Atypical teratoid rhabdoid tumor (ATRT) is a rare and highly aggressive embryonal tumor of the central nervous system with a dismal prognosis and no definitive guidelines for treatment, especially at relapse or in case of refractory disease. Metronomic chemotherapy (MC) has emerged as a new treatment option in solid malignancies, with lower toxicity and is frequently combined with drug repositioning. We report a case of ATRT in an 8-year-old boy who progressed during multimodal therapy including surgical resection, chemotherapy and radiotherapy. He was treated with MC involving continuous oral celecoxib with alternating metronomic etoposide and cyclophosphamide, in combination with biweekly bevacizumab and monthly intrathecal liposomal cytarabine. To date, he remains clinically and symptomatically disease-free with a follow-up of 10 months. The treatment was well-tolerated. Metronomics represent a possible alternative regimen for children with recurrent or progressive ATRT. Atypical teratoid rhabdoid tumor (ATRT) is a rare and highly aggressive embryonal tumor of the central nervous system with a dismal prognosis . BecauseA recent review of the US Cancer Database from 2004 to 2012 shows that trimodality therapy seems to offers the best outcome . AnotherMetronomic chemotherapy (MC) is an emerging alternative approach to conventional chemotherapy, relying on more frequent and low-dose drug administrations with minimal drug-free breaks. It is a multi-targeted therapy which targets tumor angiogenesis, increases the anticancer immune response and induces tumor dormancy . MC is fHerein, we report a case of refractory metastatic ATRT which was successfully put into sustained complete remission with metronomics.The patient, an 8-year-old boy without significant past medical history, presented with seizures in October 2015. Initial magnetic resonance imaging (MRI) examination revealed a heterogeneously enhanced solid and cystic mass with hemorrhagic areas in the left frontal cortex. The solid part of the tumor showed isointense signal with heterogeneous enhancement on T1-weighted images and slightly hyperintensity on T2-weighted images. There was MRI evidence of tumor in the intracranial leptomeninges at diagnosis. On admission, a partial tumor resection was performed. Pathologic diagnosis of ATRT grade IV of the World Health Organization (WHO) was confirmed by immunohistochemistry demonstrating loss of nuclear expression of INI1. No malignant cell was found into the cerebro-spinal fluid (CSF).2 D1), cyclophosphamide (800 mg/m2 D1–D3), and doxorubicin  but he progressed rapidly. In December 2015, the patient presented with clinical neoplastic meningitis, confirmed by identification of circulating tumor cells in the CSF and leptomeningeal enhancement with no local evolution of the tumor on the brain and spinal MRI. Patient received a second line of chemotherapy consisting in cisplatin (20 mg/m2 D1–D5), etoposide (100 mg/m2 D1–D3), and ifosfamide (1.5 g/m2 D1–D5) while waiting for radiation therapy. Radiation therapy was started 2 weeks later with a craniospinal irradiation of 36 Gy and a focal boost of 54 Gy. The follow-up MRI a month after the end of radiotherapy was clear. Patient received a second course of chemotherapy consisting in cisplatin, etoposide, and ifosfamide. In April 2016, the patient relapsed with neoplastic meningitis and presented headaches and mild alteration of consciousness MC was started right away according to the MEMMAT-derived protocol (Table 1).Because of the aggressive nature of the tumor a second surgery for further resection was performed on admission day 20. Chemotherapy was then started. The patient received two courses of chemotherapy with vincristine . No circulating tumor cells could be found in the CSF any longer.Symptoms rapidly subsided. MRI showed regression of the leptomeningeal enhancement a month after starting therapy and cleared up at month 4  and so is CSF examination. We plan to keep him on treatment for 12 months.He has had no complaints and has resumed normal activities, including going to school and participating in physical education. At last follow up, brain and spinal MRI is clear (More clinical experience of MC in pediatric oncology has been gained over the past few years . Peyrl eMetronomic chemotherapy is generally well-tolerated in trials, with reports of predominantly mild toxicities. But careful patient selection is key; a recent study exploring the effect of metronomic thalidomide, etoposide, and celecoxib in children with various refractory or high risk tumor showed an improvement in quality of life measured by the Karnofsky-Lansky scores, although the majority of patients declared grades III–V adverse effects . The mosHow the metronomics combination have led to achievement of a complete response in a refractory/relapsing disease can only be speculative and surely rely on several mechanisms. Indeed, MC has been reported to be able to inhibit angiogenesis as well as restore the anticancer properties of the immune system . MoreoveLong term and high volume trials of metronomic chemotherapy in ATRT are not available. We offer experience in a complete and sustained clinical-radiological response to MC in a case of early-relapsing ATRT.This patient with a refractory disease for which no guideline at relapse are available was treated on a case to case basis. Consent was obtained from both parents and the child.NA was in charge of the patient, directed the work and wrote the manuscript. MB wrote the manuscript. LP was the treating radiotherapist and checked the final version of the manuscript. AR was in charge of the patient and checked the final version of the manuscript. GP-G was the surgeon in charge of the patient and checked the final version of the manuscript. DF-B was the neuro-pathologist in charge of the patient and checked the final version of the manuscript.The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.


Sinonasal inverted papilloma is a locally aggressive tumor arising from the Schneiderian membrane which lines the nasal cavity and paranasal sinuses. Aggressive surgical approaches, such as lateral rhinotomy, were used until recently for complete removal of the inverted papilloma. Currently, endoscopic resection is the gold standard in the treatment of inverted papilloma. However, there are situations that justify an open approach. For example there are studies that report a higher postoperative recurrence rate after endonasal endoscopic resection, particularly in the treatment of recurrent diseases. While endoscopic resection performed by an experienced surgeon is definitely a minimally invasive therapy, an open approach is not necessarily associated with functional and aesthetic disadvantages.This case report describes the treatment of inverted papilloma by an open approach. This has been described before but the new gold standard of endoscopic resection has to be taken into account before any treatment decision is made nowadays.2 polycyclic arranged mucosal thickening with cystic and solid contrast affine shares at the antral laterocaudal area of his right maxillary sinus, extending from his right lateral nasal wall to his maxillary sinus floor.Contrast-enhanced magnetic resonance imaging of the head and neck area was indicated in a 72-year-old white German man who presented with suspected squamous cell carcinoma of his lower lip. Magnetic resonance imaging additionally revealed a 3×2 cmHe received antral polypectomy with medial maxillectomy via a unilateral LeFort I osteotomy approach. His pterygoid plate was preserved. A histological examination demonstrated a tumor composed of hyperplastic squamous epithelium protruding into the stroma , thus producing a grossly convoluted cerebriform appearance. Two weeks later, the patient regained a well-formed maxilla without any restrictions. He has remained disease-free for 25 months following the surgery and surveillance was continued in our tumor clinic.Endoscopic resection of an inverted papilloma continues to be the gold standard. However, some cases require a radical approach. This does not necessarily increase patient morbidity. Inverted papilloma (IP) is an uncommon lesion that accounts for less than 0.5 to 4 % of all sinonasal tumors . The treA 72-year-old white German man presented with a 5-month history of a growing lip mass. A review of his medical history revealed that he had arterial hypertension, diabetes mellitus, peripheral arterial disease, and a one pack-daily cigarette smoking history. Four years earlier, he underwent a resection for prostate carcinoma.2 polycyclic arranged mucosal thickening with cystic and solid contrast affine shares at the antral laterocaudal area of his right maxillary sinus, extending from his right lateral nasal wall to maxillary sinus floor. There was no evidence of bony erosion or other cervicofacial lesions. Under general anesthesia, he received the following procedures: an excisional biopsy at the lip lesion with 5 mm safety margins and bilateral sentinel lymph node biopsy, and antral polypectomy with medial maxillectomy via a unilateral LeFort I osteotomy approach with preservation of his pterygoid plate. Histological examination of the hematoxylin-eosin specimens demonstrated well-differentiated squamous cell carcinoma of the lip (not shown). The antral lesion was a tumor composed of hyperplastic squamous epithelium protruding into the stroma , thus producing a grossly convoluted cerebriform appearance.A clinical examination revealed no abnormalities other than a firm mass on the right side of his lower lip Fig. , withoutHe was hospitalized for 2 weeks (12 days) after the surgery and did well postoperatively. Two weeks later, he regained a well-formed maxilla without any restrictions. Eleven months after our therapy, a cutaneous malignant melanoma on his right upper arm was diagnosed  and he underwent a tumor resection and axillary sentinel lymph node biopsy in the clinic for dermatology of the University of Leipzig. He has remained disease-free for 25 months following the first surgery and surveillance was continued in our tumor clinic. This case substantiates our procedure of radical resection.Our patient presented with an asymptomatic IP of his maxillary sinus accidentally found with squamous cell carcinoma of his lower lip. This case required particular attention because a carcinoma could have been associated with the IP. A relatively aggressive approach for IP was, therefore, applied at the same time as the cancer surgery.Currently, no generally accepted treatment for IP of the sinonasal area exists. Some authors prefer an endonasal micro-endoscopic removal , while oIt is very important to correctly diagnose a tumor in its early stage to cure or treat the patient as soon as possible. A wrong diagnosis for IP has fatal consequences for patients. It must be decided from case to case which treatment is the best one for the patient. If a tumor is detected at an early stage and in an accessible location, endonasal micro-endoscopic surgery is a very elegant solution and can be done by any accordingly trained surgeon, mostly an otorhinolaryngologist. To achieve low recurrence rates of IP (and oncocytic papilloma), surgeons performing an endoscopic approach should consider drilling, cauterizing, or completely excising the bone underlying the tumor base instead of mucosal stripping alone . WheneveCurrently, the treatment for IP is surgical but the type of surgical approach is still controversial. Endoscopic surgery has become the gold standard for the treatment of the vast majority of IP. Antral polypectomy with medial maxillectomy via a unilateral LeFort I osteotomy is much more aggressive than the other approaches but might be justified especially in higher stages of disease . There iAnother discussion is about the possible malignant transformation of IP. Some authors also recommend radiotherapy for advanced and/or recurrent papillomas . This ofMore studies have to be performed in order to analyze and record the recurrence rates and complications for different approaches , 8. TodaIn the light of the above-mentioned options and the different specialties possibly involved we strongly recommend treating IP within a multidisciplinary tumor board setting. Any treatment choice should only be made under consideration of the above-mentioned options of surgery (either endoscopic or open) and/or radiotherapy. In particular, an endoscopic surgeon needs to be “on board” since whatever treatment choice is made the endoscopic technique is always to be considered for at least preoperative biopsy and follow-up .Endoscopic resection of an IP continues to be the gold standard. However, some cases require a more radical approach. This does not necessarily increase patient morbidity. Even so, endoscopic resection offers a great new treatment option; radical surgery is definitely not obsolete. Taking into account that radiotherapy is also worthy of discussion, a multidisciplinary discussion of any case is strongly recommended.


