The occurence of cholesteatoma and cochlear implant is rare. Secondary cholesteatomas may develop as a result of cochlear implant surgery. Primarily acquired cholesteatoma is not typically associated with congenital sensorineural hearing loss or cochlear implant in children. The occurrence of congenital cholesteatoma during cochlear implant surgery has never been reported before, partly because all patients are preoperatively submitted to imaging studies which can theoretically exclude the disease.We have reported a rare case of congenital cholesteatoma, found during sequential second side cochlear implantation in a 3-year-old child. The child underwent a computed tomography (CT) scan and magnetic resonance imaging (MRI) at 12 months of age, before the first cochlear implant surgery, which excluded middle ear pathology. The mass was removed as an intact pearl, without visible or microscopic violation of the cholesteatoma capsule. All the areas where middle ear structures were touching the cholesteatoma were vaporized with a laser and the cochlear implant was inserted uneventfully. Further follow-up excluded residual disease.We believe that primary, single stage placement of a cochlear implant (CI) with simultaneous removal of the congenital cholesteatoma can be performed safely. However, to prevent recurrence, the capsule of the cholesteatoma must not be damaged and complete laser ablation of the surface, where suspicious epithelial cells could remain, is recommended. In our opinion, cholesteatoma removal and cochlear implantation should be staged if these conditions are not met, and/or the disease is at a more advanced stage. It is suspected, that the incidence of congenital cholesteatoma in pediatric CI candidates is much higher that in average pediatric population. Cholesteatoma is an uncommon condition that has been rarely associated with cochlear implantation. Primary acquired cholesteatoma is not typically associated with congenital sensorineural hearing loss (SNHL) or CI in children. In case of secondary acquired cholesteatomas – they can develop as the result of cochlear implant surgery due to a breach of the posterior wall of the ear canal from drilling the posterior tympanotomy . The ideCongenital cholesteatoma was initially described by Cawthorne and Griffith . In 1965Congenital cholesteatoma usually grows slowly as a spherical-shaped keratin-filled cyst in the middle ear with a long asymptomatic period. When early detected, they are located deep to the antero-superior part of the tympanic membrane in two-thirds of the cases. The diagnosis is made at an average age of 4.5 years with a male to female ratio of 1:3. There are two types of congenital cholesteatoma, defined according to their location in the middle ear. The first is an isolated pearl located deep to the anterior part of the eardrum, which is believed to result from arrested epidermal formation at 10 weeks’ gestational age. It is suggested that these formations atrophy at approximately 33 weeks of gestational age, or are evacuated through the Eustachian tube. Failure of this mechanism results in this type of congenital cholesteatoma . The secThe patient was a female child diagnosed with bilateral SNHL of genetic origin at the age of 8 months. Genetic testing identified a deletion - 35delG in gene GJB2. The patient failed the newborn hearing screen at birth with subsequent diagnostic auditory brainstem responses demonstrating bilateral, severe to profound SNHL. The child initially received hearing aids but presented significant speech delay despite conventional amplification. Referred for consideration for CI, the patient underwent thorough diagnostic testing by a multidisciplinary team as well as imaging evaluation with HRCT and MRI. HRCT of the temporal bones was performed with a standard protocol using a bone algorithm with a slice thickness of 0.625 mm and collimation of 0.3 mm.Preoperative 1.5 Tesla MRI Fig.  studies One year after, a CT of the temporal bone was performed to exclude residual disease and suspicious opacification in the facial recess area was revealed. The residual disease was excluded by the endoscopy of the middle ear through anterior tympanotomy approach. The opacification turned out to be connective tissue used for obliteration of posterior tympanotomy during the CI surgery.Imaging before cochlear implantation is used to confirm the presence of an implantable inner ear space and intact cochlear nerve, as well as to provide important information about the surgical anatomy of the ear . Both MRMRI using the conventional sequences  provides additional information enabling distinguishment of different pathologic entities, as well as accurate diagnosis of primary and residual/recurrent cholesteatomas. Even higher diagnostic specificity is achieved with diffusion-weighted (DW) echo-planar imaging, delayed post-contrast imaging, DW-non-echo-planar imaging, and DWI-PROPELLER techniques. We used a 1.5 Tesla MRI with conventional sequences in our patient, following our routine protocol, which was focused primarily on assessing the anatomical implant feasibility in young children. However, even with conventional protocols, a pathological mass greater than 2 mm in size and surrounded by air can be identified on the T2-weighted sequence. In conventional MRI, the diagnosis of sub-millimeter anatomical structures is also possible provided that there is good signal contrast between the structure and its surroundings, as in our case. For temporal bone diagnosis in clinical practice, we use a 1.5 Tesla MRI. With the development of new models, MRI at 3 Tesla or higher is becoming more common and widely available. The main advantage is shorter acquisition time but because of artifacts specific to this anatomic region, diffusion-weighted sequence acquisition is paradoxically longer, which increases the risk of motion artifacts, especially in children, making interpretation more difficult, even for experienced radiologists .Except for paper of Chung et al., to date, there has been no literature published on the incidence of congenital cholesteatoma found prior to implantation . The autIt has been suggested that if congenital cholesteatoma is found during diagnostic procedures for CI, the cholesteatoma should be removed and implantation delayed to the second stage . In our Although the laser is not universally utilized in the treatment of cholesteatoma, Hamilton concludes that the appropriate use of the laser during cholesteatoma surgery facilitates significantly the complete removal of the disease and presents no extra risk to the vital structures within the temporal bone . Also JaIt has long been recognized that a second-look procedure in cases of congenital cholesteatoma is required less often than in acquired pediatric cholesteatoma . James eAccording to Derlacki and Clemis’ criteria the patient’s cholesteatoma met the definition of a congenital cholesteatoma - it was totally asymptomatic and behind an intact tympanic membrane . CholestThe growth rate of this particular cholesteatoma had to be relatively dynamic.The original size of the cholesteatoma is important. According to the first theory of origination of congenital cholesteatoma, the diameter of the epidermoid formations frequently found in human fetuses that proceed to congenital cholesteatoma if not absorbed or evacuated through the Eustachian tube, is already known. Huang et al. reviewed 49 fetuses, ranging from 12 weeks to full term. In 16 they found epidermoid formation, which was always located at the anterosuperior edge of the eardrum . The widAssuming a linear growth for congenital cholesteatoma, James et al. calculated that closed cholesteatoma cysts enlarge by approximately 1 mm in diameter per year, based on CT measurements ( James). The explanation for the lack of identification of the cholesteatoma on the initial HRCT is that the pathology was too small to be visualized. The most likely it was a very small sub-millimeter “sleeper” congenital cholesteatoma with no growth between birth and the first year, but with subsequent rapid growth between 1 and 3 years of life.Bilateral cochlear implantation in young children is increasingly common in clinical practice. Among the benefits of bilateral cochlear implantation is the restoration of some of the advantages of binaural hearing such as localization, improved listening in noise, directional hearing, binaural summation, and squelch. Typically, young hearing-impaired children are being provided with two implants either at the same time (simultaneous implantation) or at different times in early childhood as in our case  . Our locExamining the findings in this patient, the obvious question arises whether to perform another imaging study which could preclude the existing, newly formed cholesteatoma before the subsequent CI surgery, and how much time should elapse between the first and second implantation, to perform such a study most effectively?We believe that the rarity of our particular case does not justify the additional cost and burden of repeating the standard HRCT and/or MRI in so young children. Also, our case presented with limited pathology that was controlled by a single-stage procedure. We emphasize the importance of continued follow-up and advise considering staging in either known disease or more extensive disease as a reasonable and safe option.To our knowledge, this is the first report of an incidental finding of congenital cholesteatoma during CI surgery despite presurgical imaging studies. Primary excision en bloc with the removal of associated ossicular elements and laser surface ablation is the recommended treatment for limited congenital cholesteatoma. Primary placement of an implant during cholesteatoma removal is warranted as long as there is insignificant risk of recurrence, provided that no damage of the capsule has occurred and complete and safe surface contact of epithelial laser ablation was observed. There should be a low threshold for staging the implant if any of these conditions are not met, and/or the disease is found to be more extensive. Follow-up should include regular microscopic ear examination and HRCT.There is suspicion that the incidence of congenital cholesteatoma in pediatric CI candidates is much higher than in normal pediatric population (4).The written consent was obtained from the patient’s parents to publish the details of their child’s case.


Polyps originating from the posterior septum with choanal extension, also known as “septochoanal polyps,” are uncommon, and septochoanal polyps with central calcification are extremely rare. We report the second case of septochoanal polyps with central calcification in the English literature.A 55-year-old Thai woman presented with a progressive left-side nasal obstruction. An examination of her nose revealed an irregular lobulated mass, yellow in color, with a smooth surface that arose from her posterior nasal septum and extended down to her nasopharynx. Computed tomography revealed a large choanal mass with a central ossified structure. A punch biopsy was performed and microscopic examination showed an inflammatory polyp. The mass was removed using an endoscopic surgery technique, and the histology of this lesion confirmed a typical presentation of choanal polyps.Although septochoanal polyps with osseous metaplasia are known to be very rare, physicians should be aware of them and include them in the differential diagnosis of choanal mass with central calcification lesions. Most choanal polyps arise in the lateral walls of the nasal cavity, particularly the antrochoanal polyp, which originates from the maxillary sinus antrum. Some unusual origins, such as the sphenoid sinus, ethmoid sinus, middle turbinate, inferior turbinate, and nasal septum, have also been reported in the literature . Choanalet al. . There iet al. . Here weA 55-year-old Thai woman had a 3-month history of progressive, left-side nasal obstruction. She had no history of nasal discharge, anosmia or any sign of allergy. She was otherwise healthy. A physical and nasal endoscopic examination revealed an irregular lobulated mass, yellow in color, with a smooth surface that arose with a thin pedicle from the left-side of her posterior nasal septum. This mass extended down to her nasopharynx and crossed over to the right-side of the choana. An axial view computed tomography soft tissue window demonstrated a mass with a central ossified structure at the posterior septum, which filled her nasopharynx. There was mucosal thickening in her left maxillary sinuses, while the aeration of her other paranasal sinuses was normal Fig. . A punchet al., these polyps can arise from the maxillary sinus , sphenoid sinus , and ethmoid sinus  [et al. in 2003 [The first description of choanal polyps was reported by Killian in 1906; Killian defined them as an isolated, solitary paranasal sinus mass or cyst, which protrudes into the boundaries between the nasal cavity and the nasopharynx, or even down to the oropharynx . Accordi polyps) . The mos polyps) . The caset al. first mentioned the term “septochoanal polyps” with the third reported case in 2009 [Hakan Birkent  in 2009 . In this in 2009 . Again tThe first report of a septochoanal polyp with central calcification was published by Woo Sung Kim in 2010 . This caFor this present case, we performed a punch biopsy, under local anesthesia, for diagnosis; a pathological examination showed an inflammatory benign polyp. After endoscopic excision, a histological examination confirmed atypical polyp: mature trabecular bone tissue covered with respiratory polypoid mucosa without eosinophils. From the prognosis standpoint, the key to successful treatment of septochoanal polyps is the same as for other choanal polyps : the comWe reported the case of a 55-year-old Thai woman who presented with an uncommon nasal mass. Physicians should be aware, at the time of treating, of a nasal mass with central calcification arising from the posterior septum. A septochoanal polyp with OM could be the differential diagnosis. An endoscopic approach with complete surgical removal of the polyp and its pedicle origin is the standard treatment.


