Low-grade myofibroblastic sarcoma (LGMS) is a rare type of tumor. Previous research has paid much attention to reporting pathological analyses of LGMS. However, only few systematic clinical and/or radiological studies have been conducted.This study recruited 14 cases  of LGMS. X-ray or computer tomography (CT) scan were performed on 11 cases. MRI was performed on 5 cases.X-Ray and CT scan: Five cases developed LGMS in bones, including 3 cases in the distal femur, 1 in the right shoulder blade, and another 1 in the right inferior ramus. Massive infiltrative and vermiform bone destruction with poorly-circumscribed lesion margins and partial soft tissue masses were observed. The other 9 cases were developed in soft tissues. Out of them, 4 cases presented slightly irregular hyper- or lower-density masses with poorly-circumscribed margins. 2 cases presented massive calcification and ossification. Significant enhancement was observed in 1 case, while no obvious enhancement was seen in the other 2 cases.MRI: MR images of 5 cases revealed homogeneous iso- or hyper-signal intensity on T1WI and homogeneous or heterogeneous hyper-signal intensity on T2WI. Enhanced MRI revealed homogeneous enhancement in 2 cases and rim enhancement in 1 case.Our findings show that LGMS is characterized by invasiveness, metastases and calcification. Different radiological tools should be employed to make an accurate diagnosis. Low-grade myofibroblastic sarcoma (LGMS) is a rare type of malignant myofibroblastic tumor. Though it may occur to any body part, the most common location is the limbs, head and neck region, particularly the tongue and mouth . PreviouThis study recruited 14 cases of LGMS from two hospitals (one of the 14 participants had converted to LGMS from multiple relapses of an inflammatory myofibroblastic tumor). The imaging findings and biological characteristics were different from those of previous studies. Therefore, we report them in this article, hoping to provide further insights into LGMS.Esophageal angiography was performed on the participant with LGMS in the pyriform sinus (Case 4). X-ray or computer tomography (CT) scan were performed on 11 cases before surgery. Dual energy CT (Siemens SOMATOM Definition) images revealed slice thickness of 5–10 mm, tube voltage of 120 kV, tube current of 559 mA, pitch of 3.2, and gantry perpendicular to the CT table. Multi-planar reformatting of CT images was performed by a workstation .1WI:TR/TE 440/8.2 ms, T2WI: TR/TE: 4000/142.5 ms; slice thickness: 5 mm; NEX:4.0, FOV; 38 cm × 38 cm; and matrix: 256 ×224~ 512 × 446. Fat-suppressed T1-weighted transverse images: a gadopentetate dimeglumine of 0.1 mmol/kg  was injected intravenously into each patient with an injection rate of 2.0 ml/s. Axial view: T1WI: TR/TE 560/8.0 ms; slice thickness:6 mm; FOV 38 cm×38 cm; and matrix: 320×192~ 512×446. Corona view: T1WI: TR/TE 560/8.2 ms; slice thickness:5 mm; FOV 40 cm×40 cm; and matrix: 320×192~ 512×446.Magnetic resonance imaging (MRI), without and with contrast materials, was performed on five cases using a 3.0 T MRI scanner (GE Signa Excite). Dedicated coils were used to image different body parts and the regions of interest. MR images were acquired with spin-echo pulse sequences. Axial view: TAnalysis of all images was done by two board-certified radiologists specializing in musculoskeletal imaging. CT images were analyzed on tumor location, morphology, size, margins, density and the presence of calcification. MR images were evaluated for tumor morphology, margins, signal intensity and enhancement, necrosis, hemorrhage, and peritumoral edema. All imaging findings were in line with those of pathological analysis.Tumor specimens obtained after surgical excision were fixed in a 10% formaldehyde solution for 24 h for dehydration, and the paraffin-embedded specimens were sliced and stained with hematoxylin and eosin (H&E). Immunohistochemistry streptavidin-biotin staining (S-P) link staining with 3,3′-Diaminobenzidine (DAB) color rendering was performed on some specimens. Cytoplasmic brown precipitation was considered to be positive. Histopathological characteristics were analyzed by 2 board-certified pathologists specializing in musculoskeletal specialty.A total of 14 LGMS cases were recruited from two hospitals from April 2005 to July 2015, including the hospital where we worked. These 14 participants included 8 male and 6 female patients with a history of from 1 to 8 years. All of them had been clinically diagnosed and undergone surgical procedures, pathological analysis, and post-operation follow-ups in the two hospitals. Details of tumor location and medical history are listed in Table X-ray and CT scan results are illustrated in Table LGMS was located in bones in five cases, including 3 in the distal femur, 1 in the right shoulder blade, and 1 in the right inferior ramus of the pubis symphysis Fig. , and 3, There were a total of 9 cases of LGMS in soft tissues. Preoperational esophagus angiography performed on Case 4 revealed lobulated filling defects with smooth margins in the right pyriform sinus and epiglottic vallecula region. Preoperational X-ray was performed on Case 6 who had a lobulated mass with well-circumscribed margins in the lower quadrant of the left breast Fig. . There wMRI results are illustrated in Table During surgical procedures, tumors were taupe and pale yellow in color and were partially circumscribed or non-encapsulated. They adhered to the surrounding tissues and invaded muscular structures and adipose tissues. Large amounts of blood vessels were seen in some tumors with a rotten-fish-like look. Microscopic examinations of the tumor cells revealed diffuse infiltrative growth without distinguishable margins with surrounding tissues. Some tumor cells were bulky, fusiform or irregularly shaped, undergoing occasional mitosis and mild interstitial myxoid change and accompanied by a small amount of inflammatory cell infiltration. Tumor necrosis was present with local hyaline degeneration. Four cases presented calcium deposits and bone metaplasia. Immunohistochemistry analysis results were Vim (+), CD99 (+), desmin (+), NSE (some cells +), CD117 (−), SMA (+), S-100 (−), and ki-67 (1–60%).“Pathology and Genetics of Tumors of Soft Tissue and Bone” in 2002, and characterized by intermediate grade  malignancy. Tumor cells were considered as the origin of myofibroblasts. The WHO 2013 document maintained the classification of this disease [Myofibroblastic tumor cell is a type of contractible fusiform mesenchymal cell. It integrates the morphologic features of fibroblasts and those of smooth muscle cells into itself . It was To the best of our knowledge, only a few studies about LGMS have been conducted, reporting 65 cases of this disease –9. TumorAlthough LGMS is classified as an intermediate grade tumor, high recurrence and metastasis rate highlight the need for more pathological analyses. Among the 65 cases mentioned above  and the Scarcity of imaging studies of LGMS makes imaging characteristics of this disease still poorly understood. To our knowledge, imaging findings about 4 LGMS cases have been reported so far , 7–9, anBreast LGMS is very rare. To our knowledge, 1 of our 14 cases represents the fourth reported case –13. It iLGMS is a rare type of tumor. Only few systematic studies of its diagnostic and radiological features have been conducted. In this study, we find that LGMS is characterized by several imaging features: invasiveness, metastasis and calcification. Inflammatory myofibroblastic tumors are likely to progress into LGMS after several recurrences. A large portion of the tumors in our study were located in bones. This study has two limitations. Only small amounts of patients were recruited. And more imaging examinations should have been performed on each patient. Therefore, further exploration is need to understand imaging features of LGMS better, for example, the most preferred site. Although it is classified as an intermediate grade tumor, LGMS has high rate of recurrence, metastasis and calcification, which also highlights the need for further clinical and pathological analysis. With accurate diagnosis comes effective treatment.