Extranodal natural killer/T-cell lymphoma (ENKTCL) is a rare form of non-Hodgkin lymphoma. This neoplasm is more prevalent in regions of Asia and Latin America and most commonly involves the sinonasal tract, presenting with signs of nasal obstruction, epistaxis, or sinus infection. It is a locally destructive and angioinvasive neoplasm. The treatment of ENKTCL is dependent on the extent of the tumor. For localized disease, the treatment is chemoradiation. For disseminated disease, treatment is mainly chemotherapy-based. This report describes a case of a 41-year-old Hispanic woman who initially presented with signs of nasal congestion for four weeks and was subsequently diagnosed and treated for chronic sinusitis. The patient underwent endoscopic surgery for persistent chronic sinusitis, with a presumptive diagnosis of allergic fungal rhinosinusitis based on clinical and radiographic presentation. The pathologic exam revealed a diagnosis of ENKTCL. The patient underwent three cycles of chemotherapy comprised of steroid (hydrocortisone), methotrexate, ifosfamide, pre-asparaginase, and etoposide (SMILE) followed by radiation, resulting in clinical and radiographic remission. On review of the literature, ENKTCL is very rare in the United States and diagnosis is commonly delayed due to non-specific signs. We report this case to increase awareness of this disease entity and remind clinicians to include this in the differential diagnosis of nasal obstruction.  Extranodal natural killer/T-cell lymphoma (ENKTCL), previously known as lethal midline granuloma, is a rare form of non-Hodgkin lymphoma. It comprises 7-10% of all non-Hodgkin lymphomas in Asians and Latin Americans but only 1% of Caucasians worldwide. ENKTCL most often involves the nasal cavity but rarely may also involve the gastrointestinal tract, skin, soft tissue, or testes. This neoplasm can take on the phenotype of either natural killer (NK) or T-cells and is virtually always Epstein-Barr virus (EBV)-positive. This tumor is locally destructive and invasive and carries a poor prognosis due to non-specific presenting signs that often delay diagnosis . CurrentA 41-year-old Hispanic woman presented to our otolaryngology office complaining of severe congestion associated with postnasal drainage, mucorrhea, and frontal headaches with photophobia and phonophobia for two month's duration. Additionally, she complained of loss of smell and taste. One month previously, she was treated empirically for chronic rhinosinusitis with a six-day pack of Sterapred, 5 mg, Keflex, 500 mg twice daily for two weeks, and Dymista (azelastine hydrochloride and fluticasone propionate), 137/50 mcg once daily.On examination, the septum deviated to the right and turbinates were edematous. No palpable cervical lymphadenopathy was noted and the remainder of the head and neck exam was unremarkable. Nasal endoscopy revealed a polypoid mass in the region of the left nasal cavity and thick mucorrhea was noted.A computed tomography (CT) scan showed opacification of the left frontal sinus  Figure , left maBased on the CT scan findings and failure to respond to conventional treatment, allergic fungal rhinosinusitis was suspected. The patient was scheduled for left-sided image-guided endoscopic sinus surgery and started on 60 mg of prednisone for five days. Appropriate consent was obtained, and preoperative labs were unremarkable. The patient successfully underwent left-sided endoscopic sinus surgery consisting of a left frontal sinusotomy, left ethmoidectomy, and left sphenoidotomy with some resolution of the headache and congestion. Although allergic fungal rhinosinusitis was suspected initially, the intraoperative appearance of the tissue removed did not support this diagnosis. All specimens were sent to pathology for gram stain and cultures, which were negative.The histopathology report showed an atypical lymphocytic infiltrate of mostly large cells with an angiocentric pattern Figure . ImmunohSubsequently, the patient underwent further imaging for any evidence of distance metastases; positron emission tomography-computed tomography (PET-CT) and magnetic resonance imaging (MRI) were all unremarkable. One month after her diagnosis she was started on steroids (hydrocortisone), methotrexate with leucovorin, ifosfamide, pre-asparaginase, and etoposide (SMILE). She received three cycles of treatment followed by local radiation. The patient tolerated chemotherapy with the expected side effects of pancytopenia, drug-induced hepatitis, and hypotension. Biopsy of ethmoidal and maxillary sinus tissue after completion of two rounds of chemotherapy showed no evidence of lymphoma. PET scans at one-year post-treatment showed no evidence of lymphoma. Subsequent CT scans have also been negative for local recurrence Figure .ENKTCL is a rare form of non-Hodgkin lymphoma more common in the regions of China, Japan, Korea, Mexico, and Peru than in North America. ENKTCL most commonly involves the sinonasal cavity and has a 3:1 male to female ratio with a mean age of onset of 52. The etiology of this tumor is uncertain; however, it is known to have a strong association with EBV infection. The tumor is composed of mostly active NK cells and some cytotoxic T-cells [ENKTCL, nasal type, occurs in the upper aerodigestive tract involving the nasal cavity, nasopharynx, paranasal sinuses, hypopharynx, and larynx. The typical initial presentation is with non-specific symptoms, such as nasal obstruction, epistaxis, and facial swelling . As in tFlexible and rigid nasal endoscopy can be used to visualize the nasal cavity, and biopsies should be performed if there is any suspicion of a destructive pathology . In ordeHistologically, ENKTCL shows an angiocentric pattern of growth with vascular destruction and necrosis. Tumor cells range from small to large and are dispersed among plasma cells, lymphocytes, eosinophils, and histiocytes. Flow cytometry and immunohistochemistry stains demonstrate the presence of T and NK cell markers CD2, CD56, CD3), along with cytotoxic molecules, such as granzyme B, TIA-1, and perforin. Unlike T-cells, NK cells do not display surface CD3. The case reported was positive for CD3 and CD2, confirming T-cell origin, but negative for CD56. This neoplasm was unique in that it was more represented by T-cell phenotype as opposed to NK cells, the typical phenotype [, CD3, alOn imaging, ENKTCL nasal type can be seen as diffuse mucosal thickening along the nasal turbinates or as a destructive midline mass. This can be difficult to distinguish from a fungal infection, Wegener’s granulomatosis, or other neoplasms . CT is rIn the case reported above, the patient was suspected of having fungal sinusitis from the CT imaging. Tumor staging was not done prior to surgery; however, a PET-CT and MRI were performed after tissue diagnosis and were negative for local and distant disease.The treatment for ENKTCL has evolved over the years. Previously, a cyclophosphamide, doxorubicin, vincristine, and prednisolone (CHOP) therapy was used with poor results due to NK cells displaying high levels of P-glycoprotein that confers resistance to anthracyclines . The curENKTCL is an aggressive neoplasm with a five-year survival of 40-59% following treatment. However, 30-40% of patients show relapse of the disease; most recurrences occur within the first two years . The patIn summary, we describe a case of an aggressive, frequently lethal lymphoma, rarely seen in the United States in order to increase awareness and aid in the diagnosis. ENKTCL presents with non-specific signs and symptoms that can lead to misdiagnosis and delayed treatment, so practitioners must include this in their differential for suspected chronic sinusitis that does not respond to medical therapy. Current treatments include asparaginase-based chemotherapy regimens (SMILE) and radiation. If diagnosed early, this devastating disease can be treated successfully.


We present a case of a combined hamartoma of the retina and retinal pigment epithelium associated with a subfoveal acquired vitelliform lesion induced by vitreomacular traction. The purpose of this report is to present a unifying hypothesis of these concurrent findings, as aided by multimodal imaging.A 25-year-old white man presented with a 6-month history of a visual disturbance in his left eye. At presentation, ophthalmic assessment showed a combined hamartoma adjacent to his optic nerve that had caused marked corrugation within the inner retinal surface. An acquired vitelliform lesion was present in the macula with an associated epiretinal membrane as demonstrated on spectral-domain optical coherence tomography. Optical coherence tomography angiography corroborated the clinical diagnosis of combined hamartoma.We are not aware of previous cases of a combined hamartoma associated with an acquired vitelliform lesion. As previously proposed in acquired vitelliform lesions related to epiretinal membrane and vitreoretinal traction, we believe that macular tractional forces might interfere with retinal pigment epithelium phagocytosis of shed outer segments, leading to the occurrence of this acquired vitelliform lesion. Combined hamartoma of the retina and retinal pigment epithelium (henceforth referred to as combined hamartoma) was first described by Gass, in 1973, as an uncommon tumor in a series of seven patients , 3. SeveIn the largest studied series of combined hamartomas (79 eyes from a single institution) Shields et al. found that common clinical characteristics included intralesional corkscrew vessels (65 %), retinal traction (81 %), and epiretinal membrane (61 %) . AccordiA healthy 25-year-old white male presented complaining of a gradual and progressive visual disturbance in his left eye for 6 months. His Snellen best corrected visual acuity was 20/20 in each eye and the anterior segment examination was unremarkable bilaterally. Dilated fundoscopy of the right eye was normal; fundoscopy of the left eye showed a slightly elevated greyish lesion with scattered pigmentary changes superior to the optic nerve which extended in a fan-like distribution from the optic nerve Fig. . There wSD-OCT through the tumor showed thickening and disorganization of the inner and outer retina with marked saw-tooth like corrugations along the vitreoretinal interface Fig. . The subFundus autofluorescence showed intense hyperautofluorescence from the vitelliform lesion as well as granular hypoautofluorescence inferotemporal to the lesion Fig. . A lineaOCTA revealed significant distortion of the retinal microcirculation intralesionally along the radial peripapillary capillary network superior and superotemporally to the optic nerve head with vessels appearing fine and tortuous in a corkscrew pattern Fig. . SurrounSimilar to the description of combined hamartomas by Gass in 1973 . In a siThese observed clinical characteristics are confirmed and further elaborated with the aid of multimodal imaging, including novel techniques such as OCTA, which allowed for depiction of the tumor’s vasculature without the need for intravenous dye injection and without the limits in resolution arising from extravasation of fluorescein dye . OCTA coOur patient manifested many of the characteristics mentioned above. However, he presented uniquely with a subfoveal acquired vitelliform lesion in the setting of traction occurring at the vitreoretinal interface. Acquired vitelliform lesions are known to occur in tractional maculopathies in which significant vitreomacular traction is present , 17. ForA combined hamartoma can cause visual impairment in several ways, but vision loss most commonly occurs when retinal dragging causes foveal distortion , 14, 21.Diffuse retinal folds seen emanating from the tumor and extending over the macula indicate the presence of significant macular contraction where the subfoveal vitelliform lesion lies. SD-OCT served as a diagnostic adjunct to further localize the area of traction occurring along the vitreoretinal interface as evidenced by the presence inner retinal corrugations with an overlying epiretinal membrane. AVLs have been noted to occur in tractional maculopathies caused by epiretinal membranes, and we believe that the tractional forces described above resulted in the formation of an AVL in our patient. We additionally attribute the epiretinal gliosis and subsequent traction on the inner retinal vessels to have caused the linear retinal hemorrhage seen in the superior macula.Both combined hamartomas and AVLs are capable of compromising vision if the integrity of the ellipsoid is compromised, either through direct traction or due to late outer retinal changes often seen in acquired vitelliform lesions. Despite the significant retinal striations and contraction seen in our patient, his foveal architecture remained generally undisturbed with relative sparing of the outer retinal landmarks accounting for his preserved visual acuity. Furthermore, as our patient did not exhibit any evidence of edema or CNV in association with the combined hamartoma, there was no rationale for the use of anti-inflammatory agents or antiangiogenic injections. Given these findings and the lack of consistent favorable outcomes after surgical intervention, close observation was recommended to the patient.In summary, the advent of novel imaging modalities including SD-OCT has allowed for detailed characterization of combined hamartomas, particularly at the vitreoretinal interface. We are not aware of previously published OCTA imaging of combined hamartomas, which, in our patient, revealed features of the tumor’s microvascular morphology hitherto only seen in histopathological studies.Written informed consent was obtained from the patient for publication of this Case report and any accompanying images.