We report a case of bladder alveolar soft part sarcoma in an 18-year-old Thai male patient who had been treated with testicular radiation and systemic chemotherapy for acute lymphoblastic leukemia with testicular relapse. He presented with recurrent dysuria and gross hematuria. Cystoscopy revealed a 2-centimeter irregular sessile mass at the bladder base adjacent to left ureteral orifice. Transurethral resection of the tumor was performed. The histopathological diagnosis was alveolar soft part sarcoma. Chest and abdominal computed tomography showed no evidence of metastasis. He was treated with partial cystectomy and left ureteral reimplantation with negative surgical margin. No evidence of recurrence was found during a 28-month follow-up period with surveillance cystoscopy and computed tomography of the chest and abdomen. Alveolar soft part sarcoma (ASPS) is a rare soft tissue neoplasm, accounting for 0.5 to 1% of all soft tissue sarcomas. It typically arises in the deep soft tissue of buttock and thigh but rarely grows in the genitourinary system. Herein we report a case of alveolar soft part sarcoma arising in the urinary bladder, which to our knowledge is the first reported case that occurred as a second primary tumor following radiation therapy.An 18-year-old Thai male patient presented with recurrent painful hematuria over a year. He was initially treated as urinary tract infection but did not respond to several courses of antibiotics. Later, a renal and bladder ultrasound revealed 1.7 × 1.1 cm hyperechoic nodule at the left lateral aspect of his urinary bladder. Subsequent computed tomography demonstrated normal upper urinary tracts and no lymphadenopathy. He was diagnosed with acute lymphoblastic leukemia (ALL) at the age of 4 and treated with systemic chemotherapy. Bone marrow and extramedullary relapse in the testis occurred few years after treatment completion. Salvage chemotherapy and testicular radiation led to complete remission when he was 11 years old.βE12, vimentin, RCC, p504s, desmin, S-100, HMB45, melan-A, chromogranin-A, CD 34, and CD 68. A diagnosis of alveolar soft part sarcoma was established based on the compatible histomorphological finding, presence of crystal, and the positivity of TFE3 on IHC staining and by exclusion of other bladder tumors.Upon presentation to our department, cystoscopy demonstrated a friable two-centimeter broad-based nodule at the bladder base adjacent to the left ureteral orifice with surrounding hypervascularization . A transThe patient underwent partial cystectomy with left ureteral reimplantation. Open midline transperitoneal approach was selected. Part of left-sided bladder wall and left distal ureter was removed en bloc. Transvesical technique was applied for left ureteral reimplantation. Pelvic lymph node dissection was performed with a standard template. Postoperative course was uneventful with a hospital stay of 5 days. The histopathological finding demonstrated benign urothelium lining with underlying edematous stroma and focal foreign body granulomas. No residual tumor was documented. Six reactive lymph nodes were found without evidence of tumor.After the discussion in our multidisciplinary tumor board, we opted for clinical observation and regular surveillance without adjuvant therapy for this patient. Subsequent surveillance consisted of periodic cystoscopy along with chest and abdominal computed tomography. Until now, no evidence of local recurrence or distant metastasis is detected during a 28-month follow-up duration.The entity of alveolar soft part sarcoma (ASPS) was first described by Christopherson et al. in 1952 . In the ASPS of the urinary bladder is exceedingly rare with 3 reports in the literature. The first published case was a 31-year-old male presenting with hematuria and large pelvic mass . Tumor rThe second case was a 37-year-old male presenting with a slow growing suprapubic mass and bilateral ureteral obstruction . CystoscGiven the paucity of ASPS cases, it remains unclear whether surgery alone or surgery with addition therapy is superior to others. As in other soft tissue sarcomas, radical surgery is the main therapy for localized disease with an ultimate goal of achieving R0 resection. Most series in other soft tissue ASPS have suggested that this tumor is relatively resistant to conventional cytotoxic chemotherapy . HoweverOur case represents the first case of bladder ASPS arising as a second primary malignancy. This patient was treated successfully with organ-preserving surgery and close observation. Although the risk factor predisposing to ASPS is unknown, prior exposure to radiation therapy and cytotoxic chemotherapy in this patient may play some roles in carcinogenesis. His bladder ASPS has met all criteria for diagnosis of postradiation sarcoma defined by Cahan et al. as follows: (1) lesions of different histologic features to that of the original lesion, (2) being located within the field of radiation, and (3) arising after a latency period of more than four years . This paIn conclusion, the optimal treatment for bladder ASPS has to be further defined. Radical resection remains the therapy of choice for localized disease, but the organ-preserving surgery with stringent follow-up in selected patient is a reasonable option, which may improve the quality of life without compromising clinical outcomes.


