and without notable drawbacks.When surgical resection of epidermoid cysts is indicated, we believe that the ideal technique is laparoscopic partial splenectomy, allowing the removal of the lesion while preserving the splenic parenchyma. Dissection of the splenic hilum is the key step during the procedure. The use of ICG-mediated fluorescence facilitates dissection and allows the vascularization of the splenic remnant to be checked, which increases the safety of the procedure.To the best of our knowledge and by going through the previous publications in PubMed, this is the first article regarding the use of fluorescence during a laparoscopic partial splenectomy in a pediatric patient.


Primary lacrimal sac melanoma is an extremely rare condition with fewer than 50 cases reported so far. Clinically, its symptoms resemble those of dacryocystitis, leading to frequent misdiagnosis. During diagnosis, imaging examination is often performed to differentiate tumors from inflammation. In this report, we present a case of primary lacrimal sac melanoma and summarize the CT and MRI characteristics of lacrimal sac melanoma.We report a 50-year-old female patient who had undergone a dacryocystectomy for the left lacrimal sac mass. Postoperative pathological examination confirmed the presence of primary malignant melanoma. Three months later, a lump in the lacrimal sac area was found. The patient underwent CT and MR examinations. CT scan demonstrated a partially well-defined soft mass in the fossa of left lacrimal sac extending into the nasolacrimal duct and anterior ethmoid sinus. MRI revealed an intermediate signal intensity on T1 and iso-or hyper-signal on T2 weighted images. Histopathological examination on biopsy confirmed recurrence of primary lacrimal sac melanoma.None has described the CT and MR findings of primary lacrimal sac melanoma so far. Typically, MR images show hyperintensity signal on T1-weighted images and hypointense signal on T2-weighted images owing to the paramagnetic properties of melanin. In contrast to previous reports and the present case, most cases do not present these typical signals. Thus, reporting such radiological findings is important to create awareness on variant images of primary lacrimal sac melanoma. This will reduce misdiagnosis and mistreatment. Primary malignant melanoma of the lacrimal sac is an extremely rare condition. Yet, the lesion is highly malignant, can metastasize at a relatively early stage and patients with such lesions have poor prognosis. Early diagnosis is therefore essential. Notably, this condition is often misdiagnosed as chronic dacryocystitis. For accurate diagnosis, imaging examination is recommended to assess the severity of the disease, differentiate between tumors and inflammation and characterize tumor features. Here, we present a case of primary malignant melanoma of the lacrimal sac. We provide a summarize of the computed tomography (CT) and magnetic resonance imaging (MRI) findings and review previous literature on this condition.A 50-year-old female patient who had a mass in the lacrimal sac area for more than 3 months is presented in this report. The mass exhibited slow but progressive swelling. She experienced occasional epiphora and pain, without blood discharge or impaired vision. After 6 months, the patient underwent dacryocystectomy for the left lacrimal sac mass. Postoperative pathological examination confirmed the presence of malignant melanoma. Three months after the operation, a lump in the lacrimal sac area had gradually increased. Local recurrence was suspected and she was referred to ophthalmology department for further treatment.A physical examination revealed a scar from the previous operation. In addition, a 1.5 cm × 1.5 cm firm, localized and relatively clearly defined mass was observed in the left medial canthus. There was no cervical lymphadenopathy. A CT scan demonstrated a partially well-defined soft mass in the fossa of left lacrimal sac extending into the nasolacrimal duct and anterior ethmoid sinus   Table . BecauseOf note, CT is not sufficient for tumor diagnosis because CT values do not show the number of melanin granules. In contrast, MRI images provide effective diagnosis for malignant melanoma. MRI has a high soft-tissue resolution, making it the best imaging technique for evaluating malignant melanoma currently. Typically, MR images show the presence of hyperintensity signal on T1-weighted images and hypointense signal on T2-weighted images owing to the paramagnetic properties of melanin in most other parts of malignant melanomas. But this condition is rare in malignant melanoma of lacrimal sac. To the best of our knowledge, only six case reports have been published describing the MRI radiological features of this condition. Four cases (4/7) (including our case) show intermediate signal intensity on T1 weighted image, and only one case presented with typical signals on T2 weighted images , malignant lymphoma (7.8%), neural tumors, inflammatory lesions and metastatic tumor . Cases wTo date, none has summarized the CT and MR findings on primary lacrimal sac melanoma. Based on previous reports and our case, most cases do not present typical signals. Thus, reporting such radiological findings is important to create awareness on variant images of primary lacrimal sac melanoma. This will reduce misdiagnosis and mistreatment.


