The ganglioneuroma is a rare tumor arising from sympathetic nervous system, which is composed of gangliocytes and mature stroma.A retroperitoneal ganglioneuroma was found in a 14 years old Saudi boy, presented with recurrent vomiting. CT image showing a retroperitoneal cyst near the inferior vena cava and right iliac vessels. Treated by laparoscopic cystic excision, histopathology of the cyst showing ganglioneuroma, completely excised.Ganglioneuromas arise from the sympathetic chain, The most common sites of presentation are the posterior mediastinum, retroperitoneum, head and neck region. GN are usually asymptomatic found incidentally on abdominal imaging. The treatment of retroperitoneal mass is complete surgical excision.Ganglioneuroma (GN) is a rare benign tumor, usually asymptomatic. One of the most common site of presentation is retroperitoneum, the main treatment for that is complete surgical excision. They arise from the neural crest include neuroblastoma, ganglioneuroblastoma (nodular or intermixed), and ganglioneuroma .The most benign tumor is the ganglioneuroma, which is composed of gangliocytes and mature stroma. Ganglioneuroblastoma is composed of both mature gangliocytes and immature neuroblasts and has intermediate malignant potential. Neuroblastoma is the most immature, undifferentiated, and malignant tumor of the three .2In January 2019, A 14 years old Saudi boy not known to have any medical illness, presented to the clinic complained of recurrent vomiting for the last 2 months. The vomiting was food content. No history of abdominal pain, nausea, change on bowel habit, weight loss and fever. No family history of any malignancy nor history of radiotherapy or smoking.On examination: Conscious oriented alert, not on respiratory distress, not pallor neither cyanosis, he looks underweight. Systemic examination unremarkable. Cardiovascular and chest examination were unremarkable. Abdominal examination swelling was notice at epigastric and right upper quadrant area, abdomen was soft, with no tenderness, normal bowel sound.9/l hgb12.9 g/dl platelets 272 × 109/l, albumin 47 g/l. Abd x-ray reveled normal distribution of bowel gas. CT abdomen His workup wbc 7 × 10Surgical management was considered for him including: laparoscopic exploration with cystic excision, patient was on supine position. Intraoperative finding was showing encapsulated mass measured 10 × 9 × 6 cm in size adherent to the IVC, its was carefully dissected from it using blunt dissection and electrocautery Histopathology of the cyst showing ganglioneuroma, completely excised, composed of numerous spindle cells consistent with shawanian cell mixed with ganglion cells, no neuroblastic component. Patient was discharge on good health condition during his visit on the clinic, improve of his complain for regular follow up.3Ganglioneuroma (GN) is a rare benign tumor, its either happen spontaneously or during the therapy for neuroblastoma with either chemotherapy or radiation therapy. Association with Turner’s syndrome and multiple endocrine neoplasia II has been reported .Ganglioneuromas arise from the sympathetic chain. The most common sites of presentation are the posterior mediastinum, retroperitoneum, head and neck region .GN are usually asymptomatic found incidentally on abdominal imaging or having nonspecific symptoms related to mass effect and compression of surrounding organs.Back pain due to spinal deformity, vomiting and abdominal pain from compressing the stomach or bowel. The compression of the diaphragm muscle patients exhibited dyspnea. Changes in gait, weakness of the muscles and senses, from the mass compresses the spinal cord. Some patient may have Hypertension and diarrhea if the tumor secretes catecholamine and vasoactive intestinal peptide .The imaging modality of choice is contrast enhanced CT scan and MRI imaging. For identifying the relationship of the tumor with surrounding organs and vital structures. The ganglioneuromas appear as an oval, well-defined mass in the adrenal gland or in extra-adrenal location with low attenuation on unenhanced CT scans, homogeneous low signal intensity on T1-weighted images and homogeneous high signal intensity on T2-weighted image on MRI. The definitive diagnosis is made by the histopathology .The treatment of retroperitoneal mass is complete surgical excision. One of the limitation factor for complete excision the tumor surrounding vital organs and blood vessels. The surgical resection of these tumors can be undertaken via laparoscopic or open technique. Open surgery preferred while operating on tumor in close proximity of or surrounding important blood vessels in the abdominal cavity .Follow-up is needed the tumor have slow progression potential and late recurrence .4Ganglioneuroma (GN) is a rare benign tumor, usually asymptomatic. One of the most common site of presentation is retroperitoneum, the main treatment for that is complete surgical excision. Regular follow-up is needed due to late recurrence.All of authors have no conflict of interest.This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors.In our institute, ethical approval is exempted, depend on acquired patient consent.Written informed consent was obtained from the guardian on behalf of the patient for publication of this case report. A copy of the written consent is available for review by the Editor-in-Chief of this journal.Nahlah arab and Ashwag alharbi contributed to manuscript preparation, manuscript editing, manuscript review.We don’t need to register this work.The Guarantor is DR. Ashwag Alharbi.Not commissioned, externally peer-reviewed.


Neuroendocrine tumors (NETs) of small intestinal origin are generally slow-growing tumors with a relatively high propensity for metastases to surrounding organs and lymphatic tissue. We present the first case of an ileal NET metastasizing to the submandibular gland in a woman with metastatic carcinoid syndrome.A 55-year-old female presented with a four-month history of a palpable, left-sided neck mass. The patient had a history of metastatic neuroendocrine tumor of ileal origin, initially treated with primary resection 4.5 years previously, with known subdiaphragmatic metastases to the liver, mesenteric nodes, and peritoneum. Four years following primary resection she developed carcinoid syndrome leading to therapy with radiolabelled metaiodobenzylguanidine (MIBG), as well as telotristat etiprate in the context of a clinical trial due to progressive symptoms. A fine needle aspiration biopsy of the neck mass revealed an immunohistochemical staining pattern consistent with ileal NET. The patient underwent a left level 1b neck dissection and submandibular gland excision. Pathology was consistent with metastastic ileal NET.We report the first case of ileal NET metastasis to the submandibular gland. Familiarity with the carcinoid syndrome and associated physiology should be maintained as it can affect the head and neck on rare occasions. Maintaining a broad differential is key in diagnosis of undifferentiated neck masses. They are the most common malignancies of the small intestine, with most originating in the ileum, and can give rise to carcinoid syndrome if metastases to the liver develop. The classic carcinoid syndrome is due to circulating serotonin secreted by metastatic, functional small intestinal NETs and results in progressive facial flushing, diarrhea, and wheezing .Rare cases of metastasis from bronchopulmonary NETs to the head and neck have been reported, including the parotid glands  and thyr2A 55-year-old female presented to the Otolaryngology – Head & Neck Surgery clinic with a four-month history of a left-sided neck mass. The patient had a history of metastatic ileal NET with metastases to the liver, mesenteric nodes, and peritoneum. She underwent primary right hemicolectomy and small bowel resection in 2010. Pathology revealed two foci of well-differentiated malignant NET in the terminal ileum, with muscle and serosal invasion, and three of nine regional lymph nodes involved. There were two to three mitoses per ten high power fields (HPFs) with no necrosis and the Ki-67 index was 3%. Immunohistochemistry revealed positivity for synaptophysin and chromogranin. Pre-operative imaging revealed metastatic involvement of an aorto-caval node, thus the disease was staged as pT4(m)N1M1. Post-operatively, treatment began with long acting somatostatin analogue (SSA). Liver metastases were detected eleven months after her initial surgery. In 2014, she developed carcinoid syndrome leading to therapy with radiolabelled metaiodobenzylguanidine (MIBG). Subsequently she received telotristat etiprate due to progressive symptoms.On presentation of the neck mass in 2015, she had no additional otorhinolaryngological symptoms. Examination of the neck revealed a smooth, mobile, painless, 2-cm mass located in the submandibular triangle. The remainder of the physical examination was unremarkable, including flexible nasopharyngoscopy which revealed no mucosal masses or lesions in the upper aerodigestive tract.Fine needle aspiration (FNA) revealed malignant cells with mildly pleomorphic nuclei and salt and pepper chromatin. Immunohistochemistry was positive for chromogranin and synaptophysin, and consistent with metastatic NET. Computed tomography imaging of the mass revealed left submandibular gland enlargement and no cervical lymphadenopathy . The mulAn uncomplicated left level 1b neck dissection was performed and there were no permanent post-operative sequelae. Gross pathology revealed a circumscribed tumor mass within the submandibular gland that measured 2.2 × 1.5 × 1.4 cm, and appeared as a firm, smooth nodule with a grayish white color. The tumor displayed focal abutment of the margin, but did not extend outside of the gland. Immunohistochemistry was again positive for chromogranin and synaptophysin, consistent with the previous FNA and was .Fig. 2PeUnfortunately, the patient passed away one year later due to progressive disease in the mesentery and liver.3Neuroendocrine tumors, although uncommon, are increasing in incidence. Data from the Surveillance, Epidemiology, and End Results (SEER) program found an approximate 5-fold increase in incidence between 1973–2004 , similar to data from Ontario . They arMultiple synchronous NETs are discovered in approximately 25% of patients with tumors of small intestinal origin, as was the case in our patient ,8. CurreSynchronous metastatic disease is present in approximately 20% of patients at diagnosis, with a further 35–40% developing metastatic disease at some point . To our Therapeutic goals for NETs include curative-intent surgery when feasible, and tumor proliferation and symptom control in the setting of unresectable metastatic disease . First-l4NETs of small intestinal origin are slow-growing tumors with a relatively high propensity for metastases to surrounding organs and lymphatic tissue. We report the first case of an ileal NET metastasizing to the submandibular gland. For all clinicians, awareness of NETs and the carcinoid syndrome is important due to rising incidence and the fact that diagnosis is often delayed due to the symptom non-specificity or absence. For otolaryngologists, maintaining a broad differential for the initial presentation of a neck mass in the setting of an uncommon metastatic disease is also important. Expert pathology review and comparison with original tumor specimens is critical to minimize risk of misdiagnosis.The authors declare that they have no conflict of interest.This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectorsNo research ethics approval was necessary for this case report. Written informed consent was obtained from the patient for publication of this Case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.Written informed consent was obtained from the patient for publication of this Case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.DF: Methodology, validation, investigation, data curation, writing original draft, writing reviewing and editing, visualization. PC: writing original draft, methodology, investigation. MB: writing reviewing and editing, visualization. DR: writing, reviewing and editing. RDH: writing, reviewing and editing. JRT: writing, reviewing and editing. MHR: conceptualization, methodology, writing reviewing and editing. All authors read and approved the final manuscript.N/A.Dr’s David Forner and Matthew Rigby are to be considered the co-guarantor’s for this manuscript.Not commissioned, externally peer reviewed.