Myeloid sarcoma (MS) of the central nervous system (CNS) is a rare presentation of leukemic mass infiltration outside of the bone marrow. It may involve the subperiosteum and dura mater and, on rare occasions, can also invade the brain parenchyma. The disease is most commonly seen in children or young adults; however, it has been described in multiple age groups. MS can be seen in patients with acute myeloid leukemia (AML), chronic myeloid leukemia and other myeloproliferative disorders. This entity has the potential to be underdiagnosed if the MS appearance precedes the first diagnosis of leukemia. The main reason is that their appearance on CT and MRI has a broad differential diagnosis, and proper diagnosis of MS can only be made if the imaging findings are correlated with the clinical history and laboratory findings. Herein, we describe the intracranial CNS manifestations of MS in patients with AML on CT and MRI involving the brain and/or meninges. This study is based on a systematic review of the literature. In addition, three case reports from the author’s institution with AML and intracranial involvement of MS are included. Our aim is to enhance the awareness of this entity among both clinicians and radiologists. Central nervous system (CNS) myeloid sarcoma (MS), a rare manifestation of acute myeloid leukemia (AML), chronic myeloid leukemia and other myeloproliferative disorders were first described by Burns in 1811 [et al., [(1)They may develop during the active phase of leukemia;(2)They may develop concurrently with known chronic myeloproliferative disorders;(3)They may manifest as a relapse after months or years after clinical remission of AML, particularly after bone marrow transplantation;(4)They may precede the AML diagnosis and may be detected in previously healthy patients who have a normal peripheral blood cell count and who have no blast infiltration in the bone marrow (0.6% of cases as described by Krause ). In thiAccording to Audouin et al., , there aIn this review of the literature, we have identified 21 reported cases of myeloid sarcoma in patients with AML and describe the diagnostic and imaging findings pertinent to the brain parenchyma and/or meninges. In addition, three patients from our institution with AML and positive intracranial involvement were included.A PubMed database search using the descriptors chloroma, myeloid sarcoma, myeloid sarcoma and brain, intracranial, central nervous system and cranial nerves retrieved 56 relevant citations from 1971 to 2014. Reviewing the literature, 21 reported cases of myeloid sarcoma with a sufficient description of the diagnosis and imaging findings related to the brain and/or meninges were identified. Leukemic conditions other than AML, such as chronic myeloid leukemia and myeloproliferative disorders, were not included. Only articles in English that were available on the PubMed central publishing platform were considered.versus extra-axial location, single versus multiple lesions and CT and/or MR imaging characteristics  with AML and CNS myeloid sarcoma were included in this review. Gender was not available for one case report . NineteeOut of the 21 patients, a total of 24 intracranial myeloid sarcoma lesions were described. Of those, 13 lesions (54%) were described in the intra-axial compartment of the brain and 11 lesions (45%) in the extra-axial brain compartment. One patient had sequential lesions develop in more than one anatomic site in the brain . Anothern = 6), frontal lobe (n = 4), cerebellum (n = 4), parietal lobe (n = 3), occipital lobe (n = 3), cerebellopontine angle cistern (n = 1), corpus callosum (n = 1), basal ganglia (n = 1) and subdural space (n = 1). Six patients described also showed either concurrent or sequential lesions in locations outside the brain. These extra-cranial locations included the temporal bone (n = 1), both kidneys (n = 1), multiple bones (n = 1), nasopharynx (n = 1), cervical and lumbar spine (n = 1), thoracic and sacral spine (n = 1), scalp tissues (n = 2) and infratemporal fossa (n = 2). One intracranial vascular lesion involving the superior sagittal sinus was described in one patient (n = 1).Overall, the sites of intracranial myeloid sarcomas included the temporal lobe  appeared hyperdense on noncontrast CT. Out of all 11 hyperdense lesions, six lesions were intra-axial and five extra-axial. Twenty-two lesions (91%) exhibited avid homogeneous enhancement. One lesion demonstrated inhomogeneous thick peripheral enhancement, indistinguishable from a brain abscess 1% appear.Seven intra-axial MS lesions were assessed with brain MRI. MS demonstrated either a hyper, iso- or hypo-intense signal on T2-weighted images. T2 hyperintensity was described in four lesions, while T2 iso- or hypo-intensity was described in three lesions. Nine extra-axial MS lesions were assessed with MRI. T2 hyperintensity was described in two extra-axial lesions; however, T2 signal findings were not described for the remaining seven extra-axial reported lesions assessed with MRI. Primary bone destruction was evident in one patient, resulting in erosion of the petrous portion of the temporal bone and subsequent intraparenchymal involvement of the right temporal lobe .Vasogenic edema surrounding the enhancing leukemic masses was identifiable on both CT and MRI. This was seen in 11 lesions (45%). Among these, six lesions were in the extra-axial and five in the intra-axial compartment of the brain.CT and MR imaging characteristics of three patients from our institution presenting with acute myeloid leukemia and intracranial CNS manifestations of myeloid sarcoma are described. Case 3 was previously reported in the literature by one of the authors .Case 1: A 69-year-old female patient with a recent history of worsening headaches and flu-like symptoms diagnosed with AML and more than 90% myeloid cells in the blood count during her first admission .Case 2: A 56-year-old female patient with acute myelomonocytic leukemia, French-American-British (FAB) classification M4, developed a first relapse of AML with 26% blasts in the blood count during admission. She underwent double umbilical cord blood stem cell transplantation during the remission phase after being successfully treated with induction chemotherapy for her first relapse. On Day 39 of the post-transplant period, she presented with persistent headaches, muscle weakness, falls at home and the onset of leukemia cutis with new skin lesions .Case 3: A 53-year-old man with a three-month history of polydipsia and polyuria and indication of myelodysplastic syndrome (MDS) presented with refractory anemia and 16% blast cells in the bone marrow biopsy suggesting a diagnosis of refractory anemia with excess of blasts (RAEB). Diabetes insipidus was confirmed by inappropriately low urine osmolality and low antidiuretic hormone (ADH) levels. AML induction chemotherapy (seven days of cytarabine and three days of idarubicin) along with intrathecal methotrexate after cerebrospinal fluid (CSF) analysis was initiated .i.e., myeloid sarcoma, the imaging features can be mistaken for some conditions other than the myeloid sarcoma. Myeloid sarcoma seen in leukemic patients may involve any part of the body. The most common sites for myeloid sarcoma deposits are the bones, soft tissues, lymph nodes and the skin. Rarely, they may manifest as single or multiple intracranial lesions [CNS leukemic infiltration may present in one or more of the following intracranial forms: (1) meningeal disease, as “carcinomatous meningitis”; (2) intravascular tumor aggregates throughout the brain, as “carcinomatous encephalitis”; or (3) focal tumor masses, as “myeloid sarcomas”. When AML manifests as a solid tumor outside the bone marrow,  lesions , most co lesions . In our et al., of a total of 61 biopsied proven myeloid sarcomas, twenty-two lesions were seen in 15 patients with no known history of acute myeloid leukemia [Myeloid sarcoma can occur at variable ages (1–81 years old), often after the diagnosis of AML. However, in approximately 25% of the patients, MS appears before the initial diagnosis of AML by months or years . Accordileukemia . Most ofRadiologically, intracranial MS in patients with acute myeloid leukemia most commonly presents as an extra-axial hyperdense mass on noncontrast CT scan. In this review of the literature, all 24 intracranial lesions, except for one, appear as hyperdense masses on the noncontrast CT studies. The differential diagnosis of hyperdense intracranial masses is broad and includes meningioma, B-cell lymphoma and intracranial metastasis. Interestingly, myeloid sarcomas can express B-cell antigens  and, thus, potentially lead to a histologic misdiagnosis of CNS lymphoma, if no immunohistochemistry or flow cytometry analysis is available . Other lVasogenic edema was present on either CT or MR in 11 lesions (45%) out of the 24 intracranial myeloid sarcomas that were included in this review. Further characterization of other CT and/or MR imaging features should be used to distinguish the intracranial MS lesions from other commonly-found intracranial lesions, such as meningioma, lymphoma or brain metastasis, which also commonly show vasogenic edema and can potentially occur concurrently with myeloid sarcoma in patients with acute myeloid leukemia.Leukemic cell infiltrates are capable of migration from the bone marrow of the periosteum and dura mater into the underlying brain parenchyma once there is disruption of the pial-glial barrier. Despite the close involvement of the extra-cranial and intra-cranial tissues, destructive bony changes are not commonly seen with myeloid sarcomas. Only one patient showed apparent bone destruction of the temporal bone and concomitant involvement of the adjacent right temporal lobe parenchyma .One of the limitations of this study is the small sample of the reported cases available in the literature, since we exclusively included AML patients with intracranial MS lesions. Other forms of acute or chronic leukemias and myeloproliferative disorders could also present with intracranial MS lesions and were not included in this study. Potential selection bias of the study population is another limitation, since the majority of patients included were young adults. Since only one pediatric patient with AML and intracranial CNS myeloid sarcoma was present in our systematic review, our findings cannot be generalized to all age groups.Myeloid sarcoma of the intracranial CNS is a rare presentation of AML. This can be detected before the diagnosis of leukemia or any time throughout the course of the disease during routine neuroimaging studies. Proper CT and MR imaging interpretation in conjunction with relevant clinical information, including the age at the time of diagnosis, symptomatology, such as the presence of headaches, seizures, focal neurological deficits and/or cranial nerve palsy (-ies), a history of hematologic malignancies, prior bone marrow or solid organ transplantation status and immune system status, can help to narrow the clinical diagnosis of intracranial MS. Finally, prompt clinical and laboratory evaluation for acute myeloid leukemia relapse is necessary in patients with a known history of leukemia presenting with meningeal and/or parenchymal lesions.


Dermatomyositis is an autoimmune disease characterized by proximal myopathy, cutaneous Gottron papules and heliotrope rash; intestinal involvement associated to acute vasculitis is less common but could be a life-threatening condition.A 21-year-old woman, affected by dermatomyositis, presented to our attention with a three-day story of severe abdominal pain, no bowel movement and biliary vomit. She was diagnosed with acute abdomen. A CT scan with bowel contrast demonstrated the presence of a leakage from the retroperitoneal aspect of duodenum. The surgical and clinical management in the light of literature review is presented.Our first approach consisted in primary repair of the duodenal perforation with omentopexy. Post-operative course was complicated by hemorrhage. A reintervention showed a new perforation associated with multiple ischemic intestinal areas. We performed a gastroenteric anastomosis with functional exclusion of the damaged duodenum and positioning of drainages to create a biliary fistula. A nutritional enteric tube and an open abdomen vacuum-assisted closure system to monitor the fistula creation and to prevent abdominal contamination and collections were positioned. To reduce the amount of biliary leakage, a percutaneous transhepatic biliary drainage was placed, with progressive fistula flow disappearance in four months.In patients with dermatomyositis, when clinical findings and symptoms suggest abdominal vasculitis, it is very important to be aware of the risk of bowel and particularly duodenal perforations. Open abdomen treatment favors control of contamination by gastrointestinal contents, offers temporary abdominal closure, helps ICU care and delays definitive surgery. Dermatomyositis (DM) is an autoimmune disease characterized by cutaneous heliotropic rash, Gottron papules and proximal myopathy associated to dysphagia, dysphonia, Raynaud phenomenon, fatigue and non-erosive inflammatory polyarthritis . VasculiWe performed a literature review by searching on PubMed : only few cases of bowel perforation associated to dermatomyositis are described in literature, and surgical approach is not always mentioned or specified -19. In lWe report the case of a 21-year-old patient affected by DM presenting with rapid onset acute abdomen associated to severe vasculitis and complicated duodenal perforation, and discuss the surgical and clinical management in the light of literature review.A 21-year-old female diagnosed with DM in 2008, on treatment with prednisone and cyclosporine with moderate disease activity until December 2012, presented to our Emergency Department (ED) with a three day history of diffuse, acute abdominal pain, no bowel movement and biliary vomit. She underwent laparoscopic cholecystectomy in 2010 for symptomatic calculosis. The patient was admitted to our Department with a bowel perforation suspect. An oral follow-through was negative but a CT scan with oral contrast demonstrated a small leakage from the posterior aspect of the third duodenal portion . Argatroban was administered obtaining progressive increase in PLT count (354.000/μm3). Three months after surgery she had seizures with MRI scan positive for vasculitic diffuse encephalic lesions, treated with levetiracetam and metilprendisone. During hospitalization we observed nasal regurgitation of fluids, nasal speech and hoarseness probably due to loss of pharyngoesophageal muscle tone and increase and reduction in hepatic stasis values of unknown origin. After 8 months of follow-up, no signs or symptoms of abdominal disease were reported.During her ICU stay, the patient presented signs of renal vasculitis, therefore she underwent cycles of continuous veno-venous hemodialysis (CCVHD), plasmapheresis and intravenous immunoglobulin (IVIG), showing clear improvement of her renal function and negative immunological test. Low molecular weight heparin (LMWH) treatment was complicated by heparin induced thrombocytopenia (HIT) with low platelet (PLT) count (99.000/μmDM is an autoimmune disease characterized by cutaneous heliotropic rash, Gottron papules and proximal myopathy associated to dysphagia, dysphonia, Raynaud phenomenon, fatigue and non-erosive inflammatory polyarthritis . VasculiSurgical treatment is not standardized because of the rarity and variety of the gastrointestinal DM presentations that can affect the entire gastrointestinal tract. In literature we found few descriptions of ischemic gastrointestinal perforation in DM. Surgical treatments and outcomes reported were anecdotic and varied, including resection of the affected tract or sutures of the lesions Table . High reWhen clinical findings and symptoms suggest possible abdominal vasculitis in a young subject known for DM, it is very important to consider bowel and particularly duodenal perforation. We found very helpful CT scan with oral contrast to support diagnosis and we had to face the more life-threatening condition of multiple ischemic intestinal ulcerations conditioning duodenal multiple perforations. To manage this challenging condition we used open abdomen treatment with exclusion of the duodenal ischemic perforated tract through a gastroenteroanastomosis and PTBD with the creation of a guided fistula to decrease the flow and obtaining progressive healing with improvement of patient’s general conditions. This surgical treatment must always be accompanied by DM specific medical treatment to avoid further vasculitic complications and to obtain control of the disease activity.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.CAPS: Anti-phospholipidic catastrophic syndrome; CT: Computed tomography; CVVHD: Continuous veno-venous haemodialysis; DM: Dermatomyositis; ED: Emergency Department; HIT: Heparin induced thrombocytopenia; ICU: Intensive care unit; IVIG: IntraVenous immunoGlobulin; LAC: Lupus anti coaugulant; LMWH: Low molecular weight heparin; MRI: Magnetic resonance imaging; NGT: Naso-gastric tube; PTBD: Percutaneous transhepatic biliary drainage; PLT: Platelet.The authors declare that they have no competing interests.RV made substantial contributions to acquisition and interpretation of data, was involved in conception and drafting of the manuscript. SC contributed to interpretation of data, was involved in conception, drafting and revision of the manuscript. SF and CC contributed to acquisition of data, drafted the manuscript. MV was involved in revising the manuscript critically for important intellectual content. ECA contributed to interpretation of data, gave final approval of the version to be published. All authors read and approved the final manuscript.


