We present a rare case of a four-year-old boy with a botyroid embryonal rhabdomyosarcoma of the right middle ear. Rhabdomyosarcoma is a soft tissue malignancy which is thought to originate from embryonic mesenchymal cells of striated skeletal muscle.  It is a disease primarily of children and is exceptionally rare in parameningeal regions.  The diagnosis is often delayed and easily misdiagnosed as aural polyp. Therefore, advanced disease is common at the time of diagnosis.  A four-year-old boy presented with a four-month history of recurrent left ear blood and pus discharge, otalgia and fevers. He attended his GP three times and paediatric A&E 13 times where he received antibiotics for presumed otitis media and externa. He was eventually referred to the otolaryngology department and underwent an examination under anaesthesia of ear and excisional biopsy of a suspicious aural polyp.  Staging chest CT and PET scan showed no loco-regional spread or distal metastasis. Magnetic resonance imaging demonstrated absence of invasion into adjacent organs.  Histology confirmed embryonal rhabdomyosarcoma, botryoid subtype.  Subsequent to the initial excision of the polyp, he was started on an ifosfamine, vincristine and actinomycin (IVA) chemotherapy regime in three weekly cycles for nine cycles with concomitant radiotherapy. Two weeks subsequent to his first chemotherapy dose he presented with a House-Brackmann II-III facial nerve palsy but no other middle ear complications. He was started on intravenous antibiotics and dexamethasone. The facial nerve palsy incompletely resolved with treatment. The commonest areas of the body to be affected are the head and neck, the bladder and reproductive organs. They are rare elsewhere and are exceptionally rare in parameningeal regions, including the ear and mastoid.Rhabdomyosarcoma is a rare soft tissue malignancy with unknown aetiology. It is thought to originate from embryonic mesenchymal cells of striated skeletal muscle. It is a disease primarily of children, with two-thirds of patients presenting under the age of six yearsSymptoms depend on the location of the tumour. Aural involvement often has non-specific symptoms such as fever and otalgia. If the tumour is significant in size and protrudes through the tympanic membrane, then otorrhoea and hearing loss may result. Rarely, facial nerve palsy may occur. The diagnosis is often delayed and easily misdiagnosed as aural polyp. Therefore, advanced disease is common at the time of diagnosis.We present a rare case of a four-year-old boy with a botyroid embryonal rhabdomyosarcoma of the right middle ear and review the literature.A four-year-old boy presented with a four-month history of recurrent left ear blood and pus discharge, otalgia and fevers. He attended his general practitioner (GP) three times and Paediatric Accident & Emergency 13 times where he received oral, topical and intravenous antibiotics for presumed otitis media and externa. He had no relevant family history. He was eventually referred to the otolaryngology department after 19 months and was listed for an examination under anaesthesia of and excisional biopsy of a suspicious aural polyp 2 weeks after presentation to the otolaryngology department team. Intra-operatively, a large lesion extending from the middle ear through the tympanic membrane and into the external auditory canal was noted which was incompletely resected as only tissue lateral to the tympanic membrane was resected. The lesion was found to be highly vascular.Histopathological findings were in keeping with embryonal rhabdomyosarcoma, botryoid subtype. This rare diagnosis required a second opinion from the Histology Department at the Royal Marsden Hospital. Immunochemical staining revealed sheets of rounded tumour cells with scant cytoplasm and inconspicuous nucleoli, which were distinguished by the formation of polypoid and grapelike tumour masses. Malignant cells in an abundant myxoid stroma were observed. Staining was positive for desmin and muscle-specific actin.Magnetic resonance imaging (MRI) of the head was performed within two weeks of resection of the tumour, which demonstrated absence of invasion into adjacent organs. Staging chest MRI showed no evidence of metastases and PET scan showed no loco-regional spread or distal metastasis. Bone marrow trephine and CSF were negative. The patient’s haemoglobin levels were borderline anaemic but all routine blood tests were otherwise within normal limits.2 on days 1 and 2); vincristine ; and Dactinomycin (at 1.5 mg/m2 on day 1) in three weekly cycles with concomitant radiotherapy.At 5 weeks after initial presentation he was started on nine cycles of IVA chemotherapy: ifosfamine ; ear; aural; middle ear; inner ear; ear canal, parameningeal; ear neoplasm in July 2018.Inclusion criteria were: diagnosis of rhabdomyosarcoma of the middle ear; published in a peer reviewed journal; any geographical location or language; any publication date; any age of patient.In total 11 case reports were found. Of these, there was an equal male-female split. The clinical presentations were similar throughout the case reports, mimicking recurrent otitis media/externa. The average survival across the case reports was 7.2 months. We summarise the case reports in2. Around 30% of cases of paediatric rhabdomyosarcoma occur in the head and neck involving the orbits, base of skull, nasal cavity and nasopharynx3 but only 3% occur in the ear or mastoid4. In total, 63% of rhabdomyosarcomas occur in children under 10 years old, with a peak between 2 and 5 years old5.Rhabdomyosarcoma of the middle ear and mastoid is rare6. By the time of diagnosis, a facial nerve palsy is usually present and there is involvement of both the middle and external ear and petrous bone7. Metastases commonly spread to the lungs, liver, bones and extremities, and are present in approximately 30% of cases3.Presentation often involves purulent or blood-stained discharge, hearing loss, aural polyps and granulation tissue. Clinical diagnosis is often delayed since the presentation is similar to that of chronic suppurative otitis media3. Alveolar is the second most common, usually presenting in the deep soft tissues of the extremities4.According to the International Classification of Rhabdomyosarcoma, these tumours are histopathologically classified into five categories: botryoid, spindle cell, embryonal, alveolar, and undifferentiated. Embryonal rhabdomyosarcoma is the most common, accounting for 70–80%, occurring mostly in the orbit and parameningesin utero, lower socioeconomic status and use of antibiotics soon after birth8. Several genetic syndromes, including some commonly known to otolaryngologists such as Rubinstein-Taybi syndrome and Beckwith-Wiedemann syndrome, are associated with increased prevalence of rhabdomyosarcoma.Risk factors for rhabdomyosarcomas are poorly understood. Epidemiological studies have suggested a link with radiation and recreational drug9; however, surgical removal is often difficult in head and neck cases given the stage of the neoplasm and anatomy. Consequently, the mainstay of treatment is often chemoradiotherapy. Parameningeal tumours, especially of the middle ear and mastoid, have a poor prognosis because of the proximity to the brain6. Of the categories, embryonal have the best prognostic factor and alveolar the worst and children have a better prognosis than adults4.Historically, rhabdomyosarcomas were invariably fatal. Now, 5-year survival rates of around 80% have been reportedRhabdomyosarcoma of the ear is a rare presentation but one which should be considered in young children with recurrent otitis media. If missed, facial nerve involvement is a common as well as local meningeal and distant metastasis. Multi-disciplinary involvement and patient education is mandatory in ensuring the highest survival rates. Treatment consists of a combination of surgery, chemotherapy and/or radiotherapy.All data underlying the results are available as part of the article and no additional source data are required.Written informed consent for publication of their clinical details as obtained from the parent of the patient study. This article describes a case of botyroid embryonal rhabdomyosarcoma of the right middle ear in a four year old child. It covers the clinical presentation, workup and subsequent management of this rare entity and reviews the pertinent literature of other case descriptions. The case report is very well written, informative, covers an interesting and unusual topic, and adequately covers all of the objectives set out in the guidelines.  The background of the case’s history and progression are described in excellent detail. Description of the physical examination and workup of the patient are detailed and methodical, as are the descriptions of diagnostic tests, treatments and outcomes. Emphasis is placed on the rarity of this clinical entity and thus it's contribution to the literature is a useful one. There were no novel treatments in this case report, however the description of the chemotherpay regimen will be useful for other clinicians. Description of the recurrent presentations to the GP and A&E departments are a sobering reminder of the need for these clinicians to recognise unusual clinical patterns, not rely on previous diagnoses and seek help from specialists early in such scenarios.  Images of the histology would have been a nice to see given the rarity of this clinical entity.I confirm that I have read this submission and believe that I have an appropriate level of expertise to confirm that it is of an acceptable scientific standard. This is a generally well written case report detailing a rare disease process in an unusual anatomical location. It covers pathogenesis, presentation, problems with diagnosis, treatment and prognosis. It has multiple grammatical errors which need review and correcting: 'Recreational drugs in utero.' The diagnosis is often delayed and easily misdiagnosed as AN aural polyp 'A four-year-old boy presented with a four-month history of recurrent left ear blood and pus discharge' - please revise to A four-year-old boy presented with a four-month history of recurrent haemo-purulent discharge from the left ear. In addition I have the following comments/suggestions: Intra- operatively, a large lesion extending from the middle ear through the tympanic membrane and into the external auditory canal was noted which was resected. The lesion was found to be highly vascular.  Was it totally resected? Via a tympanotomy? Or was the tissue lateral to the TM resected. If a tympanotomy was performed what was the status of the ossicles? You say that the facial nerve palsy incompletely resolved. Please give details using House-Brackmann or other suitable classification. How do you arrive at '3% of paediatric rhabdomyosarcoma occurring in the head and neck involving the …………..'? There are only 11 cases reported. You would need 100/3 = 30 cases or more to be able to state this? Could intra operation images and high res CT image be included? Please state the disease free followup to date of your patient. Please comment on hearing outcome. There is no mention specific mention of MDT discussion? You state a second opinion was sought but surely this case went through an MDT?I confirm that I have read this submission and believe that I have an appropriate level of expertise to confirm that it is of an acceptable scientific standard. Many Thanks for your comments.  We have adjusted the manuscript to reflect your feedback. This is an interesting, concise and well-constructed case report and review of the relevant literature surrounding rhabdomyosarcoma affecting the middle ear. This is a rare entity and as such additional case reports are valuable in adding to the existing literature. The case report contains adequate description of the patient and detail of the presentation and initial investigations, which would be in line with the reviewers own practice. The case highlights the need for high clinical suspicion when treating non-resolving recurrent ear infections in children which is a useful message for clinicians. The literature review results are presented clearly and contain all relevant information regarding the case’s reported. However, there is a lack of detail surrounding which chemotherapy/radiotherapy regimes were used. It is also unclear if they were adjuvant or primary treatment modalities for the other cases described.I confirm that I have read this submission and believe that I have an appropriate level of expertise to confirm that it is of an acceptable scientific standard.