Endobronchial carcinoid tumors are the most common intrabronchial tumors in children and adolescents. Common signs and symptoms include persistent cough and wheezing not responsive to bronchodilators, hemoptysis, and recurrent fever. Diagnosis is frequently made by imaging and direct visualization with flexible bronchoscopy; surgery remains the gold standard treatment, and lung-sparing resections should be performed whenever possible. Though carcinoid syndrome—characterized by flushing, palpitations, wheezing, shortness of breath, and diarrhea—has been found in association with adult bronchial carcinoid tumors, to our knowledge only one previous study has reported the presence of carcinoid syndrome in a pediatric patient with an endobronchial carcinoid. Here, we report a case of a 14-year-old girl with chronic cough found to have an endobronchial carcinoid tumor and signs and symptoms consistent with carcinoid syndrome. Pulmonary tumors in the pediatric population are rare and account for only 0.2% of all cases of childhood cancer . CarcinoA 14-year-old girl was referred to a large, regional tertiary children's hospital for evaluation of chronic cough and fatigue. She complained of a 6–8-month history of fatigue and a 2-3-month history of chronic cough that was precipitated by a viral illness. She described her cough as dry and nonproductive. A trial of inhaled corticosteroids and albuterol led to minimal improvement in her cough, and her symptoms persisted despite a five-day course of azithromycin. She also complained of low-grade fevers that occurred 1-2 times per week, night sweats, headaches, palpitations, chest pain, dyspnea, intermittent facial flushing, and diarrhea. Her past medical history was notable for mild allergic rhinitis. Her family history was significant for maternal thyroid disease but negative for other known autoimmune, oncologic, or chronic lung diseases. She lived in the Pacific Northwest, with frequent travel to the Midwest, and had exposure to horses and cows. There were no other travel or other known exposures to smoke or infectious agents. On physical examination, she had a dry cough but was not tachypneic and her oxygen saturation was 96% in room air. Additional pertinent negative exam findings included the absence of wheezes, crackles, or a prolonged expiratory phase on respiratory exam, no digital clubbing, and no cervical or supraclavicular adenopathy.3  with a neutrophil predominance, a c-reactive protein of 0.9 mg/dL , and negative virologic and serologic testing for Epstein-Barr virus, Cytomegalovirus, Mycobacterium tuberculosis, Cryptococcus neoformans, histoplasmosis, and Coccidiomycosis. A chest X-ray was obtained and was concerning for a mediastinal mass along with left upper lobe consolidation concerning for infection. A computed tomography (CT) scan of the chest demonstrated a soft-tissue density protruding into the distal aspect of the left main bronchus with fluid filling the distal left upper lobe airway . Pediatric thoracic surgery performed an open lobectomy and sleeve resection of the left upper lobe due to the mass location at the distal portion of the left upper lobe bronchus; a concurrent sampling lymphadenectomy was also performed. The tissue was sent to pathology which confirmed the presence of a unifocal, well-differentiated, typical endobronchial carcinoid tumor without lymph-node metastasis, extensive chronic obstructive pneumonitis, and acute bronchopneumonia. Her postoperative course was uncomplicated, and one month following the procedure she had no further cough, dyspnea, facial flushing, or fatigue.The patient was admitted to the hospital and subsequently underwent a diagnostic flexible bronchoscopy and bronchoalveolar lavage to evaluate the lesion. The presence of a reddish-orange smooth tissue mass was noted to be completely obstructing the take-off of the left upper lobe bronchus, with purulent secretions noted around the mass . A broncEndobronchial carcinoid tumors in adults are rare  but are the most common intrabronchial tumor in children, accounting for 80–85% of primary malignant lung tumors , 4. The Chest CT scans (particularly with contrast) are more useful than chest radiographs to define the tumor location and surrounding anatomy and to guide the surgical team for subsequent biopsy and/or removal. Bronchial carcinoids in children tend to arise most frequently in regions of bronchial bifurcation in the main, lobar, or segmental bronchi . In ordeWhen directly visualized, bronchial carcinoids typically present as smooth, pink-reddish or yellow endobronchial masses that are highly vascular . Though Surgery represents the treatment of choice for all bronchial carcinoids; in children and adolescents, lung-sparing resections  are recommended when possible. Surgical resection should be combined with lymph-node dissection to confirm the absence of local regional metastases . The preCarcinoid syndrome—characterized by flushing, palpitations, wheezing, shortness of breath, and diarrhea —is causeOur patient presented with signs and symptoms consistent with carcinoid syndrome, including facial flushing, dyspnea, chest pain, diarrhea, and palpitations. In addition, although her serum chromogranin A and neuron-specific enolase were within normal limits, her serum serotonin level was elevated. Finally, one month following surgical removal, our patient no longer had any systemic or respiratory complaints.Carcinoid syndrome is extremely rare found in conjunction with pediatric endobronchial tumors and should be considered in the setting of persistent systemic symptoms with recurrent pneumonia. Initial management consists of referral to a pediatric pulmonologist and pediatric thoracic surgeon to expedite diagnosis and surgical resection. Early diagnosis limits risk of metastatic spread and facilitates disease-free survival.


Mesenteric cysts are documented as a rare entity in pediatric population. They are considered as benign intra-abdominal tumors with an unknown etiology. Symptoms are not specific and knowledge of such condition is essential in order to establish a proper management. We report three pediatrics cases of mesenteric cysts managed between 2000 and 2009 in the pediatric surgery Department of Monastir College Hospital. We described the clinical, radiological and operative findings. Two males and a female were managed . Two patients were presented with an intestinal obstruction. A preoperative diagnosis was made basing on imaging. Thus, abdominal ultrasonography was performed in all of our reported cases and showed a cystic mass in all cases. The cystic nature of the mass, its margins and its extension were better described on tomographic images. The mesenteric cyst was completely and successfully removed in all cases. The histopathological report confirmed the diagnosis and showed a multiloculated cyst with columnar mesothelial lining, without any defined muscular layer or cellular atypia and without any evidence of malignancy. The children were evaluated post-operatively with a mean follow-up of 2 years and a half. No recurrence was noted in our patients during the follow-up period. It is known that clinical features are not specific of such anomaly but once the diagnosis is made, the complete surgical removal of the cyst remains the treatment of choice with excellent outcomes. Mesenteric cysts are histologically benign tumors. It was demonstrated that their proliferation is caused by lymphatic channels’ obstruction in the mesentery leading to communication rupture with the rest of lymphatic system. Mesenteric cysts are frequently located in the mesentery of the small intestines. Most of them present as symptomless, the preoperative diagnosis is more frequently based on clinical and radiological findings. The histological report confirms the diagnosis. Minimally invasive surgery is the surgical approach of choice. A complete laparoscopic excision of mesenteric and omental cysts is generally feasible. Mesenteric cyst diagnosis pose a challenge as there are no pathognomonic signs and these may mimic pathologies such as cystic lymphangioma and gastrointestinal duplications. In this study, we present completely resectable mesenteric cysts in three case reports, and we specify the clinical presentation as well as the radiological and histological features of these benign tumors.Our experience was based on a retrospective study conducted in the Pediatric Surgery Department of Monastir  from 2000 to 2009. Three children were managed. The preoperative diagnosis was primarily based on the clinical features and the radiological assessment. The histopathological report confirmed the diagnosis in all the cases. The children were evaluated post-operatively with a mean follow-up of 2 years and a half.A 10-day newborn female baby was admitted for an abdominal cystic mass discovered prenatally. On Physical examination, there was a mass of about 7*5cm at the left lower abdomen. Biological tests were unremarkable. A plain X-ray of the abdomen revealed a radio opaque lesion located at the left iliac fossa which compressed adjacent digestive system. The ultrasonography and the CT scan of the abdomen  confirmeA 4-year-old male child presented with a 2-day history of recurrent abdominal pain, fever and non-passage of both flatus and stools. The physical examination revealed a healthy child with an intra-abdominal voluminous mass measuring 10 cm long axis, on the right upper quadrant with a well-defined margin and smooth surface. The Complete Blood Count was normal. The ultra-sonography and the CT scan of abdomen and pelvis showed a large, cystic well-defined lesion anteriorly displacing the bowel . That maA 5-year-old male child was admitted for vomiting, anorexia and weight loss. The routine observations revealed low-grade pyrexia of 37.5%. On physical examination, we noted a palpatory tenderness and a painful mobile mass in the lower left quadrant measuring 4cm long axis. The initial blood tests were all within normal limits. A plain X-Ray abdominal revealed multiple air-fluid levels with a radio opaque lesion located at the left lumbar fossa . We diagThe first case of mesenteric cyst reported in the literature was by Benevieni in 1507 . SimilarAs confirmed by our experience, a mesenteric cyst can be diagnosed pre operatively. The knowledge of this rare entity is so important to suspect the diagnosis because there are no specific symptoms. A complete laparoscopic excision of the cyst remains the surgical approach of choice.Mesenteric cyst is a rare pathology;Symptoms are not specifics;Confirmation of this pathology is based on the histological examination.Insists the particularities of this pathology;The interest of prenatal diagnosis and screening of this pathology since complications;Minimally invasive surgery is the surgical approach of choice to treat and confirm this pathology.