Introduction: Surgical treatment in oncology is one of the main part concerning the surveillance rate of the patient in case of tumor recurrence. Metastatic suspected lesions are mostly located in the abdomen or pelvis and are diagnosed by PET, MRI, or CT scan. Especially surgery of small lesions in recurrent disease for diagnostic or therapeutic purpose is often challenging.Material and Methods: We report a case series of 3 patients who were treated in our department due to a metastatic suspected lesion in PET-CT in follow up. For histological confirmation we performed a laparoscopy using a near infrared camera (NIR) for an improved visualization of the metastatic suspected lesion during surgical treatment. Previously the lesion was marked with an amount of Indocyanine Green (ICG) via computer tomography-guided percutaneous injection. The lesion was identified via NIR camera. While changing the camera in NIR mode, it show up as a blue spot due to the fluorescent signal. After correct identification it was removed and send to pathology.Results: In all 3 cases they confirmed the diagnosis of a metastatic lesion. Complication occur in just one case, where the metastatic lymph node infiltrated the external iliac vein, which led to a high blood loss. In this case a vascular interposition had to be done.Conclusions: Because of separate wavelengths, which are used for illumination and recording, only the marked area is visible, not the background.Due to correct identification, resection of the lesion was improved and healthy surrounding tissue could have been spared. Metastatic suspected lesions are mostly located in the abdomen or pelvis and are diagnosed by PET, MRI or CT scan Figure . EspeciaAs a tracer Indocyanine Green is mostly used for SLN mapping in Cervical and Endometrial Cancer and showed increased detection rates compared to the Conventional Dyes  , 3. BecaIn our department we treated three patients due to metastatic suspected lesion in the abdomen/pelvis caused by cervical cancer (2) and carcinosarcoma (1). The first patient was a 35-year old women with a metastatic suspected lesion 2 years after cervical cancer. Initial surgical treatment on FIGO IIA was performed as Total Mesometrial Resection with sentinel lymph node biopsy and was followed by radiation and chemotherapy. The lesion was located on peritoneal site paracolic left. It was resected without difficulties after mobilization of the colon descendens. In this case an expander was inserted to protect the colon in radiotherapy.The second patient was a 30-year old women with metastatic suspected lesion near external iliac vein 2 years after cervical cancer. Initial surgical treatment on FIGO IB1 was performed as extended cervical conization with sentinel lymph node biopsy, pelvic lymphadenectomy right and paraaortic lymphadenectomy and was followed by radiation and chemotherapy. The lesion was marked on the left side without complication. Due to infiltration of the external iliac vein a severe bleeding was caused by trying to dissect the lesion. Therefore a laparotomy had been performed to stop bleeding. Patientin three, a 74-year old women, had a suspected lesion near external iliac vein 2 years after carcinoasarcoma. Initial operation was performed on FIGO IA by hysterectomy, adnexectomy, pelvic and paraaortic lymphadenectomy and omentectomy. No radiation or chemotherapy has been followed. In this case we performed a prophylactic clamping of the external iliac vein to avoid a mayor complication. The vein was blocked with bulldog-clamps. The operation was performed laparoscopically without any complications. This case is demonstrated in the video.No ICG was used prior to mark the lymphnodes. Therefore we used ICG to mark the metastatic suspected lesion computer tomography guided in all three cases.One vial of 25 mg ICG powder was suspended with 5 ml of sterile water to get a maximum of concentration. We tried to insufflate as less as possible due to little capacity of the lymph node. After running a conventional CT with contrast agent first to demonstrate the lesion, 0.2–0.3 ml of ICG was injected into the lesion via 20-Gauge needle computer tomography guided (Figure We performed a conventional laparoscopy with a NIR camera. In all three cases the suspected lesion show up as a blue spot Figures , 4. The Complication occur in just one case, where the metastatic lymph node infiltrated the external iliac vein, which led to a high blood loss. In this case a vascular interposition had to be done.In our video we are explaining how it is possible to detect small lesions laparoscopically after preoperative computer tomography guided Indocyanine Green injection. In a case series of 3 patients we were able to demonstrate an effective method of correct excision. Especially in the age of modern hybrid operating theaters, which are not yet in routine use in gynecology, a preoperative computer tomography guided injection with a fluorescent dye is a possible method which may lead to shorter operation times and less trauma by correct identification of the suspect lesion. Despite the fact that we do know the limitation of our case series, we truly believe that this cases could pave the way for the establishment of a new operation procedure.All subjects involved in this case series gave written informed consent in accordance with the Decleration of Helsinki.AK and AG conception or design of the work and drafting the article. AK, GM, RL, and JF data collection. AK, GM, and AG data analysis and interpretation. AK, RL, JF, GM, and AG critical revision of the article. AK, RL, JF, GM, and AG final approval of the version to be published.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.


Mucocele is a common benign lesion otherwise rarely located in the sphenoid sinus. Some complications after head and neck irradiation have been described in the literature until now. To our knowledge, this is the first report of a sphenoid sinus mucocele in a North African patient treated some years before with radiotherapy for a nasopharyngeal carcinoma (NPC). We extend the literature review about this infrequent finding, of which head and neck surgeons should be aware. Key words:Mucocele, sphenoid sinus mucocele, nasopharyngeal carcinoma, radiotherapy, North African. Isolated sphenoid sinus mucocele is a rare entity. Its incidence has been reported to be 1-3% of all mucoceles.Diagnosis of sphenoid sinus mucocele is often delayed due to its late symptoms that are not evident until it expands and compresses neighbouring structures. Furthermore, this location makes it impossible to direct examination . CorrelaA 49 year-old female patient originally from Maghreb was referred to our Department due to some abnormalities found on a magnetic resonance imaging (MRI) developed for temporomandibular joint dysfunction.The patient had undergone radiotherapy for a NPC 11 years before.MRI revealed a cystic mass located in the clivus area Fig. a,b. ContThe patient had gone to the Emergency Unit complaining about visual disturbances some weeks before.Under general anaesthesia marsupialisation of the mucocele was performed, and a thick yellowish mucopurulent fluid was aspired from the mass Fig. .Pathological findings of the lining mucosa showed a mucous retention cyst with no evidence of tumor cells Fig. . IntraopInformed consent was obtained by the patient.Mucoceles are considered the most frequent expansive lesions of the paranasal sinuses . They arMucoceles are considered to arise due to sinus ostium obstruction, preceded by fibrosis, infection, trauma, surgery (35-66%), fibrous dysplasia, osteoma or ossifying fibroma. Radiotherapy for a NPC is a rare ethiology for this entity; scarcely any case has been described in the literature before .Although being rare in Caucasian, it is endemic in southern China. Southeast Asia, North Africa, the Middle East and the Arctic are considered intermediate-risk regions .Nasal endoscopy followed by a CT scan or an MRI are useful tools for the diagnosis, since physical examination might be normal. CT scan would be characterized by a sinus homogeneous isodense mass which does not enhance with contrast administration; it totally fills the sinus cavity and surrounding structures. Mucoceles usually show moderate or low signal intensity on T1 weighted MRI images and high signal intensity on T2 weighted images, although in some cases there is a high protein content and density, which leads to T1 high intensity images, as in the present case .et al. analysed paranasal sinuses CT scans before and after radiotherapy in patients with NPC. They could find an increase in chronic sinus disease due to mucous membrane atrophy, as a late complication of radiotherapy  after radiotherapy, since only a 9% of recurrence arising 5 years after radiotherapy has been reported (Endoscopic drainage, which is effective and secure, is considered the outstanding treatment for this entity .et al. in 1991. In that case authors supported the idea that sinus ostium was occluded by scarred mucosa following radiotherapy, leading to mucocele formation (Only few cases of sphenoid mucocele after irradiation for NPC have been reported in the literature, most of them being chinese male patients . Common Sphenoid sinus mucocele developing after radiotherapy for NPC has rarely been reported and it has been mostly described in chinese man. Its presentation may sometimes mimic recurrence and hence, clinical history, examination, CT scanning and MRI are useful tools for the diagnosis. It is crucial for oral surgeons and otorhinolaryngologists to keep this uncommon entity in mind in order to allow early diagnosis and treatment and prevent possible complications developing in the orbits or intracranially.


Gastrointestinal stromal tumors (GISTs) grow relatively slowly and without specific symptoms; therefore, they are typically incidental findings. We report a rare gastric GIST in the mediastinum associated with chest discomfort and an esophageal hiatal hernia.An 81-year-old woman with chest discomfort was admitted to the hospital, where barium esophagography showed a sliding esophageal hiatal hernia and a tumor of the lower esophagus and gastric wall. Esophagogastroscopy confirmed the presence of a huge submucosal tumor that extended from the lower esophagus to the gastric fundus. According to computed tomography, the mediastinal mass measured 12.7 cm and had heterogeneous low-density areas. A submucosal gastric tumor, which we suspected to be a GIST, was diagnosed in association with an esophageal hiatal hernia. Using thoracolaparotomy, we performed a total gastrectomy, a lower esophagectomy, and a Roux-en-Y reconstruction with the jejunum. The presumptive diagnosis was confirmed through immunohistochemical examination; immunostaining yielded results positive for CD34 and c-kit. The patient was discharged from the hospital 13 days after surgery with no complications and remained disease-free at follow-up 24 months after surgery.GIST should be considered in the differential diagnosis of tumors growing in the mediastinum. Gastrointestinal stromal tumors (GISTs) are the most common mesenchymal tumors of the gastrointestinal tract , althougAn 81-year-old woman was admitted with chest discomfort. She had a history of appendectomy, hypertension, colon polyps, and osteoporosis. Physical examination revealed no tenderness and no palpable mass in the abdomen. Laboratory investigation yielded unremarkable results, and the values for hemoglobin and tumor markers (including carcinoembryonic antigen and CA19-9) were normal. Barium esophagography, ordered because of the clinical findings, revealed a sliding esophageal hiatal hernia associated with a defect of the lower esophagus and the gastric wall that was caused by a huge tumor  of the eight tumors were diagnosed before treatment. Pathological study revealed positivity for c-kit and CD34 in all cases. Risk for recurrence was high in 50% of the patients, according to Miettinen’s criteria, and in 75% according to Joensuu’s criteria , 20. It Several studies have shown that neoadjuvant chemotherapy is useful for treating primary or secondary GIST , 22. We Immunohistochemical examination revealed a c-kit-positive tumor with a mitotic index of 2 per 50 high-power fields and a diameter of 14.0 cm at its largest aspect. Thus, the tumor was classified as having moderate risk of recurrence, according to Miettinen’s criteria,  and at hWe have reported our experience with a rare case of GIST located in the mediastinum as a result of an esophageal hiatal hernia. This diagnosis should be considered in the differential diagnosis of tumors growing in the mediastinum.