Ulnar hemimelia, also referred to as post-axial longitudinal deficiency of the upper limb, is a very rare skeletal anomaly characterized by the partial or complete absence of the ulna. The majority of the reported cases are sporadic and more common in males. The disorder is mostly unilateral, right-sided and incomplete. A slight shortening of the forearm, radial bowing and ulnar-sided hand drift are the anomalies which often accompany ulnar hemimelia. Ulnar hemimelia may also be seen in association with complex wrist and hand anomalies. The absence of post-axial metacarpal and digital bones are frequent findings in patients with this rare disorder. Cases with additional digital abnormalities such as post-axial syndactyly and camptodactyly are also present in the literature. However, a case of ulnar hemimelia in association with mesoaxial synostotic syndactyly has never been reported to date. A 20-year-old male with left upper limb disability admitted to Dışkapı Yıldıım Beyazıt Training and Research Hospital for mandatory health screening before military service. He had no remarkable symptoms or signs indicative of else pathology other than the skeletal disorder involving his left upper limb. He is the second of three sons born to non-consanguineous healthy parents. His brothers do not have any skeletal or other systemic disorders. There is no family history of any kind of congenital skeletal abnormalities in the extended family. His mother was at the age of 27 when she gave birth to our patient. There is no history of any drug, smoke, alcohol or radiation exposure during pregnancy. Our patient was born uneventfully at full-term through normal vaginal delivery. No other significant health problem is present in the history of his childhood. He stated that he had never received surgical or non-surgical treatment for the deformities involving his left upper extremity.On physical examination, his left upper limb was short and forearm was slightly curved . There w–5th metacarpals as well as fingers were absent . There was symmetric triangular-shaped synostosis of the proximal phalanges of the 2–3rd fingers while the respective metacarpals were normal . There were two metacarpophalangeal but only one proximal interphalangeal joint in this synostotic finger. Fixed flexion of the proximal interphalangeal (camptodactyly) and fixed extension of the distal interphalangeal joints of the synostotic finger were evident on lateral hand radiograph. The thumb was normal.Anteroposterior radiograph of the left arm showed that the shoulder joint and humerus were normal . AnteropBased on the characteristic radiographic findings, the patient was diagnosed as having ulnar hemimelia with postaxial oligodactyly and mesoaxial synostotic syndactyly.A regular and continuous physiotherapy plan was arranged in order to optimize the functionality of the elbow joint and prevent the development of disuse atrophy. However, no surgery was planned.1 Different from the radial hemimelia, ulnar hemimelia is mostly non-syndromic and the majority of the reported cases are sporadic. It may also appear as a component of skeletal dysplasias. In rare cases, ulnar hemimelia may present with some syndromes such as Poland, Goltz-Gorlin, Cornelia De Lange, or Femur Fibula Ulna syndromes. The disorder is more common in males compared to females. In approximately 70% of the cases, ulnar hemimelia is unilateral, mostly right-sided and incomplete.2Hemimelia is the name given to the congenital skeletal anomaly that presents as the absence or underdevelopment of one side of the distal half of a limb. Ulnar hemimelia, also referred to as post-axial longitudinal deficiency of the upper limb, occurs at a rate of about 1 in 150,000 live births. It is a very rare type of hemimelia in which the ulna is completely or partially absent.3 Some shortening of the forearm, radial bowing and tendency of the hand to drift to the ulnar side of the wrist which usually accompany ulnar hemimelia were all present in the case we currently present. Other skeletal anomalies such as humeroradial synostosis, radial head dislocation and carpal coalition may also be seen in cases with ulnar hemimelia.2–5 While there were a humeroradial joint distance reduction and a fixed flexion of about 45° of the elbow joint in our patient, a characteristic humeroradial synostosis which typically causes fixed flexion or extension of the elbow joint was not present.Ulnar hemimelia presents as the hypoplasia