Congenital teratomas occur in extragonadal locations, the commonest site being the sacrococcygeal region. This report describes a rare case of antenatally detected, large, immature retroperitoneal teratoma. The diagnostic and therapeutic challenges of dealing with such a case have been discussed and the relevant literature reviewed. The recurrence of the tumor after gross surgical removal indicates a definitive role of administering chemotherapy in such a case.  Congenital teratomas tend to be extragonadal and histologically benign but carry a high mortality due to premature delivery of the baby or the development of hydrops fetalis [1]. Improvement in the results of treatment and survival rates of patients with teratomas diagnosed in utero raises important questions regarding the prognostic significance of immature germ cell elements which are frequently present in these tumors. This is because these immature elements often show maturation with time [2]. We report a case of congenital immature retroperitoneal teratoma that had been detected antenatally. The management of this rare condition is discussed and the relevant literature reviewed. A full-term male neonate weighing 2.6 kg was referred for surgical treatment of an abdominal mass, which had been detected by an antenatal ultrasound scan (US) at 28 weeks of gestation. He was born by caesarian section, to a third gravida and second para mother. Abdominal examination revealed a firm central abdominal mass occupying almost all quadrants with restricted mobility of the mass. Plain X-ray abdomen showed the bowel gas shadows pushed to the left upper quadrant by a large space-occupying lesion. Postnatal US confirmed a large abdominal mass consisting of solid and cystic areas with foci of calcification. A computed tomographic (CT) scan of the abdomen at day five of life revealed a heterogeneous retroperitoneal mass (7cm× 5cm× 8.5cm) with calcification . The serum alpha-feto-protein (AFP) level was 484 ng/ml. A pan-systolic murmur was detected over the precordium. Doppler echocardiography showed a large muscular ventricular septal defect (VSD) with a left to right shunt and severe pulmonary hypertension. After consultation with the cardiologist, a decision was taken to proceed with the abdominal surgery. The child underwent laparotomy at 21 days of life. A large retroperitoneal tumor (8 cm×10cm) consisting of solid and cystic areas was found occupying mainly the right flank and central abdomen. The tumor was adherent to the surrounding structures namely the root of mesentery, superior mesenteric vessels, duodenum, colon, right kidney with renal vessels. However, there was no infiltration into any vessels or organs neither was any significant lymphadenopathy noted. Due to its friable nature, the tumor ruptured during removal resulting in intra-peritoneal spillage; however, gross complete excision of the tumor was performed . Post-operatively the child developed congestive cardiac failure, which was treated with digitalis and diuretics. The child was discharged on the 10th postoperative day on full oral feeds. Histopathological sections from the tumor showed features of high-grade (grade III) immature teratoma with presence of immature neuroepithelial cells, glands, tubules, retinal tissue, hyaline cartilage and woven bone. Since the patient was a neonate and also had features of congestive cardiac failure, chemotherapy was not considered at that stage. The child underwent closure of the VSD at four months of age. At five months of age, the child presented with recurrence of the tumor, mainly on the left side of the abdomen, and a significantly elevated serum AFP level. The patient was re-operated for removal of the recurrent tumor. During surgery, a large recurrent tumor was found to be adherent to the retroperitoneal structures leading to incomplete removal and an iatrogenic duodenal injury. The child succumbed in the postoperative period to overwhelming sepsis.Teratomas are the commonest congenital neoplasms. The usual site for congenital teratomas is sacrococcygeal, the other sites being the mediastinum, head and neck, oropharynx, pericardium, and the retroperitoneum [3]. Lack et al [4] reported 3 cases of neonatal retroperitoneal teratoma; of these, two were immature and both succumbed to the disease. Antenatally detected immature teratoma of the retroperitoneum similar to our case has been extremely rarely reported . Generally, immature teratomas are associated with increased risk of tumor recurrence and malignant change. This does not appear to be the case with congenital teratomas. Histological tumor immaturity does not appear to correlate with malignancy in congenital teratoma [2]. Clinical stage and surgical resectibility are considered the most important prognostic factors [7]. The element of microscopic foci containing immature tissues varies from less than 10% in grade 1, 10 – 50 % in Grade 2, and more than 50% in grade 3 teratoma.Immature elements like immature neuroepithelial cells, glands, tubules, retinal tissues were found histologically in the index case. The prognosis of neonatal teratomas is favorable with an 80-100% survival reported after surgical excision of the tumor and treatment of any recurrence . McKenney et al [2] in a series of 22 cases of congenital teratoma reported 100% survival and no recurrence after complete surgical resection with microscopic clearance, irrespective of histological tumor grade or immaturity; however, none of them was immature retroperitoneal teratoma. Tumor recurrence has been reported following spillage [8]. Some immature teratomas may continue to differentiate with time. Adjuvant chemotherapy was not considered in the index case being a neonate with associated congestive cardiac failure. However, for recurrence and for incomplete surgical excision, chemotherapy should be added in the treatment regimen (4-6 cycles of platinum based chemotherapy). Despite grossly complete surgical removal, the recurrence in our case could be attributed to microscopic seedling resulting from tumor rupture during surgery and not treating it with adjuvant chemotherapy. Serial serum AFP can be used as a maker to detect recurrence; Most of the recurrences occur within 7 months following excision [7], although recurrence can occur even up to 3 years after surgical excision. Therefore, a close follow-up of these patients is essential. In conclusion, the management of congenital immature retroperitoneal teratoma is complete surgical excision and microscopic clearance followed by close surveillance for any recurrence including serial serum AFP monitoring. For incomplete surgical excision or tumor spillage like in our case, post operative adjuvant chemotherapy should be given.Source of Support: NilConflict of Interest: NoneEditorial Comment: Neonatal tumors have a different biological activity and thus their prognosis is different from the children with tumors in post-infantile period. Though approximately 60% of the congenital tumors are benign, the incidence of malignancy is about 40%. Of all the congenital tumors, the extracranial teratomas have the highest frequency of occurrence of malignancy. The behavior of malignant teratomas/germ cell tumor in neonates and infants has been noted to be more aggressive with a higher incidence of recurrence and metastases in this age group. The same seems to be true for high-grade immature teratomasas reported in the index case. Taking these facts into consideration, the optimal therapy for high grade immature teratomas of congenital origin should not be just a single modality. A multimodality treatment approach would be more appropriate for long-term tumor-free survival without significant morbidity. No doubt that the tumor of these dimensions has to be surgically excised, but in case of intra-operative tumor spill, appropriate chemotherapy has to be administered postoperatively for improved outcomes. Intraoperative tumor spillage upgrades the stage of the tumor in almost all malignant tumors. Hence, much caution is warranted during dissection. When malignancy, especially a high grade, is confirmed, the patient with tumor warrants further treatment in the form of systemic therapy. The chemotherapy regimen most commonly used for immature teratomas is combination of Bleomycin, Etoposide and Cisplatin (BEP), which does not have any cardiotoxic effects. Platinum based chemotherapy has significantly improved the outcome of infants with malignant disease. Combination of Vinblastine, Actinomycin and Cyclophosphamide (VAC regimen) has also been frequently utilized for extratesticular immature teratomas of both grade 2 and 3 without any cardiotoxic effects. In neonates, the chemotherapy doses are as such administered with 50% reduction to decrease the toxic effects. Hence, the justification of not using chemotherapy in high-grade immature teratoma with intra-operative tumor spillage does not hold ground. Another major issue which needs to be addressed is recurrence. The high-grade (grade 3) pediatric immature teratomas, especially the extragonadal tumors have a much greater propensity for recurrence, as also incomplete resection. In this particular case, intra-operative tumor spillage is equivalent to microscopic residue of the disease. All recurrences of chemosensitive tumors must first be treated by systemic therapy as the extent of spread of disease can be assessed only macroscopically and the microscopic spread goes undetected, control of which can be done only by systemic therapy. Hence, upfront surgical intervention in the event of recurrence was inappropriate. The behavior of immature gonadal teratomas is different from immature extra-gonadal tumors. Contrary to the gonadal counterparts whose prognosis is not affected by the grade of the tumor and only surgical excisionis sufficient, the extragonadal immature teratomas require a judicious use of multimodality therapy depending on the grade of immaturity for long-term tumor free survival.