An otherwise healthy 36-year-old woman consulted our department with abdominal wall pain. She had a history of double Cesarean section, and lumbar scoliosis. The pain was described as cramping and soar, intensifying after physical activity. The painful area was located cranially to the right superior pubic ramus, radiating towards the right inguinal region. Abdominal ultrasound revealed a heterogeneous mass located in the rectus abdominis muscle, in proximity of its insertion. Hyperechoic and hypoechoic nodules were present, accompanied by a mild degree of internal vascularization Fig. , C. Due Endometrioma in the rectus abdominis muscle is a rare condition. The disease presents in a population of mostly young females of child-bearing age, often with previous history of Cesarean section or gynecological surgery. It is defined as the presence of endometrial tissue outside the uterine cavity. The sonographic appearance is variable. Common sonographic findings are a hypoechoic mass with inhomogeneous echo texture, scattered hyperechoic echoes and irregular margins, as well as solid or cystic lesions. A hyperechoic ring often notes inflammatory alterations. Internal vascularization can be detected in almost every case. Ultrasound is often the first diagnostic tool. Abdominal CT or MR can prove useful in the differential diagnosis. FNAC should be avoided, as spreading of the tissue can occur. It should be noted that neither of the imaging modalities can provide a certain pre-operative diagnosis. The final diagnosis is made by pathological examination after surgical excision of the lesion.The differential diagnosis can be difficult, and includes ventral hernia, hematoma, lymphadenopathy, lymphoma, lipoma, abscess, subcutaneous or sebaceous cyst, suture granuloma, soft-tissue sarcoma, desmoid tumors, and even metastatic cancer. Surgical excision provides the best therapeutic option. In the absence of a suggesting clinical history, the differential diagnosis can be difficult.The authors declare that they have no competing interests.


A retained surgical sponge remains a dreaded complication of modern surgery. Despite the increasing focus on patient safety instances of “a sponge being left in the abdomen”, are all too common in popular media. In this article we report the rare phenomenon of transmigration of a retained surgical sponge in a patient who underwent laparoscopic sterilization.A 30-year-old female presented with progressive abdominal pain for about one month and vomiting with obstipation for 2 days. The patient had undergone laparoscopic sterilization 7 years back and then underwent re-canalization one year back. She underwent an exploratory laparotomy for suspected adhesive small bowel obstruction. During surgery, an intra-luminal surgical sponge was recovered from the distal small bowel. The patient recovered and was discharged in good health.Despite numerous advances in terms of technology and the ever-growing emphasis on patient safety, the problem of a retained surgical sponge remains a dreaded potential complication. All clinicians and health care professionals should be aware of this entity and its various presentations. All over the world over 312.9 million major operations are carried out each year . Herein A 30-year-old female presented to the emergency with progressive abdominal pain for about one month, which had increased in severity for the past 2 days. The pain was initially localized to the right iliac fossa, and was described as colicky, lasting for about 4–5 min with 2–3 episodes/day, partially relieved by analgesics. Over the past 2 days, the severity of the pain had increased and had become generalized. She also had multiple episodes of severe vomiting accompanied by obstipation for the same amount of time.The patient had undergone laparoscopic sterilization 7 years ago and then underwent re-canalization one year back. The patient was initially managed at a primary health centre and was then referred to our hospital with a tentative diagnosis of small bowel obstruction due to adhesions with worsening of symptoms. On examination, the patient had tachycardia, abdominal distension with guarding and rigidity. No abdominal mass was palpable. A per rectal examination revealed hard fecal matter with rectal ballooning. A plain erect abdominal radiograph revealed multiple air-fluid levels suggestive of small bowel obstruction Fig. . SonologA retained surgical sponge is often referred to as a “Gossypiboma”. The word is derived from ‘Gossypium’, which is Latin for cotton, and ‘oma’ which is Greek for tumor/mass. Other terms with similar meanings include, “Gauzeom”, “Textiloma”, “Cottonoma” and “Muslinoma” –7. BasedTransmigration of the surgical sponge a fascinatingly rare event. A 2008 study published by Zandvoort et al. found a total of 64 cases reported worldwide . A subseIn vivo studies in dogs have revealed that the body can respond in one of two ways with respect to gossypibomas. The antigenic potential of the offending agent and the degree of inflammatory response elicited within an individual determines the nature of response .An exudative response is supported by a higher antigenicity and/or a severe inflammatory reaction. This response occurs early and begins abscess formation around the gossypiboma. Increasing pressure leads to breach of the weaker wall, which oftentimes is the gut, resulting in fistula formation and ultimately driving the surgical sponge into the lumen of gut. Peristaltic waves may further augment the deliverance of the gossypiboma into the lumen. This migration of the gossypiboma from the abdomen downregulates the inflammatory response at the site and the process of healing begins by stimulation of fibroblasts. This can often fillup the tract with or without a scar, hence leaving no proof of gut wall perforation. Once inside the lumen, the sponge is thrust onward by peristalsis. If small the sponge may pass spontaneously in stool or it may get stuck at narrow portions of the intestinal lumen. In cases of partial intestinal obstruction, undigested food residue accumulates adjacent to the site of blockage, followed by a few episodes of partial obstruction before becoming complete , 11.If the abscess abuts the abdominal wall it may appear as a septic mass and subsequent rupture of this mass can result in fistulisation as well. Close proximity to the diaphragm can lead to rupture and migration into the thoracic cavity , 11.On the other hand, the transudative response is favoured by low level of antigenicity and/or milder inflammatory response and usually occurs late. This can result in encapsulation of the sponge, resulting in the formation of a mass, or may result in the formation of adhesions, calcification, degradation and uncommonly migration of the gossypiboma as in the exudative response. In contrast to the tissue which surrounds foreign material at other anatomical sites, the tissue encapsulating foreign objects in the peritoneal cavity is avascular in nature. The presence of cytokines may produce anorexia, weakness, weight loss and fever , 10.Gossypibomas may remain asymptomatic for long periods of time depending upon the size, site, and the inflammatory response of body. It may also present with vague ill health, weight loss, fever with chills and rigors, altered bowel habits, anorexia, nausea, vomiting, tenesmus, diarrhoea, discharging sinus, non-healing wound, intestinal obstruction, malabsorption, and as an abdominal mass . The abdVarious risk factors have been described in literature. Emergency surgery, prolonged surgery, unplanned change in the surgical procedure, multiple operating teams, change of members of operating team, obesity, female gender, inexperienced staff, improper counting of surgical towels, sticking of towels, small sponges, haemodynamic instability, and poor communication amongst the surgical team have all been noted in reported cases/series , 15. RadOn sonography the sponge is often described as a well-defined mass with linear or wavy internal echogenic area with intense posterior acoustic shadowing. Computed tomography is the investigation of choice, with detection of the sponge as a mass with a well-defined capsule. On occasion, it may have a spongiform or mottled appearance due to air bubbles and rarely even barium may be seen inside sponge . On magnOnce diagnosed, the only treatment of a gossypiboma is its removal . There aBar code based, or data matrix coded sponges have also been used as an adjunct to increase the accuracy of a sponge count . HoweverAlthough it is easy to assume that the fault lies with one individual, evidence suggests that most cases of a retained surgical item are because of team/system errors . Reason’The presence of a retained surgical sponge is considered to be a ‘never event’ by the National Quality Forum of the United States of America and is also part of patient safety guidelines issued by the Health Department of United Kingdom . Shaming


Liposarcoma is a malignant mesenchymal neoplasm composed of adipose tissue with varying degrees of atypia. These tumors grow slowly and may reach an enormous size particularly if located in the retroperitoneum. We report a 40-year-old male with a 6-month history of gradual abdominal enlargement. Computed tomography (CT) of the abdomen showed a huge retroperitoneal mass with characteristic features consistent with liposarcoma. On laparotomy, the mass was noted to be encasing the right ureter for which a wide excision with en bloc ureterectomy and subsequent renal autotransplantation for organ preservation was done. Post-operative course was uneventful with excellent outcome after 6 months of follow-up. Final histopathologic diagnosis was low-grade, well differentiated liposarcoma, which has favorable prognosis following radical surgery. This was the first report of such a case in the Philippines. Retroperitoneal liposarcoma is a rare tumor and exhibits considerable histological heterogeneity. The Importance of adequate staging and grading in guiding the management approach of such cases cannot be overemphasized. Surgery is the mainstay of treatment and a macroscopically complete resection gives the patient the best chance of prolonged recurrence free survival and over-all survival. We herein report our first case of retroperitoneal liposarcoma for which radical surgery was performed followed by renal autotransplantation to salvage the organ. This case highlights the importance and application of oncologic surgical principles while giving equal importance to surgical technique.A 40-year-old male, with no known comorbidities, came in due to a 6-month history of a gradually enlarging abdomen with associated weight loss and early satiety. He complained of occasional dyspnea especially in a recumbent position and was able to do activities of daily living with some limitations. There were no other associated symptoms noted. On physical examination, there was a huge, movable, firm mass occupying the distended, nontender abdomen.Contrast-enhanced CT scan showed a soft tissue mass at the midline measuring 47 × 34 × 17 cm originating from the right retroperitoneum, predominantly fat with interspersed areas of solid soft tissue component, with note of lateral displacement of the bowel segments and right pelvic ureter ectasia. It shows a clear plane from the aorta while the inferior vena cava is compressed .rd of the ureter  of their cases, consequently, renal sparing resections can be performed % of thei. Since tAdditional technical challenges added interest in this case, as we found 2 veins and 3 arteries. Ligation of one of the renal veins was inconsequential as tributaries communicate within the kidney. Furthermore, the use of the inferior epigastric artery as an ancillary inflow source has been well established in grafts with multiple arteries yielding excellent patency rates. Although it increased our ischemia time, it was not detrimental to the kidney function as evidenced by the nuclear scan.In conclusion, retroperitoneal liposarcomas are locally aggressive tumors. Complete surgical excision with negative margins provides the best chance of attaining disease-free survival, however, in the process of obtaining oncologic margins, equal effort in organ preservation should be employed at times. Such cases may benefit timely referral to high volume centers where multidisciplinary technical expertise is available.