Masses which develop on the surface of the rib bones are rare. The differential diagnosis includes benign and malignant lesions.A 23-year-old European woman presented at an out-patient clinic with a 9-month history of a painless swelling on the right posterolateral side of her chest wall. The case reported here is of a very rarely seen parosteal osteosarcoma of the rib that was treated with wide resection and chest wall reconstruction. There was no evidence of local recurrence or distal metastasis after a 1-year follow-up.Parosteal osteosarcoma is a locally aggressive malignant tumor, and resection with a wide margin is the most appropriate treatment. Correct diagnosis of parosteal osteosarcoma is challenging for an orthopedic surgeon. Although rare, in the differential diagnosis of lesions located on the ribs, parosteal osteosarcoma should be considered and a systematic diagnostic approach should be taken. Osteosarcomas (OSs) are the most frequent primary malignant bone tumor in which neoplastic cells produce osteoids and they usually develop in an intramedullary location . Group OParosteal OSs are the most common type of surface OSs and comprise approximately 4% of all OSs . ParosteParosteal OSs are usually well-differentiated low-grade lesions with a better prognosis compared to conventional OSs . The casA 23-year-old European woman who was working as a secretary, presented at an out-patient clinic with a 9-month history of a painless swelling on the right posterolateral side of her chest wall. She did not recall any trauma that may be associated with this condition neither did she have a history of genetic disease or cancer. A physical examination revealed a hard, painless mass at the posteroinferior and lateral thoracic region. The results of her laboratory tests were all within normal limits.On a plain chest radiograph, an area of calcified opacity was observed at the ninth rib, with no destruction of the underlying bone. Computed tomography (CT) demonstrated a mass of 6 cm × 5 cm × 2.5 cm in size arising from the ninth rib Fig. . There wAfter these examinations, an incisional biopsy was performed. Histopathologic examination revealed fibroblastic and osteoblastic cells with mild nuclear atypia and pleomorphism which was consistent with parosteal OS.She was informed and a wide segmental resection was applied to her eighth, ninth, and tenth ribs with the involvement of parietal pleura Fig. . AfterwaOn gross examination, the lesion was found to be attached to the outer surface of her ninth rib measuring 7 cm × 3 cm × 5 cm. Histopathological evaluation of the resected specimen confirmed it to be parosteal OS. Our patient had no chemotherapy and throughout a 1-year follow-up, there was no evidence of local recurrence or distal metastasis. Informed consent was obtained from our patient before this report.Parosteal OSs are uncommon bone tumors and comprise only 4% of all OSs . While tThe differential diagnosis of parosteal OS from other bone-forming benign and malign neoplasms is yet another complexity . OsteochThe medical history of the patient is also very important because fracture callus and ossifying hematoma can easily simulate bone tumors. So, clinical, radiologic, and pathologic assessments of the lesions are paramount evidence for the parosteal OS differential diagnosis.Parosteal OS is a locally aggressive malignant tumor, and resection with a wide margin is the most appropriate treatment. In the current case, the posterolateral part of our patient’s ninth rib was resected en bloc with her eighth and tenth ribs. Her chest wall was then reconstructed to avoid future pulmonary compromise. In general, chest wall reconstruction is recommended for defects > 5 cm or when more than two ribs are resected to avoid flail chest. In the current case, the chest wall defect was > 10 cm and three ribs were resected with intercostal muscles and parietal pleura to achieve a clear margin. The chest wall reconstruction was applied with a collagen mesh and locked plate and no complications were observed during follow-up.A rare case of parosteal OS located on a rib is presented here. Although rare, for the differential diagnosis of lesions located on the ribs, parosteal OS should be considered and a systematic diagnostic approach should be taken.


Lymphoma originating from the middle ear is rare. The diagnosis of lymphoma as with other cancers of the temporal bone is often made late, and this has a negative implication on the treatment and prognosis of the condition. The delay of diagnosis is mainly due to the similar presentation shared with other benign conditions of the middle ear. We present a case of a 62-year-old man who was treated as a case of chronic otitis media for a period of time before presenting with advanced symptoms; a final diagnosis of lymphoma of the middle ear was given. Other similar cases in the literature are discussed and reviewed. Severe and persistent symptoms of the middle ear should raise red flags and warrant detailed investigations. Lymphoma is a cancer of the reticuloendothelial system; it has two subtypes, Hodgkin's lymphoma and non-Hodgkin's lymphoma. Furthermore, lymphomas constitute 10% of all head and neck tumors; 60% of these are non-Hodgkin's lymphoma and the other 40% are Hodgkin's lymphoma.Diffuse large cell B lymphoma is the largest subunit of non-Hodgkin's lymphoma, and it is characterized by extranodal involvement. The most common sites of extranodal involvement are the stomach and the gastrointestinal tract; however, lymphoma can present in any type of tissues. The bony involvement comprises 1-2% of malignant lymphomas and 3–5% of extranodal lymphomas; involvement of temporal bone is extremely rare with only few reported cases in the literature [A 62-year-old man presented to the otolaryngology clinic with a House-Brackmann grade 4 left lower motor facial nerve palsy with a 10-day history of left postauricular pain. This was preceded by 6 months of intermittent purulent discharge from the left ear for which he received multiple antibiotic courses. On examination, the patient was vitally stable; however, he was febrile. The left postauricular area was mildly tender on palpation and the overlying skin was normal. Otoscopic examination of the left ear was only significant for an erythematous and retracted tympanic membrane. The rest of the examination of the right ear along with a full head and neck exam was unremarkable. White blood count was elevated with 13900 .The patient was admitted to the hospital and had an initial diagnosis of mastoiditis with facial nerve paralysis. He was started on intravenous antibiotics. High-resolution computed tomography (HRCT) and magnetic resonance imaging were performed Figures –4.Due to the severe complication of the facial nerve palsy a decision was made for surgical intervention; cortical mastoidectomy with facial nerve decompression and left middle ear exploration was performed. Granulation tissue in the mastoid air cells and the middle ear were encountered and removed. Biopsies were also taken and sent for histopathology.The histopathological assessment of the tissue revealed diffuse proliferation of large monomorphic atypical lymphoid cells admixed with few medium-sized cells (centroblastic and prominent immunoblastic lymphoid cells) .The cells showed scant to moderate amphophilic cytoplasm, vesicular nuclei, or focal irregular chromatin clumping with prominent 1-2 nucleoli.Frequent mitoses and few tumor giant cells were regarded along with subendothelial infiltrate and occasional pseudorosette Figures  and 7.The proliferative fraction as detected by Ki67 immunostaining is very high (80–90% positivity) .The patient was then transferred to the oncology unit for staging and further management. There was no distant spread and the patient was treated with courses of chemotherapy. At the most recent follow-up his ear symptoms of otalgia and persistent discharge have resolved; however, he still showed a House-Brackmann grade 3 left lower motor neuron facial palsy.Carcinoma of the temporal bone is rare, and most of the pathological diagnosis in such cases turns out to be squamous cell carcinoma. In addition, adenoid cystic carcinoma, adenocarcinoma, and basal cell carcinomas along with metastasis are also reported. Other malignant tumors that can occur in the temporal bone are melanoma, rhabdomyosarcoma, chondrosarcoma, and lymphoma .Lymphoma of the middle ear can arise from the mucosa of the mastoid antrum, tympanum, or the tympanic orifice of the eustachian tube. In addition to that it can arise primarily from the temporal bone, as in our case, which is in the mastoid part of the temporal bone.Diagnosis of cancer in the middle ear and the mastoid process is seldom made early; this can contribute to the unique anatomy of middle ear and the fact that many benign pathologies such as otitis media and its complications share similar clinical presentations; most commonly reported symptoms include otorrhea, otalgia, aural fullness, tinnitus, and hearing loss .Severe hearing loss, facial nerve weakness, and vestibular dysfunction are advanced sequel of middle ear pathology that should raise red flags for a more sinister cause. In addition to malignancy, autoimmune conditions such as granulomatosis with polyangiitis and sarcoidosis and immunodeficiency conditions should also be considered in the differential diagnosis of cases of refractory chronic otitis media with facial nerve palsy. Delay in management of such cases usually occurs due to a low index of suspicion of the treating physician.Saito et al. presented a similar case in a 58-year-old woman who presented with headache and auditory disturbances in addition to otitis media; further investigations revealed that the patient has transverse sinus thrombosis. Surgery was performed 6 years after initial presentation, when only the diagnosis of lymphoma was made. The patient then recovered fully after a course of chemotherapy. They have also reviewed the literature and found 10 cases of lymphoma arising around the middle ear; the most common presentations were facial nerve palsy and otorrhea which is consistent with the presentation of our case [Roberts et al. have presented a case of a 78-year-old woman with large B-cell lymphoma of the middle ear presenting as chronic otomastoiditis. However, in their case there was no involvement of any of the cranial nerves, and emphasis was made on the early detection of temporal bone malignancy through neurotological examination and imaging as early diagnosis and aggressive chemotherapeutic treatment for primary lymphoma of the middle ear lymphoma are crucial for a good outcome [Our case presented similarly to other cases in the literature; however, earlier detection within the period where he had persistent symptoms refractory to medical treatment may have prevented the advanced complication of facial nerve palsy and increased his chances of cure.Lymphoma in the temporal bone is very rare; it may manifest with symptoms that are similar to other common benign middle ear pathologies. In severe and persistent symptoms a malignant cause should be put into consideration; this ensures early detection and thus management of the condition.