of the forearm accompanied by complex elbow and carpus abnormalities.6 It is a striking finding that there is an increased incidence of wrist and hand deficiency as one passes from the radial side to the ulnar side.3 So, the absence of post-axial carpal, metacarpal and digital bones are frequent findings in patients with ulnar hemimelia. In general, the degree of wrist and hand anomaly correlates with the degree of ulnar hypoplasia. The most common hand anomaly associated with the disorder is three-fingered hand, closely followed by monodigital hand.3,4 Cases with additional digital abnormalities such as postaxial syndactyly and camptodactyly are also present in the literature.2 Our patient presented a marked deficiency of the ulna. And parallel to the severity of the ulnar failure, postaxial carpal metacarpal and digital elements were all absent. Moreover, an unusual form of syndactyly involving the index and middle fingers was evident on the hand radiograph of the patient. A triangular shaped proximal phalanx was articulating with the second and third metacarpals and a single middle phalanx.As an indication that post-axial wrist and hand halves are uniquely formed in response to an ulnarizing influence, the anatomy of the carpal, metacarpal and digital elements alters in almost all cases of ulnar hemimelia.7 The phenotype of the digital anomaly of this case, which was almost the same as that of our patient, included a triangular proximal phalanx articulating with the second and third metacarpals and a single middle phalanx. Malik et al stated that this case was consistent with syndactyly type IX (OMIM #609432) and objected its presentation as a new syndactyly type.8–10 However, while syndactyly type IX is characterized by osseous synostosis of the third and fourth metacarpals, synostosis of the case of Weinrich et al was involving the second and third proximal phalanges. Therefore, this discussion did not result in a consensus.11 As syndactyly of our patient is not isolated but accompanies ulnar hemimelia, the present case is outside the limits of this discussion. What makes our case special is that it is the only reported case of mesoaxial synostotic syndactyly in association with ulnar hemimelia.Recently, Weinrich et al reported a case of an isolated unilateral mesoaxial synostotic syndactyly and presented this unusual case as a novel nonsyndromic syndactyly of the hand.2 It is shown that the majority of surgeries for ulnar hemimelia are performed on the hand. Hand surgeries are planned for the proper function of the thumb and fingers. Syndactyly release, thumb rotation, modified pollicization procedures and rotational metacarpal osteotomies are surgical procedures performed to increase the ability to use the hand.5,6 In selected cases, various surgical approaches including Z-plasty, elbow disarticulation, and humeral derotation osteotomy are performed to improve the range of motion of the elbow joint.2 Our patient stated that he had never received physiotherapy or surgical treatment to date. In order to improve the elbow function and avoid disuse atrophy, a regular and continuous physiotherapy was planned. However, he was not suitable for any kind of surgical intervention and no surgery was planned.The clinical presentation and course of ulnar hemimelia is determined by the severity of the ulnar deficiency and the presence of other accompanying upper limb anomalies. While patients with an isolated mild ulnar deficiency may remain asymptomatic, cases with prominent ulnar deficiency accompanied by complex upper limb abnormalities present with a severe disability. Treatment of this disorder is difficult and should be highly personalized. Continous physiotherapy and training should be given from infancy in order to avoid disuse atrophy and to obtain maximum functioning of the limb.Written informed consent was obtained from the patient for publication of this case report, including accompanying imagesUlnar hemimelia, also referred to as postaxial longitudinal deficiency of the upper limb, is a very rare skeletal anomaly characterized by the partial or complete absence of the ulna.Some shortening of the forearm, radial bowing and an ulnar-sided hand drift usually accompany this rare disorder.The absence of post-axial carpal, metacarpal and digital bones are frequent findings in patients with ulnar hemimelia.Mesoaxial synostotic syndactyly, which is a very rare hand anomaly, may accompany ulnar hemimelia.