The incidence of cholesteatoma occurring as a result of tympanoplasty is extremely rare. Understanding the cause and preventing its occurrence in the future is the main intention of highlighting this peculiar presentation.A 25-year-old woman presented with progressive hearing loss and blocked sensation in the left ear of one and a half months duration. Past history revealed a history of left myringoplasty six years prior to presentation. Clinical examination of the ear revealed a smooth, soft epithelium covered bulge in the lateral one-third of the floor and posterior wall of the left external auditory canal. HRCT and MRI of the temporal bone confirmed the presence of a soft tissue density in the mastoid. Pure tone audiometry revealed conductive hearing loss. She underwent mastoid exploration, removal of sac with soft wall reconstruction.Proper placement of the vascular strip with the skin lining the external auditory canal with approximation of the incision margins is essential to prevent iatrogenic cholesteatoma formation. Close follow-up is essential to prevent any recurrence and diffusion weighted MRI plays a vital role in detection of recurrence. Cholesteatoma is a sac containing keratinising squamous epithelium presenting in the middle ear and mastoid with a propensity to erode bone and cause complications intracranially and extracranially ,2. The iA 25-year-old woman presented to our out-patient department with progressive hearing loss and blocked sensation in the left ear for of one and a half months duration. There was no history of ear discharge, ear pain, vertigo and tinnitus. There was no history of nose or throat complaints. Past history revealed a history of left myringoplasty six years prior to presentation. Clinical examination of the ear revealed a smooth, soft epithelium covered bulge in the lateral one-third of the floor and posterior wall of the left external auditory canal which was sensitive to touch and did not bleed on touch . ExaminaHRCT of the temporal bone revealed a soft tissue density in the lateral one-third of the external auditory canal eroding the posterior wall and extending into the mastoid bone impinging on the sigmoid sinus plate . MRI with Gadolinium contrast of the temporal bone revealed a well-defined lobulated T2W hyperintense lesion  with parThe patient underwent left mastoid exploration under general anaesthesia. Postauricular incision with exposure of the mastoid cortex showed a well-defined sac with keratin debris in the mastoid which was originating from the bony cartilagenous region of the external auditory canal and eroding the mastoid cortex . The sigmoid sinus plate, mastoid antrum, dural plate and tympanic membrane were intact. The sac was removed in toto and sent for histopathological examination. The defect in the posterior external auditory canal was reconstructed with cartilage, temporalis fascia and periosteum.Histopathological examination showed the presence of keratin flakes and lamellated keratin and eroded fragments of bone along with fibro-collagenous stroma. Keratinized stratified squamous epithelium overlying fibro-collagenous stroma was infiltrated with lymphoplasmacytic infiltrate, cystic macrophages and multinucleated giant cells consistent with features of cholesteatoma .Follow-up after three months showed a well-delineated external auditory canal. Post-operative HRCT temporal bone at three months showed a well-healed cartilage graft surrounded by soft tissue without any evidence of residual disease.Cholesteatoma has been described as a sac of epithelium containing desquamated keratin debris with a propensity to erode surrounding bone . PresencEACC can be of 2 variants: primary and secondary. Primary EACC can be caused by first branchial cleft rudiment which creates a fistulous sinus and epithelium mass remnants in the fistulous sinus. It can also be caused by recurrent microtrauma or microangiopathy produced by cigarette smoking. Secondary EACC is either caused by obstruction or bony defects. Obstruction can be due to exostosis, nevi, stenosis or mycetomas. Bony defects can be post-inflammatory, post-traumatic, postoperative, radiogenic and post tumours. Sweeney et al suggested that vascular strip inversion during tympanoplasty surgery resulted in the formation of cholesteatoma. It can be a result of a shortened vascular strip, overthinning of the mastoid cortex and the influence of pressure negativity within the mastoid cavity ,5. CroniOtorrhea is the most common presentation followed by itching and fullness in the ear. On examination, the presence of keratin debris with a sac in the floor of the external auditory canal is the most common finding whereas, secondary EACC is commonly multifocal in nature. The presence of past history of surgery, repeated cotton-tipped applicator use, smoking and hearing aid use have to be determined to indicate the presence of predisposing factors for EACC. Our patient had a peculiar presentation of decreased hearing of short duration and examination revealed a smooth skin covered bulge in the posterior wall of the external auditory canal with the absence of keratin debris -4. HowevHRCT of the temporal bone is useful in diagnosis. It detects the presence of soft tissue density with bone erosion ,4. MRI wHolt proposed a 3 stage classification based on the extension of the sac from the external auditory canal to the mastoid . Naim etRecurrence following surgical treatment for vascular strip cholesteatoma is extremely rare -3. ThereProper placement of the vascular strip with the skin lining the external auditory canal with approximation of the incision margins is essential to prevent iatrogenic cholesteatoma formation. Close follow-up is essential to prevent any recurrence and diffusion weighted MRI plays a vital role in detection of recurrence.


Schwannoma arises from Schwann's cell of the neural sheath. Schwannoma of the large intestine, particularly of the appendix, is rare. We report a case of appendiceal schwannoma resected using laparoscopic surgery.  A 75-year-old man was referred to our hospital for abdominal fullness and nausea since 2 months. Abdominal CT revealed a well-demarcated oval mass of 25 mm at the tip of the appendix. Contrast-enhanced CT revealed a lesion with gradually enhanced contrast from the arterial phase to the equilibrium phase. Abdominal US revealed a well-demarcated hypoechoic tumor. Preoperative diagnosis indicated appendiceal mesenchymal or neuroendocrine tumor. Ileocecal resection with D3 lymph node dissection was performed. Pathological and immunohistochemical findings confirmed the diagnosis of appendiceal schwannoma.  For determining the surgical procedure of nonepithelial tumor of the appendix, preoperative diagnosis of mesenchymal or neuroendocrine tumors is required. However, appendiceal schwannoma is extremely rare, and its characteristic findings have not yet been established. Accumulating cases of appendiceal schwannomas is necessary for improving imaging diagnosis and surgical treatment. Gastrointestinal mesenchymal tumors are classified into three types by immunohistochemical staining, namely gastrointestinal stromal tumor (GIST), leiomyoma, and schwannoma. Schwannoma arises from the Schwann's cell, which covers the peripheral nerves . GastroiA 75-year-old man was referred to our hospital for abdominal fullness and nausea since 2 months. He had a medical history of hypertension and hyperlipidemia and a surgical history of the right inguinal hernia. The patient's laboratory findings were within normal limits. Abdominal computed tomography (CT) revealed a well-demarcated oval isodensity mass of 25 mm at the tip of his appendix. Contrast-enhanced CT revealed a lesion with gradual homogeneous contrast enhancement from the arterial phase to the equilibrium phase . No abnoPreoperative diagnosis indicated appendiceal neuroendocrine tumor (NET) G1 or gastrointestinal mesenchymal tumors, such as GIST. Malignancy could not be ruled out; therefore, laparoscopic ileocecal resection with D3 lymph node dissection was recommended. Intraoperative findings revealed a well-demarcated tumor at the tip of the appendix, with no invasion into the surrounding tissue. This observation was similar to the preoperative imaging findings. According to another intraoperative finding, dissecting the adhesion between the terminal ileum and the peritoneum, which was the effect of the past herniorrhaphy, was necessary. The operation time was 167 min, and the amount of blood loss was 100 ml.Pathological findings revealed a well-demarcated tumor originating from the muscular layer at the tip of the appendix and spindle-shaped heterotypic cells proliferating in a bundle. Vascular invasion and lymph duct invasion were not detected. No tumor cells were found in the dissected lymph node. Immunohistochemical studies revealed negative values for KIT and CD34 and positive values for S-100 protein , which cVerocay  first deSchwannoma can occur at any location throughout the body along the peripheral nerves , 12. GasGastrointestinal schwannoma assumes the form of a submucosal tumor. The incidence of schwannoma has been reported to be 2%–6% of all submucosal tumors of the intestine , 14. In Regarding appendiceal NET G1, some cases of appendicular goblet cell carcinoid, which exhibit pathological characteristics of both carcinoid and adenocarcinoma, have been recently reported. Goblet cell carcinoma of the appendix requires a treatment approach similar to that required for a colorectal malignant tumor. Some reports of additional resection surgery for lymph node dissection after appendectomy for appendiceal goblet cell carcinoma are available . MarudanGastrointestinal schwannoma and GIST show similar imaging findings ; however18F]Fluorodeoxyglucose (FDG) positron emission tomography (PET) has been used for detecting malignant tumor. However, there are several reports of gastrointestinal schwannoma with increased FDG uptake, among which is a case of appendiceal schwannoma detected by FDG-PET reported by Nishio et al. [[o et al. , 23, 24.In our case, abdominal CT revealed a well-defined oval mass of 25 mm at the tip of the appendix. Contrast-enhanced CT revealed the mass with gradual homogeneous contrast enhancement from the arterial phase to the equilibrium phase. Several 5 mm lymph nodes surrounding the ileocolic artery were identified. Abdominal US revealed a well-demarcated hypoechoic hypervascular mass and some cystic area. The CT findings were similar to the known findings of schwannoma. In the US findings, the partial cyst region was different from that reported in the general findings of schwannoma, suggesting the possibility of it depicting the Antoni B area. In the abdominal US, the wall structure was not maintained, and the evaluation of the localized layer of the wall was difficult. Abdominal US may be useful for the diagnosis rather than EUS if the digestive tract wall structure of the appendix can be depicted. According to Bucher et al. [The standard treatment for schwannoma is complete resection, and several cases of laparoscopic surgery for schwannoma of the large intestine have been reported , 12, 15.In this case, our preoperative diagnosis was appendiceal NET or mesenchymal tumor with a >2 cm mass. According to the JSCO guideline, ileocecal resection or right hemicolectomy with lymph node dissection was recommended. The presence of enlarged lymph nodes also contributed to this recommendation of lymph node dissection. Currently, regarding the diagnosis of the nonepithelial tumor of the appendix, such as mesenchymal tumor and NET, it is necessary to decide a surgical treatment according to both gastrointestinal mesenchymal tumor and NET guidelines.We describe a case of appendiceal schwannoma resected using laparoscopic surgery. There are few reports of gastrointestinal mesenchymal tumors, including appendiceal schwannoma; therefore, characteristic clinical findings of appendiceal schwannoma remain unclear. Thus, accumulating cases of appendiceal schwannoma are warranted for improving the imaging diagnosis and surgical treatment of appendiceal schwannoma.