Myxofibrosarcoma (MFS) is most often found on the limbs of aged male people, but extremely uncommon in the liver.A 52-year-old female patient with a liver mass was diagnosed as a primary MFS. It had no obvious abdominal symptoms, and the tumor was resected with an extended margin. Three years after the surgery, the patient was readmitted for peritoneal metastasis and passed away 4 months later. The tumor has a benign presentation, but malignant outcome.Comprehensive radiological inspection, intensive preoperative evaluation, careful design of operating procedures, wide margin resection, consecutive treatment, and strict periodical follow-ups should be taken to ensure a better prognosis of this kind of neoplastic disease. Angervall et al. first described myxofibrosarcoma (MFS) in 1977 as a class of fibroblastic neoplasm, which showed a wide range of cellularity, nuclear polymorphism and proliferative capability  and HollMFS is most often found on the limbs of aged male people, but extremely uncommon in the liver. Rare occurrences have been reported in cranial cavity , orbit , maxillaA 52-year-old female patient with no obvious abdominal symptoms was admitted for liver mass found by ultrasound examination. A hypoechoic solid mass ranged 5.7 * 5.6 * 5.0 cm was detected in the left lobe, with a clear outline and abundant blood flow inside the tumor. Physical examination showed no jaundice in the skin and sclera, liver palms, and any spider angioma. The abdomen was flat and soft, with no varicose veins. No tenderness or rebound pain was induced by palpation. All investigations of chest and limbs were normal. Laboratory tests showed normal blood cell count and liver functions except a slightly increase of total bilirubin to 21.3 μmol/L. Liver tumor related cell markers, including alpha feto-protein, carcino-embryonic antigen, and carbohydrate antigen 199 were all within normal values. Hepatitis serological tests were all negative. No family or personal history of malignancy disease either.Both CT and MRI examinations revealed the mass in the IVth segment of the liver with a multi-lobular appearance, clear boundary, and internal separations. The solid mass can be enhanced in the CT scan from 20Hu to 30Hu. Long T1 and T2 signal were showed in MRI, with only a weak strengthening in the delayed phase, suggesting the high probability of a benign hepatic tumor  showed staining of Vimentin Fig. , SMA, CKThree years after the surgery, the patient was readmitted because of massive ascites. Severe peritoneal effusions and omental thickening were detected by both ultrasound and enhanced CT scan. An ultrasound-guided percutaneous biopsy of the omentum was performed and followed by H.E. staining. It was found that spindle-shaped cells were distributed in a bundled or interlaced pattern with a relative high density. Multi-sized nuclei were slightly enlarged and eosinohilic cytoplasms were transparent but lack clear boundaries with surrounding myxoid stroma Fig. .Fig. 6H− 100. Combination of the clinical progress with histopathological findings indicated that these metastatic lesions were developed from a malignant tumor with a mesenchymal origin, thus considered as low-grade MFS. Pathological consultation was carried out and a historical review of the original liver tumor slices indicated a high homology between these two lesions. The second onset was therefore considered as peritoneal metastasis of the primary liver tumor. This patient passed away 4 months later due to multiple organ failure.IHC staining of the biopsy specimen showed positive of vimentin, calretinin, and D2–40, < 5% Ki67, but weak for CK and negative for CK7, CK20, SMA, CEA, CA125, GLUT-1, WT1, CD34, and SMalignant tumors in the liver are predominantly carcinomas, and sarcomas in rare cases. In our report, neither clinical presentation nor radiological examinations revealed the occurrence of a primary tumor elsewhere. This patient had no family or personal history of malignancy either. Unobvious symptoms, non-apparent signs, negative serology tests, near to normal laboratory reports, and untypical radiology appearance misled us to a diagnosis of benign hepatic tumor rather as the MFS of liver.MFS are classified into two types, solid and “tail-like” pattern by T2-weighted magnetic resonance images (MRI). In “tail-like” type, there are extensive spread along the fascial planes that extended away from the primary site of tumor . MFS witIf a biopsy was performed to suggest a diagnosis of MFS, a thorough radiological investigation to estimate the tumor extension should be mandatory. Due to its infiltrative nature, a much wider margin should be applied even for a low or intermediate-grade MFS to prevent a local recurrence . In caseIn general, low grade MFS is shown as a jelly-like multinodular tumor with a weakly invasive pattern of growth. Under microscope, spindle or stellate-shaped tumor cells are mainly distributed on a mucoid matrix (50%), mainly consists of hyaluronic acid . Extra fStringent follow-up was not required in our patient because there was no report of metastasis in hamartoma. In contrast, although low grade MFS is considered to have less potential of malignancy, it shows considerable frequency of metastases about 20 to 25%) , 20. Met to 25% ,To our knowledge, this is the first case report of a primary MFS in the liver. Although the occurrence is remarkably rare, we should learn a lesson and bear in mind of this probability. Comprehensive radiological inspection, intensive preoperative evaluation, careful design of operating procedures, wide margin resection, consecutive treatment, and strict periodical follow-ups should be taken to ensure a better prognosis of this kind of neoplastic disease.


Inflammatory myofibroblastic tumor is a rare benign neoplasm that frequently involves the lung and abdominopelvic region, and is found mainly in children and young adults. Inflammatory myofibroblastic tumor tends to be locally invasive or recurrent, and rarely metastasizes.A 76-year-old Korean man presented with a history of upper back pain for 2 months and motor weakness in both lower extremities for 2 days. Contrast-enhanced computed tomography of his chest and abdomen showed a large heterogeneous pleural mass involving the right fifth rib and vertebral body and a mass infiltrating the right renal hilum. Computed tomography-guided percutaneous needle biopsy of the pleural mass was performed. The histological findings on hematoxylin and eosin staining showed proliferation of spindle cells with infiltration of lymphocytes and plasma cells. Immunohistochemistry showed neoplastic cells positive for CD68, focally positive for smooth muscle actin, and negative for cytokeratin and desmin. Inflammatory myofibroblastic tumor was diagnosed based on the histological examination. Treatment with glucocorticoids (methylprednisolone 1 mg/kg) and radiotherapy  was started. After 1 month, chest computed tomography showed a reduction in the size of the pleural mass, and abdominopelvic computed tomography showed decreased infiltration around the right renal pelvis.Inflammatory myofibroblastic tumor is a rare neoplasm of intermediate malignant potential due to a tendency for local recurrence and it rarely develops distant metastases. Complete surgical resection is the primary treatment. However, unresectable and metastatic inflammatory myofibroblastic tumor can be treated with systemic therapy, including glucocorticoids, radiotherapy, and/or chemotherapy. Inflammatory myofibroblastic tumor (IMT) is a rare benign neoplasm that frequently involves the lung and abdominopelvic region, and is mainly found in children and young adults . The hisHere we present the case of a 76-year-old Korean man with pleural IMT involving the adjacent chest wall, with metastasis to the kidney.A 76-year-old Korean man presented with upper back pain for 2 months and motor weakness in both lower extremities for 2 days. A chest radiograph and computed tomography (CT) performed at another institution showed a pleural mass in the upper lobe of his right lung. He was referred to our hospital for evaluation of the pleural mass and paraplegia. He had been treated with medication for 4 years for type 2 diabetes mellitus and primary hypertension. He did not have a family history of malignant disease. On chest examination, he had tenderness at the level of the fifth rib on the right side. On neurologic examination, he showed paraplegia with numbness and a sensory deficit below the T5 dermatome.Laboratory findings showed an elevated C-reactive protein level of 6.84 mg/dL , blood urea nitrogen of 25 mg/dL , and a serum creatinine of 1.5 mg/dL . Urine analysis showed 4+ blood, with many red blood cells and 0–1 white blood cells/high-power field  staining showed proliferation of spindle cells with infiltration of lymphocytes and plasma cells Fig. . ImmunohAs the tumor could not be completely resected, treatment with glucocorticoids (methylprednisolone 1 mg/kg) and radiotherapy  was started. After 1 month, laboratory findings were unremarkable. Hematuria, which is thought to be caused by kidney metastasis of IMT improved after treatment  locus at 2p23 [The etiology and pathogenesis are unknown. Several theories have suggested involvement of the immunologic response to infectious agents such as Epstein–Barr virus or human herpesvirus 8, or an inflammatory reaction to an underlying low-grade malignancy . Griffin at 2p23 . These f at 2p23 . ALK-pos at 2p23 .In our case, the patient presented upper back pain and motor weakness in both lower extremities due to IMT involving pleura and vertebra body. Laboratory findings showed an elevated erythrocyte sedimentation rate and elevated C-reactive protein. In addition, urine analysis showed hematuria at admission due to metastasis to the kidney. The clinical features are determined using the location of IMT, with 15% to 30% of patients presenting with fever, weight loss, and malaise. Laboratory findings include an elevated erythrocyte sedimentation rate, elevated C-reactive protein, microcytic hypochromic anemia, and thrombocytosis . Signs aImaging findings vary according to the site of involvement. Pulmonary IMTs usually present with a solitary, peripheral, circumscribed, lobulated mass on chest radiography. Chest CT shows heterogeneous attenuation and enhancement. An amorphous, mixed, or fine fleck-like pattern of calcification has been reported in pulmonary IMTs. MRI shows homogeneous or heterogeneous signal intensity on T1-weighted and T2-weighted images. Kidney IMTs show homogeneous or cystic masses on enhanced CT –15. EnhaThe most accurate diagnostic method is surgical excision of the lesion. Fine-needle aspiration is often unable to diagnose the disease because specimens are aseptic and show a mixture of inflammatory cells . The diaet al. suggested that vinorelbine and methotrexate is a therapeutic option for adult patients with relapsed and unresectable IMT [et al. reported a sustained partial response to the ALK inhibitor crizotinib in a patient with ALK-translocated IMT [The treatment of choice for IMT is complete surgical resection. In this case report, our patient was unable to undergo complete surgical resection because IMT invaded the adjacent chest wall. So, if patients have contraindications to surgical resection, or have unresectable or locally aggressive disease, glucocorticoids, chemotherapy, and radiotherapy can be used. Steroid therapy has shown good response in many studies. Various cytotoxic drugs or regimens  have been used for treatment with unresectable and/or relapsed IMT in a case series. Recently, Maruyama able IMT . Radiothable IMT . Howeverable IMT , 20. Altated IMT .The recurrence rate varies according to the site of involvement, from < 2% for tumors confined to the lung to 25% for extrapulmonary IMTs associated with an abdominopelvic site, multinodular tumor masses, and incomplete resection. Distant metastasis is rare . DevelopWe presented a rare case of pleural IMT involving the adjacent chest wall and kidney metastasis. IMT is a rare neoplasm of intermediate malignant potential, with a tendency toward local recurrence and rare distant metastasis. Complete surgical resection of IMT is the primary treatment. However, unresectable and metastatic IMT can be treated with systemic therapy, including glucocorticoids, radiotherapy, and/or chemotherapy.