Synovial sarcomas in the head and neck are extremely rare tumors, especially in the pediatric population. 3–5% of synovial sarcomas occur in the head and neck region displaying varied imaging and histopathological features resulting in frequent misdiagnosis. These tumors have a poor prognosis; hence early diagnosis and accurate classification based on imaging, histopathology, and immunohistochemistry are critical for prompt treatment. To the best of our knowledge, imaging findings of pediatric retropharyngeal lipomatous synovial sarcoma have not been reported to date in English medical literature. We report, for the first time, a rare case of retropharyngeal lipomatous synovial sarcoma in a ten-year-old child and discuss the case-specific imaging findings in our patient using magnetic resonance imaging and computed tomography. Synovial sarcomas are well known clinical entities occurring in para-articular locations close to synovial linings, tendon sheaths, and bursae. These tumors are uncommon in the head and neck but cases have been reported in the adult population involving the oral cavity, sinonasal tract, submandibular area, parotid gland, temporal/infratemporal regions, cheek, supra-auricular scalp, maxillary/submaxillary regions, mandible, and nasolabial regions . It is iA ten-year-old male child presented with dysphagia, breathlessness, disturbed sleep, and snoring for two months. There was no history of fever or throat pain. Clinical examination of the oral cavity revealed a large smooth bulge on the posterior pharyngeal wall with intact overlying mucosa. The swelling was firm, nontender and its inferior margin could not be identified. No neck nodes were palpable on clinical examination. The patient was referred for preoperative radiological evaluation. A contrast-enhanced MRI examination and a noncontrast low-dose CT scan were performed. The imaging studies revealed a large well-defined mass with smooth margins and a complex signal appearance in the midline retropharyngeal space measuring approximately 4.8 × 1.5 cm in maximum transverse and anteroposterior dimensions and extending superoinferiorly for approximately 6.6 cm . The lesBased on the above imaging appearances and age of the patient, the differential diagnosis included teratoma, dermoid cyst, and a fat containing mesenchymal tumor.The patient underwent a wide local excision of the mass under general anesthesia via a transoral approach. The postoperative period was uneventful. Histopathological examination of the operative specimen showed polygonal to spindle shaped cells arranged in whorls and storiform pattern . ScatterA diagnosis of monophasic synovial sarcoma was made based on the above findings. The patient subsequently received radiation therapy over a period of five months and has been advised regular six-monthly follow-up at our ENT outpatient department. There was no evidence of local or systemic recurrence on the PET-CT scan carried out three months after completion of radiation therapy.The retropharyngeal space is situated posterior to the pharynx and esophagus and anterior to the prevertebral muscles. The detailed anatomy of this neck space and the extensive differential diagnosis of pathologies have been discussed extensively in existing medical literature . Benign This case report highlights the imaging findings in a child with a lipomatous synovial sarcoma of the retropharyngeal space. There were no specific imaging criteria to make a preoperative diagnosis which was confirmed only postoperatively by histopathology and immunohistochemistry. A complex signal intensity mass on MRI (even those with well-defined smooth margins) in the retropharyngeal space with solid/cystic components, fluid-fluid levels, and heterogeneous postcontrast enhancement indicates a high possibility of a malignant pathology, even in children. Presence of adipose tissue in such a complex mass further raises the index of suspicion for malignancy and all such lesions should be treated aggressively to lower incidence of metastatic cervical lymphadenopathy and distant metastases.


Ameloblastic fibrosarcoma is an uncommon odontogenic tumor composed of a benign epithelial component and a malignant ectomesenchymal component most frequently seen in the third and fourth decades of life. It mainly presents as a painful maxillary or mandibular swelling. Radiographs show a radiolucent mass with ill-defined borders. Radical surgical excision and long-term follow-up are the suggested treatment. We report the case of a 22-year-old female with a 2-month history of an asymptomatic swelling in her left mandible. Examination revealed an exophytic growth measuring 3 × 3 cm extending from the mandibular left first premolar to the second molar. The patient underwent a left hemimandibular resection. Histopathological examination revealed a biphasic tumor composed of inconspicuous islands of benign odontogenic epithelium and an abundant malignant mesenchymal component with marked cellularity, nuclear pleomorphism, hyperchromatism, and moderate mitotic figures with clear margins; one year after the surgical procedure, the patient is clinically and radiologically disease-free. Odontogenic tumors and tumor-like lesions constitute a rare group of heterogeneous diseases that range from nonneoplastic tissue proliferations to malignant tumors with metastatic potential. They are derived from epithelial, ectomesenchymal, and mesenchymal elements of the tooth-forming apparatus. Malignant odontogenic tumors are classified as odontogenic carcinomas and odontogenic sarcomas . AmeloblA 22-year-old female presented with a 2-month history of an asymptomatic swelling in her left mandible. Extraoral evaluation revealed a gross swelling over the left mandible . Upon inFour months later a panoramic radiograph and CT scan of head and neck were performed disregarding locoregional and distant metastases while also revealing again the same ill-defined radiolucent lesion around an impacted mandibular left first molar . The patAFS was first reported by Heath in 1887 describing it as a spindle cell sarcoma that also had epithelial cells resembling the cells of the enamel organ . To the AFS can arise de novo or from a previous ameloblastic fibroma (AF). Kobayashi et al. suggest that up to one-third of AFSs arise from transformation of an AF , while LAF is the main differential diagnosis of AFS. Both neoplasms have a biphasic nature; however, AF has no malignant component, unlike AFS in which the mesenchymal component presents marked cellularity, nuclear pleomorphism, hyperchromatism, and a moderate to high number of mitotic figures. Immunohistochemical markers can be helpful to distinguish AFS and AF, and the mesenchymal component of AFS is positive for p53 and PCNA unlike the negativity for these stains in AF , 13. RegAFS has a reported recurrence rate of 37% and a mortality rate of 19% . Only 2 Our patient presented an AFS in the posterior mandible. Such location is the most frequent affected site reported in the literature. Her age at presentation was 22 years old, a little younger than the mean age of presentation, but still in the third decade of life, like most patients from previous reports. Her tumor was considered a de novo AFS since she did not have a history of previous AF. The origin of our patients AFS and her age are two important features to consider since they seem to further support the observation made by Noordhoek et al., claiming that de novo AFS tends to occur in younger patients than AFS arising from an AF. Histologically our case had very few islands of odontogenic epithelium compared to the extensive malignant mesenchymal component, a feature commonly seen. Although the immunohistochemical profile of this neoplasm was identical to the one described in the literature and helped to establish the diagnosis, we agree with Kobayashi and most authors, and believe the diagnosis is essentially made by histology.In summary, AFS is a rare malignant odontogenic tumor characterized by a benign odontogenic epithelium and a malignant mesenchymal component that can arise from a previous AF or de novo. Resection with a wide margin is the optimal treatment strategy and close follow-up is advised due to its relatively high recurrence rate.


Vascular abnormalities are characterized by increasing number of vessels. Salivary gland tumors are uncommon and their overall incidence is about 3 per 100000 per year. Salivary gland hemangioma makes up 1 % of all salivary gland tumors. Trismus resulting from parotid hemangioma is so rare. The patient was a 6-month-old boy with a huge lesion in his right parotid who referred to Shahid sadoughi hospital of Yazd, Iran. The lesion appeared at 4 months of age and had rapid growth and was suspected as hemangioma after clinical examination and patient had trismus. The lesion was excised without any complications. Five months after surgery, area of the lesion appeared normal. Parotid hemangioma has low potential to turn into malignant form but early detection and biopsy are necessary for decreasing complications. Removal of the mass was the best treatment for the patients with large or complicated hemangioma; however, it should be performed with caution because the tissues may bleed profusely. Patients may have long term survival after surgery. Hemangioam is a kind of endothelial malformation and can cause significant morbidity and mortality in children and adults. The term hemangioma is applied to describe a benign vascular (with growth phase) and self-limited tumor. A hemangioma can occur anywhere on the body. Mechanisms of controlling the growth of hemangiomas are not clearly understood. Infantile hemangiomas are characterized by proliferation of vessels within the first year of life. Parotid hemangioma is a benign rare lesion  and is mIn most cases, the cause of hemangioma is unknown but low birth weight probably is the major risk factor and most of hemangiomas are asymptomatic .The duraTreatment of a hemangioma isn't usually needed but the care of children with hemangioma is the most important part of the treatment .In this report, a case of parotid hemangioma was described and as far as we know it is the first case reported in Iran and a rare case in the world.A 6 month old male infant presented with a right-sided hemihypertrophy of the face in the preauricular region. He was referred to Shahid Sadoughi Hospital of Yazd, Iran, for evaluation and treatment of the swelling that it was appeared initially as a bright red swelling with rapid growth. The lesion began at 4 months of age with rapid growth. The size of tumor was 7×7×3.5.It was a mobile, soft, and bright red swelling in the region of parotid gland.Swelling's size with changing the position of the head did not change. No evidence of facial weakness or cervical lymphadenopathy was presented, however he had some degree of trismus. The patient was taken no medications for this condition before his treatment. When the mass failed to respond to a 3-week course of steroid treatment (and trismus continued), the patient underwent surgical resection of the parotid mass. The vascular lesion was excised and submitted for histopathological examination. Histologic examination of hematoxylin and eosin stained slides showed dilation, proliferation of vessels and A large number of thin-walled capillary vascular spaces containing red blood cells. The residual salivary gland within tumor was also seen in Hemangiomas are vascular abnormalities and characterized by increasing number of vessels. The salivary glands are responsible for making saliva and enzymes and releasing them into the mouth. Hemangiomas are classified as major  and minor. The salivary gland tumors are uncommon and allocated 2-4% of head and neck neoplasms to themselves. Most (70%) salivary gland tumors are in the parotid gland. Most ofCommon differential diagnosis hemihypertrophy of the face are hemangioma, malignant neoplasm, and abscess . If biopIn some cases, hemangiomas can be potentially life-threatening. Available treatments such as corticosteroids have variable responses and side effects . TreatinHemangiomas can be deep, superficial or segmental . Therefore various treatment modalities have been attempted and consequently, surgical resection is chosen as a safe procedure with lower relapse. Surgical excision during the proliferative phase should be avoided due to risk of major blood loss and injury to the facial nerve. Recently, bleomycin and oral propranolol have been introduced but no effective medical treatment has been reported for children with large and forming hemangiomas of the parotid gland , 13. In addition, propranolol and corticosteroids are safe drugs that can be used . Furthermore, in some cases resistance to propranolol in at least 9% of thepatients is seen. Despite the efficacy of propranolol, patients must be monitored in life-threatening hemangiomas like airway hemanigiomas. As for  In this case, the lesion wasn’t a segmental hemangioma. Since the first choice was medical treatment but it didn't work. So, it was performed surgical resection. This article demonstrated that hemangiomas in the parotid gland may be more resistant to therapy with corticosteroids so the surgical procedure can be a safe treatment. Despite different recommended modalities in managing parotid hemangiomas, in cases of huge malformations, removal of the mass is the best treatment for the patients and should be performed with caution because the tissues may bleed profusely. Therefore, patients may have long term survival after surgery.