Intussusception is a relatively common condition seen in children. In comparison, adult intussusception is rare and usually occurs as a complication in patients with organic diseases. It is responsible for 1% of all bowel obstructions, in most of intussusceptions a malignant tumor is involved. Herein, we present an extremely unusual case of intussusception that occurred as a complication at the site of a functional end-to-end anastomosis.A 57-year-old female patient was diagnosed with tumors in the ascending and descending colon and was referred to our department. Laparoscopic hemicolectomy and laparoscopic descending colectomy were performed. The mechanical intestinal obstruction occurred on the 9th day postoperatively, and computed tomography scan revealed intussusception at the site of the ileocolic anastomosis. Endoscopic reduction was attempted, but the procedure was challenging. Surgery was then performed and revealed that the site of ileocolic anastomosis firmly adhered to the side wall and right retroperitoneum. However, the intestine in the oral side of the anastomosis was not fixed. Examination of the anastomotic site revealed that the ileum had passed through the anastomosis and prolapsed into the transverse colon. The ileocolic anastomosis was resected. End-to-end anastomosis was performed, and surgery was then completed. No neoplastic lesions were observed in the resected tissue of the lead point of intussusception. The postoperative clinical course was favorable, and the patient was discharged on the 11th day after the second round of surgery.There are no reports the anastomosis is involved as part of the intussception, as observed in the present case. Intussusception should thus be considered as one of the causes of postoperative mechanical intestinal obstruction. Intussusception in adults is rare, and usually occurs as a complication in patients with organic diseases. We report a case of intussusception that occurred as a complication at the site of a functional end-to-end anastomosis.A 57-year-old female patient presented with a chief complaint of abnormal findings upon medical examination. She had no significant lifestyle/family history or medical history. Regarding her history of present illness, the patient was referred to our department after undergoing a computed tomography (CT) scan in September 2017, which revealed a tumor in the descending colon.4/μl; carcinoembryonic antigen level, 3.3 ng/mL; and CA 19–9 level, 11.1 U/mL. The results were not significant.Hematological analysis revealed the following results: White blood cell count, 7900/μl; hemoglobin level, 12.6 g/dL; platelet count, 28.4 × 10Lower gastrointestinal endoscopy findings revealed a 25-mm type 0-Is lesion observed in the ascending colon and a 30-mm type Is lesion in the descending colon. In the abdominal contrast-enhanced CT findings, the tumors could not be located, and lymph node, lung, or liver metastases were not observed. Regarding histopathological findings, biopsy results revealed that the tumor in the ascending colon was a high-grade adenoma and the tumor in the descending colon was a moderately differentiated adenocarcinoma. Endoscopic tumor resection was not possible due to the difficulty in maneuvering the endoscope in the ascending and descending colon. Thus, surgery was considered.In the first round of surgery, surgery was initiated with five ports. No ascites, peritoneal dissemination, or liver metastases were observed. The ascending colon polyp was adenoma, but there was a polyp near the ileocecal valve. Therefore, it was difficult to resect the ascending colon and we chosed the right hemicolectomy. The descending colon polyp was adenocarcinoma on biopsy. However, we diagnosed intramucosal cancer and performed descending colon resection. Laparoscopic resection of the descending colon and right hemicolectomy was performed according to standard procedures. There were two functional end-to-end anastomoses. Regarding the clinical course after the first round of surgery, mechanical intestinal obstruction occurred on the 9th day postoperatively, and CT scan showed that intussusception occurred from the functional end-to-end anastomosis Figs.  and 2. AIn the second round of surgery, when midline laparotomy was performed, the site of the ileocolic anastomosis was found to be firmly adhered to the side wall and right retroperitoneum. Because the staple used for anastomosis may have adhered to the peritoneum. The intestines in the proximal side of the anastomosis were not fixed. Examination of the anastomosis revealed that the ileum had passed through the anastomosis and entered the transverse colon Fig. . AlthougIntussusception in adults is rare . Over 90Intussusception caused by functional end-to-end anastomosis is extremely rare. We searched the PubMed database for articles published between 1970 and December 2017 using “intussusception,” “anastomosis,” and “right hemicolectomy” as keywords. However, we did not find any relevant articles. “Intussusception” and “functional end-to-end Anastomosis” did not hit any human case reports.The chief complaints only few symptoms that characterize the condition are observed, and the clinical course often varies . DiagnosIntussusception is first treated with a barium enema or endoscopic reduction , 10. In Currently, idiopathic intussusception is believed to occur when a stimulus causes the circular muscle of the intestine to contract convulsively and enter the relaxed intestine that is contiguous to it in the anal side . Elechi As a surgery to prevent recurrence, there are methods to prevent invasion of the ileum into the colon by fixing the intestine to the abdominal wall, and to suppress recurrence by promoting postoperative adhesion –15. We dIntussusceptions in adults are often caused by tumors, and they are rarely caused by other factors. To the best of our knowledge, no studies have reported cases of anastomosis as a cause of intussusception, as reported in the present case. Our case indicated that intussusception should be considered as one of the causes of postoperative mechanical intestinal obstruction.