A 34-year-old Cape Verdean male with terminal chronic renal failure of unknown etiology since two years was referred to the urology department for a second opinion after finding an infiltrative mass on routine renal ultrasound. The patient was nonobese and otherwise asymptomatic. Clinical examination revealed a large palpable mass in the pelvis. Laboratory examination showed highly elevated blood levels of creatinine (10.98 mg/dL (nl: 0.67–1.17 mg/dL)) and urea (123 mg/dL (nl: 16.6–48.5 mg/dL)).We decided to perform a contrast-enhanced CT scan for further evaluation of the mass. The application of contrast agents in this patient with renal failure was permitted because these contrast agents were washed out by dialysis afterwards. The CT examination showed a large pelvic infiltration by fatty tissue with typical attenuation (–40 to –100 Hounsfield units) ; the lower intestinal tract ; and the vascular system (edema of lower extremities) can be compressed. At physical examination, one may observe pain at abdominal palpation, presence of a palpable mass in the hypogastric region, urinary retention, elevation of the prostate on digital rectal examination, lower limb edema, and arterial hypertension. Late complications are obstructive renal failure, proliferative cystitis, and bladder adenocarcinoma.The etiology of pelvic lipomatosis is unknown. Some authors have raised the hypothesis that this disease is a manifestation of generalized obesity; other authors have suggested it is a response to recurrent urological infections. The disease is more prevalent in males and African Americans.Imaging studies such as CT or MRI are crucial in the diagnosis of pelvic lipomatosis. Diagnosis is based on the distinct absorption coefficient of the intrapelvic fatty tissue. The pelvis seems congested because of excessive amounts of symmetrically spread fat. Soft-tissue planes of the pelvis are preserved. There is no truly soft-tissue mass present, and after the administration of a contrast agent, the fat does not enhance. The urinary bladder is extrinsically compressed, vertically stretched out, and raised from the pelvic floor. On ultrasound, there is hyperechogenic fat tissue surrounding the pear-shaped bladder and sigmoid.Treatment options are limited. Dietary management, antimicrobial agents, corticoids, and radiotherapy have not demonstrated efficacity. Complete surgical eradication of the adherent fatty tissue is difficult, and the clinical effect of this intervention is uncertain.The possibility of making a specific diagnosis of pelvic lipomatosis without an invasive technique is useful. Therefore, one should also think about the possibility of pelvic lipomatosis in a patient with chronic renal failure of unknown etiology with an increased amount of adipose tissue in the pelvis in the absence of a truly mass lesion.The authors declare that they have no competing interests.


Killian‐Jamieson diverticulum represents a rare form of esophageal diverticulum originating on the anterolateral wall of the cervical esophagus. Despite its rarity, it is crucial to recognize this entity, with such specific imaging findings, to avoid unnecessary invasive procedures such as fine‐needle aspiration or even surgery. A 77‐year‐old male patient presented with a 1‐year history of swallowing foreign body sensation.He denied dyspnea, dysphagia, or dysphonia. No other ENT symptoms were specified.Physical exam and laboratory blood tests were unremarkable.A neck ultrasound performed in another institution identified a suspicious left lobe hypoechoic thyroid nodule, containing microcalcifications. The patient was referred for fine‐needle aspiration biopsy (FNA).The ultrasound (US) examination performed prior to FNA, revealed a communication of this nodule with the posteriorly located esophagus Figure . PassageNo further treatment was implemented and the patient is stable at six‐month follow‐up.Killian‐Jamieson diverticulum represents a rare entity, originating on the anterolateral wall of the cervical esophagus, below the cricopharyngeal muscle. This diagnosis should be considered when a thyroid nodule is located near the esophagus, especially when it ultrasound features changes after swallowing.Despite its rarity, it is crucial to recognize this entity, with such specific imaging findings, to avoid unnecessary invasive procedures such as FNA or even surgery.WS: Contributed to write the case and identify the images. AG: Reviewed and edited the case report and helped in identifying appropriate images. Click here for additional data file.


Duodenal gangliocytic paragangliomas (GPs) are a subclass of duodenal neuroendocrine neoplasms and are exceedingly rare. They have been associated with an indolent behavior; however, they can rarely metastasize. Radical resection like a pancreaticoduodenectomy is sometimes indicated. We hereby present a case requiring major surgery and perform a literature search about this disease.  A 49-year-old Caucasian female, who presented with an upper gastrointestinal bleed, was found to have a hypodense mass in the second/third portion of the duodenum. A biopsy of the mass during upper endoscopy was inconclusive. A pancreaticoduodenectomy was recommended based on the high suspicion for a duodenal adenocarcinoma and was performed successfully. Her final pathology revealed a duodenal gangliocytic paraganglioma.  The majority of duodenal GPs present as gastrointestinal bleeds while others less commonly present with anemia, abdominal pain, duodenal obstructive symptoms, pancreatitis, or abnormal incidental findings on axial abdominal imaging. Duodenal GPs were initially viewed as benign tumors of the duodenum; however, there have been increasing incidence reports of hematogenous and lymphatic metastasis. Appropriate treatment of duodenal GPs is still controversial and ranges from local endoscopic submucosal resection to major radical surgery.  Duodenal GPs are very rare tumors of the second portion of the duodenum presenting with upper gastrointestinal bleeding and local symptoms of surrounding organs. Local or radical resection is usually recommended to prevent bleeding and the minor risk of metastatic spread. Duodenal neuroendocrine tumors (NETs) are infrequent, comprising about 1–3% of gastrointestinal endocrine tumors . DuodenaA 49-year-old Caucasian woman presented to the emergency room with three-day history of palpitations, shortness of breath, pallor, and black tarry stools. She was found to be anemic with hemoglobin of 4.4 g/dL. She was transfused, and a gastrointestinal bleeding workup was initiated. The rest of the physical examination and additional blood tests were within normal limits. A computed tomography (CT) scan of the abdomen showed a possible hypodense mass in the second/third portion of the duodenum that is intraluminal with an extraluminal component abutting the uncinate process of the pancreas. There was no sign of any other disease in the abdomen and lower chest . ColonosDuodenal GPs were described initially by Dahl et al. in 1957, and they were given their current name in 1971 by Kepes et al. , 14. AftThe clinical presentation of duodenal GPs varies, with upper gastrointestinal bleeding occurring in almost half of the patients (as in the case of our patient), while 15% present with anemia , 17. TheGP are composed of three cell types: (1) epithelioid cells resembling well-differentiated neuroendocrine tumors or paraganglioma in both cytologic and architectural features; (2) spindled cells resembling those seen in peripheral nerve sheath tumors; and (3) ganglion-like cells with characteristic abundant cytoplasm and prominent nucleoli . The celDuodenal GPs were initially viewed as benign behaving tumors of the duodenum; however, there has been an increasing incidence of reports about lymph node metastasis and distant organ metastasis , 18, 19.Duodenal GP are rare tumors of the second portion of the duodenum that present with upper gastrointestinal bleeding and local obstructive symptoms of surrounding organs. They are classified as rare neuroendocrine neoplasms of the gastrointestinal tract and are usually considered benign and assigned an excellent prognosis. Appropriate methods of treatment are still controversial but range from endoscopic submucosal resection to pancreaticoduodenectomy.