As it originates from neural crest cells, Neuroblastoma (NBL) can arise anywhere along the sympathetic chain. However, its occurrence in the urinary bladder (UB) is extremely rare. We present a case of an incidentally diagnosed pelvic NBL arising from the dome of the UB in a 7-month-old infant. The mass was treated with surgical excision only after being classified as a very low risk group according to the International Neuroblastoma Risk Group staging system. The patient was disease free after 5 years of follow-up. Although rare, we suggest that NBL should be considered in the differential diagnosis of UB masses in children and investigated accordingly. US showed a well-circumscribed, solid, polypoid mass arising from the UB fundus, measuring 2 cm in diameter, with hypervascularity on color Doppler . MagnetWe proceeded to excisional biopsy through an extended suprapubic incision. The dome of the UB was opened, and the tumor felt elastic, firm, and homogenous . The maFurther assessment was made for metastatic disease, including bone scan, bone marrow biopsy, and MIBG (metaiodobenzylguanidine) scan, which were all negative. Urine creatinine, HMMA (4-hydroxy-3-methoxy mandelic acid)/creatinine, and homovanillic acid (HVA)/creatinine ratios were all within normal limits.Due to favorable prognostic factors, our patient was classified as a very low risk group according to the International Neuroblastoma Risk Group staging system (INRGSS) and was treated by surgical excision only. Annual follow-up with clinical examination and two MRI scans performed after 1 and 3 years postoperatively revealed no evidence of recurrence or residual tumor. The patient remained asymptomatic throughout and was discharged from follow-up after 5 years.NBL is the most common extracranial solid tumor of childhood.Other than cases diagnosed by screening, bladder NBL can present with microscopic hematuria and/or the presence of a palpable mass on physical examination.The preoperative staging system in NBL is the INRGSS of 2009, which has simplified the staging into L1, L2, M, and MS.NBL is known to exhibit the highest rate of spontaneous regression among malignant tumors.Treatment strategies for NBL include surgery, chemotherapy, and/or radiation. Stem cell transplant and immunotherapy are reserved for advanced or recurrent disease. Low and intermediate risk groups have a 95% survival rate with the use of surgery and/or chemotherapy. High-risk groups with combination therapy have a survival between 40 and 50%.Our patient was stage L1 by INRGSS and because of the favorable prognostic factors was put in the very low-risk group, which is amenable to treatment by surgical excision solely. Annual clinical examination and two postoperative MRI scans revealed no evidence of recurrent or residual tumor. We discharged the patient after 5 years of follow-up.Although rare, NBL should be considered in the differential diagnosis of pediatric urinary bladder masses, especially when arising from the dome.


TSC1 and TSC2, whose gene products typically act to inhibit mTOR. There are several cases of malignant angiomyolipoma which exhibit transient responses to mTOR inhibitors, forming the basis of current practice guidelines in malignant PEComa. However the tumors ultimately acquire resistance, and there is no well-established second-line option. Despite the increasing prevalence of immunotherapy across a wide range of solid tumors, little is known about the immune infiltrate and PD-L1 expression of angiomyolipoma. Furthermore, there is no reported case on the treatment of malignant angiomyolipoma with an immune checkpoint inhibitor.Malignant angiomyolipoma is an uncommon tumor of the class of perivasciular epithelioid cell neoplasms (PEComas). These tumors are characteristically driven by deleterious mutations in the tumor suppressors TSC2, and the patient was treated with the mTOR inihbitor everolimus. The patient went on to have a partial response but ultimately progressed. He was then treated with the anti-PD-1 immune checkpoint inhibitor nivolumab, and achieved a durable near-complete response which is ongoing after two years of treatment. Immunohistochemical staining of tumor tissue revealed strong PD-L1 expression and a brisk T-cell infiltrate.A 38 year-old man presented with gross hematuria and was diagnosed with renal epithelioid angiomyolipoma. Despite surgical resection, the tumor recurred and metastasized. Targeted genomic sequencing revealed a deleterious mutation in We report on the first durable systemic treatment of malignant epithelioid angiomyolipoima with the use of PD-1 antibody nivolumab. Given the absence of prospective clinical trials in this exceedingly rare disease, particularly in the second-line setting, immune checkpoint inhibitors like nivolumab should be considered. TSC1 or TSC2 mutations [Angiomyolipomas (AMLs) are neoplasms thought to arise from pericites, hence belonging to the family of perivasciular epithelioid cell neoplasms (PEComas). Though associated with tuberous sclerosis complex (TSC), most AMLs arise de novo, in the absence of germline utations , 4. GiveTSC1 or TSC2 [TSC1 and TSC2 also contribute to tumor growth via unopposed mTOR signaling, and sporadic AML is similarly characterized by somatic loss-of-function alterations in TSC2 [TSC1 or TSC2 mutations [TSC is a genetic syndrome characterized by multisystem tumor development, including renal angiomyolipoma. Most patients harbor pathogenic germline loss-of-function mutations in  or TSC2 , whose w or TSC2 , 7. This or TSC2 , a Unite in TSC2 . Multiplutations , includiutations , and spoutations , though utations . HoweverNivolumab is a fully humanized monoclonal IgG4 antibody that targets the programmed death 1 (PD-1) receptor, an immune checkpoint expressed on exhausted effector T lymphocytes, and prevents binding by its activating ligand PD-L1, leading to reinvigoration of anti-tumor immunity . NivolumTSC2 mutation. The patient exhibited a transient response to everolimus, but ultimately progressed. He subsequently achieved a significant and durable response to nivolumab. To the best of our knowledge, this is the first report on the treatment of malignant EAML with immunotherapy.Given the lack of data concerning the treatment of this rare cancer, we report a case of metastatic EAML harboring a deleterious A 38 year-old man with vitiligo and hypothyroidism initially presented in 2011 with gross hematuria. Diagnostic imaging Fig.  revealedThe patient had an uneventful course for the next 3 years until April, 2014, when surveillance imaging detected an asymptomatic 13-cm renal fossa mass for which he underwent repeat surgical resection. Surgical pathology confirmed recurrent EAML, again with negative margins. The patient’s tumor recurred again in October, 2014, prompting a third surgical resection. Pathologic evaluation this time demonstrated indeterminate margins, prompted referral to medical oncology for further management.ATRX, and a deletion in TSC2, specifically, TSC2 H1746_R1751del, which has been reported both as a somatic variant in AML [MSH2, MSH6, PMS2, or MLH1.December, 2014 surveillance imaging obtained by the treating medical oncologist (AVB) demonstrated new retroperitoneal and pelvic implants consistent with metastatic EAML. The patient’s tumor DNA was subjected to FoundationOne® targeted next-generation sequencing , which rt in AML  and as at in AML . Of noteTSC2 deletion, the patient was initiated on everolimus in January, 2015. Imaging at 3 months  was not specified. Nonetheless, despite an initial response to rapalog therapy, virtually all tumors ultimately developed resistance. In addition to mTOR inhibitors, tumor responses have been observed with the use of chemotherapy. Cibas, et al. reported a partial response to doxorubicin [Current approaches to treatment for the sporadic form of recurrent or metastatic EAML are derived from anecdotal reports of efficacy with mTOR inhibitors , 24–27. orubicin , and othorubicin , with moTSC1 and TSC2 alterations occur in approximately 5% of all solid tumors, including 4.7% of the MSK-IMPACT cohort [MTOR as the etiology of everolimus resistance in TSC2-mutant thyroid carcinoma. However, no post-treatment alterations were identified to explain the acquired resistance in the present case of EAML.Cumulatively, T cohort  and 5.2%T cohort . In the T cohort , TSC2 H1T cohort . HoweverIn contrast to the transient responses to everolimus both in our case and others’, we report the first durable response to any agent in this rare malignancy with the use of the PD-1 antibody nivolumab. To the best of our knowledge, this case is the only report on the use of immunotherapy in the management of angiomyolipoma of any primary site or histologic variant. Immunohistochemistry revealed high PD-L1 expression (> 50% of cells), and brisk cytotoxic T cell infiltrate Fig. -g. The sAlso of interest is our patient’s history of pre-existing as well as treatment-related autoimmunity. The development of immune-related adverse events has been correlated to clinical benefit with immunotherapy among melanoma patients, a subset of whom develop vitiligo . In the In this case of malignant EAML – a rare disease with no standard of care – we report a durable near-complete response to the anti-PD-1 immune checkpoint inhibitor, nivolumab. This case highlights the cross-histologic efficacy of immune checkpoint inhibition, particularly in tumors with high PD-L1 expression and brisk lymphocytic infiltrate. In the absence of prospective clinical trials, nivolumab should be considered for use in other patients with recurrent or metastatic EAML who have exhausted traditional therapeutic options.