Wilms tumor is the most frequent pediatric renal malignancy, and its usual presentation is an abdominal mass or hematuria. Unusual presentations have also been reported, such as paraneoplastic syndromes . These conditions can precede, occur concomitantly, or present in a later phase of tumor development. Precocious puberty, as paraneoplastic endocrine syndrome, has already been described in children with malignant tumors . However, little is known about central precocious puberty, as paraneoplastic manifestation of nephroblastoma or secondary to its specific chemotherapy. Here, we report a case of Wilms tumor and simultaneous precocious puberty in a 5-year-old girl. The initial diagnosis was premature telarche, but the clinical and biological pubertal progression changed our diagnosis to idiopathic central precocious puberty. Chemotherapy and nephrectomy were well tolerated, and we began treatment with a gonadotropin-releasing hormone agonist which showed favorable outcomes over the short term. We highlight the need for early diagnosis and work-up in all patients of precocious puberty, in order to institute timely management. Premature telarche (PT), a benign condition in girls younger than 8 years of age, defines an isolated breast development, without other signs of puberty. The diagnosis is usually made clinically and does not require biochemical or radiological tests. The most consistent aspect of PT management requires serial monitoring of the patient to ensure the continued absence of other sexual characteristics. The spontaneous regression of breast enlargement is usually the main evolution rule. After this self-limited event, puberty usually occurs and progresses physiologically.However, it is possible that PT progresses to precocious puberty (PP), with pubertal initiation before the age of 8 years in girls and 9 years in boys. The incidence of PP was recently estimated to be 0.2% in girls and 0.05% in boys . ApproxiPP is one of the most common endocrine-related manifestations of neoplasia in pediatric patients. Other systemic or endocrine manifestations, globally referred to as paraneoplastic syndrome, include hypercalcemia, inappropriate antidiuretic hormone secretion, ectopic adrenocorticotrophic hormone secretion, and hypoglycemia. Endocrine abnormalities can also appear subsequently, after the neoplasia has been treated, associated with drugs or radiation.Herein, we present the case of a child with Wilms tumor and concomitant PT who progressed to central PP (CPP). In addition, we provide a review of the literature relating to neoplasia-associated puberty and paraneoplastic manifestations of renal tumors.A previously healthy 5-year-old girl of African origin presented to the Emergency Department (ED) with macroscopic hematuria appeared 24 hours before presentation and isolated abdominal pain for one month previously. There was no history of fever, vomiting, urinary or systemic symptoms, weight loss, or abdominal trauma.The patient had no significant medical history, in particular, head injuries such as trauma, hemorrhage, or infection, and did not take any medications. Family history was unremarkable, with no antecedents of neoplasm or precocious puberty. Her mother was not taking any contraceptive hormonal medications, and there was no history of any other source of exposure to hormonal drugs.th percentile); height 117 cm (50–75th percentile). Her vital signs were normal, except for blood pressure at 130/90 mm Hg (>99th percentile for sex and height). She had neither dysmorphic features nor skin lesions . On abdominal examination, we palpated a mass, measuring 6 cm with regularly margins in the right upper quadrant. There was no central nervous system dysfunction . She had bilateral, symmetrical nontender enlargement of breast buds corresponding to stage 2 of the Tanner classification. There was no pubic or axillary hair, and her thyroid was normal on clinical inspection.On physical examination, the child appeared well. Her anthropometric parameters were as follows: weight 21 kg , a high suspicion of nephroblastoma was made. Histological examination confirmed the diagnosis of intermediary-risk nephroblastoma stage I.The first endocrine work-up revealed a normal thyroid function and prepubertal gonadotropin levels see . UltrasoThe International Society of Pediatric Oncology (SIOP) 2001 chemotherapy protocol (dactinomycin/vincristine) was commenced, with complete excision of the tumor performed 4 weeks later. Additional chemotherapy cycles were provided after surgery, for a total duration of 3 months. The clinical tolerance and response were excellent, without any side effects or signs of residual tumor.A second endocrine assessment was performed at the end of chemotherapy. Surprisingly, we identified an accelerated linear growth (a gain of 3 cm in 4 months) in the context of rapid breast development (passage from stage 2 to stage 3 over 4 months) with no axillary or pubic hair. The second hormonal work-up found an activated pituitary-gonadal axis  along wiMagnetic resonance imaging (MRI) of the brain showed a morphologically normal pituitary, but of pubertal size (pituitary height of 6 mm and marked convexity of the upper surface) and no congenital or acquired lesions in the pineal or hypothalamic-optic region . Human cThe definitive diagnosis of idiopathic CPP was made, and we began treatment with GnRHa . Six months later, we noted a reassuring clinical evolution, with partial regression of breast development, reduction in growth velocity (2 cm/6 months), and increasingly random LH level at 3.4 IU/L. Recently, a prospective study [The global evolution of our patient, from oncological diagnosis up to 6 months after the beginning of CPP treatment, is depicted in At the time of oncological diagnosis, our patient presented with classical PT. Her subsequent evolution, with accelerated height velocity (3 cm/4 months) in the context of somatic stress related to nephroblastoma treatment (nephrectomy and chemotherapy), was the first finding signaling the evolution to PP.PT cases progressing to PP are described in the literature, with a prevalence of 9 to 14% , 6. NeitThe diagnosis of PP requires a clinical, biological, and radiological evaluation. A detailed personal and family history and a complete physical examination including the assessment of linear growth and secondary sexual features  and their classification on the basis of the Marshall and Tanner are necessary information. Bone age  and pelvic ultrasound (uterine and ovarian dimensions) are well correlated. The diagnostic value of basal LH is variable, and in this circumstance, the gold standard of the biochemical diagnosis is based on the assessment of gonadotropins, mainly LH, after stimulation with GnRHa. A peak stimulated LH of >5 IU/L along with an LH/FSH ratio of ≥2 are consistent with the diagnosis of CPP . HoweverWilms tumor is known to be associated with paraneoplastic systemic findings  and endocrine manifestations including hypercalcemia, Cushing syndrome, and hypoglycemia –12.Given the troublesome presentation of our case, we reviewed the literature on neoplasm-related PP and effects of cancer therapy upon pubertal activation. Brain, gonadal, adrenal tumors, and hepatoblastoma are the most frequent tumors that may be associated with PP .α-subunit is identical to that of LH, induce precocious puberty due to cross reaction between hCG and the LH receptor. The clinical profile of this condition results in pubertal changes to the external genitalia, but with less testicular enlargement (secondary to the lack of Sertoli cell stimulation from FSH). The biological profile includes low levels of LH/FSH and high levels of testosterone due to the stimulatory effect of hCG on the LH receptor in the testes. Final diagnosis is made by measuring serum and cerebrospinal fluid hCG levels. Pediatric germ-cell tumors that induce this type of gonadotropin-dependent precocious puberty can also be mediastinal (particularly in Klinefelter syndrome) [CNS tumors can disrupt hypothalamic and pituitary function, with secondary CPP. The most common tumors are hypothalamic , chiasmatic (gliomas), and pineal  . Ependymyndrome) , 16, hepIn females, hCG-secreting tumors can induce PP by several possible mechanisms, including the cosecretion of hCG and estradiol, low FSH activity of the very high hCG, and high tumoral aromatase activity for some cranial dysgerminomas –19.The risk of organic pathology, particularly intracranial, is much higher in boys than in girls . Age seeCranial radiation therapy (CRT) for cerebral tumors, with high radiation doses, has direct effects on GnRH with clinical and biological CPP. CPP is among the most common complications that can occur following CNS tumors and/or CRT affecting the hypothalamus/pituitary . The risOther types of tumors that are able to induce PP include hepatoblastomas and gonadal neoplasia. A hepatoblastoma is a hepatic type of tumor which can produce hCG. This form of tumor is not uncommonly associated with PP in males (testicular stimulation by hCG). Some tumor cells are also able to secrete testosterone . GonadalAn interesting point to consider is whether the CPP in our case represented a coincidental finding. We performed a full review of the published literature indexed in PubMed up to January 2019 regarding Wilms tumor and puberty. Search terms included “Wilms tumor,” “paraneoplastic syndrome,” and “precocious puberty.” Our search failed to identify existing reports relating to the coexistence of Wilms tumor and CPP.It is possible that the pathogenesis of CPP could be related to the concomitant effect of chemotherapy along with functional alterations in the HPG axis, but none of the existing literature described similar circumstances. Chemotherapy could cause some anterior pituitary dysfunction in the survivors of childhood cancer, with hormonal deficiencies but not activation of the hypothalamic/pituitary axis. Alkylating agents, such as cyclophosphamide, lomustine, and procarbazine, are known to cause premature ovarian insufficiency and can indirectly affect pituitary function, but this adaptive pituitary response is not considered a disease .Collating data from the literature review relating to the proposed pathophysiological mechanisms underlying paraneoplastic syndrome and neoplasia-induced pubertal activation and considering this alongside our patient's clinical evolution, we finally established that the association between Wilms tumor and CPP was purely coincidental.We report a case of PT with rapid progression to CPP in a 5-year-old girl with a concomitant Wilms tumor. Our observations may shed light on the necessity to diagnose PP early in every child irrespective of the presenting illness. A complete clinical examination and prompt referral to a pediatric endocrinologist for specific work-up and treatment are helpful in limiting early and late long-term health implications .We therefore recommend that when faced with PP in a child with a concomitant tumor, the diagnosis of paraneoplastic or neoplasia-associated PP should be discussed.