Hydatid Disease (HD), or hydatidosis or echinococcosis, is an endemic infection and a major public health concern in the Mediterranean region. At times it involves the primary soft tissues, such as in the breast and muscle, though this is quite uncommon even in endemic areas.A. A 31 year-old woman complained of a gradual progressive, painless lump in the left axillary tail of spence for two years. Examination revealed a firm lump measuring 5cm × 5cm, non-mobile, in the left axillary tail of Spence. B. A 32 year-old woman presented with mild and continuous pain in lateral aspect of left thigh. On examination, there was a round, non-tender, non-mobile mass in the lateral aspect of her left thigh. Preoperative imaging studies in both patients revealed evidence of HD. Both of the patients underwent surgery and received Albendazole twice per day for 10 days, before and three months after surgery.The disease can be diagnosed by serological and radiological modalities, both of which are not definitive. Ultrasonography should be the first diagnostic modality of soft tissue HD, however, MRI can be used to understand clearly the surgical involvements of structures. The standard treatment of soft tissue HD is surgery using pericystectomy techniques, as well as anthelmintic therapy.HD should be suggestive in soft tissues if mass is slowly developing and presenting with local extension, particularly in endemic countries. Excision of HD using pericystectomy technique is the first choice of intervention for HD of soft tissues. This pa22.11 and 2). Chest X-ray and abdominal ultrasound excluded lung and liver HDs. ELISA test of Echinococcus Granulosa titer test was negative. A primary muscular Hydatid cyst was diagnosed and a pericystectomy was chosen as the preferred surgical treatment. Treatment involved 400 mg of Albendazole twice per day for 10 days before and three months after surgery. The longitudinal incision (pericystectomy) was performed under spinal anesthesia in the anterolateral aspect of the left thigh (3). Cyst cavity was thoroughly irrigated with hypertonic saline and wound closed after putting a drain (3). The surgery has been carried out under the supervision of an attending general surgeon. Histopathological diagnosis confirmed the diagnosis of Hydatid Disease. The postoperative course of the patient was favorable. As Albendazole (400 mg twice per day) started 10 days before surgery, it was continued for three month after surgery and there was no evidence of recurrence of the lesion during the 8 month follow-up.A 32 year-old woman from a rural area, presented to the general surgery clinic with mild and continuous pain in the lateral aspect of her left thigh. Concomitantly, she was complaining of gradually increasing swelling in the lateral aspect of her left thigh for about 1 year duration. Family history and drug history was unremarkable. On examination, there was a round, non-tender, non-mobile mass in the lateral aspect of her left thigh. There was no overlying skin changes, ultrasound (US) showed 15cm × 5cm solid-cystic lesion in the lateral aspect of the left thigh with multiple well-defined cystic lesions. Magnetic Resonance Imaging (MRI) showed a relative large multisystem lesion in the lateral aspect of the thigh with connection to the femoral bone, in favor of hydatid cyst 1 and 2. ft thigh 3. Cyst c a drain 3. The su2.21). The chest X-ray and ultrasonography of the abdomen of the patient were unremarkable. The patient underwent surgery for the removal of the lump. The surgery was performed by attending general surgeon and after general anesthesia a curvilinear incision in hair line margin, the cyst was removed in-toto. The excised cyst was oval in shape and measured 5 cm × 4.5 cm × 3 cm. When the cyst was opened, endocysts were found confirming it to be a hydatid cyst (2). Albendazole (400 mg twice per day) was continued and control imaging was performed.  