Atrial myxomas are a rare phenomenon and although benign, primary neoplasms of the heart can be burdensome depending on their location. Clinical symptoms are caused through a variety of mechanisms including conduction disturbances, obstruction, and valvular interference. Size and symptom development are strongly correlated and can almost always be detected by the use of echocardiography, magnetic resonance imaging or computed tomography. This is a case of a 62-year-old female with no significant past medical history presented to our facility with complaints of palpitations and associated dizziness for three months. Atrial myxomas are the most common benign primary neoplasm of the heart, accounting for close to 80% of all cardiac tumors . AlthougA 62-year-old female with no significant past medical history presented to our facility with complaints of palpitations and associated dizziness for three months. Prior work-up of her palpitations with Holter monitoring showed no irregularities. On arrival, she was in no acute distress and her palpitations had subsided. Vitals that were obtained were largely unremarkable except for a blood pressure of 142/77. Her EKG showed no acute irregularity and laboratory testing was within normal limits. On physical, a regular rate was observed, no murmurs, gallops or rubs were auscultated. She did not exhibit jugular venous distention or peripheral edema and other organ systems did not yield and irregularities. The patient was admitted for further evaluation and a transthoracic echo was performed, revealing a 4.4 x 3.0-cm mass in the left atrium attached at the interatrial septum and aortomitral intervalvular fibrosa. Additional imaging studies including cardiac magnetic resonance imaging (MRI) and transesophageal echocardiography (TEE) were obtained for further confirmation of the mass and its location Figures , 2. SurgThe patient underwent full-thickness resection of the mass, resulting in an iatrogenic atrial septal defect which was closed with a Dacron patch. Her postoperative course was complicated by the development of intermittent complete heart block with junctional bradycardia and subsequent atrial fibrillation. She was evaluated by electrophysiology and a permanent pacemaker was implanted without complication. Discharge follow-up several weeks later noted the resolution of her prior symptoms, and repeat imaging showed no evidence of mass recurrence or mitral valve prolapse. Tumors of the heart commonly present with symptoms that correlate with the location, the degree of obstruction, and interference with heart valves or circulation. Clinical symptoms demonstrated in the literature include chest pain, dyspnea, and syncope but infrequently present with palpitations for an extended period of time -7. Our pWhen the diagnosis is confirmed, prompt resection is suggested to avoid the possibility of further manifestations including embolization or sudden cardiac death -12. OutcThis patient presented in an atypical fashion which lacked commonly associated symptoms including chest pain, dyspnea on exertion, and syncope. Imaging modalities including echocardiography, CT, and cardiac MRI are considered equally efficacious for the diagnosis of atrial tumors as they provide the location within the heart. Additional studies including angiography and biopsy are commonly required for further evaluation when surgery is planned. Surgical intervention consists of tumor resection and despite a low mortality rate, complications including arrhythmias and atrioventricular conduction delays are possible.


Amyand’s hernia (AH) is rare, schistosomiasis of the appendix is very uncommon, and both conditions coexisting together is an extremely rare event. Pre-operative diagnosis of each of the two conditions is usually difficult. To the best of our knowledge, the current paper is first to report both these two conditions in coexistence.A 31-year old man who had no comorbidities was admitted electively as a day case of non-complicated right indirect inguinal hernia. Further history and physical examination were unremarkable. Intraoperatively the patient was found to have right sliding AH with appendicular schistosomiasis (AS). The patient underwent Lichtenstein repair of the hernia with appendectomy. On follow up he was referred to infectious disease clinic, and the post-operative course was uneventful.Intraoperative identification of non-typical hernia sac before its opening should alert the surgeon to the possibility of sliding hernia and the presence of an organ as a part of the sac. Rare causes of appendicular masses like schistosomiasis granuloma should be considered in endemic areas or immigrants from these areas, despite the difficulty of preoperative diagnosis. Management should follow general guidelines of appendectomy, hernia repair and dealing with the associated pathology if present. He had no comorbidities, there were no other gastrointestinal or urologic symptoms, and the systemic review was unremarkable.The patient had normal vital signs. Abdominal examination showed a right groin swelling, not reaching down the scrotum, with no skin changes or discoloration, exhibiting a cough impulse but incompletely reducible, yet not tender. Genitourinary examination was otherwise unremarkable. These findings were clinically in favor of inguinal hernia. Laboratory tests were not done as the patient was young with no comorbidities. He was booked for daycare right inguinal mesh hernioplasty (Lichtenstein repair) . The patAt follow up, the wound was healing well with no surgical complications, histopathology showed periappendiceal necrotizing granulomatous inflammation with calcified parasitic ova, consistent with schistosomiasis , Fig. 4.3Amyand’s hernia (AH) is rare , schistoIn terms of presentation, AH usually presents as usual inguinal hernia with no specific or characteristic clinical features; the same applies to the presentation of the appendix inside the AH, unless the appendix is inflamed . Hence tAs for investigations, preoperative evaluation may be uncertain in cases where there is concomitant pathology on top of AH . UltrasoIntraoperatively, the initial exploration and palpation showed that the indirect sac was thick and with a hard small mass within it. Hence, we decided to open the sac distally instead of proximally in order to avoid injury to any sliding organ should there be one. In our case, the thick hernia sac was the wall of the cecum. Such approach is in agreement with the treatment of sliding hernias . IntraopAs for the surgical procedure, Losanoff and Basson proposed the surgical treatment of AH depending on its type . These iPostoperatively, histopathology of the resected specimen revealed AS, which was an incidental finding. The patient was an emigrant from an endemic schistosomiasis area, in agreement with other reported AS ,18. Our The treatment of AS is appendectomy and a single dose of praziquantel . After t4Diagnosis of AH requires a high index of suspicion as the history may not be typical either for acute appendicitis or incarcerated hernia, and physical examination maybe inconclusive. Intraoperative identification of non-typical thick hernia sac before its opening should alert the surgeon of the possibility of sliding hernia with an organ as a part of the sac. Rare causes of appendicular tumors like schistosomiasis should be considered in endemic areas or immigrants coming from these areas, despite the difficulty of preoperative diagnosis. Management follows the general guidelines of appendectomy and hernia repair whilst dealing with any associated pathology where present. Early diagnosis and proper surgical intervention result in excellent outcomes.Nothing to declare.Approved by medical research center, Hamad Medical Corporation reference number (MRC-04-19-210).Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal on request.Ali Toffaha: study concept, data collection, interpretation, writing the paper. Walid El Ansari: data interpretation, writing the paper. Orwa Elaiwy: data interpretation, writing the paper. Munzir Obaid: data interpretation, writing the paper. Omer Al-Yahri: data interpretation, writing the paper. S. Abdelazim: data interpretation, writing the paper.1. Name of the registry: researchregistry2. Unique Identifying number or registration ID: researchregistry5043https://www.researchregistry.com/register-now#home/registrationdetails/5d3c224113e2dd0010e38582/.3. Hyperlink to the registration: Atoffaha2@gmail.com.Ali Toiffaha: welansari9@gmail.com.Walid El Ansari: Not commissioned, externally peer-reviewed.Nothing to declare.


Ameloblastoma is a slow-growing, benign odontogenic tumor derived from odontogenic epithelial components with a mature fibrous stroma. It is the second most common odontogenic neoplasm following odontome. Acanthomatous ameloblastoma histologically presents with squamous epithelial metaplastic transformation of odontogenic tissue. The present case report of a 12-year-old male exclusively elaborates the issues concerned with the aggressive nature of acanthomatous ameloblastoma (AA) which is a distinctive variant of ameloblastoma. The word ameloblastoma is derived from a combination of the French word “Amel” meaning enamel and the Greek word “Blastos” which means germ or bud. Ameloblastomas are locally invasive, slow-growing odontogenic tumors most commonly involving the mandible. Based on its size, anatomical location, histological variant, and involvement of lesion in adjacent anatomical areas, there are varying treatment modalities available for these tumors . In paedA 12-year-old boy reported to our institute with a chief complaint of swelling on the left side of the face for the past six months . The patExtraorally, there was a presence of diffuse swelling involving the left side of the face, extending superiorly to the left zygomatic arch, inferiorly to the lower border of the mandible, anteriorly to the corner of the mouth, and posteriorly to the retromolar portion of the mandible. There was no obliteration of nasolabial folds seen. On palpation, the swelling was hard, noncompressible, and nonreducible along with diffuse expansion of the middle and lower 3rd of the facial region.Intraoral examination revealed a swelling involving the retromolar trigone and molar regions with obliteration of the mucobuccal fold. On palpation, the lesion was firm to hard in consistency and nontender with expansion of left buccolingual cortical plates.Radiological examination of orthopantomogram showed the presence of unilocular radiolucencies extending from the last erupted molar to retromolar region .Based on the above clinical and radiological findings, provisional diagnosis of unicystic ameloblastoma was given. Incisional biopsy was carried out, and the specimen was sent to the Department of Oral Pathology and Microbiology. The gross specimen measured around 1 × 0.6 × 0.4 cm to 0.2 × 0.2 × 0.1 cm in dimension, which was firm in consistency, creamish brown in colour, and irregular in shape. The histopathological examination showed sheets of odontogenic islands lined by tall columnar cells enclosing stellate reticulum-like cells along with squamous metaplasia in the connective tissue stroma extending into the peripheral epithelium. Figures  and 4. TFurther radiological investigations proceeded with CT scan neck (plain and contrast) and CT Brain . Serial Surgical treatment was planned under general anaesthesia. Extraction of 74, 34 and hemimandibulectomy of the left mandible was carried out. The resected mandible was reconstructed by free fibular graft. The weight and measurement of the resected specimen was 87 gms and 9 × 6 × 4 cm, respectively. Histopathological examination of the resected specimen revealed anastomosing follicular islands of the odontogenic epithelium lined by tall columnar cells enclosing stellate reticulum-like cells. Squamous metaplasia of the stellate reticulum-like cells was observed. Areas of cystic degeneration of the stellate reticulum-like cells were also seen. A reactive change of lymphoid tissue was seen. Based on the clinical, radiological, and histopathological examination, a final diagnosis of acanthomatous ameloblastoma was made (Figures 7The occurrence of ameloblastoma is only 1% compared with all oral tumors, and among other Odontogenic tumors, the incidence is 11–18% . AcanthoInvolvement of the mandibular ramus region is seen in 80% of cases as similar to our case. These lesions are commonly encountered in the 3rd to 5th decades of life, which was not consistent with our case as our patient was in the 2nd decade of his life.Though ameloblastoma is a benign tumor in nature, its clinical presentation shows a painless, slow-growing mass, with displacement of teeth, malocclusion, loose teeth along with local aggressiveness and frequent invasion into the alveolar bone , 7. SimiRadiological findings of ameloblastoma usually show expansile, unilocular/multilocular radiolucencies and typical ‘soap bubble appearance'. In our case, the OPG revealed expansile lytic lesion along with thinning of cortical plates.Histopathological examination of acanthomatous ameloblastoma shows squamous metaplasia of stellate reticulum and the formation of keratin within the tumor islands . The chaDentigerous cyst and odontogenic keratocyst may possibly be the clinical differential diagnosis considering the age, site, size, and extent of the swelling.Radiological differential diagnosis may include odontogenic myxoma and central giant-cell granuloma.Payne et al.  suggesteAssessment of cellular proliferative activity of the odontogenic cysts and tumors is mainly done by the Ki-67 index which plays an important prognostic marker of tumor recurrence, biological behaviour, and local invasiveness. The peripheral cell layer of the epithelial islands when compared to central cells usually shows Ki-67 positivity, which suggests that peripheral cells are more proliferative .Immunohistochemical analysis of Ki-67 labelling has a brownish staining of nuclear areas as well as focal and total nuclear positivity of tumor cells  similar Our case report has various significant features which discerned it from previously reported cases of acanthomatous ameloblastoma. The case being discussed is presented with a diffuse swelling involving complete ramus of the mandible in a 12-year-old boy. To avoid higher chances of recurrence and to lessen the aggressive, rapidly growing and lytic nature of acanthomatous ameloblastoma, radical surgical treatment has been performed in our patient.