Chronically discharging ear is a common cause of morbidity in developing countries, and it is also associated with intratemporal and intracranial complications. The surgeon is often able to detect the disease. However, cholesteatoma in the “hidden areas” like anterior epitympanic recess and sinus tympani can be missed. Facial nerve involvement and cholesteatomatous erosion of the bony labyrinth are dreaded complications, the extent of which cannot be assessed completely on clinical examination. Adding to the complexity are the various variations in anatomy like high riding jugular bulb and aberrant internal carotid artery which could lead to catastrophic complications during surgery if left undetected preoperatively. HRCT temporal bone is useful to detect the extent of the disease, various complications, and guide the surgeon for pre-operative planning. In this review, we go through the various HRCT imaging features of acquired cholesteatoma, a reporting template, and a few words about imaging of the post-operative ear. Acquired cholesteatoma is commonly associated with intratemporal and intracranial complications.Various “hidden areas” in the middle ear that are not amenable to clinical examination can be easily seen with HRCT.The extent of disease and complications can be assessed.Variant anatomy can be looked for to avoid intra-operative complications.Cholesteatoma is a condition prevalent around the world, predominantly in developing countries. Acquired cholesteatoma is a common complication of unsafe/atticoantral chronic otitis media. Literally, it means the presence of “skin in the wrong place.” The cholesteatomatous sac is lined by stratified squamous epithelium constituting the matrix, which secretes the acellular keratin debris within . An outeCholesteatoma can be classified as congenital or acquired. Another system of categorization could be according to the location of the cholesteatoma, which could be in the external auditory canal or middle ear cavity and other pneumatised parts of the temporal bone. HRCT temporal bone is currently advocated in all cases suspected of cholesteatoma clinically prior to surgery for assessment of the extent of disease, complications, and presence of variant anatomy.Various theories exist regarding the formation of cholesteatoma; however, none of them explain it completely.Being the most widely accepted theory, it states that the weakest part of the tympanic membrane (TM), i.e., pars flaccida, retracts because of the negative pressure in the middle ear cavity caused by long-standing eustachian tube dysfunction. This leads to the formation of posterosuperior attic pockets. The self-cleansing mechanism of these pockets is gradually lost with the accumulation of desquamation keratinized epithelial layer of the TM and results in the formation of keratin debris. Repeated superadded infection occurs and later biofilms are also formed over these debris which make them resistant to any antimicrobial therapy .It is also hypothesized that there is an invasion of the keratinized squamous epithelium into the middle ear cavity through small perforations in pars tensa, which forms cholesteatoma.Due to the chronic irritation, the keratinized squamous epithelium is formed from the squamous or cuboidal epithelium of the middle ear.There is a presence of microscopic defects even in the intact TM, through which epithelial cells can invade. These defects serve as direct contact between connective tissue of the mucoperiosteum with a middle ear effusion. In later stages, these areas become totally or partially covered with epithelium, and these epithelial breaks become connected to each other through the organized effusion. The cholesteatoma seems to spread by using the connective tissue as scaffolding .Thus, acquired cholesteatomas can be classified based on the underlying pathogenesis as either “primary” or “secondary” where primary variety is seen behind pars flaccida of an intact retracted TM and the secondary variety grows into the middle ear through a perforated pars tensa part of TM .The etiology of bony destruction by cholesteatomas is also debatable. Destruction only by mechanical pressure is unlikely. One of the theories states that production of degrading enzymes by osteoclasts is the cause of bony destruction , 7. Due Clinically, the lesion presents with foul-smelling otorrhea, earache, and hearing loss. On otoscopic examination, it classically appears as a cluster of pearly white structures in the attic region. It is also suspected beneath polyps protruding from the pars flaccida or when there is a marginal TM perforation or granulation tissue .Erosion of facial nerve canal can lead to features of lower motor neuron palsy manifesting as an inability to close ipsilateral eye, drooping of the corner of the mouth, and drooling of saliva. A large proportion of patients with facial canal erosion may not have features of facial nerve paresis. A labyrinthine fistula will lead to vertigo and nystagmus; however, these symptoms may be present in the absence of a fistula, often due to the diffusion of toxic substances into the intact labyrinthine. Intracranial complications can be life-threatening, patients presenting with fever, headache, and nuchal rigidity due to meningitis. The most common etiology of the cerebellar abscess is otogenic and patients present with fever and ataxia and other balance abnormalities.Non-contrast axial scans parallel to the axis of and through the petrous temporal bone are acquired on a multidetector CT scanner. Axial scanning is done with helical technique , section thickness 0.6–1 mm, FOV 15–20 cm) in bone algorithm. Head is kept in neutral position and true axial images parallel to Reid’s line (joining inferior margin of the orbit to the center of the orifice of EAC) are acquired. Coronal multiplanar reconstruction is made perpendicular to the axial scans.Congenital cholesteatoma of the temporal bone is formed due to lack of involution of distinct squamous cell rest, which should have involuted to become normal endothelium [Cholesteatoma can be congenital or acquired. othelium . Middle othelium , 10. A sothelium .acquired variety. The acquired variety can be further classified according to the location of the cholesteatoma in the external or the middle ear.The other type of cholesteatoma is the EAC cholesteatoma is uncommon and usually occurs in an age group older than middle ear cholesteatoma. It appears as a soft tissue mass in the canal particularly along its floor with associated bony destruction and periostitis. It occurs due to the invasion of the squamous epithelium lining the EAC into the underlying bone. However, it is difficult to distinguish between cholesteatoma, carcinoma, and malignant otitis externa using HRCT alone [CT alone , 11.middle ear cholesteatoma, the most common type is pars flaccida variety and is seen characteristically in the Prussack’s space, a niche in the epitympanum lateral to the ossicles. These cause medial displacement of the malleus and erosion of the bony scutum, extending into the aditus and antrum posteriorly Pars tension Fig. . Anothero injury , 15 , squamous cell carcinoma, metastasis, and GCT (giant cell tumor) [The differential diagnosis of l tumor) . The prel tumor) .without bone destruction, like granulation tissue, cholesterol granuloma, post-inflammatory ossicular fixation, mucosal edema, fibrosis, scar tissue, and fluid. They are also frequently encountered in the post-operative period. MR imaging can usually aid in making the correct diagnosis as cholesteatomas are hypointense on T1WI, hyperintense on T2WI, do not show any enhancement, and demonstrate diffusion restriction on DWI. Cholesterol granulomas on the other hand show hyperintense signal on T1WI due to the presence of intrinsic blood products [HRCT is unreliable in differentiating residual or recurrent cholesteatoma from other entities which usually occur products .Dehiscent facial canalHigh and dehiscent jugular bulbAnteriorly lying sigmoid sinusLow lying tegmen and dura Specific patterns of pneumatization and aeration of mastoid .In addition to the evaluation of complications of cholesteatoma, imaging also helps in warning the surgeon of any variant anatomy that may predispose to life-threatening consequences during surgery or may affect surgical access to disease such as the following:Imaging is of particular importance in patients who have undergone canal wall up procedures where rates of recurrence range from 18–36%, and detection of residual or recurrent disease by otoscopic means is particularly tough .In the postoperative period, HRCT has a high negative predictive value in cases with no evidence of soft tissue densities. However, bony erosion in patients who have previously undergone a tympanomastoidectomy cannot be characterized, as it is impossible to differentiate between surgical changes and pathological bony destruction due to cholesteatoma. In this setting, HRCT has a sensitivity of 43%, specificity of 42–51%, and a predictive value of 28% for detection of residual or recurrent cholesteatoma. HRCT also plays a major role prior to revision surgery, as anatomy may be considerably altered, limiting the utility of normal surgical landmarks .If an HRCT shows no abnormal soft tissue at 6 or 9 months following the initial surgical procedure, the surgeon may be more confident in delaying a second-look surgery .HRCT temporal bone has certain limitations. It is difficult to differentiate cholesteatoma from granulation tissue, pus, or fluid. Early disease with the absence of osseous erosion is challenging to pick. The integrity of the facial canal cannot be accurately commented upon, especially in the presence of adjacent soft tissue in the middle ear. Due to the acquisition of the non-contrast scan, intracranial complications are frequently overlooked.Location of mass—epitympanum/mesotympanum/hypotympanumHidden areas—sinus tympani, facial recess, anterior epitympanum recessOssiclesOsseous walls of the middle earMastoid air cells, aditusTegmen tympani, sinodural plate, scutumFacial canalExternal auditory canal and tympanic membraneInner ear esp. lateral semi-circular canalPetrous apex, intracranial abnormality, post-auricular soft tissueVariant anatomyHRCT temporal bone offers an insight into the extent of cholesteatoma and the various complications before the surgeon embarks onto the definitive line of management. It also helps detects recurrent/residual middle ear disease to avoid unnecessary second-look surgery.


Cecal leiomyomas are rare benign tumors of smooth muscle arising from the colonic muscularis mucosa or muscularis propria. They are usually asymptomatic in nature and, if symptomatic, may present as pain in the abdomen, intestinal obstruction, or bleeding. In some cases, leiomyoma can cause free perforation leading to peritonitis. Contrast-enhanced computed tomography (CECT) and colonoscopy were the diagnostic modalities used for evaluation. It is extremely unusual for a benign lesion of the cecum to present as a ruptured subcutaneous abscess. A 40-year-old man presented to the surgical emergency with complaints of right loin swelling and dull aching pain for one week. The patient did not have any significant medical history. Examination revealed a 5×5 cm swelling in the right anterior lumbar region. Blood investigations revealed anemia with leukocytosis. An abdominal CECT revealed a 9×6 cm heterogeneous enhancing mass lesion arising from the cecum with hypodense areas abutting the anterior abdominal wall and tracking into the intermuscular plane. The patient underwent surgical exploration, and a 9×6 cm growth arising from the cecum with a localized abscess tracking into the intermuscular plane in the right anterior abdominal wall and forming a subcutaneous abscess was intraoperatively found. A right hemicolectomy with ileocolic anastomosis was done, with external drainage of the subcutaneous abscess. Histopathological examination of the resected specimen revealed a leiomyoma of the cecum with abscess. To the best of our knowledge, this is the first report of a case of cecal leiomyoma to rupture into the subcutaneous space and present as a flank abscess. The female genital tract and skin are the most common sites for a primary leiomyoma. The colon is an uncommon site for leiomyoma formation and constitutes approximately 3% of gastrointestinal leiomyomas and 1% of all gastrointestinal neoplasms . The tumA 40-year-old male presented to the surgical emergency with complaints of swelling in the right loin and a dull aching pain that had both been present for a week. He had no complaints of altered bowel habit. There was no history of evening rise in temperature, weight loss, or loss of appetite. The patient did not have any significant medical history. On examination, a swelling (measuring 5×5 cm) was found in the right anterior lumbar region that became less prominent on the leg-raising test. The rest of the abdominal examination showed normal findings. A hematological evaluation revealed features suggestive of anemia with leukocytosis, and all other routine investigations were normal. A contrast-enhanced computed tomography (CECT) scan of the abdomen revealed a 9×6 cm heterogeneous enhancing mass lesion arising from the cecum with hypodense areas abutting the anterior abdominal wall and tracking into the intermuscular plane Figure .Thus, the patient was taken up for an emergency exploratory laparotomy. During operative exploration, we found a 9×6 cm growth arising from the cecum with a localized intraperitoneal abscess that was tracking into the intermuscular plane in the right anterior abdominal wall and forming a subcutaneous abscess. A right hemicolectomy with an end-to-end ileocolic anastomosis was conducted along with external drainage of the subcutaneous abscess. Postoperatively, the patient recovered well and resumed normal diet.A histopathological specimen examination revealed spindle cells arranged in fascicles with moderate eosinophilic cytoplasm and elongated nuclei with blunt ends suggestive of leiomyoma. There was acute on chronic inflammatory cells suggestive of an abscess Figure .The final diagnosis was leiomyoma of the cecum with abscess formation.Leiomyoma is a benign tumor that arises from smooth muscle. In 1854, Virchow first reported a leiomyoma . The tumLeiomyoma usually presents as a single tumor and may be intraluminal, intramural, or extraluminal. In some instances, there may be multiple tumors, which constitutes part of the leiomyomatosis syndrome. Leiomyomas are mostly asymptomatic and can be found incidentally during a routine colonoscopy. If symptomatic, most commonly they present with dull aching pain or mass per abdomen. Infrequently, leiomyomas may manifest with complications such as perforation leading to peritonitis, ulceration causing intestinal bleeding, and/or intussusception leading to obstruction, which usually warrants emergency surgical management.Colonoscopy can be undertaken to visualize the colonic lumen and, further, aid tissue diagnosis. On colonoscopy, the leiomyoma can appear as intraluminal or intramural polyps and may resemble an adenoma. Ultrasonography can be conducted as the initial investigation, although imaging studies are inconclusive in diagnosing leiomyomas. On CECT, a heterogeneous mass lesion with areas of calcification and focal necrosis may be seen. Therefore, accurate diagnosis requires a combination of endoscopy and imaging modalities, such as CECT, magnetic resonance imaging, or endoscopic ultrasonography .Histologically, leiomyomas arise from the muscularis propria. The presence of necrosis, increased cellularity, increased nuclear cytoplasmic ratio, and number of mitotic figures is suggestive of malignant change. Immunohistochemistry and electron microscopy are useful to differentiate leiomyomas from gastrointestinal stromal tumor (GIST). Leiomyomas usually stain positive for smooth muscle actin or desmin, but stain negative for c-kit, S100, and CD34 .The management of leiomyoma lesions is based on size, site of occurrence, and clinical presentation. Usually, resection of the lesion with wide margins is the treatment of choice for most of the leiomyomas due to difficulty in differentiating benign from malignant tumors . EndoscoCecal leiomyomas are unusual benign tumors occurring in the elderly population. It has a variable presentation, ranging from asymptomatic to clinically significant conditions. Ultrasonography may play a role in the initial investigation. Colonoscopy and CECT are essential for arriving at a definitive diagnosis. In patients presenting with a flank abscess with intra-abdominal extension, cecal leiomyoma should be considered in the differential diagnosis. Leiomyomas should be differentiated from common lesions, such as Crohn’s disease, colonic diverticulitis, abdominopelvic tuberculosis, leiomyosarcoma of the cecum, and malignant adenocarcinoma cecum. Prompt diagnosis is necessary for early treatment and to avoid the associated morbidity. Surgery is the mainstay of the treatment for a symptomatic leiomyoma.