There was no evidence of recurrence of the lesion during the 1 year follow-up.A 31 year-old housewife presented with a gradually progressive, painless lump in the left axillary tail of Spence since two years. There was no history of injury, no discharge from the nipple, and no family history of breast cancer. She did not provide a history of close contact with any animal. Examination revealed a firm lump measuring 5cm × 5cm, non-mobile, in the left axillary tail of Spence. The rest of the left breast and nipple were normal. Collaborative ultrasonography study of the breasts revealed a thick walled cystic lesion with floating membranes and internal echoes in the region 1. The chtid cyst 2. Albend3Hydatid Disease (HD) or hydatidosis or echinococcosis is an endemic disease and a major health concern in many parts of the world, including the Mediterranean region and the Middle East . Iran is4Hydatid Disease should be suggestive in soft tissues presenting with slowly developing masses with local extension, particularly in endemic countries. MRI is the most useful diagnostic modality of HD of the soft tissues and for planning the surgery, whenever ultrasonography or serological tests do not sufficiently characterize the disease. Excision of the hydatid cyst using pericystectomy technique is the first choice of treatment for HD of the soft tissues.There is no conflicts of interest.None.This is a case report paper.Informed consent was obtained from the both of the patients, 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.MS and JZB conceptualized the study, Sh.Q acquisition of data, and drafting the manuscript, D.E.L.P revising for critical intellectual concept and approved of the version to be submit.Not applicable.Corresponding author is Dr. Javad Zebarjadi Bagherpour accept full responsibility for the work and/or the conduct of the study, had access to the data, and controlled the decision to publish.Not commissioned, externally peer-reviewed.


Gastrointestinal ischemia is rare after small pelvis surgery. Minimal invasive robotic surgery requires adaptation of the surgical approach for cystectomy and derivation construction such as the use of pneumoperitoneum and Trendelenburg positioning of the patient. Two cases with gastric ischemic complications after robot-assisted radical cystectomy are described. The first case was a 68-year-old female who had prolonged gastroparalysis and blood in a replaced gastric tube at day 10 after robotic cystectomy and Bricker urinary derivation. Gastroscopy revealed ischemia of gastric and proximal duodenal mucosa while computed tomography showed multiple calcifications and thrombi in the coeliac trunk branches and splenic infarcts. The stenosis of the origin of the mesenteric superior artery was stented via an endovascular procedure, and the patient recovered with normal gastroscopy 1 month postoperatively. The second case was a 73-year-old male who developed abdominal pain and fever 5 days after robotic cystectomy and Bricker. On abdominal computed tomography imaging, subcutaneous emphysema, intra-abdominal air, and calcification at the origin of the coeliac trunk were found. At laparotomy 5 days after the cystectomy, a 3 cm hole in the fundus of the stomach was found which was removed with the major stomach curvature. Gastroscopy 5 days after hemigastrectomy revealed no remnant ischemia. The prolonged pneumoperitoneum during robotic cystectomy, the deep Trendelenburg position, and the preoperatively impaired vascular system can be the reasons of our first two cases of gastric ischemia. This rare complication should be kept in mind in patients with