However, in the pathological findings, this cyst diagnosed advanced enteric duplication cyst cancer and not originated from pancreas. Enteric duplication cysts are rare and, in addition, isolated enteric duplication cysts are lower morbidity prevalence rate. These cysts lack a connection to the gastrointestinal tract or the adjacent mesenteric vasculature and have only been reported in 10 case reports. In these reports, only two reports were cases with malignant transformation. Our case was a report for the advanced cancer of the isolated enteric duplication cyst.The patient was a 43 year-old woman with slightly abdominal pain and mass formation. The abdominal contrast-enhanced computed tomography showed 130 × 100 × 90 mm huge cystic mass existed in right upper peritoneal cavity. The cystic mass had thickened wall and many enhanced nodules. As these imaging findings suggested a tumor originated from pancreas and the preoperative diagnose was suspect of mucinous cystic neoplasm. In operative findings, the tumor originated from pancreatic head and did not attach to gastrointestinal tract. Final pathology indicated the cyst was an isolated advanced enteric duplication cyst cancer and not originated from pancreas.We experienced an extremely rare case of completely isolated advanced enteric duplication cyst cancer. Unique to this case, the preoperative diagnosis was suspect of mucinous cystic neoplasm arising from pancreas head and partial pancreatectomy was performed. However, in the pathological findings, this cyst diagnosed advanced enteric duplication cyst cancer. The more than half of the cases are diagnosed early childhood due to symptoms, abdominal pain, gastrointestinal bleeding, intestinal obstruction, abdominal mass, and so on . DuplicaMalignant transformation in isolated duplication cysts is extremely rare. Here we reported a resected case of advanced cancer arising from an isolated enteric duplication cyst surrounding the pancreas that initially diagnosed a mucinous cystic neoplasm (MCN).This report is a work based on Consensus-based surgical case report guidelines, SCARE criteria .2A 43 year-old female was admitted to our hospital with a chief complaint of right upper pain and palpable mass. On physical examination, there was slight tenderness and semimobile mass in the right upper abdomen. Clinical examination did not demonstrate any persistent weak abdominal pain and mass. Her past medical and surgical history was unremarkable. Moreover, Family history and psychosocial history were also not particular.In abdominal contrast-enhanced computed tomography (CT), 130 × 100 × 90 mm huge cystic mass was demonstrated in right upper peritoneal cavity. The cyst had thickened wall and many enhanced nodules. Many nodules and the thickened wall were slightly enhanced in early phase A and graShe was taken to the operating room by a surgical oncologist who primarily specialized in pancreatic resections and had been in practice for over 10 years. In surgical findings, this tumor did not attach to gastrointestinal tract and originated from pancreatic head A. The asFinal pathology demonstrated that the cystic mass had well-formed cyst wall with an inner mucosal lining, submucosal layer, and muscularis propria as gastrointestinal tract A–C. As tDuring hospital stay in post-operative period, she was counseled that we identified an isolated enteric duplication cyst with advanced cancer. We recommend treatment by S-1 as adjuvant chemotherapy according to the adaptation for criteria of advanced gastric cancer. She had taken S-1 for 1 year without rest of medication and lived for 1.5 year after surgery without any evidence of malignancy.3Intestinal duplication cyst are rare, seen in approximately 1/100,000 births ,7. TheseThe differential diagnoses for intra-abdominal cystic tumor around pancreas are MCN, pancreatic pseudocyst, mesenteric and omental cysts, and so on. In imaging modality, the cystic wall indicated thickened and enhanced partially. As the intra-cystic fluid had seen serous or bloody, the density and intensity were various. Therefore, the definitive diagnosis was difficult and this case was diagnosed with MCN. CEA and CA19-9 level in intra-cystic fluid was often high in MCN and IPMN and it was unuseful to distinguish malignancy .Histologically, in stricture and histomorphology, enteric duplication cysts are similar to normal bowel wall and consist of mucosa, submucosa, muscularis propria and serosa. The mucosal type is reported as gastric and jejunal type by thickness of mucosa and submucosa and structure formation of villi and crypt .-1 , as advanced gastric cancer. No standard treatment has been established for advanced isolated duplication cyst cancer because of its rarity and variable extent of this disease, and the management would be performed based on one of gastrointestinal tract cancer for the time being.As it was not reported treatment for an advanced isolated duplication cyst cancer, there is no standard treatment and surgical resection have been only managed. This case was advanced cancer and we have treated her by adjuvant chemotherapy, SThis case was second report of the isolated intestinal duplication cyst with advanced cancer and performed R0 resection. The work indicated the findings of clinical examinations, pathology, and course of treatment.4We experienced an extremely rare case of completely isolated advanced enteric duplication cyst cancer. Unique to this case, the preoperative diagnosis was suspect of mucinous cystic neoplasm arising from pancreas head and partial pancreatectomy was performed. However, in the pathological findings, this cyst diagnosed advanced enteric duplication cyst cancer.All authors have no conflicts of interest.All authors declare no sources of funding. I state in the text (page 7).This study of case report is exempt from ethnical approval in ethics committee of our institution.This manuscript includes a statement to this effect in a consent section at the end of the manuscript.Patient’s names, initials, or hospital numbers is not used. Identified information in the images of the patient is not also used and she has a right to privacy.Clinical treatment: Shinsuke Nakashima, Terumasa Yamada, Go Sato, Takaaki Sakai, Yoshinao Chinen, Hiroaki Itakura, Ryo Kato, Masami Ueda, Yujiro Tsuda, Katsuya Ohta, Jin Matsuyama, and Masakazu Ikenaga.Collected data: Shinsuke Nakashima, Yujiro Tsuda and Terumasa Yamada.Assessment and discussion: Shinsuke Nakashima and Terumasa Yamada.Wrote the paper: Shinsuke Nakashima and Terumasa Yamada.We confirm that the work is not necessary to registry UIN. This work was not included its first in man ie the first time a new device or surgical technique. The report of one case is not necessary to ethnical approval in our institution.The guarantor of this study is Terumasa Yamada, corresponding author.Not commissioned, externally peer reviewed.


Most of the endobronchial lesions are malignant in origin. In rare instances, benign lesions occupying the endobronchial tree can mimic malignant neoplasms on conventional imaging tests. We present a case of a middle-aged male patient who was admitted to our hospital with recurrent hemoptysis concerning for lung cancer on computed tomography (CT) of the chest. Biopsy of the mass obtained via bronchoscopy revealed a benign lesion most consistent with lipomatous hamartoma, which is known to constitute only 10% of all pulmonary hamartomas. We also present the data of a comprehensive literature review of the epidemiology, clinical symptoms, diagnosis, treatment, and prognosis of patients with endobronchial hamartomas. Pulmonary hamartomas are the most common benign lung tumors . It is e A 49-year-old male of Mediterranean origin without a significant past medical history presented to the emergency department for evaluation of hemoptysis and shortness of breath. He stated that his symptoms started one day ago. He denied fevers, chills, chest pain, and purulent cough. There was no weight loss, hematemesis, hematochezia, rash, or mucosal bleeding. He denied a history of recent travel and had no history of tuberculosis exposure. He is a former smoker . He drank alcohol socially and worked at a local restaurant.Upon further questioning, the patient admitted to having similar symptoms about seven months ago. At that time, he was seen by his primary care physician, diagnosed with bronchitis, and treated with antibiotics. No recurrence of symptoms was noted prior to the current presentation. Vital signs, as well as physical findings, were unremarkable. The complete blood count, metabolic panel, and coagulation profile were normal. Chest X-ray showed decreased lung volumes and a very small lingular infiltrate versus atelectasis. Computed tomography (CT) of the chest with intravenous contrast revealed a 14-mm endobronchial mass in the left upper lobe (LUL) bronchus, resulting in subsegmental atelectasis of the lingular segment -guided transbronchial needle aspiration (TBNA) of an adjacent lymph node was performed as well. The patient was discharged on Day 3 and recommended to follow up with pulmonology. The endobronchial biopsy showed a lipomatous hamartoma , were negative for malignant cells. One month after discharge, the patient continued to complain of shortness of breath, and no hemoptysis was reported. It was decided to proceed with repeat bronchoscopy. No bronchial obstruction was noted. Biopsy at the previous site was obtained, which showed only focal fibrosis with giant cell reaction. His symptoms eventually resolved, and he continues to follow-up regularly with pulmonology.The term “hamartoma” was originally introduced by Albrecht in 1904. It is derived from the Greek “hamartia” (defect) and “oma” (tumor) . A hamarPulmonary hamartoma is the third most common cause of pulmonary nodules . It is aEndobronchial hamartoma, although considered the most common endobronchial benign neoplasm, is known to be a very rare cause of an endobronchial mass. Notably, most of the endobronchial tumors are malignant. Interestingly, all benign endobronchial tumors combined together constitute about 2% of all lung tumors .We performed a comprehensive literature search in PubMed/Medline to find all the published case reports and case series of patients with endobronchial hamartoma from inception till December 20, 2018. We used the following terms: “endobronchial,” “pulmonary,” and “hamartoma.” The Boolean operators “OR”  and “AND” were selected to specify the search outcome. All terms were searched using the “Abstract/Title” field. We only included cases of adult patients. Only original case reports and case series were included for further review. Cases that did not have abstracts and were published elsewhere were excluded. We were able to identify 80 articles that met the inclusion criteria . OverallDespite the fact that pulmonary hamartoma is the most common type of benign lung tumor, its endobronchial localization is known to be rare. In contrast to the peripheral subtype, endobronchial hamartomas usually manifest with recurrent respiratory symptoms. Chest X-ray may be unremarkable in patients with endobronchial hamartoma, therefore, more advanced diagnostic tests are required for further work-up. CT of the chest can usually detect a low-attenuation endobronchial mass. Diagnostic and therapeutic bronchoscopy is the test of choice when an endobronchial mass is suspected. Of note, in rare instances, endobronchial biopsy findings may reveal metaplastic changes that can present a diagnostic challenge. Endoscopic intervention is a safe and effective therapeutic option for selected cases. The prognosis of endobronchial hamartoma is generally favorable. There is no consensus regarding the follow-up interval and its frequency. Once removed, the chances of the recurrence of endobronchial hamartoma are very low.