Mesenchymal chondrosarcoma is a rare and aggressive chondrogenic neoplasm arising from the bone or the soft tissue. Mesenchymal chondrosarcomas develop outside the osseous structures in about one-third of cases, and the majority of these occur in the meninges and the brain parenchyma. Intramuscular extraskeletal mesenchymal chondrosarcoma (EMC) is exceedingly rare, with very few cases reported in the literature. Although mesenchymal chondrosarcoma has a high potential for metastasis, there have been no reports of pulmonary metastasis from an EMC of intramuscular origin. Here, we describe a patient who came to our facility with a history of progressively worsening left lower extremity pain and swelling, and was found to have pathology-proven EMC originating in the left adductor magnus, with complete workup demonstrating multiple bilateral pulmonary metastases in addition to a possible metastatic focus in the right adrenal gland discovered during the interval surveillance period. In the largest clinicopathological study of EMCs, which included 111 cases, it was found that this tumour is more aggressive than other types of chondrosarcomas, with a high incidence of distant metastasis.4First described in 1959 by Lichtenstein and Bernstein,3 Very few EMC cases with primary intramuscular origins have been documented.3When compared with conventional chondrosarcoma, EMC differs in both sex predominance and the site of origin. Skeletal and extraskeletal conventional chondrosarcomas show a male predilection and typically arise in the pelvis and femur. There is a slight female preponderance in EMC, and a significant proportion of these develop in the central nervous system, meninges, maxillary sinuses, thyroid and soft tissues of the face.2 EMC has a biphasic histological appearance that comprises sheets of undifferentiated mesenchymal cells and islands of well-differentiated cartilage. The poorly differentiated mesenchymal portions include sheets of tightly packed small cells that vary in morphology from round or elliptical to short and spindular. The nuclei of mesenchymal tissue are hyperchromatic and the cytoplasm is scant and indistinct. Immature cartilaginous islands are often situated at the periphery of the small cells and represent a zone of transition from the undifferentiated mesenchymal tissue to the more distinct hyaline cartilage. Larger, more mature cartilaginous islands seen in histological samples of mesenchymal chondrosarcoma may appear calcified or ossified.5While the exact aetiology of EMC is not clear, cytogenetic analyses have elucidated that its tumorigenicity may be attributed to an identical Robertsonian translocation involving chromosomes 13 and 21 [der ]. There have also been studies that suggest similarity of EMC pathogenesis to Ewing’s sarcoma, including a reciprocal translocation  and trisomy 8. Furthermore, one case of mesenchymal chondrosarcoma demonstrated extra unidentifiable material on chromosome 22 with a breakpoint at 22q13, which is close to the breakpoint found in Ewing’s sarcoma at 22q12.2 The S100 protein, which is a commonly used marker for differentiating chondrogenic neoplastic tissue, is only positive within the cartilaginous islands in mesenchymal chondrosarcoma tissue samples. There is also partial positivity for vimentin, an intermediate filament protein expressed in mesenchymal cells, within the sheets of poorly differentiated small cells of mesenchymal chondrosarcoma.3 However, Ewing’s sarcoma, another entity in the differential diagnosis for mesenchymal chondrosarcoma, demonstrates CD99 expression, which is confounded by CD99 expression in mesenchymal chondrosarcoma as described by Müller et al.7With regard to histopathological diagnosis of EMC, immunohistochemical analysis provides assistance in narrowing the differential diagnosis. Mesenchymal chondrosarcoma stains positively for S100 protein and is negative for the presence of cytokeratins (CKs), which are found in synovial sarcoma and spindle cell variants.5 CT scans of patients with EMC revealed a variety of calcification patterns among eight patients. One of the most valuable imaging findings related to EMC in this cohort was a ring-and-arc pattern of mineralization. In their study, patients with peripherally distributed EMC exhibited a wide and dense calcification, while there was only one case (one-third) of centrally distributed EMC that had focal calcification. Because the cohort was too small to analyze for any sensitivity and specificity of this imaging finding with any reasonable power, the diagnostic worth of calcification patterns in EMC requires further substantiation.Plain radiograph, while important as an initial step in the radiological evaluation of suspected EMC, often has limitations secondary to potential overlapping of the adjacent structures and lower density resolution. CT images of EMC eliminates the uncertainty as to whether the structures are overlapping or if there is contiguity with the bone, which would posit the more probable existence of conventional chondrosarcoma. In a study by Chen et al,T1 and heterogeneously hyper- and hypo-intense signals on T2 weighted images. Intratumoral mineralized and non-calcified components of peripheral EMC exhibit low and high signal intensity on T2 weighted images, respectively. This finding of hyperintensities surrounding areas of hypointense signals is known as the “black pepper” sign and is considered to be one of the most important imaging characteristics of the disease. Diffuse heterogeneous or nodular enhancement can be found in both mineralized and non-mineralized areas. This is suggestive of the increased vascularity within the EMC tissue and is also an important diagnostic finding.5MRI of EMC yields a more extensive evaluation of soft tissue involvement. EMC has been demonstrated as isointense and hypointense signal on 2Owing to the rarity and the variable nature of the disease, treatment of mesenchymal chondrosarcoma is incompletely studied and EMC even less so. Surgery is the primary form of local therapy, with patients undergoing wide resection of the lesion demonstrating better survival. Currently, the National Comprehensive Cancer Network guidelines recommend that mesenchymal chondrosarcoma be treated with vincristine, doxorubicin and cyclophosphamide alternating with ifosfamide and etoposide. This is a treatment regimen used typically in cases of Ewing’s sarcoma. The efficacy of adjunctive radiation therapy is poorly defined, with purely anecdotal reports of its value.2 It has been reported that some patients die shortly after diagnosis, while others may live for long periods even in the presence of metastatic disease. Decreased survival has been associated with haemangiopericytomatous features, but prognostic indicators are again largely conjectural, as no single adjuvant treatment protocol has been adopted.2Prognosis of all cases of mesenchymal chondrosarcoma is widely variable, with 10-year overall survival rates ranging from 21% to 67%.T1 weighted images and moderately hyperintense to muscle on T2 weighted images. Following intravenous administration of gadolinium, moderate enhancement of the mass, excluding the central portion, was observed. Superiorly and inferiorly, the margins of the mass were indefinite, and the mass demonstrated peripheral vasculature, which was most prominent at the cephalad and caudad margins. The mass was centred within the adductor magnus muscle fibres that were displaced around the mass. Anterolaterally, the mass was very closely approximated to the posterior cortex of the femur and the fascial margins that separated the quadriceps and the posterior compartments (Supplementary videos). No obvious signal abnormality was noted within the femur to indicate invasion or primary osseous origin. Post-excision pathology confirmed the presence of mesenchymal chondrosarcoma.A 32-year-old male with no significant past medical history presented to our facility with a history of worsening pain and swelling in the medial aspect of his proximal left lower extremity over the past several years. Anteroposterior (AP) and “frog leg” lateral radiographs of the left lower extremity revealed a dense lesion consistent with dense matrix, adjacent to the medial aspect of the femur . An MRI In the interval, surveillance imaging for metastatic disease with CT scan of the chest, abdomen and pelvis was performed. At 18 months, multiple bilateral non-calcified pulmonary nodules concerning for metastasis were identified . The mosT2 weighted images in combination suggest the diagnosis, although all of these features need not be present, these features were present in the case at hand.This case of EMC highlights a rare tumour arising solely within the adductor magnus muscle. To date, there have been no documented cases of intramuscular EMC with metastatic foci within the lungs or adrenals. Given the low incidence of this malignancy, the literature is rather limited with regard to diagnostic approach, treatment and prognosis. Several radiological features have redemonstrated support for the diagnosis of EMC, although there are many shortcomings inherent to these diagnostic modalities. The densely calcified masses on plain radiograph with ring-and-arc calcification on CT scan and the “salt and pepper” sign on The road to a more definitive diagnosis is paved with wide excision of the mass as the initiation of therapy. The excised tumour may be assessed with a series of histopathological markers. EMC has shown S100, vimentin and CD99 positivity, along with the absence of CKs, but even these markers are not completely sensitive and may confound the differential diagnosis. Cytogenetic studies have begun to show a link with Ewing’s sarcoma, and genetic testing will likely become the diagnostic and therapy-guiding modality of choice. With rapid diagnosis, the uncertain natural course of the tumour may be altered by initiating therapy that could possibly prevent haematogenous spread as seen in our patient. Furthermore, more efficient diagnosis of this entity through radiological, histopathological and cytogenetic studies may extend survival among EMC patients, allowing for more tailored therapies to be developed.EMCs are rare, malignant soft-tissue sarcomas that typically arise in young adults.EMC has a predilection to arise in the meninges and spinal dura, but can also be found in the lower extremity.On CT scan, EMC appears as a soft-tissue mass, isodense to muscle with heterogenous enhancement. Calcifications in a “ring-and-arc” configuration may be seen.T1 weighted imaging. On T2 weighted imaging, heterogenous hyperintense and hypointense signal may be observed, reflecting intratumoral mineralized foci. This is known as the “salt and pepper” or “black pepper” sign.MR evaluation reveals isointensity to muscle on The treatment of EMC includes wide local excision and chemotherapeutic agents used in the treatment of Ewing’s sarcoma.The prognosis of EMC is variable, with 10-year survival ranging from 21% to 67%.