Echinococcus multilocularis that is potentially fatal if left untreated. It primarily involves the liver but can be disseminated to other organs like the lungs and the brain by hematogenous route. Multiorgan involvement and the aggressive appearance of lesions make alveolar hydatid disease easy to confuse with a metastatic malignancy. For this reason, histopathological confirmation is essential for definite diagnosis. We present the imaging features of this disease in two patients in order to emphasize that these lesions can be easily misdiagnosed as malignancies.Alveolar hydatid disease or alveolar echinococcosis is a disease of the parasite  Echinococcus granulosus and seen worldwide, and alveolar echinococcosis (AE), caused by Echinococcus multilocularis and restricted to the northern hemisphere [Echinococcosis, also referred to as hydatid disease, is a general term used to define zoonosis caused by Echinococcus tapeworms, or cestodes. The two main types are cystic echinococcosis, caused bymisphere . Humans misphere , 2.A 40-year-old male patient presented to our hospital with complaints of right upper quadrant pain and weight loss. The patient was in good general condition, with no respiratory distress. Except for mild hepatomegaly, physical examination was completely normal. It was noted in the patient's detailed history that he lived in rural Eastern Anatolia. Hemogram results were normal, with no signs of peripheral eosinophilia.b values of 500 sec/mm2 show signal hyperintensity in the central areas of the mass  revealed a large heterogeneous solid mass lesion containing cystic and calcified areas in the right lobe of the liver. Thoracoabdominal computed tomography (CT) revealed a heterogeneous solid mass lesion measuring 15 × 10 cm that contained multiple hypodense cystic areas and calcified foci . T2-weigthe mass . Echinococcus multilocularis in the liver parenchyma that were consistent with hepatic alveolar echinococcosis  scan  scan . As the CT) scan . Liver tAlveolar echinococcosis is a disease with poor prognosis and may be fatal if left untreated , 2. SympAdrenal gland involvement in AE is very rare. Kamishima et al.  first retotal for type 3 lesions were 1.73 ± 0.41 × 10−3 mm2/s. Our ADC-values were consistent with Becce et al. [AE is diagnosed with a detailed history, physical examination, radiological imaging, and serological examinations . While aAs seen in both of our patients, contrast enhancement is not seen in liver lesions except the minimal enhancement in the peripheral zones. In the second patient, abdominal CT angiography clearly demonstrated the extensive collateral veins that had formed between the left renal and hemiazygos veins as the result of inferior vena cava constriction and also showed the right portal vein branch thrombosis.In case of brain involvement, cranial CT or MRI may reveal AE lesions –4. MRI iAE can be easily confused with primary hepatic malignancies and metastases and is misdiagnosed more often as compared to other helminth diseases , 3. Whil18F-FDG PET-CT has been considered as a reliable method for differentiation between inactive and active AE lesions [Recently,  lesions . In hepa lesions . Patient lesions . PeriopeIn conclusion, radiologic imaging findings may contribute to the diagnosis of alveolar hydatid disease. AE lesions may be misdiagnosed as malignancies, and it is important for radiologists to have good command of typical imaging features of AE and to provide early diagnosis for patients from high prevalence areas, thereby guiding clinicians in the correct direction.


To report the rare occurrence of peripheral retinal sea-fan neovascularization in a patient with chronic rhegmatogenous retinal detachment and describe the surgical management.A 29-year-old female patient was referred to our department by her ophthalmologist for investigation and treatment of peripheral retinal neovascularization in her right eye(RE). Visual acuity(VA) at presentation was 20/200 RE and 20/20 LE. Fundoscopy of the RE revealed a chronic inferotemporal retinal detachment and peripheral neovascularization with a sea fan configuration. Fundoscopy of the LE was without any findings. Fluorescein angiography confirmed the sea fan neovascularization in the RE with leakage of the newly formed vessels and peripheral ischemia while the LE did not demonstrate any neovascularization angiographically. Family history was negative for retinitis pigmentosa and haemoglobinopathies. Patient underwent full blood count and haemoglobin electrophoresis to exclude thrombocytosis and sickle cell anaemia, and serum angiotensin-converting enzyme (SACE) measurement to exclude sarcoidosis. Examination with scleral indentation of the RE revealed 2 peripheral small retinal holes close to the ora serrata . The patient underwent a scleral buckling procedure with a small segmental buckle limited to the area of the holes and cryotherapy. Ccryotherapy was not applied to the area with neovascularization and no subretinal fluid drainage was performed. The detached retina was successfully re-attached surgically and the subretinal fluid was gradually absorbed within three months from the time of surgery. Complete regression of neovascularization was evident 2 months postoperatively and VA improved to 20/30. Three years later the clinical and functional findings remain unchanged.Our case illustrates the rare but possible association of chronic retinal detachment with peripheral retinal sea-fan neovascularization; although the incidence is rare it may pose diagnostic and treatment dilemmas. In such cases and in the presence of retinal breaks, cryotherapy and a segmental buckle limited to the retinal holes and not on the neovascularization seems to suffice for the regression of the new vessels. Rhegmatogenous retinal detachment is defined as the detachment between neurosensory retina and the pigment epithelium layer, caused by a retinal break or hole which allows fluid from the vitreous cavity to enter the subretinal space. Its incidence is estimated to be 1/10,000 people per year [Although it is an acute, vision threatening condition, in rare cases it is presented in a chronic, slowly progressive form, which is not usually diagnosed until it has reached an advanced stage. Younger age and myopic refraction are factors related to the appearance of the chronic rhegmatogenous retinal detachment(RRD) , 3. PeriWe report a case of a 29-year-old female myopic patient with a chronic rhegmatogenous retinal detachment, combined with sea-fan retinal neovascularization and describe its course after surgical management with scleral buckling.A 29-year-old female patient was referred to our department for investigation and treatment of peripheral retinal neovascularization in her right eye(RE). She had been complaining of longstanding superior scotoma and a more recent decrease of RE visual acuity. She was a myope  with otherwise unremarkable ocular and general medical history. Her RE visual acuity at presentation was 20/200. Visual acuity of her left eye was 20/20. Fundoscopy of her RE revealed a chronic inferotemporal retinal detachment and significant neovascularization with sea-fan configuration. Left eye examination was unremarkable. Optical Coherence Tomography(OCT) Figure  and FluoChronic rhegmatogenous retinal detachment is a rare form of retinal detachment, with estimated prevalence estimated between 4, 5 – 29% . In rareSea fan neovascularization is the hallmark of sickle cell retinopathy, but it is also reported in medical conditions, such as thrombocytosis , sarcoidAngiogenic factors such as vascular endothelial growth factor(VEGF) and basic fibroblast growth factor(bFGF), have been demonstrated to trigger the development of sea fan neovascularization in cases of sickle cell retinopathy . In our Our case illustrates the rare but possible association of chronic retinal detachment with peripheral retinal seafan neovascularization which maybe located quite away from the retinal holes; although the incidence is rare it may pose diagnostic and treatment dilemmas. In such cases, cryotherapy and a segmental buckling limited to the retinal holes and not on the neovascularization seems to suffice for the regression of the new vessels.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.