Endometriosis is a common entity among fertile women which unfortunately manifests through variable symptomatology. Intestinal involvement in endometriosis is quite common and can simulate several diseases such as Crohn's disease, appendicitis, tubo-ovarian abscess, or malignant tumors. Intestinal obstruction due to endometriosis is rare, and preoperative diagnosis is difficult because the signs and symptoms are nonspecific and can be easily confused. In the case of patients without a history of endometriosis, diagnosis is further complicated. We present a case of a 41-year-old female patient. She presented to the emergency room with complete bowel obstruction and a mass in the cecum. Surgery was decided, and the patient underwent full recovery. Endometriosis was the final diagnosis for the observed condition. Endometriosis is the presence of benign endometrial tissue in extrauterine sites, a condition with a prevalence of up to 10% to 15% of the general population of women . HoweverEndometriosis is common yet complex, as it is associated with a broad spectrum of clinical presentations. Despite chronic pelvic pain being common, women having endometriosis in unusual sites or experiencing complications may present with acute abdominal pain in up to 8% of the cases and require urgent medical attention , 2.The patient was a 41-year-old female with past medical history of appendectomy and dysmenorrhea. She presented to the emergency department with nausea, severe vomiting, and acute pain in the lower abdomen. She reported having experienced asthenia and weight loss for one month. On clinical examination, abdominal distension and tenderness were discovered. Blood tests revealed leukocytosis with neutrophilia, and a contrast-enhanced abdominal computed tomography (CT) showed a 7 × 7 × 4 cm hyperenhanced mass in the cecum that caused complete bowel obstruction . Also, aWith these findings, particularly the observation of a mass through the CT scan, and due to the evident weight loss that the patient had undergone, neoplasia could not be ruled out. Surgery was decided, and at laparotomy, a volume of 200 ml of inflammatory fluid was found in the cavity. Most of the loops of the distal ileum were dilated, and a 7 × 7 × 3 cm cecum mass was discovered, which compromised the ileocecal valve and caused complete bowel obstruction. Surgical decision was straightforward, the cecum mass was completely resected, and a right hemicolectomy was executed. An ileocolic anastomosis was also performed during the procedure. Furthermore, the right adnexal mass that was previously identified through the CT scan (measuring 4 × 3 × 2 cm) was observed to be firmly attached to the ovary and the fimbriae and displayed a pale external capsule surrounded by a cystic component. Gynecology consultation was required, and due to the size of the mass and its characteristics, surgical removal of the right adnexal mass was performed. After completion, closure of the abdominal wall was performed, and the remainder of the procedure continued without any complications.Pathology revealed a 4 × 3 × 2.5 cm blueish heterogenic mass that occluded 90% of the lumen of the cecum and the ileocecal valve. Microscopy revealed that the colon wall was invaded by glands and endometrial stroma. The colonic epithelium showed inflammatory changes and was negative for malignancy Figures . In the The postoperative course of the patient was uneventful. She initiated clear liquids a day after surgery and was discharged once full diet was resumed. On follow-up controls, the patient was completely asymptomatic, without any pain or complications.Ever since Sampson et al. theorized in 1927 that endometriosis could result from retrograde deposition of endometrial remains during menstruation, various theories for the cause of this condition have been proposed, including the coelomic metaplasia of the peritoneum or the dissemination of endometrial particles through lymphatic and hematogenous pathways . The mosEndometriosis is present in up to 12% of fertile women, with the number of cases diagnosed peaking between the ages of 29 and 39 years . Women mThe involvement of the bowel in intestinal endometriosis is typically associated with the disease at other sites , as presBowel obstructions due to endometriosis are rare, occurring in less than 1% of all patients . When thIn our case, it was not possible to establish an accurate preoperative diagnosis due to the clinical features and the images seen in the CT. However, due to the intestinal obstruction, surgery was decided and the patient successfully recovered. Our case also demonstrates in a unique way that despite its rarity, surgeons should be aware that endometriosis can present itself as complete intestinal obstruction and should be considered when approaching women of childbearing age with acute abdominal pain.


To describe an unusual presentation of acquired hypoventilation syndrome treated successfully with noninvasive positive pressure ventilation. We report a case report of a 48-year-old male who presented to the emergency room for recurrent syncope. He was found to have a ventricular colloid cyst causing uncal herniation. The patient was noted to be intermittently apneic and bradypnic. Transient hypoventilation was successfully treated with noninvasive positive pressure ventilation and the patient made a full neurological recovery following transcallosal resection of the colloid cyst. Subsequently, the hypoventilation resolved. With prompt surgical intervention, full neurological recovery is possible after cerebral uncal herniation. In rare circumstances, this can result in transient alveolar hypoventilation. Bilevel noninvasive positive pressure ventilation can be used to successfully manage the hypoventilation. Central hypoventilation syndrome (CHS) is characterized by the disruption of integration signals in ventilatory control system resulting in hypoventilation, hypoxemia, and hypercapnia. The congenital form of CHS is linked to genes involved in the neuronal development of autonomic nervous system . Brainst2 of 35 mm Hg. NIPPV was discontinued and the patient made a full functional neurologic recovery. He underwent a successful transcallosal resection of the colloid cyst.A 48-year-old male presented to the emergency room for recurrent syncope. He became acutely unresponsive while in the emergency room. His pupils became fixed and dilated bilaterally. Emergent computed tomography (CT) of the head revealed a ventricular colloid cyst with hydrocephalus and uncal herniation as shown in Figures Congenital central hypoventilation syndrome (CCHS) usually affects infants, and symptoms often become apparent shortly after birth. Hypoventilation is typically more severe during sleep than wakefulness . EtiologACHS, in contrast, usually results from brainstem lesions or insults affecting the medullary centers that control respiratory drive , 7, 8. CTransient ACHS due to uncal herniation is rare and management strategies for the hypoventilation are lacking. In our case, the transient ACHS was successfully managed with bilevel NIPPV intermittently during wakefulness and continuously during sleep. Although rare, the clinician should be aware of the potential for recovery from ACHS after uncal herniation. In such cases, NIPPV can be considered to manage gas exchange abnormalities.Given the paucity of literature, we aim to bring awareness of this life-threatening presentation of acquired hypoventilation syndrome and add to the clinical experience of favorable outcome when treated appropriately with both noninvasive ventilation support modality and the correction of underlying etiology.


Pneumatosis intestinalis is a rare condition that may be idiopathic or a sign of numerous underlying gastrointestinal, pulmonary and systemic diseases.Herein, the case an otherwise-healthy 82-year-old female patient with vague abdominal pain due to total colonic pneumatosis 20 days after completion of R-CHOP chemotherapy for a stage IIE primary non-Hodgkin’s lymphoma of the terminal ileum submitted to right hemicolectomy and ileal resection 6 months previously is presented. As no evidence of intramural bowel gas was present on pre-operative CT, pneumatosis coli considered to be secondary. As no worrisome clinical, laboratory and imaging findings were present, pneumatosis coli seemed to be benign. As no other etiologic factors identified, pneumatosis coli considered to be chemotherapy-induced. The patient treated conservatively with cessation of enteral nutrition and broad spectrum antibiotics with uneventfull recovery.Pneumatosis intestinalis can be benign or life-threatening. Bowel obstruction, perforation, ischemia and severe colitis represent the most life-threatening causes. In clinical practice it is often challenging to distinguish between life-threatening and benign pneumatosis intestinalis, a decision which should be based on the presence or absence of worrisome clinical, laboratory and imaging findings.In analogous cases, the main dilemma for the physicians is to identify whether surgical intervention is required or not. Given the potential severity of pneumatosis intestinalis, early diagnosis and recognition of its severity is critical as it would dictate surgical or non-surgical management. The patient had a history of right hemicolectomy and ileal resection 6 months previously for a stage IIE primary non-Hodgkin’s lymphoma of the terminal ileum submitted to 8 cycles of R-CHOP chemotherapy. Abdominal CT at initial presentation revealed the presence of total colonic pneumatosis which considered being secondary as no evidence of intramural bowel gas was present on pre-operative staging CT. The question that arised immediately was what was the cause. However on primary survey, it is emerging for the physician not to thoroughly delineate etiology but to distinguish between benign or worrisome secondary PI; ascertaining whether a life-threatening cause such as bowel obstruction, perforation, ischemia and severe colitis exist or not based on clinical, laboratory and imaging evaluation is the primary priority.3.2In 2013, the Eastern Association for the Surgery of Trauma, in a retrospective multicenter study of 500 patients with PI, showed that lactate value of more than 2.0 mmol/L, hypotension, peritonitis, acute renal failure, active mechanical ventilation and absent bowel sounds were significantly correlated with worrisome PI. The authors concluded that the presence of a lactate value of more than 2.0 mmol/L and hypotension had a predictive probability for life-threatening PI of 93.2% . In 20173.3In 2017, Goyal et al, in a retrospective study of 167 patients with PI, showed that location in the small bowel, bowel dilation, mesenteric stranding, bowel enhancement, portal vein gas, mesenteric vein gas and moderate mesenteric edema were imaging findings significantly correlated with worrisome PI . In 20133.4On primary survey it is of primary priority for the physician to ascertain whether a life-threatening cause of PI such as bowel obstruction, perforation, ischemia and severe colitis exist or not based on the presence of worrisome clinical, laboratory and imaging findings. The above surgical emergency situations should always kept in mind during secondary surveys and once excluded further delineation of the exact etiology should be initiated . In the Chemotherapy agents, such as fluorouracil and docetaxel, and molecular targeting agents, such as bevacizumab and sunitinib, may rarely cause PI. The underlying mechanisms are thought to be increased mucosal permeability and decreased submucosal lymphoid tissue . Althoug3.5PI patients with worrisome clinical, laboratory and imaging findings should undergo surgical treatment. In the absence of worrisome findings, patients should undergo a trial of non-surgical treatment under strict re-evaluation. Non-surgical management should focus on treating the underlying gastrointestinal or systemic cause and discontinuation of possible instigating medications. Antibiotics, especially metronidazole 500 mg per os three times daily for up to 3 months or until documented resolution of PI, can be used to target intraluminal and intramural bacteria in order to reduce anaerobic production of hydrogen gas . CessatiOxygen therapy has long been recognized as an effective therapy for PI leading to cyst regression on imaging and symptoms resolution by the following mechanisms: (a) increased tissue oxygenation may facilitate phagocytic activity and directly target the gas-producing organisms via anaerobic impairment; and (b) increased arterial oxygen tension forces oxygen into the hydrogen-containing cysts by diffusion from areas of high oxygen tension in the artery to low oxygen tension in the cysts. In turn, oxygen accumulation in the cysts increases the partial pressure of hydrogen in the cysts causing hydrogen to diffuse out of the high-pressure cyst into the low-hydrogen bloodstream. Cyst resolution follows as the oxygen leaves the cyst via re-absorption for use in cellular metabolism. A trial of hyperbaric oxygen therapy can be used at 2.5 ATA for 2.5 h for at least 3 sessions or until 2 days after the disappearance of cysts to reduce the risk of recurrence .4PI is a rare condition that may be idiopathic or a sign of an underlying gastrointestinal or systemic disease. Given the potential severity of this condition, early diagnosis and recognition of the severity by evaluating certain worrisome clinical, laboratory and imaging findings is critical. Treatment includes surgery or non-surgical treatment by bowel rest, cessation of instigating medications, antibiotic therapy and oxygen therapy.None.None.Ethical approval has been exempted by our institution.“Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal on request”.Boulas K was responsible for the study concept and design. Paraskeva A, Kariotis I, Kariotis S, Keskinis Ch and Barettas N equally contributed in writing the paper. Blouhos K and Hatzigeorgiadis A had the final approval of the paper.No unique identifying number requested for this case report.Hatzigeorgiadis A.Not commissioned, externally peer reviewed.