Despite the partial attachment of a complex odontoma to an impacted tooth, it is possible to guide the tooth to erupt normally and preserve it by step lesion excavation and the prevention of infection. Despite the partial attachment of a complex odontoma to an impacted tooth, it is possible to guide the tooth to erupt normally and preserve it by step lesion excavation and the prevention of infection. An impacted tooth that is partially attached to a complex odontoma without the presence of inflammation can be erupted normally by removing the lesion and preventing the infection.Odontomas often disturb the eruption of the adjacent teeth and are classified into two types: complex and compound odontomas.2A male patient aged 6 years and 8 months was referred to our pediatric dental clinic for hard‐tissue removal around the crown of an impacted maxillary right first molar. The medical histories of the patient and his family were normal. Panoramic radiography and cone‐beam computed tomography (CBCT) revealed that less than one‐fourth of the root was formed, and it was shorter than that of the corresponding left maxillary molar; additionally, the crown was covered by a radiopaque lesion Figure . The difAt 8 years and 2 months of age, CBCT revealed remnants of the lesion, which appeared to be attached to the pulp Figure . TherefoThe removed lesion fragments had enamel‐like, glossy, and irregular surfaces. Histopathology conducted using hematoxylin and eosin staining revealed the presence of multiple holes accompanied by concentric cross‐striation rings, while microcomputed tomography  showed a highly calcified lesion with an X‐ray impermeable area at the center Figure . Accordi3The cross‐striations observed in the sections obtained from the lesion were similar to that normally seen in the enamel. Moreover, X‐ray impermeability in the microCT images indicated the presence of areas with enamel‐like hardness. During the initial operation, the removal of the lesion was abandoned as it could not be separated from the molar. Due to the nature of this lesion, utilization of an ultrasonic bone scalpel and diamond bur in a step excavation would be reasonable.Previous studies have reported that treatment4An impacted tooth that is partially attached to a complex odontoma without the presence of inflammation can be erupted normally by removing the lesion and preventing the occurrence of infection.The authors declare no conflicts of interests.MM: mainly performed the dental treatment. TK: contributed the oral hygiene management during the treatment and observation period. AO and KM: examined the radiographic and pathological findings, and previous study. KM, MM, TK, and AO: drafted and reviewed the manuscript, and all approved the submission.


Dedifferentiated liposarcoma is recognized as a type of liposarcoma that usually occurs concomitantly with the well-differentiated type. In this report, we discuss the case of a 65-year-old man who developed a dedifferentiated liposarcoma with second recurrence of an atypical lipomatous tumor. The patient first presented to us with an atypical lipomatous tumor of the right elbow for which he underwent a marginal resection. After five months, the patient experienced tumor recurrence for which he underwent another extended resection. Approximately 10 months following this surgery, the tumor recurred a second time for which he underwent another extended resection. Histopathological analysis of the second recurring tumor revealed a dedifferentiated liposarcoma. So far, two years following this resection, recurrence has not been observed. This is the first case of an atypical lipomatous tumor that dedifferentiated after the additional extended resection. A liposarcoma is a malignant mesenchymal tumor that can present as an atypical lipomatous tumor, well-differentiated liposarcoma, dedifferentiated liposarcoma, myxoid liposarcoma, and pleomorphic liposarcoma . In 1979A 63-year-old man presented with a 2 x 2 cm mass on the lateral side of his right elbow. Previously, he had visited a nearby doctor, and was then referred to us for a surgical consultation. After his surgical consultation, the patient underwent a marginal resection of the mass Figure . The hisApproximately 10 months following this resection, tumor recurrence was identified on MRI Figure , 2B. He The resected specimen was yellow-white in color and had a hard, elastic texture Figure . A histoOne year following this surgery, the tumor recurred as indicated on MRI imaging Figure , 3B. AgaWe diagnosed dedifferentiated liposarcoma based on the histological findings. So far, two years have passed, and no recurrence has been observed.Dedifferentiated liposarcoma usually coexists with well-differentiated liposarcoma or recurs after resection of well-differentiated liposarcoma , 6. An eDedifferentiated tumors generally indicate some differentiated traits; however, cells and tissues eventually lose their differentiation traits and develop into an undifferentiated state, which enhances the proliferative capacity of the tissue. In general, when a liposarcoma dedifferentiates, its histology shows the features of malignant fibrous histiocytoma (MFH). Research also suggests that the time to dedifferentiation is 7.7 years on average (1–23 years) . In the We described a case of atypical lipomatous tumor that dedifferentiated after the first recurrence and after additional resections of the well-differentiated tumor. All musculoskeletal oncologists should keep in mind that atypical lipomatous tumor can dedifferentiate with recurrence after additional resection.


The chordoma is a benign cartilaginous tumor whose sphenoidale localization is exceptional. This tumor has considerable difficulties of both diagnosis and treatment. We report the observation of a Tunisian adult who presented features of hypopituitarism set wrongly on account of a prolactinoma. Chordomas are rare slow growing tumours which diagnosis is very difficult. In this report we describe a patient who presented features of intracranial hypertension, and was thought to have a prolactinoma, which in fact turned out to be an intrasellar chordoma.A 60 years old man presented with headache and visual field. MRI of the brain showed an expansive intrasellar process of 3.7cm . The diaPseudohyperprolactinemia is a frequent situation which pose a diagnostic challenge. Only four cases of pseudohyperprolactinoma have been reported previously where chordoma was associated with increased prolactin values -3. The mEssentially characterized by its great diversity, tumor pathology of the sphenoid is also distinguished by the relative difficulty of radiological diagnosis because of the variable volume of the lesions, tumour extension type as well as the poor evocative clinical features. This case is unusual and shows that chordomas can mimic pituitary adenomas. We have to recognize this disorder not only for appropriate treatment but also for prognostic.The authors declare no competing interests.


A few reports to date have described the effectiveness of surgical resection for recurrent intrahepatic cholangiocarcinoma (ICC). We report in this study a patient who achieved long-term survival after surgical resection for recurrent hepatic and pulmonary metastases of ICC.A 62-year-old man was referred to our hospital for examination of a tumor in the left lobe of the liver. Computed tomography (CT) scans of the abdomen revealed a hypovascularized tumor, 30 mm in hepatic segment 2 (S2). The patient was diagnosed with a mass-forming type of ICC. A left lateral sectionectomy with regional lymph node dissection was performed. Histopathological examination showed moderately differentiated adenocarcinoma in the hepatic S2 with lymph node metastasis. There were two intrahepatic metastases around the main tumor. The pathological stage of the ICC was pT2pN1M0pStageIIIB. The patient did not receive adjuvant chemotherapy after surgery. Twelve months after surgery, liver lesions in S4/S8 and S7 were detected on CT scans. A partial hepatectomy was performed. The histopathological features were similar to those of the previous ICC. The patient did not receive adjuvant chemotherapy after the repeat hepatectomy. Four years and four months after this repeat hepatectomy, CT scans showed multiple nodes in S4 and S10 of the left lung and in S1 of the right lung. Wedge resection of the left upper lobe and sectionectomy in S10 of the left lung were performed. Histopathological findings of the resected lung nodules were compatible with metastatic ICC. The nodule in S1 of the right lung was too small to be diagnosed as metastasis; therefore, it was not resected. After pulmonary resection, the patient was treated with gemcitabine and cisplatin for 6 months. After chemotherapy, the size of the nodule in S1 increased gradually. One year and ten months after the pulmonary resection, we performed wedge resection of S1 of the right lung, and the histopathological findings were compatible with metastatic ICC. The patient is alive without evidence of disease 8 years after the initial surgery and 8 months after the last pulmonary resection.ICC with poor prognostic factors can frequently recur; however, surgical resection for recurrent ICC might, for selected patients, enable long-term survival. Intrahepatic cholangiocarcinoma (ICC) has a poor prognosis. Surgical resection, which includes hepatectomy and regional lymph node dissection, is the only established curative therapy . AlthougA 62-year-old man was referred to our hospital for examination of a liver tumor in the hepatic left lobe. He had diabetes mellitus and prior hepatitis B virus infection. Laboratory test results for carcinoembryonic antigen (CEA) and carbohydrate antigen 19-9 (CA 19-9) were unremarkable. Computed tomography (CT) scans of the abdomen and gadoxetic acid-enhanced magnetic resonance imaging (EOB-MRI) revealed a hypovascularized tumor, measuring 30 mm in hepatic segment 2 (S2), and no enlarged regional lymph nodes  and cisplatin (25 mg/m2) which were infused on day 1 and day 8. This regimen was repeated at 21-day intervals for 6 months. After chemotherapy, the size of the nodule in S1 of the right lung increased gradually. One year and ten months after the pulmonary resection, we confirmed that there were no other metastatic lesions and we performed a wedge resection of S1 of the right lung. Histopathological findings were compatible with metastatic ICC. On immunohistochemistry, the carcinoma cells were positive for CK 7 and negative for CK 20 and TTF-1. The patient is alive without evidence of disease 8 years after the initial surgery and 8 months after the last pulmonary resection.Twelve months after surgery, liver lesions in S4/S8 and S7 were detected on CT scans Fig. a, b. No –40% [p = 0.006), periductal growth type (PI), or PI + MF subtype  and lymph node metastasis . Miura et al. [p < 0.001), T3/4 tumors , and R1/R2 surgical resection . Our patient had several risk factors, such as multiple intrahepatic lesions, lymph node metastases, and vascular invasion, for recurrence of ICC after surgery, and he was recommended for adjuvant chemotherapy. However, the patient rejected adjuvant chemotherapy and was carefully monitored.The 5-year overall survival (OS) rate of ICC after curative intent surgery was reported to be only 15–40% . The sur–40% –9. For i–40%  in theira et al.  similarl–66.6 months. Although the majority of the research has documented a small number of patients, Si et al. [< 1 year. In our patient, because he had risk factors of poor prognosis, such as multiple recurrent tumors and short TTR, he was observed for 3 months before repeat hepatectomy was performed and no other lesions were confirmed.The resection of recurrent ICC demands challenging. Recently, repeat hepatectomy for recurrent ICC has been documented and has provided survival benefits , 3. The There have been a few reports to date of resection procedures for recurrent ICC, other than hepatectomy. Pulmonary resection for recurrent ICC has been described in several case reports , and theOur patient had several poor prognostic factors, and he did not receive adjuvant chemotherapy after the initial surgery. By performing repeat hepatectomy and pulmonary resection for recurrent ICC at the appropriate time after careful follow-up, a favorable outcome was conferred on the patient. Surgical resection for recurrent ICC might contribute toward long-term survival.