This report describes a two-month-old girl who presented with signs and symptoms of a distal small bowel obstruction. She underwent an abdominal ultrasound that revealed a right lower quadrant cystic mass. A Technetium-99 scan revealed increased activity in the right lower quadrant consistent with a Meckel's diverticulum. Following a nondiagnostic laparoscopic evaluation, a laparotomy was performed to allow direct palpation of the small bowel and colon. Direct palpation of the ileum revealed a soft intraluminal mass at the ileocecal valve. The child underwent an ileocecectomy and anastomosis incorporating the intraluminal mass. Pathologic analysis revealed an intraluminal enteric duplication cyst containing ectopic gastric mucosa. This case represents the first report of such an entity in an infant. A discussion of the diagnostic and therapeutic aspects of the case and enteric duplication cysts is provided. Enteric duplication cysts are rare malformations with a reported incidence of 1 in 4500 with 80% of these diagnosed before the age of two [A two-month-old girl presented for evaluation for bilious emesis. She was a full-term female with an unremarkable perinatal history. At one month of age she developed pyloric stenosis and underwent an uneventful laparoscopic pyloromyotomy. She recovered well from this surgery and had been eating and gaining weight. On the day of presentation, she developed bilious emesis. She presented to the emergency room and underwent an abdominal X-ray that demonstrated small bowel dilation. Abdominal ultrasound was nondiagnostic but revealed a 4.8 × 2.8 cm fluid collection versus cyst to the right of midline. Her complete blood count revealed anemia with a hemoglobin count of 8.1 g/dL and her complete metabolic profile revealed a mild metabolic acidosis with a bicarbonate of 19 mEq/L.Following resuscitation, a limited upper gastrointestinal series was performed which showed normal intestinal rotation. An abdominal ultrasound was repeated which demonstrated a possible short-segment ileal-ileal intussusception with ileocecal inflammation. The previously identified fluid collection on prior ultrasound was noted to be in the area of the intussusception in the ileocecal region . She wasBased on these findings, the patient was taken to the operating room on hospital day 3 for a diagnostic laparoscopy. The entire small bowel was investigated from the ligament of Treitz to the terminal ileum which did not reveal Meckel's diverticulum or any other abnormality. Consideration for an intraluminal lesion led to laparotomy. The abdomen was opened and the terminal ileum, cecum, and appendix were eviscerated. A spherical soft mass was palpated within the lumen of the terminal ileum. Resection of the terminal ileum and a portion of the cecum was performed incorporating the mass with a primary anastomosis. Postoperatively, the child did well and was discharged. Final pathology revealed an ileocecectomy specimen with a 3.2 cm segment of ileum, a 2.2 cm segment of cecum, and a 6 cm appendix. Adjacent to the ileocecal valve was a gastric-mucosa-lined intraluminal cyst (1.7 cm) with associated abscess formation. These findings, as demonstrated in Enteric duplication cysts can occur anywhere from oropharynx to rectum and have three major characteristics: 1) the presence of a smooth muscle coat, 2) an epithelial lining representing a type of intestinal mucosa, and 3) an anatomic association with a portion of the gastrointestinal tract (most commonly on the mesenteric aspect of the intestine) [ the pres an epith an anatoEnteric duplication cysts can be classified into three subtypes: saccular , tubular, and small intramural . SacculaHere we present a rare case of a completely intraluminal duplication cyst similar to an intramural cyst. This duplication cyst was in close association with the ileocecal valve, resulting in obstructive symptoms and signs of intussusception. The diagnosis in this case was further confounded by the recent pyloromyotomy and potential adhesive small bowel obstruction. When she presented with emesis, there was initial concern that the pyloromyotomy had been inadequate. However, the bilious nature of the emesis suggested a more distal bowel obstruction. Our differential diagnoses included malrotation, volvulus, intussusception, constipation, or a distal intestinal obstruction from adhesions from prior surgery. The initial workup was most consistent with an ileoileal intussusception. We manage this nonoperatively with nasogastric decompression and resuscitation. If the child's condition deteriorates or the child develops a complete bowel obstruction operative intervention may be necessary, but surgical intervention is uncommon , 11. It The minimally invasive approach to intussusception and Meckel's diverticulum is logical and well described –13. The In conclusion, we present a rare case of an intraluminal enteric duplication cyst containing gastric mucosa causing intussusception in an infant. To our knowledge, this is the first report of such a case. This case highlights the importance of considering both Meckel's diverticulum and an enteric duplication cyst in the differential of causes for intussusception in children. If the Meckel scan is positive, we recommend the need for direct palpation of the intestine if initial laparoscopy is negative to rule out a small intraluminal anomaly.


Brodie’s abscess is a variety of subacute osteomyelitis with a long duration of presentation and intermittent pain. It usually involves the metaphyseal region of long bones of the lower limbs. Brodie’s abscess of pelvic bone is very rare. Involvement of posterior ilium with gluteal syndrome is extremely unusual and can be easily missed or misdiagnosed. We present a 9-year old boy who reported to us with intermittent low back pain of three months duration without any other constitutional symptoms. Clinically, there was mild tenderness over the posterior ilium. Computed tomography showed a lytic lesion in the posterior ilium with a breach in the outer cortex. MRI and bone scan were suggestive of inflammatory pathology. Keeping infective, tubercular and benign bone tumors as differential diagnoses, open biopsy and curettage were done. Staphylococcus aureus was cultured and histopathology was suggestive of osteomyelitis. The patient received appropriate antibiotics for six weeks. He was asymptomatic till 18 months of follow up without any recurrence. We present this case because of its rarity and unusual presentation as gluteal syndrome and low back pain, and its resemblance to other pelvic and sacroiliac joint pathologies which are often missed or misdiagnosed in paediatric patients. It may present as lumbar, gluteal and abdominal syndromes depending upon the direction of spread of the inflammation. Spread through the outer cortex into the gluteal muscles presents with pain and swelling in the gluteal region and called gluteal syndrome. We describe here the clinical, radiological and diagnostic features, the management and prognosis with a brief review of the literature.Brodie’s abscess is a type of subacute osteomyelitis first described by Sir Benjamin Brodie in 1832 involving the metaphyseal region of long bones of the lower limb. Pelvic osteomyelitis constitutes only 2 to 3% in children and adolescents, and the ilium is most commonly affectedA nine-year old male student presented with low back pain of three months duration. The pain was intermittent in nature with no history of fever or other constitutional symptoms. There was no history of night pain or contact with tuberculosis. On physical examination, there was a vaguely palpable tender swelling over the right posterior iliac crest just superolateral to the sacroiliac joint. There was no local warmth over the swelling. Clinically, pelvic compression was negative, and the sacroiliac joint appeared normal. There was no history of any intramuscular gluteal injection in the recent past.Routine blood investigations revealed haemoglobin of 12.6 g/dl, total leucocyte count 10000/mm3, neutrophils 80/100, ESR 31mm at one hour, negative C-reactive protein, serum ADA (adenosine deaminase) 29 IU/L and a negative Mantoux test (2mm). Plain radiograph of the pelvis showed a well-defined lytic lesion with surrounding osteosclerosis in the right posterior iliac crest just superolateral to the ipsilateral sacroiliac joint. CT scan revealed a pure lytic lesion (15 x 10 x 7.5mm) with a breach in the outer cortex of ilium with surrounding sclerosis . MRI of Staphylococcus aureus sensitive to oxacillin, gentamycin, and vancomycin. Histopathology was suggestive of osteomyelitis. The child received cloxacillin 500mg qid for a total period of six weeks . He was followed up for 18 months, and no recurrence was noted.The patient was planned for open biopsy and curettage under general anaesthesia. With the patient in prone position, incision was made over the vague swelling on the right posterior iliac crest. A breach in the outer cortex was noticed with an intramedullary cavitary lesion filled with unhealthy granulation tissue extending into the subcutaneous plane . The les1. The ilium is more commonly affected most probably because of its larger area and rich vascularity.Brodie’s abscess is one variety of subacute osteomyelitis which involves the metaphysis of long bones with indolent clinical features. Pelvic osteomyelitis with gluteal syndrome is very rare in healthy children2, pelvic osteomyelitis can present as three different clinical syndromes, namely lumbar, gluteal or abdominal syndromes depending on the spread of inflammation. In the lumbar type, the lesion breaches the inner cortex of ilium to the true pelvis and irritates the lumbar plexus and manifests as low backache, hip pain, thigh pain and difficulty in walking. When the inflammation enters the gluteal muscles through a breach in the outer cortex, it presents as gluteal syndrome. The most common complaint is buttock pain. Clinically there is local tenderness with or without swelling, and pelvic compression is usually positive. Very rarely, a sinus may be present. In the abdominal syndrome, it spreads into the iliac fossa and may present as an acute abdomen (acute appendicitis) or as chronic abdominal pain. Out of five cases of pelvic osteomyelitis in children reported by Beslikas et al1, two of them had gluteal syndromes, one abdominal syndrome, one presented with lumbar pain and one had features of both gluteal and abdominal syndromes. Similarly, in a study carried out by Rand et al3 two of four cases had gluteal syndromes while the other two presented as hip pain.According to Beaupre and Carroll4. Plain radiograph of Brodie’s abscess shows lytic lesion with peripheral osteosclerosis. Balaji et al5 in a similar type of case recommended a routine oblique view radiograph of the pelvis in these cases. But in the early stage, radiograph as well as computed tomography are negative. A bone scan is helpful in early diagnosis. MRI has been shown to be very sensitive and in some cases specific for diagnosis. The characteristic pattern includes an outer ring of hypointensity, an inner ring of intermediate signal intensity on T1 and high signal intensity on T2-weighted images. But bacteriological investigations are required for definite diagnosis.Diagnosing pelvic osteomyelitis is not easy because of its rarity, indolent clinical course, lack of early radiographic signs and absent or borderline haematological parametersOur patient had predominant low back pain probably because of the lesion located more towards the midline in the posterior iliac crest. Clinically there was a vague palpable swelling with tenderness in the posterior ilium adjacent to the sacroiliac joint. The routine haematological profile was inconclusive. Plain anteroposterior radiograph of the pelvis showed an osteolytic lesion in the posterior iliac crest. CT showed well-defined lytic lesion with a breach in the outer cortex. Bone scan and MRI were suggestive of infective pathology.Staphylococcus aureus is cultured in 50% cases, including in our case), but in 20% of cases, the culture is negative. Other organisms often reported are Streptococcus, Escherichia coli, Pseudomonas and Salmonella. Thorough curettage combined with antibiotic therapy (six weeks) gives excellent outcome in these cases5. At the end of 18 months, our patient was asymptomatic without any evidence of recurrence.Low virulent organisms are thought to be the usual infective agents. In conclusion, ruptured Brodie’s abscess of posterior ilium with gluteal syndrome is very unusual which is often missed or misdiagnosed because of its subtle clinical presentation. Strong clinical suspicion along with the knowledge of possible pelvis and sacroiliac joint pathologies will help the treating clinicians while evaluating cases of paediatric low back pain.