symptoms of gastric ischemia since it can result in gastric perforation. Radical cystectomy is the gold standard for the treatment of muscle-invasive bladder cancer and high-risk nonmuscle invasive disease. Despite the widespread application and the increasing surgical experience with robot-assisted radical cystectomy (RARC), Clavien − Dindo ≥ 2 complications have been reported in 35-40% of the patients treated with RARC . IschemiA 68-year-old female with a prior history of multiple sclerosis, peripheral vascular atherosclerosis with an arterial stent in the left external iliac artery developed a multifocal nonmuscle invasive high-grade bladder cancer. Due to a low compliance bladder, multifocal disease, and suspected/potential muscle-invasive disease, it was decided to perform an anterior exenteration with the removal of bladder, urethra, uterus, and adnexes using a robot-assisted surgical approach. The procedure took 4 hours and included the construction of an intracorporeal Bricker urinary derivation. During the procedure, no bowel perfusion abnormalities were noted by the surgeons, but a remarkable short small-bowel mesentery was reported. Postoperative recovery was characterized by prolonged gastroparalysis and blood in a replaced gastric tube at day 10 postoperatively. Gastroscopy revealed ischemia of gastric and proximal duodenal mucosa . The BriA 73-year-old male was seen with BCG-resistant carcinoma in situ of the bladder. The patient was known with hypercholesterolaemia, hypertension, and pulmonary embolism but no significant vascular disease. Five years after the primary diagnoses, RARC and pelvic node dissection with urine derivation according to Bricker was performed. Five days postoperatively, the patient developed abdominal pain, fever, and elevated serum CRP. On abdominal CT imaging, subcutaneous emphysema and intra-abdominal air were visible with perigastric and subphrenic fluid and renal ischemia on the left side, as well as calcification at the origin of the coeliac trunk  pneumoperitoneum . It has  hours pnPatient-related risk factors can also impair splanchnic perfusion. Specific risk factors include advanced age, diabetes, hepatic and renal impairment, atherosclerosis, low cardiac output, cardiac arrhythmias, and medications (such as diuretics and beta-blockers) which can reduce intestinal perfusion . In our 2 pneumoperitoneum and a long surgical procedure can impair cardiac and vascular function, which could result in decreased gastric perfusion.Prolonged Trendelenburg position can be also associated with gastric ischemia since it has been reported to be the cause also of acute intestinal distress syndrome . This sy2 insufflation, absorption, and elimination. Immediate gastroscopy in patients with symptoms of gastric ischemia  will allow an early diagnosis and improved survival. Treatment should include vascular support, antibiotic therapy, and vigorous use of proton-pump inhibitors. Patients with signs of ischemic stomach perforation require abdominal exploration, endovascular reconstruction, and/or partial gastrectomy.Measures to prevent this rare complication can be an increased intraoperative hydration, application of the lowest insufflation pressure possible, intermittent abdomen decompression, and use of novel smoke evacuation systems which combine stable intrabdominal pressure with constant suction via a mechanism of valve and insufflation. It has been associated with reduced need of COThe prolonged pneumoperitoneum during RARC, the deep Trendelenburg position, and the preoperatively impaired vascular system can be the reasons of our first two cases of gastric ischemia. This rare complication should be kept in mind in patients with symptoms of gastric ischemia since it can result in gastric perforation.