Most colorectal cancer patients in the early stages of the disease do not display any alarming symptoms. A total percentage of 9-27% of colorectal cancer patients present with acute abdomen, bowel obstruction, perforation, or bleeding. Perforation as the first presentation of the disease is seen in no more than 2.6-10% of patients. Intestinal perforation may be found on either the site of the tumor or on a more proximal site, caused by distention of the bowel due to peripheral obstruction. This is a case of a 75-year-old female patient who presents in the emergency department with retroperitoneal cecal perforation due to an obstructing tumor of the ascending colon. She underwent an emergency right hemicolectomy and washout of the retroperitoneal space. The cecum is not an unusual site of distention and subsequent perforation in the case of colonic obstruction, especially in the presence of a competent ileocecal valve. While the mechanism of diastatic cecal perforation is well described, it is the first time in the literature that this does not occur on the anterior surface of the organ. In our case, cecal perforation presents as a retroperitoneal abscess without peritoneal spillage. Nonetheless, it still carries a grim prognosis and urgent surgical intervention is needed. The reported incidence of complete obstruction ranges from 8% to 40%, while being responsible for up to 85% of emergency colorectal operations, whereas the incidence of perforation ranges from 2.6% to 10% [Colorectal cancer (CRC) is the most common gastrointestinal cancer, representing the 3ng women . Colorec9/L) and neutrophilia. An emergency CT scan of the abdomen was performed, which revealed free air and fluid collection in the retroperitoneal space in close relation to the cecum  . Chen etThe cecum is the most common location of a diastatic perforation, owing to the fact that, according to the law of Laplace, “in a long pliable tube, the site of largest diameter requires the least pressure to distend.” Therefore, in the presence of distal large bowel obstruction, usually in the left colon, and with the prerequisite of a competent ileocecal valve, the cecum is the most common site of perforation . In otheRisk factors heralding the progression of the ischemic changes to frank perforation include a cecal diameter of 12 cm, a long-standing dilation, and an intraluminal pressure greater than 80 mmHg. Additionally, a closed-loop bowel obstruction may only occur in the presence of a competent ileocecal valve, which barium studies have exhibited in only 10-30% of the tested population .V > 2000 mL), probably due to diluted fecal material spilling through the perforation [In case of complications arising from the presence of a CRC or even, as often is the case nowadays, in the evaluation of nonspecific abdominal pain (NSAP), computed tomography (CT) represents the most commonly utilized imaging technique that is also more readily available. In the case of an obstructing lesion, CT is a sensitive imaging modality and it may provide additional information on the transition point of the obstruction. In an obstructing colorectal cancer with a competent ileocecal valve (closed-loop obstruction), CT may identify the obstructing mass causing severe dilatation of the proximal colon, while the small bowel will not be dilated. In such cases, the finding of a cecal diameter over 12 cm or cecal pneumatosis is often interpreted as a sign of ischemia and imminent perforation of the cecum may ensue. Perforation of the colon may also be demonstrated by CT with the depiction of a defect in the colon wall that may be furthermore accompanied by a fluid-density abscess, free air, or stranding of the pericolic fat. Identifying the irregular wall thickening of the adjacent colon is critical for making the diagnosis of underlying colon cancer, thus differentiating a perforation caused by benign causes. However, in some cases, the severe pericolic inflammation accompanying a large abscess or peritoneal spillage may make it difficult to make an accurate preoperative diagnosis . Anotherforation .A retroperitoneal abscess may be of multifactorial origin, and it manifests usually secondary to infections of the gastrointestinal or genitourinary tract. If no definite etiology can be discovered, it is characterized as primary. Important predisposing factors are considered to be diabetes mellitus, muscle trauma, and individuals tested positive for HIV. The most common causes of retroperitoneal abscesses are infections of the duodenum, pancreas, terminal ileum, appendix, and ascending and descending colon. Retroperitoneal abscesses, in addition, may result from microbial agents such as tuberculosis, Staph. aureus, E. coli, Bacteroides species, or other rare bacteria, such as the Actinomyces species . Since cThe principles of damage control surgery may also apply when the surgeon confronts a diastatic colonic perforation, namely, source control and further resuscitation of the patient in the intensive care unit. A right hemicolectomy and an ileostomy with a distal colonic fistula without intraperitoneal anastomosis are usually warranted , as the anastomotic leak rate is very high in generalized peritonitis. In the frailest of the patients, the perforation may be exteriorized as a colostomy, providing thus source control and obviating the need for a major procedure . In our A diastatic perforation carries grim perioperative mortality, reported to be as high as 90%, as well as a worse long-term outcome, compared to tumor site perforation , 12, 15.Since the role of radiotherapy and chemotherapy in CRC is expanding, and due to the fact that the population is aging, the prevalence of diastatic colonic perforation may be on the rise. Although at present it is an infrequent event that many surgeons may not encounter during their whole careers, one should bear in mind that a pneumoperitoneum is not always due to tumor perforation and that an adequate search for the perforation site should include the whole colon.


Head and neck neuroendocrine tumors (NET) are a rare type of cancer. NET can be classified according to the histopathological features. The typical carcinoid tumor is a well-differentiated tumor that is the least common among other types. Owing to its indolent behavior and variable radiological and pathological features, treatment of carcinoid tumors remains a challenge. We report a case of a 54-year-old man presenting with a non-operable carcinoid tumor arising in the sphenoid sinuses treated with radiotherapy with stable disease control after three years follow-up. Neuroendocrine tumors (NETs) are a group of neoplasms with various clinical presentations and growth rates. Although rare, neuroendocrine carcinoma has been well-described in head and neck cancers . Of all A 54-year-old man presented in 2015 with a 12-month history of persistent left nasal obstruction and nasal discharge, as well as episodes of self-resolving epistaxis. He was first treated for presumed nasal polyps and sinusitis without improvement. The patient is known to have hypertension. He denied any history of tobacco or alcohol use and had no prior history of radiation.On physical examination, the patient had a Karnofsky Performance Status (KPS) of 90%. On fiber-optic examination, there was evidence of a mass filling the left nasal cavity. No palpable cervical lymphadenopathy was noted. There was no evidence of neurological deficits, and cranial nerves, II to XII, were intact.A computed tomography (CT) scan and magnetic resonance imaging (MRI) of the head and paranasal sinuses revealed a large, enhancing soft tissue mass (6.1 x 4.9 x 4.1 cm) centered in the sphenoid sinus with an invasion of the base of the skull and clivus, chromogranin A (CgA), CD56, and cytokeratin AE1/AE3. They were negative for CK7, CK20, and S-100. The Ki-67 proliferative index was less than 1%. The pathological diagnosis was a typical carcinoid neuroendocrine tumor of the sphenoid sinus  scan revealed no evidence of regional or distant metastasis. Clinical staging of this tumor, based on the American Joint Committee on Cancer (AJCC) TNM staging system for the nasal cavity and paranasal sinuses , was T4bThe patient was seen by a head and neck surgeon who considered that the tumor was unresectable. The patient was treated with definitive intensity-modulated radiation therapy (IMRT). He received a total dose of 60 Gy in 30 fractions  that showed a significant decrease in metabolic activity. The size was stable, and there was evidence of central necrosis Figure -4B. DuriHead and neck tumors represent around 3% of all cancers in the United States . SquamouThe clinical and histological heterogeneity of NETs makes it a challenge for the pathologists to diagnose and the oncologists to treat. Well-differentiated NET (carcinoid) produces abundant secretory granules with intense immunoexpression of neuroendocrine markers, such as Syn, CgA, CD56, and cytokeratin AE1/AE3. These are characteristically arranged in a well-developed “organoid” or neuroendocrine shape with nesting, trabecular, or gyriform/serpentine growth patterns. Both the mitotic rate and the presence of necrosis represent criteria used to distinguish between typical and atypical carcinoids. Typical carcinoids are characterized by a low mitotic rate (< 2 mitoses per 2 mm² 10 high-power field (HPF)) and the absence of necrosis, whereas atypical carcinoids show a moderate mitotic rate (2 - 10 mitosis per 2 mm² 10 HPF) and/or the presence of necrosis. Immunostaining to S-100 is typically negative for NETs, and Ki67 index is usually low (< 3%) in carcinoid tumors . Since tTo date, the treatment of head and neck NETs remains challenging and controversial, with surgery being the mainstay treatment. This has been illustrated in previously reported cases for patients with typical carcinoid tumors arising from the nasal cavity or paranasal sinuses -12. HoweNETs represent a rare entity of head and neck cancer. We report a rare case of non-operable typical carcinoid tumor arising from the sphenoid sinus and achieved local control with IMRT alone. This treatment approach in patients with unresectable typical carcinoid of the head and neck warrants further investigation.