Papillary intralymphatic angioendothelioma (PILA) is a locally aggressive, rarely metastasizing vascular tumor, generally occurring in the soft tissues, with less than 40 cases described in the literature and only three cases reported in bone.We describe the case of a 51-year-old male with an intraosseous PILA of the proximal edge of his left clavicle and two other lesions evident on imaging. The patient was treated with marginal resection of the clavicle lesion but was lost to follow-up 1 month after surgery.PILA can also occur in bone, albeit very rarely, and has to be considered in the differential diagnosis of vascular bone tumors. Papillary intralymphatic angioendothelioma (PILA) is a rare vascular tumor defined in the latest edition of the WHO tumor classification  as a “raThe aim of the present study is to report a new case of PILA occurring in bone, describing the clinical, radiological, and histological features.Only one patient with intraosseous PILA was referred to and treated at our center between 1901 and 2016. This patient was a 51-year-old male with a painful lesion of the proximal edge of his left clavicle, which had been present for 2 months.Imaging studies Fig. A revealeTwo other lesions were radiologically evident in the right clavicle and left distal femur.The patient underwent a needle biopsy. Histologically, a vascular lesion was evident, with hypercellular areas and the neoplastic cells had a focal epithelioid appearance. A final diagnosis of low-grade hemangioendothelioma was made.Partial resection of the clavicle was performed. Grossly, a gray-reddish lesion was present in the clavicle, with infiltration of the soft tissues Fig. A. The tuThe patient was lost to follow-up 1 month after surgery.PILA is a rarely metastasizing lymphatic vascular neoplasm first described in 1969 by Dabska et al. . To dateClinically, this extremely rare tumor more commonly occurs in infants, children and young adults , 13, 15,Only three cases have been reported in bone , 13, 15,The pain and radiological features of the tumor are consistent with a well-circumscribed radiolucent lesion, generally with sclerotic margins on CT-scan, and are suggestive of osteoid osteoma , 13. LanHistologically, PILA is composed of a proliferation of spindle- to polygonal-shaped, slightly atypical cells forming numerous interconnecting capillary and cavernous vascular cavities , 13, 15,Two vascular lesions, retiform hemangioendothelioma and papillary endothelial hyperplasia (Masson’s hemangioma), may be confused with PILA due to the presence of “hobnail” cells and papillary projections , 15. How“Hobnail” cells and papillary projections associated with podoplanin immunohistochemical expression are also helpful in distinguishing PILA from epithelioid hemangioma and lymphangioma-like Kaposi sarcoma .PILA can be locally invasive with the potential to metastasize. Lymph node metastases have been reported  and a caDue to the low numbers of intraosseous PILA reported in the literature, the difference between intraosseous and soft tissue PILA in tumor prognosis is unknown; similarly, no consensus has been reached as to the proper treatment of intraosseous PILA , althougIn conclusion, we describe a new case of PILA of bone, with multifocal presentation on imaging. This very rare vascular tumor generally occurs in soft tissues, but can also be present in bone, thus extending the spectrum of vascular bone tumors.


The differential diagnosis of retroperitoneal mass includes liposarcoma, leiomyosarcoma, malignant fibrous histiocytoma, neurofibroma, stromal tumor, teratoma, and lymphoma. Leiomyosarcoma is rare with poorer prognosis than other soft tissue sarcomas. Soft tissue sarcoma of retroperitoneal origin often remains asymptomatic until tumor enlargement, leading to diagnosis at advanced stages. Multiple lung tumors were detected at a routine medical checkup at the office on a chest X‐ray in a 46‐year‐old woman. CT scanning identified a 150‐mm solid tumor with irregular density and some areas of weak enhancement in the left retroperitoneum, and multiple liver/lung/bone metastases Figs . What isA diagnosis of leiomyosarcoma was given based on a liver biopsy showing atypical spindle cells, which were positive for smooth muscle actin (SMA) and negative for KIT, CD34, and S‐100 immunohistochemically (Fig. The authors state that they have no conflict of interest.TT: gastroenterologist, responsible for the hospitalization and outpatient follow‐up of the case, and also had the major role in writing the manuscript. DA: gastroenterologist, responsible for the hospitalization and outpatient follow‐up of the case. YF: pathologist, responsible for pathological examination. SW: Professor of Department of Gastroenterology, Juntendo University School of Medicine; had the whole supervision of the case.


In rare cases of hypereosinophilic syndrome, nodular lesions of liver due to infiltration of eosinophilic granulocytes has been described. In such cases, a computed tomography of the abdomen could mimic a metastasizing disease while a spontaneous regression of the lesions can be expected. We will present such a case and discuss how this misdiagnosis can be avoided. A 59-year-old woman consulted her general practitioner for a two-week episode of widespread itchiness, enlarged cervical nodes on the left side and right upper quadrant pain. She felt feverish. Her medical history included appendectomy and hysterectomy. Medication consisted of benzodiazepines. Initial laboratory examination was normal except for leukocytosis of 1500/µl  and eosinophilia of 4400 cells/µl . Her general practitioner requested abdominal computed tomography (CT). It showed multiple focal low-attenuation lesions in the liver Figure .The patient was referred to the urology department, because the lesion in the left kidney could possibly represent the primary tumor. However, this preliminary hypothesis was rejected, as the renal lesion did not show typical features of renal cell carcinoma on a dedicated triphasic renal CT. The liver and lung manifestations were therefore considered potentially as a metastatic disease of unknown etiology.The patient was referred to the oncology department for further investigation. Fluorodeoxyglucose positron emission tomography was performed and didn’t show any pathological tracer accumulation in the lung, liver or kidney. Laboratory tests showed (besides eosinophilia of now 540 cells/µl), normal CA 125, CA 15.3, CA 19.9, CEA and NSE tumor markers. As a next step, an ultrasound-guided liver biopsy was performed. The pathology report described a dense infiltrate of eosinophilic granulocytes without any malignant epithelial cell. Because clinicians still considered malignancy very likely, a second liver biopsy was requested and showed normal liver parenchyma with rare lymphocytes and eosinophils, again without any signs of malignancy. The hypothetic diagnosis of hypereosinophilic syndrome was assumed. Because of the spontaneous symptomatic improvement and the rather mild peripheral eosinophilia, a “wait and see” attitude was adopted, and the patient was discharged with close follow up. After two months, a follow-up CT of the thorax and the abdomen showed complete regression of the lung lesions and significant volume decrease of the liver lesions. Laboratory results showed peripheral eosinophils within the normal range.Hypereosinophilia in the peripheral blood is defined as an absolute eosinophil count of more than 1500 cells/µl on two examinations separated in time by at least one month and/or pathologic confirmation of tissue hypereosinophilia . In our 3In this case, the diagnosis was put on the wrong track by misinterpreting the CT scans, unfortunately, leading to an anxious patient and a second (unnecessary) liver biopsy. The challenge in this case is to distinguish eosinophilic lesions in the liver from metastatic liver disease, which is especially important in patients with a known primary tumor. The different radiographic characteristics of eosinophilic liver disease and malignant nodules are well described in several reports. On CT, in the portal venous phase, the eosinophilic lesions and malignant nodules are both low-attenuation lesions. Eosinophilic lesions are characterized by multifocal, small (< 2 cm) oval or round lesions with irregular margins, whereas malignant nodules are usually (but not necessarily) significantly larger and well-circumscribed . More he3Eosinophilic disease is a rare condition that may mimic liver and lung metastases. The radiologist should be aware of the existence of this condition and add it to the list of rare differential diagnostic considerations, particularly in patients with peripheral eosinophilia and normal tumor markers. CT findings in favor of eosinophilic lesions include multifocal, small <2 cm) oval or round lesions with irregular margins. Imaging-guided biopsy together with correct interpretation of imaging findings and follow-up allow correct diagnosis. Especially in patients with concurrent neoplastic disease, this may avoid misdiagnosis and even unnecessary chemotherapy 2. cm oval