Neuroendocrine adenomas of the middle ear are rare tumors that represent less than 2% of primary tumors of the ear. In this paper, we describe a case of a 40-year-old woman who developed neuroendocrine adenoma of the middle ear. The specific management strategy for this rare tumor is unclear; information in the available literature on the management of this tumor is varied. However, an extensive demolition seems to be the gold standard treatment for this tumor to avoid recurrence and regional metastases in the lymph node or distant metastases. For the present case, we performed an incisional biopsy to confirm the diagnosis, and thereafter, we performed a canal-wall-down tympanoplasty. For cases like the present one, careful long-term clinical and instrumental follow-up is required to monitor progress and facilitate patient recovery. Neuroendocrine adenoma of the middle ear was described for the first time in 1976 . The claNeuroendocrine adenomas show a low aggressive biological behavior with slow local growth, but they can recur and cause local and distant metastases, suggesting they should be classified as having low-grade malignancy . ReachinHerein, we report a case of a 40-year-old woman treated for a neuroendocrine adenoma in her right ear and present a brief review of the available literature on this rare tumor.A 40-year-old woman presented to our clinic in February 2019 with a four-year history of aural fullness in her right ear associated with recurrent otalgia, itching, headache, and postural instability. She did not have any other otologic and neurologic symptoms or signs.In Oct 2015 and April 2018, the patient went to an emergency room because her headaches worsened during these periods. The headaches were cluster headaches characterized by severe pain in the frontal and temporal regions. In 2018, the headaches were associated with a mucopurulent otitis in her right ear. The patient underwent two brain computed tomography (CT) scans, one for each hospitalization. In 2018, the second CT scan revealed signs of inflammation of the hypotympanum and mesotympanum of her right ear. The patient was treated with antibiotic therapy.At the time of presentation to our clinic, she had no history of neoplastic pathologies, smoking, or alcoholism. Clinical examination showed that her right tympanic membrane was intact, but some whitish material could be seen at the retrotympanic level. Measurement of her audiometric threshold showed that she had bilateral normal hearing levels .Magnetic resonance imaging (MRI) of the ear did not reveal any signs of cholesteatoma  in the right ear ; therefoAfter the diagnosis was made, examination using positron emission tomography/CT did not reveal any local or distant metastases. Therefore, a new MRI was performed to monitor the state of the local disease. The imaging showed some pathological residual tissue .A multidisciplinary team, which comprised a radiologist, a radiotherapist, an oncologist, and an otolaryngologist, determined surgical treatment to be the best therapeutic choice to treat the disease. A radical demolition to eradicate the residual disease was carried out by performing a canal-wall-down tympanoplasty removing the stapes and footplate and with preservation of the optic capsule. The patient was hospitalized for six days; this period included the duration of the surgery and the postoperative period.Postoperative audiometric examination revealed the presence of good auditory residue in the operated ear . The patUntil today, the patient was not treated to restore the hearing loss. We retain to suggest bone-anchored hearing aids after 3–5 years from the treatment of the tumor.In this report, we present the details of a rare case of neuroendocrine adenoma of the middle ear. The pathological and immunohistochemical findings of this case were consistent with those described in the existing literature –7.The pathogenesis of this tumor is currently a topic of discussion, with different authors proposing different origins. Hyams and Michaels speculated that it may originate from the cells of the mucosa of the middle ear ; they alPatients with this tumor usually present with hearing loss, otalgia and ear fullness, and an intact eardrum behind a visible brown-red mass. A diagnosis is often delayed due to the rarity of this tumor, and biopsy is usually needed to reach a definite diagnosis. In most cases, such as in ours, cholesteatoma is the first suspect. The differential diagnoses of a neuroendocrine adenoma include jugulotympanic paraganglioma, vascular malformations, acoustic neurinoma, meningioma, endolymphatic sac papillary tumor, rhabdomyosarcoma, teratoid tumor, and adenocarcinoma –10. HoweSeveral authors have proposed different classification systems because this neoplasm, although seldom, tends to exhibit local invasion, local recurrence, and metastatic potential , 8, 11. In the 2005 World Health Organization classification, these tumors were classified as neuroendocrine carcinomas with low-grade epithelial differentiation (grade I), whereas in the recent 2017 classification, they were classified as adenomas with neuroendocrine features .Marinelli et al. in a multi-institutional retrospective study on 32 cases proposed a “T/N/M/S” staging system . In accoBased on information in the existing literature, a complete surgical resection is the primary treatment method for neuroendocrine adenomas of the middle ear. Although four metastatic cases treated with adjuvant radiotherapy have been previously reported, chemotherapy has not been described as a viable treatment method , 13. LocA middle ear adenomatous neuroendocrine tumor is a rare tumor that is characterized by an indolent course and an uncertain malignant potential. Due to its ability to recur locally, the regional and distant metastases reported in the available literature, and the absence of sufficiently valid data to predict the progress of this tumor, extensive demolition with negative margins and careful long-term clinical and instrumental follow-up are required to monitor its biological progress.