Ectopic hamartomatous thymoma, which usually occurs in the lower neck, is a rare benign tumor containing spindle cells, epithelial nests, and adipose tissue. Although the origin of this tumor is still unknown, recent reports suggest that the designation of this tumor is inappropriate.A 38-year-old with an anterior cervical mass in the suprasternal region of her neck was referred to our hospital. An ultrasound examination showed that the well-defined oval mass was 31 × 23 × 17 mm in size. A non-enhanced computed tomography scan of the neck revealed that the distinct neck mass in the subcutaneous tissue had a mixture of soft tissue and fatty components. The cervical tumor was clinically diagnosed to be an unusual lipoma with degeneration. The patient underwent the neck mass extirpation. During the surgery, the cervical mass was well demarcated and did not adhere to the surrounding tissues. The postoperative course was uneventful. The gross pathology report showed that the neck mass measured 3.0 × 2.5 × 2.0 cm. Microscopically, the tumor was composed of spindle cells, epithelial nests, and mature adipose tissue. Immunohistochemical examination revealed that both spindle cells and epithelial nests were positive for cytokeratin AE1/AE3. These histopathological findings were consistent with the features of ectopic hamartomatous thymoma. Over a follow-up period of 30 months, this patient exhibited no evidence of recurrence.Ectopic hamartomatous thymoma should be considered in the differential diagnosis of subcutaneous tumors in the lower neck, when the CT shows the tumor has the mixed components of fat and soft tissues. Ectopic hamartomatous thymoma (EHT) usually occurs in the lower neck, and it is a rare benign tumor containing spindle cells, epithelial nests, and adipose tissue. The first case of EHT was reported by Smith and McClure in 1982 [A 38-year-old woman with a 3-month history of an anterior cervical mass located in the suprasternal region of her neck was referred to our hospital. Physical examination revealed that the 3-cm movable neck mass was firm and slightly tender on palpation and had a distinct margin from surrounding tissues. An ultrasound examination showed that the well-defined oval mass was 31 × 23 × 17 mm in size and exhibited heterogeneity. Furthermore, a non-enhanced computed tomography (CT) scan of the neck revealed that the distinct neck mass in the subcutaneous tissue had a mixture of soft tissue and fatty components Fig. . As thesHistorically, tumors designated as “EHT” were first described by Smith and McClure in 1982  named thAlthough EHTs are usually benign (including our case), three of 79 EHT cases  showed mAn accurate nomenclature to describe this rare neoplasm is still under discussion as the exact origin of EHT remains unknown. In 2004, Fetsch et al.  proposedAlthough this tumor is rare, EHT should be considered in the preoperative diagnosis when CT shows a tumor in the lower neck with mixed components of fat and soft tissues. Finally, more case reports and discussion might be necessary before an appropriate nomenclature of this tumor can be proposed.


Granular cell tumors (GCT) are usually benign, soft tissue tumors that are mostly found in the oral cavity, skin, and subcutaneous tissue. GCTs in the gastrointestinal (GI) tract are mainly located in the esophagus.A 63-year-old male was referred to the gastroenterology clinic for a major complaint of six months of painless rectal bleeding. Laboratory results showed mild macrocytic anemia. He denied any prior colonoscopies and hence, a lower endoscopic procedure was done. The colonoscopy showed multiple polyps, one of them located at the cecum. The cecal polyp showed polygonal cells with abundant eosinophilic infiltration and S100 stain positive. This confirmed a diagnosis of GCT.GCTs are thought to be derived from the neural tissue (Schwann cells). This entity is usually asymptomatic; however, tumors located at the lower GI tract can present with hematochezia. Only 2% of GCTs follow a malignant course, with associated poor prognosis. This case is being presented because of its asymptomatic nature. It is important to monitor these lesions in order to recognize early signs/symptoms concerning for malignancy. Granular cell tumors (GCT) are usually benign, soft tissue tumors that can be located in any part of the body, but are mostly found in the oral cavity, skin, and subcutaneous tissues . These rA 63-year-old male with a past medical history of hypertension, human immunodeficiency virus on treatment, late latent syphilis, and chronic anemia secondary to folate deficiency was referred to the gastroenterology clinic due to painless intermittent rectal bleeding for six months. He denied any associated abdominal or rectal pain, melena, tenesmus, or mucus in his stools. Laboratory results showed a hemoglobin of 11.6 g/dL, with a mean corpuscular volume (MCV) of 105.3 fL. Since the patient did not have any prior colorectal screening procedure and given his present symptoms, a colonoscopy was indicated. The colonoscopy showed multiple polyps: one 15-mm polyp at 65 cm proximal to the anus, a 10-mm polyp at the ascending colon, a 5-mm polyp at the ileocecal valve, and a diminutive at the cecum Figure .GCTs are mainly found in patients in their fourth to sixth decades of life and have a female predominance with a 2:1 ratio -4. FurthGCTs are frequently asymptomatic, and tumors are found incidentally on endoscopic procedures. When symptoms are present, these depend on the location of the tumor. Esophageal GCT can present as gastroesophageal reflux or dysphagia/globular sensation when the tumor is located at the cervical esophagus. Patients with small intestine tumors can present with upper gastrointestinal bleeding or abdominal pain. Colorectal GCT can present with hematochezia, abdominal pain, and change in bowel habits ,6. The pApproximately 20% of all the gastrointestinal GCTs are located in the colorectal region. Usually these present as solitary tumors; however, approximately 7% to 25% can be found in aggregates . DiffereThe gold standard for the diagnosis, regardless of the lesion site, is based on histologic findings: there are nests of epithelioid or spindle cells, with a small round nucleus, and abundant eosinophilic granular cytoplasm. Stains are positive for S-100, CD56, CD68, SOX-10, and neuron-specific enolase . If maliSince there are no established guidelines for the treatment of colorectal GCT, some physicians recommend performing endoscopic mucosal resection (EMR), endoscopic submucosal dissection (ESD), or polypectomy for tumors less than 2 cm . Follow-Colorectal GCTs can be a diagnostic challenge due to its asymptomatic nature. Although there is a low chance for malignancy conversion, it is important to recognize this feature in order to monitor it with follow-up endoscopic procedures.


Cystic ganglionosis is an unusual benign condition, which presents as multiple ganglion cysts involving multiple joints. The case we report here is probably the first case of an adult reported in the literature. The only other case of multiple ganglion cysts reported in the literature is that of an 11-year-old child with ganglion cysts in multiple joints. A 57-year-old female presented with an approximately 4-year history of pain and swelling below the left knee and in the right hand. An MRI of the left knee showed multiloculated cystic-intensity lesions around the knee joint with intra-/extra-articular and intraosseous components. Similar lesions were noted around the right knee joint, right wrist and ring finger. With all the above findings and in view of multiple joint involvement, the possibility of cystic ganglionosis or multiple giant ganglion cysts was considered and confirmed by biopsy of one of the knee lesions. It is because of this rarity and the unusual presentation involving multiple joints that we have reported this case. The importance of the diagnosis is that unnecessary surgical intervention and hospitalization can be avoided, provided the patient’s symptoms are not severe. To our knowledge, no case of multiple ganglion cysts in adults has been reported in the medical literature. Only one case of multiple ganglion cysts has been reported in an 11-year-old child.2 We have reported this case because of this rare multiplicity, the unusual presentation involving the bilateral knees and Hoffa’s fat pad, together with multiple intraosseous lesions in a single patient. This is probably the first case of an adult reported in the literature.Ganglion cysts are common lesions presented by the patients. Ganglia are not true tumours. Ganglia arise from the joint capsules, bursae, ligaments, tendons and subchondral bone. Hands, wrists and feet are the sites where they are commonly seen. They are usually asymptomatic; however, the mass effect on the adjacent tissue can sometimes be the clinical presentation.T1 weighted and hyperintense on T2 weighted sequences around the knee joint with intra- and extra-articular components  with limitation of motion and paraesthesia.2About 50–70% of soft-tissue masses in the wrist region are ganglions.9 Before surgery, it is important to confirm the cystic nature of these lesions to determine their relationship to the joint and evaluate the joint for the presence of associated intra-articular disease. A demonstration of the relationship of the cyst to the capsule may be important in differentiating the ganglia from the other lesions, as failure to remove the capsular components of the cyst may lead to recurrence.10 A high rate of spontaneous resolution and recurrence after surgery argue against surgical intervention to treat them.Watchful waiting, non-operative aspiration and surgical removal are the main treatment options. High recurrence rates are noted with cyst aspiration. Surgery is better in terms of lower rates of recurrence, but has a higher incidence of complications.To our knowledge, no case of multiple ganglion cysts in adults has been reported in the medical literature. Only one case of multiple ganglion cysts has been reported in an 11-year-old child. The multifocal distribution of the ganglion cysts in our patient is unique, also bilateral involvement of the knees has never been reported previously. These may imply some inherent susceptibility of the patient to develop these lesions. The need to screen other joints and look for multifocality is highlighted by this case. We have reported this case because of this rare multiplicity, the unusual presentation involving the bilateral knees and Hoffa’s fat pad, together with multiple intraosseous lesions in a single patient. The importance of the diagnosis is that unnecessary surgical intervention and hospitalization can be avoided, provided the patient’s symptoms are not severe.Ganglion cysts are commonly seen around the joints and tendons, appearing as cystic lesions on imaging, and should be included in the differential diagnosis of any cystic lesion around the joints and tendons.Cystic ganglionosis (multiple ganglion cysts) is a very rare condition, where ganglion cysts involve multiple joints and tendons, as described in our patient. This has been hardly reported in the literature, hence its prevalence and associations are not known and further studies are needed in this regard.Awareness and recognition of this condition by the radiologist is important, as they can guide the clinician on management, which is mainly conservative.