Open adrenalectomy was performed for this highly suspicious adrenal mass. Histopathology revealed benign AO that was BRAFV600E negative, with low Ki-67, and no somatic mutation found on NGS. Thyroidectomy revealed invasive, BRAFV600E-positive PTC. At 6 months follow-up, androgen levels returned to normal, and no recurrence was seen on imaging. To our knowledge, this is the first report of an androgen-secreting AO with concomitant PTC. Possibly the simultaneous discovery of two independent neoplasms was observed. In conclusion, this case highlights that care should be given to exclude concomitant neoplasms. Long-term and regular imaging with biochemical follow-up is warranted, since the outcome and clinical behavior of AO remains uncertain.Adrenal oncocytoma (AO) is an extremely rare adrenocortical neoplasm and little is known about its malignant potential, secretory properties, and hereditary origin. We present the case of a benign AO with concomitant incidentally found papillary thyroid cancer (PTC) and review similar cases in the literature. Immunohistochemistry and next-generation sequencing (NGS) were performed. A 66-year-old women was incidentally found to have a large, androgen-secreting right adrenal mass.  Fine needle aspiration (FNA) biopsy does not allow for the diagnosis of AO, and since their biological behavior is uncertain, adrenalectomy remains the only reliable diagnostic and therapeutic option.Oncocytic neoplasms occur in various organs such as the kidneys, thyroid, parathyroid, or salivary glands. An adrenal location, called adrenal oncocytoma (AO), is extremely rare with about 150 reported cases. They have been considered benign non-functional neoplasms; however, in about 20% of cases, they show malignant potential, and in 15% of cases, adrenocortical hormone secretion was detected (BRAF (most commonly V600E) and RAS genes as well as RET/PTC and PAX8/PPARγ chromosomal rearrangements. The type of alteration in PTC seems to be linked to specific etiologic factors. Rearrangements have a strong association with exposure to ionizing radiation and possibly DNA fragility, whereas point mutations probably arise as a result of chemical mutagenesis (The incidence of thyroid cancer (TC) is about 3 cases per 100,000 population, and papillary thyroid cancer (PTC) represents 85% of TC, with a good prognosis . MutatioMenin gene) is known to present with multiples combinations, including ACC but not PTC (RET gene) include pheochromocytoma and medullary TC, but do not traditionally include PTC or ACC. ACC is known to appear in Li–Fraumeni syndrome with TP53 mutation. There are four reports that attribute the association of ACC and PTC to coincidence, but do not discount a potential genetic or hereditary link  Type 1  revealed a hypoechoic mass, and CT scan revealed a 7.6 cm × 5.6 cm × 7.4 cm right adrenal mass, hyperdense (>50 Hounsfield units) with delayed wash out after contrast injection  with a mass effect on the liver .V600E immunostaining and a low Ki-67 proliferation index (<2%)  Figure D. In addd CTNNB1 ]; 2,800  Figures A–D, stag131 radioactive iodine therapy. Postoperative work-up at 2 months showed normalization of DHEAS to 1.7 μmol/l and morning cortisol at 464 nmol/l, as well as testosterone level at 0.8 nmol/l. Corrected calcium was normal at 2.32 mmol/l and thyroglobulin at <0.10 μg/l. For the thyroid lesion, the patient will be followed up by measuring stimulated thyroglobulin after radioactive iodine therapy, and aiming for a suppressed TSH, as well as by performing a neck ultrasonography and thyroglobulin at 6-month intervals. For the adrenal lesion, at 6-month follow-up, the androgen levels stayed normal (1.7 μmol/l), and no recurrence was seen on CT scan imaging. Regular imaging and biochemical follow-up at 12 and 18 months for a minimum of 5 years are planned.Postoperative recovery was uneventful, and patient was started on thyroid hormone replacement therapy. According to ATA guidelines  and locaWe describe a female patient with a highly suspicious adrenal mass that reveals to be a benign lesion based on the pathological criteria, without somatic mutations, and who presents with a simultaneous endocrine malignancy of the thyroid gland.18F-FDG, which revealed a highly metabolic adrenal mass and the possibility of another synchronous primitive tumor or metastasis  is detected, the tumor is considered malignant. Large-sized tumors (>10 cm and/or >200 g), necrosis, capsular, and sinusoidal invasion are minor criteria to define an oncocytic tumor as borderline/uncertain malignant potential (V600E negative, and no somatic mutation was found, while the PTC was BRAFV600E positive. However, we did not perform germline mutation analysis. In literature, two cases of functional adrenal adenoma (p53 and no mutation in the Menin gene, thus concluding the absence of a hereditary cancer syndrome. Only one case of borderline malignant non-functional AO with simultaneous PTC has been reported (To our knowledge, no hereditary syndrome is known comprising AO and PTC. The AO was BRAF adenoma , 6 and t adenoma , 8. Wantreported . HoweverIn conclusion, we present the first case of an androgen-secreting AO with concomitant PTC. We probably observed the simultaneous incidental discovery of two independent endocrine neoplasms. Regular and long-term imaging with biochemical follow-up is recommended, since the outcome and clinical behavior of AO remain uncertain.The patient provided written informed consent for research participation and for the publication of indirectly identifiable data.Wrote the first draft of the manuscript: MPod. Contributed to the writing of the manuscript: SS. Made contributions to the acquisition of the clinical data: MPod, MPu, CT, MPro, FT, and SS. Performed pathological analyses: MPu. Agreed with manuscript results and conclusions: MPod, MPu, CT, MPro, FT, and SS. Made critical revisions and approved final version: MPu, FT, and SS. All the authors revised and approved the final manuscript and agreed to be accountable for the content of the work.The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest. The reviewer JL and handling editor declared their shared affiliation.


Mediastinal thymic seminomas are rare male germ cell tumors with extragonadal origin that appear predominately with a cystic appearance.A 22-year-old male was referred to our department for further investigation of a mediastinal mass discovered incidentally during routine chest X-ray. The patient has denied any symptoms including dyspnea, chest pain, cough, fever, dysphagia, hemoptysis, weight loss, and weakness. His past medical history was remarkable for orchitis, for which he had undergone a bilateral testicular biopsy, without the latter however, indicating the presence of a germ cell tumor or a premalignant lesion. Contrast-enhanced chest computed tomography revealed a lobulated and well-marginated cystic lesion in the anterior mediastinum. Differential diagnosis included mostly a multilocular thymic cyst, a lymphoma, a seminoma, or a soft tissue tumor. Resection of the mass revealed a primary thymic seminoma.A surgical approach for the management of these tumors might be reasonable considering that an extensive sampling is mandatory to gain an appropriate biopsy preoperatively in order to securely confirm or refute the presence of a mediastinal extragonadal tumor. Orchitis might be a sign of a general disorder of the germ cells which might transform in time. Male germ cell tumors with extragonadal origin (EGCTs) represent only a 2–5% of all male germ cell tumors . Germ ceThere are two major theories about the molecular biology of the pathogenesis of germ cell tumors. The first one  targets The second one  suggestsOccurrence of a primary mediastinal thymic seminoma with a predominate cystic appearance has rarely been reported.A 22-year-old male was referred to our department for further investigation of a mediastinal mass discovered incidentally during routine chest X-ray Fig. . The patClinical examination was unremarkable and did not reveal a palpable mass on the chest wall, neither has demonstrated any signs of a vena cava occlusion syndrome, while it also did not show depletion of the muscle strength regarding all muscle groups.Blood tests excluded relevant hematological disorders or signs of anemia, whereas chemistry tests revealed an elevated LDH one and a half time more than the reference rate. Alpha-fetoprotein (AFP) and human chorionic gonadotropin (hHG) values were not elevated. Contrast-enhanced chest computed tomography revealed a lobulated and well-marginated cystic lesion in the anterior mediastinum Fig. . The difSurprisingly, the histopathological examination revealed a primary thymic seminoma with a predominate cystic appearance Fig. . MacroscThe patient received adjuvant cisplatin-based chemotherapy and 38 months after surgery he is alive and remains disease-free.Two major theories for the pathogenesis of EGCTs have been described. Firstly, it has been hypothesized that these tumors are the result of a mismigration of germ cells along the urogenital ridge, which occurs during embryogenesis. Secondly, they might arise from normally distributed germ cells of the organs .The cystic morphology of thymic seminoma has previously been described  in theseAccording to the International Germ Cell Cancer Collaborative Group (IGCCCG), the treatment of extragonadal seminomas with good or intermediate prognosis is based on chemotherapy . The rolInterestingly, one third of the patients with EGCT may harbor TIN in clinically normal testicles. The risk of developing metachronous testicular cancer 10 years after treatment is 6.2% for tumors with a primary mediastinal location. However, due to the fact that the vast majority of these patients receive a platin-based chemotherapy, which might eliminate TIN, the standard use of testicular biopsies after treatment in the follow-up period is not recommended according to the European consensus guidelines .Although the presence of orchitis might reveal a germ cell tumor of the testicles, our patient had negative bilateral biopsies but still developed an EGCT. In the study of Bokemeyer and colleagues  regardinA surgical approach for the management of these tumors might be reasonable considering the fact that extensive sampling is mandatory to gain preoperatively an appropriate biopsy sample in order to securely confirm or refute the presence of a mediastinal extragonadal tumor. Orchitis might be a sign of a general disorder of the germ cells which might transform in time.