Vascular malformations of the thyroid gland represent a very rare, often accidentally diagnosed, disease that in the case of eufunctional goitre may be the cause of mechanical neck syndrome. The authors present here the complex differential-diagnosis and treatment approach and stress the importance of histopathology for determining the final diagnosis.Using various imaging methods , the cause of breathing difficulties in a 64-year-old old man from the Czech Republic with normal thyroid gland function was found to be an arteriovenous malformation of the left lobe of his thyroid gland, 80×70×55mm in size, reaching retrosternally between the major arteries branching from his aortic arch and displacing his trachea 10mm to the right. In preparation for surgery, he underwent a radio-interventional procedure with embolisation of the arteries supplying the left lobe. This was followed by a lobectomy on the left via a partial sternotomy. The definitive histology result confirmed that the arteriovenous malformation was the benign cause of the mechanical neck syndrome.The case report presented here extends the differential diagnostic options in cases of mechanical neck syndrome. It describes a very rare disease of the thyroid gland, which prior to surgery may arouse suspicion of malignancy. It stresses the importance of close team cooperation between the endocrinologist, interventional radiologist and surgeon within the framework of preoperative diagnosis as well as preparation for surgery. Determination of the definitive histopathological diagnosis requires a pathologist experienced in such issues. The thyroid gland (TG) belongs to a group of endocrine organs, which produce hormones that participate in the management of many metabolic processes. Therefore, most diseases affecting this gland frequently involve functional disorders in the sense of either hypo or hyperfunction (hypothyroidism and hyperthyroidism respectively), and their treatment falls within the domain of endocrinologists. Such patients become candidates for surgical treatment only when conservative (medicamentous) treatment fails or when they develop mechanical neck syndrome, which may occur even in the case of glands with normal secretory activity . A second large group of TG diseases involves malignant tumours, which from the aspect of radicality always require surgical treatment. A less frequent group of TG diseases involves inflammation and these diseases are again managed by endocrinologists and may be the cause of secondary functional disorders.This article presents a very rare case report of a 64-year-old man with an unusual underlying cause of mechanical neck syndrome, demonstrating the complex differential-diagnostic and therapeutic approach along with the histopathological determination of the final diagnosis.A 64-year-old man from the Czech Republic with a history of hypertension was first examined by a cardiologist for intermittent cough, mild dyspnoea on exertion and non-constant hoarseness. He underwent an X-ray examination, which inadvertently revealed deviation of his trachea 10mm to the right. An examination specifically targeting his TG revealed the presence of goitre, with significant predominance of the left lobe. He was referred for specialist examination by an endocrinologist who performed an ultrasound (USG) of his neck. The right lobe of his TG was 20×21×49mm in size with normal findings and the left lobe of 80×70×55mm was described as hypoechogenic, lobulated, hypervascularised with propagation retrosternally. It included several cystic lesions and a vascular plexus located in its lower section with the diameter of its arteries reaching up to 12mm. No cervical lymphadenopathy was present. In order to further clarify these findings, the patient underwent multidetector computed tomography (MDCT) of his neck focusing on the TG. This described the left lobe as reaching retrosternally, significantly deforming and compressing the trachea with a large nodule located at its inferior pole, displacing the arteries of the aortic arch, with which it was in close contact  is performed with the aid of digital subtraction angiography (DSA), an invasive method with the option of selective contrast dye application and imaging of arteries supplying the lesion . Today, Therapeutic options include radio-interventional, surgical and combined approaches depending on the character and location of the AVM . BasicalAVMs of the TG are, in general, rare diseases and their preoperative diagnosis is usually difficult. Only one work has been published worldwide to date, by the Spanish authors Lizarralde and Diaz-Cano describing the issue discussed here involving two patients . Small Aet al.[TG AVM as part of the Wyburn-Mason syndrome was first described in the form of a unusual case report by the American authors Lee et al.. The Wybet al., 8. TG AMore frequent vascular malformations include haemangiomas, which on USG also give the impression of tumours, presenting as hyperechogenic nodules . In suchFrom a pathological point of view, AVM is completely benign and its location within the TG is very rare. Determination of the diagnosis in the case of small lesions is accidental. Clinically more significant manifestations include a mechanical syndrome that places the patient at risk of bleeding complications. Large and symptomatic AVMs require treatment, ideally a combination of radio-interventional embolisation of the arteries supplying the lesion and subsequent surgical resection.The case report presented here offers an extension of the differential diagnosis options in cases of mechanical neck syndrome, representing a very rare cause mimicking preoperatively a malignant disease. The authors stress the importance of close team cooperation between the endocrinologist, interventional radiologist and surgeon and point out the fundamental aspects of precise preoperative examination and preparation for the surgical procedure itself. The definitive determination of the histopathological diagnosis is not easy and requires a pathologist experienced in the given problem.I write the following to provide assistance to the case report written about my operation. I have no medical knowledge or background so I only write from my own perspective and experience.I began to have nonspecific symptoms as a cough, mild dyspnoea on exertion and non-constant hoarseness a half year ago. I was afraid of having a problem with my heart or lungs, so I decided to visit a cardiologist. The examination showed that my heart is healthy but the cause of my symptoms is deviation of trachea to the right side that was shown on X-ray. I was quickly sent to CT that revealed the possibility of AVM of the TG but did not exclude malignant disease. The same week I underwent the CTAG that gave a more precise finding and showed the main arteries supplying the AVM. The surgeons described the findings to me clearly and recommended the operation, because the findings could be danger by bleeding. They suggested the best way would be cooperation with a radio-invasive procedure which would close the main arteries before surgery and the operation will be for me safer. In 1 month I was received at the hospital, underwent selective intraarterial embolisation and 4 days later I was operated. After both proceuures I felt very well, I was very satisfied and surprised with non-problematic postoperative course. I was discharged home on 7th day after surgery.The best report I got 2 weeks after operation. It was the result of histological finding that revealed the benign disease. I had no problem with the healing of the wound; nowadays I have a nice scar. These days I go regularly to my endocrinologist, I began to take levothyroxine and my last visit was with ultrasonography of the rest of my TG and is OK.Now I admire all the specialists who shared in my treatment. They planned the procedures excellently so as to have no complications and my life could be the same as before surgery.Written informed consent was obtained from our patient for the 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.


Pulmonary hernia, also known as lung herniation or intercostal herniation, is best explained as the lung parenchyma protruding beyond the confines of the thoracic wall. This rare finding can be classified as congenital or acquired. Acquired pulmonary herniations are often the complication of blunt or penetrating trauma to the chest wall. This report describes a two-year-old male who fell onto a rigid post, striking his left lower chest. Imaging studies demonstrated a small pneumothorax as well as pulmonary herniation. The patient underwent a diagnostic thoracoscopy and repair of a pulmonary hernia within the 7th intercostal space without complication. In this case report, we aim to add to the limited body of existing literature on the surgical management of pulmonary hernias. Herniation of the lung is defined as the protrusion of pulmonary tissues through the confines of the thoracic cage . In the A two-year-old male fell onto a rigid post approximately one centimeter in diameter while playing. Physical examination of the left lateral inferior chest wall revealed a mass with paradoxical motion during respiration . There wRadiographs of the chest demonstrated a tubular lucency within the soft tissues of the 7th intercostal space. No pneumothorax or rib fractures were evident . ComputeThe patient underwent diagnostic thoracoscopy. Findings included an intact left hemidiaphragm as well as a small area of contusion to the lingular portion of the left lung. Associated with this was a disruption of the pleural lining and the overlying musculature within the 7th intercostal space . SurgicaThe patient was seen in follow-up seventeen months following repair. The parent reported good health since surgery, and the wound was well-healed. There was no evidence of recurrent hernia.Intercostal hernias, otherwise known as pulmonary hernias, are a protrusion of body cavity contents through adjacent ribs due to a defect located in the musculature . PulmonaAcquired hernias are usually associated with some type of blunt trauma to the chest wall. Blunt trauma resulting in herniation of the lung is extremely rare in the pediatric population. It is postulated that the elastic nature of a child's thoracic wall absorbs enough of the kinetic energy to prevent such herniation . It has On physical exam, symptoms can be very subtle or can speak for themselves. Since this is so rare, the diagnosis is often missed. If an intercostal mass is present, it can be expected to vary in size with respiration and to have paradoxical protrusion during expiration. The intercostal mass will be reducible and elastic and may be associated with an overlying crepitus . On chesTreatment options include observation or surgical repair. Controversy exists regarding which treatment option should be employed. Factors associated with repair include the location of the hernia and the presence of symptoms. Due to the small size of the defect, our patient was treated with suturing of the muscular fascia. Larger defects, however, may require repair with mesh. Some authors have reported that supraclavicular herniations may be observed, while intercostal herniations require immediate repair . It has In our case, we elected to proceed with surgical repair of the pulmonary hernia due to risks of future strangulation and the noted size and site of the defect. Though there is a limited body of literature on pulmonary hernia, it is reasonable to argue that entrapment of herniated lung may lead to ischemic complications of lung tissue . We also


Chyloptysis is a relatively rare embodiment of disease that encompasses a lengthy differential and provides many diagnostic and therapeutic challenges. Presented here is the case of a young woman with massive chyloptysis due to a thoracic cavernous lymphangioma arising in the peripartum period. The severity of her condition mandated the use of cardiopulmonary bypass to resect her lymphangioma. We believe that the extent of her symptoms, etiology of disease, and surgical management represent a unique scenario in the literature. The overwhelming majority arise during fetal development and childhood but there have been rare reported cases of such structures being unveiled later in life.2 The most widely accepted classification includes (a) cystic lymphangioma , (b) cavernous lymphangioma, and (c) lymphangioma simplex. Largely asymptomatic, cavernous lymphangioma can present a diagnostic challenge often requiring lymphoscintigraphy and magnetic resonance imaging (MRI) to identify. Once recognized and treated with surgical resection, as in our case, the long-term prognosis is excellent.Lymphangiomas account for up to 4.5% of mediastinal tumors.A 26-year-old Hispanic lady without antecedent history was admitted to the intensive care unit with acute respiratory failure and massive chyloptysis requiring mechanical ventilation. Two years prior she began to experience a persistent, frothy white productive cough, dyspnea, and fatigue following an uncomplicated caesarean section. Various pulmonary conditions, including recurrent community-acquired pneumonia, pulmonary alveolar proteinosis, granulomatous inflammation, and bronchial anthracofibrosis had been entertained and a VATS (video-assisted thoracoscopic surgery) lung biopsy revealed nodular perivascular anthracosis with chronic inflammation and local fibrosis. She had developed bilateral chylous effusions (right greater than left) and parenchymal consolidations. Infectious etiologies were meticulously ruled out. Chemical analysis of her sputum revealed a triglyceride level of 251 mg/dL, a cholesterol of 8 mg/dL, bilirubin <1 µg/mL, hemoglobin of 6 µg/mL, and the presence of a large quantity of chylomicrons. A lymphatic disorder was suspected and she underwent an exhaustive workup, including a high-resolution computed tomography (CT) scan of the head/neck/chest as well as an I-125 albumin lymphoscintigraphy  and 2. TShe was taken to the operating room and underwent thoracic duct ligation via a minimally invasive approach through the right hemithorax with the intent of treating an underlying thoracic lymphangiectasis. After a transient improvement in her condition and in her chylous output, she deteriorated rapidly and experienced multisystem organ failure and the decision was made to return to the operating room for a more extensive exploration. Given her circulatory collapse and hypoxemia, she could not tolerate single-lung ventilation. In addition, the planned diagnostic and therapeutic bronchoscopy would preclude the use of a double lumen tube and require prolonged periods of imperfect to inadequate ventilation. With this in mind and to provide optimal exposure to the proposed operative field, she underwent median sternotomy and was placed on cardiopulmonary bypass.Dissection of the mediastinum revealed a singular lymphatic structure arising near the take-off of the right carotid artery. It coursed posterior to the innominate vessels and branched above the right pulmonary artery with tributaries extending toward the right and left main stem bronchi. There was communication with the right middle and lower lobes  and no eInterpretation of the specimens by anatomic pathology described the lung parenchyma as having been nearly replaced by chyle-filled alveolar spaces consisting of lipoid material. Periodic acid–Schiff staining was negative thus ruling out pulmonary alveolar proteinosis. Further histopathologic investigation ascertained the presence of an abnormal proliferation of enlarged vascular channels lined with endothelium. Positive staining by D2-40 of these endothelial cells affirmed that these channels were indeed lymphatic proliferations -8.Cavernous lymphangioma usually originates in the head and neck or axilla and is characterized by its isolated location and benign proliferation as opposed to lymphagiomatosis, which is more aggressive, arises in multiple anatomic locations and is a more common cause of chyloptysis.3 In our review of the literature there were infrequent citations of this association and no series of patients with thoracic cavernous lymphangioma manifesting as chyloptysis. The largest and most important series of 25 lymphangiomas from the Mayo Clinic did not identify chyloptysis as a presenting symptom.1 Likewise, the use of cardiopulmonary bypass in this exigent setting represents a novel report in the literature. There have been case reports describing the use of cardiopulmonary bypass for the resection of primary cardiac cystic lymphangiomas but not for the resection of a primary thoracic cavernous lymphangioma.4Chyloptysis is a rare manifestation of lymphangioma. It is more typical of other lymphatic disorders such as the aforementioned lymphangiomatosis, lymphangioleiomyomatosis, thoracic lymphangiectasis, yellow nail syndrome, and iatrogenic injury.5 MRI may have identified a characteristic high-intensity lesion on T2-weighted images.The frustrating aspect of our case was the difficulty in diagnosis of the source of chyloptysis and localization of this lady’s lymphangioma. Lymphoscintigraphy has been the historical, noninvasive, imaging modality of choice. It is limited, however, by its low specificity and inability to pinpoint individual anatomic structures. More recently high resolution CT scan and MRI have best been used to diagnosis mediastinal lymphangiomas although most authors admit that radiographic diagnosis alone remains challenging and elusive.6 We believe this is the first documented case of a peripartum thoracic cavernous lymphangioma. Quack Loetscher et al6 postulated that this phenomenon is likely due to the presence of increased vessel-inducing factors, for example, vascular endothelial growth factor, during the course of pregnancy. We believe that our case fits the pattern previously described given the onset of chyloptysis following her caesarian section.As to the etiology of the patient’s disease, previous case reports have described lymphangiomas that arise during pregnancy. There have been 2 cases of axillary lymphangioma occurring in the third trimester and a single case of abdominal lymphangioma discovered during the 14th week.Although a benign disease, most thoracic lymphangiomas that come to light will become symptomatic over time and the treatment of choice is surgical resection. Complete resection portends an excellent prognosis with equivalent life expectancy in age matched populations. There have been reports of up to a 30% recurrence rate but given the paucity of large series, resection should remain the mainstay of treatment. With the presentation of chyloptysis, it is imperative to recognize a lymphatic disorder or injury and proceed with timely surgical intervention as necessary and not be forced to intervene under duress.