A spontaneous diaphragmatic hernia (SDH) occurs when intra-abdominal contents extend into the thoracic cavity through a defect in the diaphragm after a sudden increase in intra-abdominal pressure. SDH is one of the rarest surgical emergencies with less than 30 reported cases in the literature. A patient presenting to the emergency department (ED) in respiratory distress with unilateral breath sounds does not have an extensive differential diagnosis. In a trauma scenario a pneumothorax or hemothorax quickly comes to mind. It is taught that a chest tube should be inserted without further diagnostic testing. In the non-traumatic patient a spontaneous pneumothorax, pleural effusion, pneumonia, or airway foreign body are among the most likely etiologies. Spontaneous diaphragmatic hernia (SDH) is not often considered, and thus may be a missed diagnosis during initial evaluation.SDH is one of the rarest surgical emergencies comprising less than 1% of all diaphragmatic hernias, with fewer than 30 reported cases in the literature.2A 94-year-old woman with chronic obstructive pulmonary disease, hypertension, and breast cancer presented to the ED in respiratory distress. She reported dyspnea starting the night prior to presentation with no history of trauma. She was normothermic, had a normal heart rate and blood pressure, but was tachypneic and hypoxic to 88% on room air. Physical exam revealed significant accessory muscle use, no stridor, no jugular venous distention, normal heart sounds and diminished breath sounds in the left hemithorax.While the nurse gathered a 14-gauge needle and a chest tube tray for needle decompression followed by tube thoracostomy, a bedside ultrasound was performed. The ultrasound showed bilateral pleural sliding without significant B-lines or effusion. Portable chest radiograph revealed that a large amount of intra-abdominal contents had entered the thoracic cavity resulting in a shift of the mediastinum . A nasog(CT) scan to further characterize the defect in the diaphragm  occurs when intra-abdominal contents extend into the thoracic cavity through a defect in the diaphragm after a sudden increase in intra-abdominal pressure.What makes this presentation of disease reportable?SDH is one of the rarest surgical emergencies with fewer than 30 reported cases in the literature.What is the major learning point?The diagnosis of SDH should be considered in patients presenting in respiratory distress with unilateral or asymmetrical breath sounds.How might this improve emergency medicine practice?Increased awareness of this rare diagnosis could prevent the unnecessary and potentially harmful placement of a chest tube in this patient population.Diagnostic imaging options in the ED include chest radiograph, CT, ultrasound, magnetic resonance imaging, and upper gastrointestinal (GI) contrast studies. The most readily available options are radiograph, point-of-care ultrasound, and CT.9The most accurate method of making the diagnosis is via exploration in the operating room.Spontaneous diaphragmatic hernia is a rare diagnosis that is often missed on the initial patient encounter. While the treatment is surgical, therapeutic measures can be taken in the ED to alleviate symptoms and suffering. The utility of bedside ultrasound in the diagnosis of SDH is yet unproven. However, in our case it rapidly excluded a pneumothorax and prevented the unnecessary placement of a chest tube in an elderly woman.Documented patient informed consent and/or Institutional Review Board approval has been obtained and filed for publication of this case report.


Mesenteric cysts are a rare and often asymptomatic incidental finding on imaging. The diagnosis and management of mesenteric cysts remains a clinical challenge since those presenting with symptoms, often have vague and nonspecific symptoms, owing to variability in cyst location and size. This case report will aim to discuss the presentation, investigation and management options available.A 73-year-old female presented with abdominal swelling and a palpable right sided mass. Examination revealed a right sided mass and abdominal distention with vital signs within normal limits. Ultrasound scan (USS) revealed a right-sided 12 cm × 11 × cm × 8 cm thin walled cyst.Mesenteric cysts are mostly asymptomatic but can cause nonspecific symptoms of abdominal pain, abdominal distension, altered bowel habit, nausea, vomiting and an abdominal mass. Complete surgical excision, either laparoscopically, or through a laparotomy is typically considered first line treatment. If size or location of the cyst precludes complete surgical excision, partial excision with marsupialisation of the opening of the cyst into the abdominal peritoneal cavity is a second option.Mesenteric cysts represent a rare cause of intra-abdominal mass. Owing to low prevalence, literature is limited as is guidance on management. Careful pre-operative planning is essential so as to avoid operative complications. Imaging such as USS is of great importance; however Computed Tomography (CT) and Magnetic Resonance Imaging (MRI) may be of more benefit, owing to the complex anatomical relations within which mesenteric cysts can present. Surgical excision is widely reported as the surgical treatment of choice for symptomatic cysts. Mesente2A 73-year-old female presented to her General Practitioner (GP) with a history of abdominal swelling and a right sided abdominal mass. She also complained of early satiety and intermittent bloating but was otherwise systemically well with no fever. Notably absent were symptoms of: abdominal pain, vomiting, urinary symptoms, change in bowel habit or significant weight loss. Her past medical history included hypertension and atrial fibrillation, for which she was taking regular antihypertensive medications and warfarin. Previous surgeries included a hysterectomy and appendicectomy. She was fully independent, an ex-smoker, with no significant alcohol history. There was no family history of relevance or any congenital abnormality. On clinical examination, vital parameters were within normal limits with no lymphadenopathy, jaundice, pallor or pedal oedema present. On abdomen examination, her abdomen was distended but soft, with no obvious shifting dullness. A well-defined right sided intraabdominal mass could be felt on palpation, which was non-tender, with well-defined margins and cystic in consistency.Blood tests including full blood count, urea and electrolytes, c-reactive protein and liver function tests were conducted which were unremarkable and within normal limits. Tumour markers were not requested. In the first instance an ultrasound of the abdomen was arranged which revealed a right-sided 12 cm × 11 × cm × 8 cm thin walled intrabdominal cystic mass. The origin of the cyst was difficult to determaine but USS suggest the mass may reporesent an exophytic right renal cyst. A follow up CT scan was requested, with the patient being referred to Urology multi disciplinary team (MDT) clinic. Once completed the CT scan report demonstrated that the cystic lesion in the abdomen on the right side was abutting the right kidney but did not appear to be arising from it. The organ of origin was still uncertain. There were no radiological features suggestive of malignancy, and no significant abnormalities of the solid abdominal or pelvic viscera .Fig. 1CTFollowing the CT scan findings, which suggested that the cyst was separate from the right kidney, the case was referred on to the general surgery team. MDT discussions concluded that this large cystic mass was likely to represent a ‘mesenteric cyst’ and operative management was recommended. The differential diagnosis of a mesenteric cyst includes: pancreatic pseudocysts, omental cyst, ovarian cyst, haemangiomas, endometriosis, loculated ascites , mesothelioma and lymphoma .Surgical management was deemed the most appropriate intervention starting with a laparoscopic approach, which showed that the right ureter appeared adherent to the anterior cyst wall, a relationship not previously identified on imaging. Given this anatomical relationship the surgery was converted to an open laparotomy. A mesenteric window was opened to access the cyst, and the ureter was preserved . Due to 3Mesenteric cysts are rare intra-abdominal lesions located anywhere in the mesentery of the gastrointestinal tract from duodenum to rectum. The incidence is 1 in 250,000 hospital admissions . They we4Mesenteric cysts are a rare cause of an intra-abdominal mass. They are found in 1 in 250 000 hospital admissions and represent an uncommon cause of abdominal mass. The aetiology is unclear but one suggested theory is that the mesenteric lymphatic tissue proliferates and fails to communicate with the core lymphatic system. Although mesenteric cysts are mostly asymptomatic they can cause nonspecific symptoms of abdominal pain, abdominal distention, altered bowel habit, nausea, vomiting and an abdominal mass. A careful history, examination and investigation are essential for both the diagnoses and management of these cases. The recommended management option of choice is complete surgical excision either laparoscopically or through a laparotomy. If this is not an option due to the size of the cyst or location; partial excision with marsupialisation of the opening of the cyst into the abdominal peritoneal cavity is a second option.We do however note the rarity in incidence, of mesenteric cysts. There remains a significant clinical need for trials to help determine optimum management, surgical approaches and indeed potential novel surgical techniques for management.Farzan Dholoo – Nothing to declare.Amanda Shabana - Nothing to declare.Rebecca Nunn – Nothing to declare.Waseem Hameed – Nothing to declare.Farzan Dholoo – Nothing to declare.Amanda Shabana - Nothing to declare.Rebecca Nunn – Nothing to declare.Waseem Hameed – Nothing to declare.The following statement applies for all listed authors:Appropriate consents, permissions and releases have been obtained from the patient – We have a copy of the signed consent form.The following statement applies for all listed authors:Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal on request”.We do not have ethics committee approval as this was not a study on multiple patients.No identifying details are included.Amanda Shabana and Farzan Dholoo are primary authors.Rebecca Nunn is a second author.Waseem Hameed is the responsible Consultant Surgeon, who: oversaw care of the patient; oversaw the project; edited the manuscript up to submission and supervised/facilitated the project up to submission.The following statement applies for all listed authors:There was no research involving human participants.There was no trials or observational research undertaken.This is a case report only.Mr Waseem Hameed .Waseem Hameed, Wexham Park Hospital, Frimley Health NHS Foundation Trust, General Surgery, Berkshire UK.Not commissioned, externally peer-reviewed.