Schwannomas, also known as neurilemmomas, are benign peripheral nerve sheath tumors. They originate from any nerve covered with schwann cell sheath. Schwannomas constitute 25–45% of tumors of the head and neck. About 4% of head and neck schwannomas present as a sinonasal schwannoma. Brachial plexus schwannoma constitute only about 5% of schwannomas. Cervical vagal schwannomas constitute about 2–5% of neurogenic tumors.  We present a case series of 5 patients of schwannomas, one arising from the maxillary branch of trigeminal nerve in the maxillary sinus, second arising from the brachial plexus, third arising from the cervical vagus, and two arising from cervical spinal nerves.  Complete extracapsular excision of the tumors was achieved by microneurosurgical technique with preservation of nerve of origin in all except one.  Head and neck schwannoma though rare should be considered as a differential diagnosis of a unilateral slow growing mass in the head and neck region, particularly in an adult. Schwannomas are always a diagnostic dilemma as they are asymptomatic for long time, and histopathology is the gold standard for diagnosis. As a rule, treatment is surgical and dictated by the location of the tumor and nerve of origin. Due to its rarity, complex anatomical location and morbidity risk postexcision, they can pose a formidable challenge to surgeons. This study aims to describe the presentation, workup, surgical technique, and outcome. Neurilemmoma also known as schwannoma is a benign tumor of nerve sheath origin, arising from any nerve covered with a schwann cell sheath, which includes the cranial nerves (except for optic and olfactory), the spinal nerves, and autonomic nervous system , 2.Schwannomas occurring in the head and neck region are frequent, with 25–45% of all reported schwannomas being found in this region .We present our surgical experience with five such tumors, one arising from the maxillary branch of the trigeminal nerve in the maxillary sinus, second arising from the brachial plexus, third arising from the cervical vagus, and two arising from cervical spinal nerves. The clinical presentation, surgical treatment, and outcomes of patients with this pathology are described.A retrospective analysis was done at MGM Medical College and Hospital, Aurangabad, from January 2015 to December 2016. Of the five patients, two were female and three were males, with a mean age of 33 years (ranging from 23 to 40 years). Complete excision of the tumors was achieved by extracapsular excision using microneurosurgical technique with operating microscope. Preservation of nerve of origin was achieved in all except one where conventional extracapsular excision technique was applied. Surgical observation, histopathology, and immunochemistry confirmed the tumors to be benign schwannomas in all five cases. There was no recurrence in any of the five patients  at one year follow-up.We describe a rare case of schwannoma arising primarily from the maxillary sinus extending into the orbit and intracranially. A 40-year-old female presented with headache since 2 years, left nostril discharge since 1 year, and left eye progressive vision loss since 1 year.Diagnostic nasal endoscopy (DNE) revealed left-sided nasal mass, pale, firm, pinkish in colour, not bleeding on touch. Clinical examination revealed reduced vision in the left eye with finger counting less than five feet, with mild proptosis of the left eye. There was no motor or sensory loss with no other cranial nerve dysfunction.Contrast-enhanced CT (CECT) scan with cisternography revealed a large lobulated mass of size 81 × 53 × 82 mm in the left zygomatic and masseteric region with intracranial extension . The lesSuperiorly, mass was extending to middle cranial fossa and displacing left middle temporal lobe. Medially, it was extending to left nasal cavity causing complete blockage. Posteriorly, it was extending to the posterior nasopharyngeal region. Laterally, it was involving and abutting the masseter and pterygoid muscles and intraorbital muscles. Superiorly, it was extending to the infraorbital region and displacing and compressing the left optic nerve. Lamina papyracea and cribriform plate were intact.Clinical features and CECT were suggestive of malignant tumor. This divisive situation steered us to keep a possibility of a malignant component in the tumor.The tumor was approached through Weber Furguson incision with subciliary extension. Left suprastructure maxillotomy was done. Tumor was firm, necrotic, moderately vascular, and grayish white  and founWe describe a 35-year-old female who presented with right-sided supraclavicular swelling since 3 years  and compPre- and postcontrast MRI neck screening revealed a well-defined, rounded space occupying lesion in the right side of the neck, that was found to be lateral to the neck vessels and superficial to the subclavian vessels. The lesion was hyperintense on T2-weighted images and STIR (short T1 inversion recovery) images and was found to be isointense on T1-weighted images with heterogeneous contrast enhancement in the lesion . These fUnder general anaesthesia, the tumor was approached using a V-shaped incision. The brachial plexus was explored. The tumor was found arising from the upper trunk of the brachial plexus and was found to be adherent to the middle and lower trunk of the brachial plexus . The tumWe describe a 23-year-old male patient who presented with right-sided painless neck swelling since one month. There was no history of trauma, fever, and systemic illness. The patient had no complains of weakness, dysphagia, and hoarseness of voice. On local examination, a single ovoid swelling was present in the right carotid triangle, anterior to the sternocleidomastoid, measuring 3 × 2 cms. Swelling did not move on deglutition or tongue protrusion. There was no loss of muscle power in all muscles, no signs of wasting, or neurological deficits.USG neck showed a hypoechoic lesion seen at bifurcation of the carotid artery. Moderate central and peripheral vascularity was seen within the lesion. Findings were suggestive of a carotid body tumor/nerve sheath tumor.CECT neck revealed a well-defined, well-encapsulated, peripherally enhancing soft tissue mass lesion of size 3.4 × 1.8 cm . The masThe tumor was approached by an anterior approach along the medial border of sternocleidomastoid. Platysma and fascia were dissected to reach the tumor. There was a medium-sized globular smooth mass, well-encapsulated seen engulfing the vagus nerve Figures . ExtracaPostoperatively, the patient had mild dysphagia which resolved in two days. The patient reported a minimal change in voice. There were no signs of aspiration. Postoperative videolaryngoscopic assessment revealed right vocal cord palsy. The patient's voice improved with speech therapy.We present a 30-year-old male patient who presented with left-sided painless neck swelling since two years. It was insidious in onset and gradually progressed in size. The patient had no complains of weakness, dysphagia, and hoarseness of voice. On local examination, a single ovoid swelling was present in the left carotid triangle, anterior to the sternocleidomastoid, measuring 5 × 3 cms. Swelling did not move on deglutition or tongue protrusion. There was no loss of muscle power in all muscles, no signs of wasting, or neurological deficits.MRI neck with angiography and CECT neck revealed a moderately enhancing lesion of size 63 × 45 × 34 mm in left carotid space causing splaying of internal and external carotid artery, anteriorly abutting the submandibular gland with no evidence of encasement of the vessels .The tumor was approached by an lateral approach along the lateral border of sternocleidomastoid, 3.5 cms below the auricle. External jugular vein ligated. Tumor was found attached to the C1 spinal nerve. Extracapsular tumor excision was done in toto by microneurosurgical technique . MinivacPostoperatively, the patient had mild ptosis of the left eye lid with marginal mandibular nerve paresis and features suggestive of first bite syndrome, all of which improved with physiotherapy. There was no vocal cord paralysis. The patient is currently free of all symptoms at one year follow-up.We present a 35-year-old male patient who presented with right-sided neck swelling since six months. The patient had complaints of right-sided mild ear pain and no complains of weakness, dysphagia, and hoarseness of voice. On local examination, a single ovoid swelling was present in the left carotid triangle, anterior to the sternocleidomastoid, measuring 4 × 4 cms. There was no loss of muscle power in all muscles, no signs of wasting, or neurological deficits.MRI neck revealed an oval solid lesion of size 42 × 41 × 28 mm in the right carotid triangle, laterally abutting sternocleidomastoid and posteriorly the paraspinal muscle. The lesion was hyperintense on T2W and FLAIR (Fluid Attenuated Inversion Recovery) images and hypointense on T1W images .The tumor was approached by anterior approach along the medial border of sternocleidomastoid. The tumor was seen involving the cervical spinal nerve. Extracapsular tumor excision was done in toto by microneurosurgical technique, preserving the involved nerve. Post-op recovery was uneventful and devoid of any complications or deficits.Schwannomas can arise from any nerve which has schwann cells except optic and olfactory nerve as they lack those cells .These tumors are generally encapsulated except from the sinonasal tract and nasopharynx .Most often these tumors are solitary but can occur in multiple areas also. Multiple occurrences are usually associated not only with neurofibromatosis II but also schwannomatosis , 5. HeadSinonasal schwannoma contributes about 4% of head and neck schwannomas .It remains asymptomatic for a quite long period because of slow growth. Nasal obstruction is the main symptom of this tumor. Pain, bony dehiscence including skull base and neural symptoms develop later as the tumor enlarges. It can also simulate rhinosinusitis as clinical features are similar .Brachial plexus tumors are rare comprising of only 5% of all tumors of upper limb . The mosThe two most common brachial plexus region tumors are the schwannomas and neurofibromas, both of which are benign and arise from the nerve sheath .Most often, early in the course, minimal neurological deficit will be present, or there will be none at all. Manipulation of the mass can produce paraesthesias or “shocks” in the distribution of the affected nerve, and this can be an important aid to diagnosis. Side-to-side mobility greater than longitudinal mobility of the mass from the nerve is more common in cases of benign lesions, whereas malignancy is associated with firmness and immobility , 13. In Computerized tomography scanning is optimal at revealing osseous erosion around the spine or changes in neural foramina. A newer modality, MR neurography (MR-magnetic resonance), has the potential to demonstrate the entire course of visualized peripheral nerves . SchwannCervical vagal schwannomas constitute about 2–5% of neurogenic tumors . These tMicroscopically , spindleNeurilemmomas are resistant to radiotherapy , and as Conventional extracapsular excision can damage the normal fascicles during dissection of the capsule. Intracapsular excision with gentle dissection between the tumor capsule and normal fascicles minimizes the risk of nerve damage. The epineurial layer covering the tumor capsule should be dissected in a manner similar to peeling an onion to allow for safe removal of the tumor, which should be approached by its proximal and distal poles . The fasComplete extracapsular excision preserving the nerve of origin should be attempted when feasible , but forThe choice of surgical approach is dictated by the size of the tumor, its location, its relationship to the great vessels, and the suspicion of malignancy.This case report emphasises the rarity of schwannomas in maxillary sinus involving the trigeminal, arising from the brachial plexus, vagus nerve, and cervical spinal nerves, respectively. Among surgical techniques, complete extracapsular excision using microneurosurgical technique with operating microscope is the preferred technique advocated. A schwannoma though rare should be considered as a differential diagnosis of a unilateral slow growing mass in the head and neck region particularly in an adult. They are difficult to diagnose preoperatively. Radiologic findings are usually nondiagnostic. Diagnosis relies on clinical suspicion, and confirmation is often obtained by means of surgical pathology. Thorough preoperative counselling of patients to inform them of the potential occurrence of neurological deficit is important. Long-term surveillance is not recommended, even though these tumors are benign and mostly asymptomatic. Recurrence is rare.