A patient with a congenital intralabyrinthine cholesteatoma is presented. High-resolution computerized tomographic scans and intraoperative photomicrographs display features of intralabyrinthine extension. We discuss pathogenetic theories for the development of congenital intralabyrinthine cholesteatoma. The distinction of this condition from congenital cholesteatoma with labyrinthine erosion is discussed.  The presence of a white spheroid mass in the anterosuperior mesotympanum seen through an intact tympanic membrane in an otherwise asymptomatic patient, without prior history of otologic surgery, is diagnostic for congenital cholesteatoma. Ossicular erosion can occur and in unusual cases, extension into the mastoid can be seen . CongeniWe describe the first known case of a patient with congenital intralabyrinthine cholesteatoma. High resolution computerized tomographic (HRCT) scans of the temporal bone and intraoperative photomicrographs display features of intralabyrinthine origin and extension. We discuss pathogenetic theories and discuss the distinction of congenital intralabyrinthine cholesteatoma from congenital cholesteatoma with labyrinthine erosion.A 27-year-old male presented with a long history of right hearing loss. There was no history of tinnitus, disequilibrium, vertigo or facial paresis/paralysis. The past medical history was significant for trauma to the head from a basketball at the age of two. There was no history of fracture or concussion. There was no family history of conductive hearing impairment.Otoscopic examination revealed normal tympanic membranes with no visible mass in the mesotympanum. The remaining parts of the otolaryngologic examination were normal.Audiometry revealed a right moderate conductive hearing loss with excellent speech discrimination scores and an absent right stapes reflex to ipsilateral and contralateral stimulation . A HRCT A mastoidectomy operation revealed a “bony cap” over the labyrinth. When the “cap” was removed, cholesteatoma was seen filling the horizontal and superior semicircular canals Figures  and 4. TPostoperatively, the patient experienced a temporary vestibulopathy and right anacusis. Pathologic examination revealed cholesteatoma. During a second-look procedure, a formal labyrinthectomy was performed with removal of additional fragments of cholesteatoma. Following the latter procedure, the vestibulopathy nearly resolved.An astute clinician can make the diagnosis of congenital cholesteatoma. A white mass, in the anterosuperior mesotympanum seen through an intact tympanic membrane, in a patient with no prior history of otologic surgery is diagnostic for this condition. Congenital cholesteatoma can cause ossicular erosion and conductive hearing impairment; however, labyrinthine erosion is rare. Labyrinthine involvement more commonly consists of focal erosion of the horizontal or superior semicircular canal and exposure of the membranous labyrinth. Cholesteatoma matrix can be seen contacting the membranous labyrinth. It is thought that the mechanism of otic capsule erosion is enzymatic destruction or pressure-related remodeling.Labyrinthine invasion and intralabyrinthine extension, as seen in our case, is exceedingly rare. In our opinion, the tiny volume of middle ear cholesteatoma contiguous with the intralabyrinthine component seen in our case helps confirm an intralabyrinthine site of origin. Spingarn et al.  report oMany theories have been popularized to explain the genesis of congenital cholesteatoma. Incomplete involution or persistence of the epidermoid formation in the middle ear cleft is the most widely accepted theory , 7–9. LeThese aforementioned theories do not explain the mechanism of entry and extension within the labyrinth. During the third week of embryogenesis, the otic placode, a thickened area of ectoderm adjacent to the rhombencephalon, forms. By the fourth week of development, this otic placode invaginates to form the otocyst, the precursor of the membranous labyrinth. Surrounding neural crest and mesodermal tissue form the otic capsule. Perhaps in congenital intralabyrinthine cholesteatoma, the process of invagination may entrap pluripotential cells that later differentiate into keratinizing squamous epithelium and lead to intralabyrinthine cholesteatoma.Hearing preservation in congenital cholesteatoma with labyrinthine erosion has been reported . In thesDiagnosis of congenital intralabyrinthine cholesteatoma should be suspected in patients with unilateral conductive hearing loss, as in our case, or anacusis. A mesotympanic mass may not be seen behind an intact tympanic membrane. This case underscores the importance of HRCT imaging in patients with conductive hearing impairment. In congenital intralabyrinthine cholesteatoma, HRCT imaging may reveal dilated intralabyrinthine spaces. The conductive hearing impairment is presumed to be of inner ear origin. Vestibulopathy may or may not be present depending on the degree of central vestibular compensation.The diagnosis can only be confirmed intraoperatively. Resulting anacusis can be habilitated with use of CROS  technology or with osseointegrated implants. Postoperative vestibulopathy resolves with central vestibular compensation. In some cases, vestibular rehabilitation may hasten recovery.Congenital intralabyrinthine cholesteatoma is exceedingly rare. Suspicion should be aroused in patients with unilateral conductive hearing loss or anacusis. HRCT of the temporal bone can reveal a lesion within the labyrinth with dilation of the intralabyrinthine space. It is the author's opinion that, in most cases, hearing preservation with surgery is not possible.


Pulmonary glandular papillomas are rare neoplasms, and their very slow or absent growth over time generally facilitates establishing the diagnosis.In an 84-year-old woman who underwent surgery for sigmoid colon cancer, a growing solitary pulmonary nodule was identified on postoperative follow-up computed tomography. A computer tomography-guided needle biopsy was performed under suspicion that the nodule was malignant. The histopathological findings suggested a glandular papilloma. Right basilar segmentectomy was carried out, and the lesion was completely resected. Postoperative histopathological examination revealed a benign glandular papilloma accompanied by mucus retention in the surrounding alveolar region.A malignant neoplasm is usually suspected when a pulmonary tumor shows rapid growth. However, glandular papillomas associated with mucus retention also tend to grow in some cases, and should be included in the differential diagnosis. Solitary pulmonary papillomas are rare neoplasms usually arising from the bronchial surface epithelium and forming endobronchial tumors-3. Most An 84-year-old woman was found to have an abnormal shadow on a chest X-ray during postoperative follow-up for colon cancer Figure , and wasIn situ hybridization for human papilloma virus DNA was not performed in this case because there had been no reports of malignant transformation of glandular papillomas so far.Macroscopically, a whitish mucous nodule measuring 17 mm in diameter with a yellowish endobronchiolar tumor approximately 5 mm in diameter in the center was observed Figure B. HistopThe patient had an uneventful postoperative course. At present, 12 months after lung surgery, she remains recurrence free.Papillomas of the lung have two clinical presentations: multiple and solitary,4. SolitGlandular papillomas occur predominantly in the central tracheobronchial tree,11. TheyIt might be difficult to distinguish a glandular papilloma from active inflammation, granulomatous disease, carcinoid, and lung cancer based only on imaging findings. BronchoAlthough there are occasional recurrent cases, most glandular papillomas are cured by excision only. There hWritten informed consent was obtained from the patient for the publication of this case presentation and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.CT: computed tomography; PET: positron emission tomography.The authors declare that they have no competing interests.SS and TG wrote the manuscript. SS and TG performed the surgery. KE and YH carried out the pathological examination. TG was involved in the final editing. All authors approved the final manuscript.