Trichophagia is the habit of chewing hair. On an average, only 1% patients with trichophagia develop trichobezoar. They are usually symptomless until they reach a large size. It is common in young females usually with an underlying psychiatric disorder. The gold standard for diagnosis is upper gastrointestinal endoscopy (UGIE). Here we present a case of trichobezoar in an 11-year-old girl who presented in the emergency with complaint of pain abdomen and symptoms of obstruction.A high index of suspicion among physician can lead to early diagnosis and management in these cases (trichobezoar).How to cite this article: Prasad A, Jain A, Gupta A, Kamra A. Trichobezoar: Ravenous for Hair. Euroasian J Hepato-Gastroenterol 2018;8(1):97-98. This term is believed to originate from the Arabic word “Badzehr,” Persian word “Padzahr,” or Turkish word “Panzehir,” all of which mean substance that act as “antidote” or counterpoison.2Bezoars can be classified according to the content, such as trichobezoar (hair), phytobezoar (vegetable fibers), lactobezoar (milk products), pharmacobezoar (drugs), diospyrobezoars (persimmon fibers), cotton bezoars (cotton fibers).3 Trichopha gia is the habit of chewing the hair. On an average, only 1% patients with trichophagia develop trichobezoar. The slippery surface of the hair tufts resists the gastric peristaltic propulsion and tends to collect in the gastric mucosal folds. As more and more hair accumulates, a ball of hair is formed, which becomes too large to escape and results in gastric atony and subsequently takes the shape of stomach.Trichobezoars were first described by Baudomont in 1779.Here we present a case of trichobezoar in an 11-year-old girl who had the habit of chewing hairs and who presented in the emergency with pain in abdomen and symptoms of obstruction.An 11-year-old thin-built, anemic girl was brought to the emergency with complaint of pain in upper abdomen and recurrent vomiting since 1 week. Abdominal examination revealed a nontender lump in the epigastric region, which was moving with respiration. Rest examination was normal. In laboratory examination, microcytic hypochromic anemia was seen; other parameters were normal. Parents gave history of disturbed behavior of child for past few months and decreased appetite. Sibling of the patient had seen her chewing her hairs infrequently, but did not think of it to be a major problem.She was initially admitted in other hospitals for this, where conservative management was given, but there was no relief. Along with other routine investigations, UGIE was done in which there was a large blackish mass in the stomach and the sample taken from it showed mostly hairs. Based on the history and examination, diagnosis of trichobezoar was made and patient was taken for surgery. Anterior gastrotomy was done  and larg9 in which a dense compact mass of hair (trichobezoar) was found in the stomach with extension into intestine through duodenum in patients with a history of psychiatric disorder. The name “Rapunzel” syndrome comes from the Grimm Brothers’ fairy tale of a 12-year-old princess who was shut into a tower with neither stairs nor doors by an enchantress who climbed up the tower’s walls with the help of Rapunzel’s long tresses.10Rapunzel syndrome was first described in the literature by Vaughan et al in 1968Trichobezoars are usually symptomless until they reach a large size. Clinical presentation is deceptive and vague, ranging from an abdominal mass to gastrointestinal symptoms, wasting and cachexia. Large bezoars are often palpable and may be indentable (Lamerton’s sign).Severe halitosis and patchy alopecia provide clues on physical examination. Imaging may show the bezoar as a mass or filling defect. The gold standard for diagnosis is UGIE. In addition to direct visualization, one can take sample and therapeutic intervention can also be done in some cases.1Complications of trichobezoars include gastrointestinal obstruction (26%), bleeding (10%), perforation, malabsorption, and nutritional deficiencies. If bezoars are left without treatment, the mortality rate can reach 30% because of the associated complications.In our case, the patient had psychiatric symptoms but the family did not take it seriously. The clinical presentation was also vague, which made the diagnosis of trichobezoar difficult in the initial stage. The finding of UGIE led to the diagnosis and then surgery was planned as endoscopic removal was not possible.There should be awareness among the general public for any mental disturbance or altered behavior, which should be consulted with the physician or psychiatrist and treatment should be started before any delay leads to complications, whether physical, mental, or social.A high index of suspicion among physicians can lead to early diagnosis and management in these cases (trichobezoar).


Extra-abdominal desmoid tumors (DTs) are rare tumors of apparent fibroblastic origin with unpredictable clinical behavior. Though histologically benign and slow growing, DTs can be proliferative, aggressive tumors, invading the surrounding areas. DTs located extra-abdominally are most commonly found in the extremities or proximal structures like the shoulders, chest wall, and neck. Spinal involvement is very rare. Here, we describe a case where an extra-abdominal DT mimicked a schwannoma in the posterior cervical spine. A 67-year-old female patient presented with acute neck and bilateral shoulder pain. After attempting conservative treatments with no symptomatic relief, a magnetic resonance imaging of the cervical spine was obtained, showing a paraspinal mass in the posterior elements from C2 to C4. The computed tomography guided needle biopsy showed rare spindle cells, suggestive of a spindle cell neoplasm, and complete surgical resection was performed. The pathology report was consistent with fibromatosis, leading to a final diagnosis of the extra-abdominal desmoid. This case demonstrates a rare presentation of an unusual tumor that often manifests with nonspecific symptoms or no symptoms at all. Extra-abdominal desmoid tumors (DTs) are rare tumors of apparent fibroblastic origin with unpredictable clinical behavior. Desmoid-type fibromatosis accounts for less than 3% of all soft tissue tumors and about 0.03% of all neoplasms -3, affecDue to the high rate of invasion and local recurrence, as well as their variable natural history, DTs are difficult to treat clinically and can lead to significant morbidity and death. Prognoses are worse for tumors in the head and neck due to negative local consequences. Diagnosis and evaluation of the tumor is accomplished by using magnetic resonance imaging (MRI), while treatment for both intra- and extra-abdominal tumors requires a multidisciplinary approach -3. ManagDesmoids occurring extra-abdominally are most commonly found in the extremities or proximal structures like the shoulders, chest wall, and neck. Spontaneous formation is rare overall, but accounts for the majority of DTs . As of 2Interestingly, previous cases have identified a link between resection of schwannomas and subsequent development of extra-abdominal DTs , 8. HoweCaseA 67-year-old woman with a history of C5-C7 anterior cervical decompression and fusions presented with acute neck and bilateral shoulder pain. The patient did not have a history of trauma, significant family history, or syndromic findings suggestive of Gardner syndrome. Initially, she was managed conservatively using NSAIDs for pain management and physical therapy with minimal symptomatic relief. Her neurological exam, including motor, sensory and reflex testing was nonfocal. Due to the failure of conservative treatments, MRI of the cervical spine was obtained. This showed an approximately 3.5 cm x 1.7 cm x 1.6 cm paraspinal mass in the posterior elements from C2 to C4 (Figures Surgical observationsThe mass was identified between spinal levels C2 and C4, below the muscular plane on the left side. The tumor was large, firm, and surrounded by muscle. The lesion was delineated from its attachment to the muscle in different planes. Direct stimulation of the lesion did not elicit an electromyographic response.Postoperative careThe patient had an uneventful postoperative course and was discharged home on postoperative day 2. A postoperative MRI confirmed gross total resection of the tumor Figures -6. GivenPathologyThe tumor was a spindle cell neoplasm of low to moderate cellularity without significant pleiomorphism Figures -8. It inExtra-abdominal DTs, or "aggressive fibromatoses" are locally aggressive, slow-growing neoplasms with no metastatic potential, but a substantial local recurrence rate. DTs typically infiltrate adjacent skeletal muscle cells and are otherwise within a densely collagenous or myxoid matrix, as seen in sections of the tumor from this patient.The World Health Organization classifies DTs as intermediate or locally aggressive soft tissue tumors. This is to account for the absence of a demonstrable, metastatic potential combined with the high incidence of local recurrence, requi ring wide surgical margins to decrease the likelihood of recurrence . Extra-aThe incidence of DTs changes depending on age and gender. In pediatric populations, most DTs are extra-abdominal and occur in both males and females equally . The samDepending on the location of the tumor, extra-abdominal fibromatoses affect patients differently. DTs are often painless and most patients are asymptomatic. Patients seldom present with decreased joint mobility, neurological symptoms, or pain . InteresAs treatment of DTs requires an interdisciplinary approach, management strategies are determined individually for each patient.An extra-abdominal desmoid is a rare tumor. This case demonstrates a rare presentation of an unusual tumor that often manifests with nonspecific symptoms or no symptoms at all. Clinical suspicion and a tissue biopsy are required for diagnosis