The patient underwent an endoscopic endonasal approach for retroclival mass resection three weeks later. The tissue analysis of the mass was consistent with ecchordosis physaliphora. This could have been misdiagnosed as idiopathic intracranial hypertension had the MRI of the brain not been performed.We report a rare case of ecchordosis physaliphora presenting with headache, nausea, and diplopia. On neurological examination, the patient was found to have left abducens nerve palsy. CT of the head without contrast was unremarkable. Brain MRI demonstrated a non-enhancing retroclival mass with a mass effect upon the ventral pons. The mass had increased signal intensity on T2 and decreased signal intensity on T1-weighted sequences. Lumbar puncture revealed an opening pressure of 37 cm H Ecchordosis physaliphora is a congenital benign hamartomatous lesion derived from notochord remnants. It is usually located in the retroclival prepontine region but can be found anywhere from the skull base to the sacrum . Unlike 2O; 28 CC of CSF was removed, and the subsequent closing pressure was 22 cm H2O. The CSF examination was unremarkable ]. She also reported a 30-lbs unintentional weight-gain over the past year. Neurological exam revealed impaired left eye abduction without papilledema or other neurological deficits. CT head without contrast was unremarkable Figure , 1B. In le Table , 2C, 2D.Three weeks later, the patient underwent an endoscopic endonasal approach for retroclival mass resection. The hematoxylin and eosin-stained, formalin-fixed, paraffin-embedded sections of the lesion showed physaliphorous cells and lymphocytic infiltrates in adjacent tissue; immunohistochemical stains showed strong reactivity of tumor cells for cytokeratin AE1/AE3) and weak reactivity for S100. The surgical finding and tissue analysis of the mass were consistent with ecchordosis physaliphora (Figure  and weak2O. Given the brain MRI findings and follow-up outcomes, we believe that direct compression from the tumor and increased intracranial pressure contributed to the patient’s symptoms.Ecchordosis physaliphora is a congenital benign hamartomatous lesion. It is usually asymptomatic and incidentally found in 0.5-2% of autopsies. Our patient presented with an acute severe headache and sudden-onset diplopia. Lumbar puncture demonstrated intracranial hypertension with an opening pressure of 37 cm HThe diagnosis of ecchordosis physaliphora can be challenging without the aid of an MRI. The initial head CT was read as normal in this case. In cases of intracranial hypertension and obesity, idiopathic intracranial hypertension is often atop the list of differential diagnoses. In this case, however, there was no papilledema on the fundoscopic exam. Brain MRI with and without contrast revealed the possible diagnosis of ecchordosis physaliphora with specific features: non-enhancing retroclival mass with increased signal intensity on T2-weighted sequences and decreased signal intensity on T1-weighted sequences ,9. The mNo previous case reports regarding ecchordosis physaliphora have mentioned raised opening pressure in the lumbar puncture. Our patient's initial brain MRI showed non-enhancing retroclival mass with mass effect upon the ventral pons and the left sixth cranial nerve stretch. The mass with a size of 17 mm x 15 mm x 19 mm at the prepontine region might cause intracranial hypertension even though brain MRI did not show any radiographic features for increased intracranial pressure. While the patient did not have papilledema, we could not rule out idiopathic intracranial hypertension completely due to the patient’s history, which was the reason why the patient was placed on Diamox initially. A lesson learned from this case is that a brain MRI with and without contrast is essential for the diagnosis of ecchordosis physaliphora and that this diagnosis should be considered in cases that show similarity to idiopathic intracranial hypertension. In our patient, ecchordosis physaliphora could have been misdiagnosed as idiopathic intracranial hypertension had the MRI of the brain not been performed.In this report, we discussed a rare case of symptomatic ecchordosis physaliphora presenting with headache, nausea, abducens nerve palsy, and intracranial hypertension. The diagnosis was revealed by brain MRI with and without contrast as well as tissue analysis.