Ossifying fibromyxoid tumor of soft parts (OFMT), is a rare but morphologically distinctive neoplasm of uncertain histogenesis that most frequently affects middle-aged male adults. Clinically, it usually presents as a slowly enlarging, small, circumscribed mass, which in most cases is painless. OFMT is most frequently found within the subcutaneous tissues of extremities or trunk, and rarely in the oral/head and neck region. We present an unusual case of this tumor in the submandibular region, and, based on the current medical literatures this is probably the first case described in this anatomical location.A 32-year-old male presented to our outpatient clinic with a right submandibular mass with 1-year of evolution. Excisional biopsy showed that it was characterized by ossification along the periphery of the lesion. The neoplastic cells were spindle-like with scant eosinophilic cytoplasm. These cells were arranged with uniform cell-to-cell space in a fibromyxoid stroma. Small and large clusters of calcifications were present within the tumor. Immunohistochemically, the case showed positive staining of S-100 protein, vimentin, nestin, calponin, SMA, GFAF, desmin, INI-1, caldesmon, and CD34. It also showed negative staining of CK, CK7, CK8/18, NF, and EMA. About 2% of neoplastic cells showed positive staining of Ki67. Based on these features, the final pathological diagnosis was OFMT.It is hoped that a greater understanding of OFMT in the head and neck region will avoid potential misdiagnosis, and contribute to determining the correct management, which appears to be complete surgical excision with close follow-up for recurrence surveillance. Ossifying fibromyxoid tumor of soft parts (OFMT) was first described by Enzinger et al. in 1989, and is a rare but morphologically distinctive neoplasm of undetermined histogenesis . ClinicaOFMT is typically composed microscopically of lobules of small polygonal to spindle-shaped cells with vesicular nuclei, discernible nucleoli, and eosinophilic cytoplasm, arranged in cords, trabeculae, or clusters in a loose fibromyxoid matrix . AlthougThe objective of our study is to describe an unusual clinical presentation of this tumor in the submandibular region. Based on the current medical literatures, this is probably the first case described in this anatomical location. Additionally, a literature review was performed with particular emphasis on previously reported cases affecting the oral/head and neck region.2) mixed lesion with defined margins that was adjunct to the right submandibular gland  with1-year history of a right neck mass; the patient’s past medical history was non-contributory. Extraoral clinical examination revealed a soft and slightly movable mass in the right submandibular region with normal overlying skin Fig. ; the remand Fig. . Additioand Fig. . The priExcisional biopsy was performed through an extraoral approach with submandibular gland preservation Fig. . Gross sThe tissue sections were fixed in formalin, embedded in paraffin (the thickness is 5 μm) and then were stained with haematoxylin–eosin and immunohistochemistry. Following heat-induced epitope retrieval, slides were incubated with antibodies specific for: S-100, nestin, calponin, vimentin, cytokeratin(CK), cytokeratin 7 (CK7), CK8/18, calponin, smooth muscle actin (SMA), glialfibrillary acidic protein (GFAP), neurofilament (NF), desmin, caldesmon, CD34, epithelial membrane antigen (EMA) and INI-1 . Detection of the immunoreactionwas performed using second antibodies and ZLI-9017/9018/9019 DAB kit .The literature review was performed using the PubMed/MEDLINE electronic database to identify relevant publications from January 1989 through to December 2016 using the following search terms: “ossifying fibromyxoid tumor AND head and neck neoplasms.” From the publications obtained in this search, those pertaining to cases of OFMT in the oral/head and neck region were included in the review –26. AddiExamination of the biopsy specimen showed a 5.5 cm × 5 cm × 3 cm, rubbery, fragile, gray-white colored, well-defined tumor that was surrounded by dark yellow lobulated soft tissues Fig. . Histolon = 88). According to the data reviewed, OFMT in the head and neck region usually presents as a painless subcutaneous mass in middle-aged men. OFMT in the oral cavity was seen in 8 of 88 cases (9%) and a less common location was the nasal cavity , where it was associated with pain and nasal congestion. The preferred surgical treatment in the majority of cases was local excision; however, more radical excisions were performed in recurrent disease. Increased mitotic activity of > 2 per 10 high-power fields (HPFs) and cytological atypia was seen in 12/45 (27%) and 8/68 (12%) cases, respectively. At least 1 episode of recurrence was described in 15 of 70 cases (21%) and was associated in almost all cases with an increased mitotic activity of > 2 per 10 HPFs . Distant metastasis was a rare event that occurred in only 4 of 78 cases (5%), and in all cases, it was associated with recurrent disease and increased mitotic activity. Two patients with distant metastasis died of the disease.Twenty-eight articles with well-documented OFMT cases affecting the oral/head and neck region were reviewed; all articles were published between 1989 and December 2016. Table OFMT is a rare musculoskeletal tumor of borderline malignant potential , 27. TheSince the first description by Enzinger et al. , severalThe clinical presentation of OFMT in the head and neck does not differ much from that in other anatomical regions. Head and neck OFMT presents as a well-circumscribed, slow-growing, painless, and subcutaneous/submucosal mass. However, nasal cavity OFMT usually presents as pain and nasal congestion. These tumors normally have a longstanding clinical course, ranging from 1 to 20 years, or even longer  , 32. On OFMT histopathology is characterized by the presence of uniform lobules, round to fusiform-shaped cells arranged in nests and cords, and set in a variably fibromyxoid stroma . ApproxiAtypical and malignant OFMT have been described based on certain histopathologic criteria , 15, 27.Recurrences have been reported in a considerable number of OFMT cases . The cliSurgical management is the initial treatment of choice for head and neck OFMT. Local excision is described as the primary therapy, which is curative in most cases. However, close follow-up is recommended, especially in atypical and malignant histopathologic types, because of the previously discussed propensity for local recurrences and distant metastases. In recurrent cases, wide and more radical excisions with secure margins would be the appropriate treatment. Adjuvant radiotherapy does not seem to be indicated, except for the management of distant metastasis or unresectable disease.It is hoped that a greater understanding of OFMT in the head and neck region will avoid potential misdiagnosis, and contribute to determining the correct management, which appears to be complete surgical excision with close follow-up for recurrencesurveillance.


Foreign-body ingestion is a common phenomenon and foreign bodies are mostly excreted in stool. Once sharp bodies are ingested without being realized, perforation of intestine is possible and misdiagnosis may be made. We report 2 toothpick ingestion cases that were both diagnosed accurately.We present 2 cases of middle-aged persons who suffered from abdominal pain. They did not realize and provide any information of having the history of swallowing foreign bodies.No serious problem was discovered in the examination and blood test. There were somewhere abnormal in computed tomography (CT) images and ultrasound (US). Then a toothpick was found penetrating the wall of intestine into the adjacent viscera in the laparotomy.Both patients in the 2 cases were undergone operation to remove the toothpicks.The 2 cases’ prognoses were good.When accepting patients with abdominal pain, suitable examining means and careful observation should be given to find easily ignored lesions. CT is recommended in the diagnostic process of swallowed foreign mass. When there is a vague place, US can be used for further diagnose. Toothpicks are ingested accidentally for various reasons—the ingestion happens to people who are edentulous, alcoholics, or who are used to picking their teeth.,5,7–9 About 54% of patients cannot explain when or how the toothpicks get into their bodies. The symptoms of perforation are significantly different from relapsing blunt abdominal pain to typical peritonitis,,4,5,7 mainly depending on the location where a toothpick lodges. Overall, the low morbidity and confusing clinical presentations tend to misguide physicians. A rapid and accurate diagnosis is necessary to avoid severe outcomes. Therefore, we present 2 cases of intestinal perforation caused by toothpicks.Foreign-body ingestion is a common phenomenon.2Written informed consent was obtained from both patients for the publication of this manuscript and accompanying images.2.1A 33-year-old man presented to our facility with chronic mild transferable abdominal pain 2 months after having a midnight snack. His vital signs were normal. Abdominal x-ray showed nothing abnormal before admission. The pain from epigastrium to hypogastrium could be relieved by the management of omeprazole pills (20 mg/day) for a few days. The mimic blunt pain relapsed after drinking. After admission, there was no obvious tenderness or mass in his abdomen during the body check-up. However, ultrasound (US) and abdominal computed tomography (CT) scans showed a linear shadow in the left liver lobe. The patient was taken to the operating room and underwent exploratory laparotomy, which revealed a wooden toothpick perforating intestinal wall with one end into the left liver lobe and the other end into the duodenum with surrounding inflammation and adhesion. The toothpick was successfully removed by snare extraction without complications. The patient made an uneventful postoperative recovery. When informed of this unusual finding during his follow-up, he recalled the probable accidental ingestion of a toothpick after drinking.2.29/L, and a granulocyte count of 12.1 × 109/L (79.1%), while other relevant laboratory data were within reference values. The contrast-enhanced CT scan of the abdomen revealed a linear high-density shadow penetrating the descending colon and pointing to the tail of the pancreas with secondary surrounding inflammation , stomach (20%), duodenum (23%), small intestine (18%), and large intestine (37%).,5 The main impressionable adjacent viscera include liver, pancreas, kidney, and vasculature.,5,10 Complications of perforation include fistula, sepsis, bleeding, and even death.,11 The location that a toothpick lodges determines the symptom.Less than 1% of cases after swallowing a foreign body lead to perforation.,9,12 However, the sensitivity of these methods depends on the organs examined. X-ray is easily examined and can reveal indirectly symptoms of digestive tract injury .,14 As for the abdominal x-ray, it can be used for initial screening and the diagnostic significance is small. The sensitivity of US in the diagnosis of damage to the solid viscera is high, and US has the advantage of real-time dynamic observation from different angles.,15 CT and MRI have superior sensitivity in any gastrointestinal injury. Through observing the continuing fixed plane angle, it is easy to find the differential part if the imaging readers report the result carefully and prudently. But MRI is not only more expensive but also less accurate than CT. On the other hand, longer time should be used in MRI. Endoscopy can directly show the location of toothpicks in the early stage, and corresponding treatment can be made at the same time.,5Intensive patients were directly referred for exploratory surgery, and then the foreign bodies were found. The remaining cases were examined out the existence of foreign bodies with more conservative methods, such as x-ray, US, magnetic resonance imaging (MRI), CT, and endoscopy.In conclusion, when accepting patients with abdominal pain, suitable examining means and careful observation should be given to find easily ignored lesions. CT is recommended in the diagnostic process of swallowed foreign mass. When there is a vague place, US can be used for further diagnose. If necessary, we can choose endoscopic to diagnose and treat at the same time. In case of emergency, laparotomy is necessary. A more accurate diagnosis and corresponding treatment should be made to reduce the pain of the patient and help them recover without delay.