Cystic lesions located in the paravaginal region are rare. When present, paravaginal cysts are typically benign and are incidentally found on routine gynecological exams; however, rarely they can be malignant. Treatment options for paravaginal cancers are not well studied and early diagnosis may help improve prognosis in these patients. Our case describes a 55-year-old female with a recurrent paravaginal cyst that was remarkable for serous papillary adenocarcinoma despite biopsy and fluid cytology negative for malignancy. This case demonstrates that malignancy should be considered highly with a recurrent paravaginal cyst, especially when present over a short interval. Large paravaginal cysts are rare and when present are most commonly benign. These cysts can be either congenital or acquired. While congenital cysts are associated with developmental abnormalities and are typically derived from embryological remnants, acquired cysts are most commonly caused by trauma . There hA 55-year-old postmenopausal female, gravida 0, presented for an annual gynecologic exam. Her surgical history was significant for a total abdominal hysterectomy with bilateral salpingo-opherectomy in 1986 for severe endometriosis. She had been taking conjugated estrogen daily since the operation due to postmenopausal symptoms and denied any vaginal bleeding or vaginal discharge. On bimanual examination, the uterus and cervix were noted to be surgically absent; however, a large pelvic mass was palpated. This mass was smooth, approximately 8 cm in diameter, and located at the apex of the vaginal vault. It was palpable rectally with no evidence of generalized lymphadenopathy. Stool guaiac test was negative.Diagnostic tests to further investigate the mass included vaginal cuff cytology, imaging, and laboratory studies. A Papanicolaou test was benign. Serum Cancer Antigen-125 was normal at 8.4 U/mL. Computed tomography of the abdomen and pelvis (CT) revealed a 10 cm mass posterior to the bladder, compressing the rectum to the left . The cenApproximately two weeks later, the patient presented to clinic with right upper and lower quadrant pain, vaginal spotting, and watery vaginal discharge. There was no rebound tenderness or guarding present. A pelvic exam was unremarkable; the vaginal cuff was intact with no evidence of any masses or lesions. CT was notable for a right sided cystic mass that displaced the bladder and rectosigmoid colon to the left. Ultrasound-guided aspiration of the 7.67 x 7.76 cm loculated cystic mass was performed revealing 150 cc of serous fluid, with immediate relief in the patient's symptoms . Due to Three weeks following aspiration of the cyst, the patient returned to clinic with complaints of right pelvic pain. Transvaginal ultrasound showed a recurrence of the 7 cm cyst, which was aspirated to reveal 270 cc of serosanguinous fluid. Cytology of the fluid was negative for malignancy and consistent with a benign cyst. Interestingly enough, a third recurrence of the cyst was noted with 230 cc of subsequent serous fluid drainage. Given the multiple recurrences and unpredictable nature of cyst, a laparotomy and right cystectomy was scheduled.Surgical findings were significant for a large fluctuant mass in the right pelvis extending to the posterior peritoneum. The posterior-lateral capsular wall of the cyst was noted to be nodular and extensively adherent to the posterior peritoneum and pelvic side wall. Because of the extent of the adhesions, a combined transvaginal and abdominal resection was necessary. The right vaginal wall was subsequently entered, and the cyst wall was dissected from the deep pelvic side wall. Intraoperative frozen section of the cyst was notable for serous papillary adenocarcinoma. At this point in time, Gynecology Oncology was consulted intraoperatively and an omentectomy and peritoneal washings were performed which were also negative for malignancy. A thorough exploration of the pelvic cavity was only notable for a small cystic mass at the top of the sigmoid colon, which was excised and found to be a benign serous cyst.Final pathology of the paravaginal cyst wall was remarkable for a benign squamous lining with scattered cell fragments of degenerated papillary configuration consistent with serous papillary carcinoma. The nodules on the posterior wall of the cyst showed necrotic stroma with dystrophic calcification representing infiltration of moderate to poorly differentiated serous papillary carcinoma of unknown primary. Further review of tumor markers demonstrated that the wall of the cyst was estrogen receptor (ER) and progesterone receptor (PR) negative; however, it was positive for Herceptin, CA-125, and focally positive for Wilms Tumor (WT-1), consistent with serous papillary carcinoma .2 with concurrent external beam radiation plus interstitial brachytherapy. After receiving three courses of her chemotherapy, the patient refused additional treatment due to inability to tolerate the side-effects. Despite undergoing external beam radiation plus interstitial brachytherapy for three months, the patient experienced rapid clinical deterioration with subsequent vesicovaginal fistula formation, and the decision was made for palliative therapy and pain management after three months of aggressive treatment.Preoperative imaging of the patient was unremarkable for metastasis or any suspicious lesions. Given the findings, a postoperative head CT was performed which revealed no evidence of intracranial metastasis. The patient was subsequently referred to Gynecology Oncology where thorough discussions were held regarding palliative therapy versus chemotherapy and radiation. She was counseled on the limited data currently available on the treatment of vaginal cancer, and all acute and long-term serious sequelae, along with her poor prognosis, were further explained to the family. After many discussions, she was planned to start on once weekly cis-platinum chemotherapy given intravenously at a dose of 50 mg/mParavaginal cysts are asymptomatic and are incidental masses that are usually found during routine gynecological exams . These cCurrently, treatment options and studies existing for the treatment of paravaginal cancers are extremely limited. The combination of external beam radiation and chemotherapy has been studied extensively by numerous investigators –9. McCurThis case demonstrates a serous papillary carcinoma that was not found on primary aspiration of a paravaginal cyst, but was found in the cyst wall once it was excised after three subsequent recurrences. It illustrates that malignancy should be considered highly on the differential diagnosis with a recurrent paravaginal cyst, especially when they persist within a short interval. Perhaps, if it had been discovered earlier, the patient would have had a better prognosis with concurrent radiation and chemotherapy, but given the multiple recurrences and the delay in time to treatment, the patient in this case deteriorated rapidly.


Glomus tumors are rare, benign, vascular neoplasms arising from the glomus body. Although they can develop in any part of the body, they commonly do so in the upper extremities, most frequently subungual areas. They can be either solitary or multiple. Most typically they present as a small, round, bluish nodule visible through the nail plate with a classic triad of symptoms: hypersensitivity to cold, heightened pinprick sensitivity, and paroxysmal pain. Several tests can help in diagnosing these tumors with multiple imaging tools such as X-ray, magnetic resonance imaging, and ultrasonography. However, only histology can confirm the diagnosis. Complete surgical excision of the tumor is the only effective treatment to achieve pain relief and low recurrence rate.We report here a rare case of a 54-year-old Moroccan Berber woman presenting with a 10-year history of a glomus tumor at an unusual site. Dermoscopy and histology were helpful to confirm the diagnosis.We aim to discuss clinical, dermoscopical aspects of this tumor and surgical modalities. We also emphasize the importance of keeping this tumor in mind among the possibilities of differential diagnosis of painful digital nodules. Glomus tumors, as first described by Masson in 1924, are rare, benign, vascular neoplasms arising from the glomus body, which is a contractile neuromyoarterial structure found in the reticular dermis . ApproxiA 54-year-old, right-handed unemployed Moroccan Berber woman from an urban area reported a personal medical history of intermittent epigastric pain without a history of diabetes or chronic disease, nor any special psychosocial background, and with a familial history of allergic rhinitis. She presented with a 10-year history of progressively intense pain, cold sensitivity, and severe tenderness to palpation of the pulp of her left little finger, with no gross abnormalities of her fingers, and no previous trauma history. The pain increased when her digit was exposed to cold. Furthermore, the tip was excessively sensitive to touch, and her pain increased at night. She had seen a primary care doctor, with no definitive diagnosis. Moreover, she reported occasional intake of omeprazole for intermittent abdominal pain. She had no history of active or passive tobacco smoking or alcohol intake. She also had no past intervention. She was referred to our department for surgical excision with histopathological examination.A clinical examination showed a well-oriented, apyretic, and eupneic patient, with normal cardiac frequency and regular blood pressure, presenting with a painful subcutaneous nodule of approximately 1.5 cm, of firm consistency and pinkish red coloration, streaked with multiple telangiectasias on the pulp of the distal phalanx of her left little finger Fig. . A neuroThe differential diagnosis at the time of examination included glomus tumor, schwannoma, mucoid cyst, and neurofibroma. An X-ray study was done for her left hand. No bony lesions were identified by radiographic studies Fig. . She didSurgical intervention was performed. A paramedian volar incision was made of the pulp of the distal phalanx of her left little finger. The mass was well circumscribed and removed with blunt dissection and sent to pathology Fig. . It was Here we present a rare case of a histologically confirmed glomus tumor of the pulp, which is an unusual site of such tumors, with a 10-year history of intense pain, cold sensitivity, and severe tenderness to palpation of the pulp. Through this case, we emphasize the dermoscopical features of this tumor in such a location, which are lacking in the literature, and we detail histological aspects and operative procedure.These tumors, also known as Barré–Masson syndrome, are rare benign hamartomas that arise from the normal glomus apparatus, located in subcutaneous tissue . They weThe etiology of glomus tumors is unknown and it may be related to sex, age, trauma, or inheritance. Some authors have proposed that a weakness in the structure of a glomus body could lead to reactive hypertrophy after trauma . Such riIt has a variable evolution time between days and decades; it is a difficult tumor to diagnose due to its rarity, which justifies long delays in diagnosis and the establishment of therapy .Glomus tumors are categorized as either solitary or multiple, according to their clinical presentation . The mosAlthough the cause of pain in glomus tumor is not clearly understood, several hypotheses have been proposed : the capThe dermoscopic appearance of glomus tumors can be very subtle. In subungual tumors, nail plate dermoscopy can find the presence of linear vascular structures , whereasImportantly, the diagnosis of glomus tumor must be made through the history and clinical examination of a patient . TypicalIn addition to the classical presentation, there are three useful tests that help in diagnosing these tumors : Love’s In this case, X-rays revealed the mass to be in the distal pulp of our patient’s little finger. Further radiological investigations were not necessary in our patient because of the clinical features of the tumor and the presence of paroxysmal pain exacerbated by cold.Histopathological analysis reveals variable composition of glomus cells, blood vessels, and smooth muscles . It may Although glomus tumors are essentially benign, sarcomas accompany benign glomus tumors in rare cases to form glomangiosarcoma . HistoloSolitary glomus tumors need to be ruled out from painful tumors, such as leiomyoma or eccrine spiradenoma . MoreoveComplete surgical excision of the tumor is the only effective treatment . For subMeticulous care needs to be taken at the first operation to completely remove all lesions  because We report the case of a glomus tumor arising in the unusual location of the pulp of a finger, with typical symptoms of long-term pain and sensitivity to touch. Here we are the first to describe the dermoscopic features of a glomus tumor in the rare location of the pulp of a finger. We aim to emphasize the importance of the inclusion of the glomus tumor among the possibilities of differential diagnosis of painful digital nodules, despite its low occurrence. Clinicians should also keep the possibility of these tumors in mind and perform careful examinations and preoperative tests. Complete surgical excision is mandatory to get complete relief from the symptoms and to avoid recurrence.