Herlyn–Werner-Wunderlich syndrome (HWWS) is an uncommon congenital anomaly of the female urogenital tract, characterised by uterus didelphys, obstructed hemivagina, and ipsilateral renal agenesis. We reported the difficult pregnancy course complicated by an extremely rare and unique case of this syndrome associated with ectrodactyly, a clinical combination never described in literature.A 28- year-old nulliparous woman previously diagnosed for HWWS associated with ectrodactyly of the right foot and with a history of abdominal left hemi-hysterectomy, ipsilateral salpingectomy, vaginal reconstruction when she was an adolescent. She suffered from threats of abortion in the first trimester, recurrent urinary tract infections during all pregnancy. At 33 weeks + 5 days of gestational age, she was hospitalized for premature rupture of the membranes and uterine contractions and a caesarean section was performed because of breech presentation. Postpartum period was complicated by a pelvic abscess resolved with parental antibiotic therapies.Our literature review shows an unusual aspect in our case: HWWS is not classically associated with skeletal anomalies. Moreover, the most frequent urogenital side affected is the right, not left side as in this woman. Preterm spontaneous rupture of membranes and fetal abnormal presentation represent frequent complications and probably post-caesarean infections are related to pregnancies in the context of this syndrome. Herlyn –Werner-Wunderlich syndrome (HWWS) is a rare congenital anomaly of the female genital tract, characterised by the triad of uterus didelphys, blind hemivagina, and ipsilateral renal agenesis , 2. It i3/ml ; C-reactive protein (CRP) was 19.98 mg/l  and procalcitonin was 0.22 ng/ml . The pelvic ultrasound and the computerized tomography (CT) demonstrated a pelvic abscess neighboring to the lower anterior wall of the uterus with dimensions of 53 x 47 mm , immediately became apparent. The karyotype analysis was normal 46 XX. At age 1, she underwent a surgical correction of this anomaly with consequent partial improvement of a functional deficiency. An upper abdominal ultrasound, performed after a history of recurrent urinary tract infections and pyelonephritis, revealed the absence of the left kidney and the right megaureter. At 12 years, after 2 months from menarche, due to severe acute pelvic pain, a pelvic ultrasound and a magnetic resonance imaging (MRI) were performed. MRI showed a left blind hemivagina with hematocolpos, uterus didelphys with hematometra in the left hemiuterus and ipsilateral hematosalpinx. These imaging findings were later confirmed by the diagnostic laparoscopy which showed normal right uterus, right fallopian tube and both regular ovaries. Consequently, she underwent a surgical reconstruction of the vagina consisting in the drainage of hematocolpos and the removal of the vaginal septum, whereas an abdominal left hemi- hysterectomy and ipsilateral salpingectomy were performed through a Pfannenstiel incision. Her obstetric history was significant for two spontaneous abortion at the age of 26, occurred at 7th and 12th weeks respectively. She had no problem of fertility in anamnesis. The woman came to our observation for the first time at 15 weeks of pregnancy for abortion threats resolved with vaginal progesterone. Singleton fetus was anatomically in the norm. The patient had a moderate protenuria of 1400 mg in 24 hours, so she started a proper diet and a monitor of urine proteins. Close and regular surveillance  was initiated. The obstetric ultrasound controls revealed adequate growth of a fetus without major malformations, and normal Doppler indices of the fetal, feto-maternal and utero-placental vessels. During the three trimesters, frequent urinary infections occurred that were appropriately treated after urine culture and antibiogram test. At 33 weeks + 5 days of gestational age she was admitted to our hospital for premature spontaneous rupture of membranes (pPROM): she reported light amniotic fluid leak one hour before our observation. The admission assessment detected a reduced amniotic fluid index, a regular fetal growth and posterior placenta in the norm; the umbilical artery Doppler values were in the range, the fetal cardiac monitoring was regular and uterine contractions were present. The vaginal examination revealed a soft cervix, 80% effaced, dilated about 2 cm, and the fetus was in breech at station -3. The ultrasound cervical length was 24 mm. A single course of antenatal corticosteroid therapy for fetal lungs maturity induction and tocolytic drugs were administered. On the second day of hospitalization, an emergency caesarean section was performed because the cervix was modified (dilatation about 4 cm), uterine contractility increased while persisting breech presentation (Fig. Müllerian duct anomalies affect 2-3% of women . The comIn our experience, we focused on the course and management of the pregnancy complicated of this disorder. The pregnancy we described was at high-risk and the surgical treatment performed during adolescence was the least recommended by literature, however the obstetric outcome was positive thanks to a straight maternal-fetal follow-up. Our case was characterized by two complications of the pregnancy: preterm labour with pPROM and breech presentation, which are frequent in HWWS in literature. Additionally, pPROM was related to uterine anomaly and probably promoted by the frequent infections of urinary tract  that mayHWWS is an unusual congenital anomaly with clinical significance and different options for surgical management. An early correct diagnosis and treatment are the goal to relieve symptoms and prevent complications to preserve sexual and reproductive abilities. Despite this, the pregnancies of these women are at an increased risk for unfavorable obstetric outcomes and can be characterized more frequently by complications that must be managed promptly by an accurate and regular maternal and fetal follow-up.


Extramedullary involvement of the gastrointestinal tract (GIT) is a rare entity as most patients present with lymphoreticular organ involvement. Its detection and diagnosis can be extremely challenging, as these patients would present with unusual clinical symptoms. We diagnosed and managed a patient with leukemic infiltration of GIT who presented with perianal pain. Prompt use of MRI played an important role in detecting underlying pathology, and effective tissue sampling confirmed the diagnosis. This resulted in overall successful management of the patient. Extramedullary involvement of the gastrointestinal tract (GIT) with leukemia is relatively rare and not easily detectable . HoweverWe present a case of a 70-year-old male, who presented with two-week history of perianal pain accompanied by urgency to defecate and anorexia. He was passing small stools with severe pain, sweating, and burning sensation. There was no history of abdominal pain, constipation, rectal bleeding, fever, and diarrhea or weight loss. Past history included coronary artery bypass graft and hiatus hernia repair. He had worked at a cattle farm and was a nonsmoker.At presentation, he was haemodynamically stable with normal systemic examination. The perineum looked unremarkable with no blood or mucous. Digital examination was aborted due to pain. His hemoglobin was normal with raised inflammatory markers. Liver function tests were mildly elevated. A provisional diagnosis of deep perianal abscess was made.Following this, magnetic resonance imaging (MRI) was done which demonstrated inflammatory changes involving mucosa of the anus, rectum, and internal sphincter with no definite fistulous tract or collection see . His bloFollowing admission, he became febrile; hence, full septic screen was done before starting intravenous antibiotics. An examination under anesthesia in theatre showed a generally inflamed, edematous rectum without contact bleeding. There was no mass, fistula, or abscess identified. A rectal polyp found at 5 o'clock position was excised and sent for histology. Several biopsies of the inflamed mucosa were also sent for histological analysis . HistoloThe hematologist performed a trephine bone marrow biopsy that reported markedly hypercellular bone marrow aspirate showing excess blasts consistent with the diagnosis of acute leukemia, morphologically AMML. Flow cytometry showed an increased population detected with the phenotypes CD13+, CD33+, CD34−, CD65+, HLADr+, and CD117+ and aberrant expression of CD7+. His cytogenetic studies showed no molecular evidence of a translocation involving the KMT2A (MLL) gene at 11q23. No molecular/next generation sequencing was performed because of involvement of high cost of the test. Diagnosis of acute myeloid leukemia was hence made. Histology and bone marrow trephine biopsy results showed French American British (FAB) classification [Extramedullary involvement of GIT with leukemia is rare as most patients are more likely to present with involvement of lymphoreticular organs and sanctuary sites such as the brain, testes, and ovaries. When it does occur, leukemic involvement of GIT has been described in locations from the mouth to the anus, in both solid and hollow organs.Iwamoto et al.  comment The reported autopsy incidence of gastrointestinal involvement by leukemia ranges from 5.7%–13% . The incCornes and colleagues  concludeLeukemic involvement of the GIT must be confirmed histologically. Michiko et al. highlight the importance of tissue sampling in their paper and stress that biopsies should be taken on all abnormal-appearing and sometimes normal-appearing tissues for histologic confirmation of suspected pathology.The challenge with this patient was getting an initial diagnosis, as he had no prior diagnosis of leukemia. The course of disease experienced by this patient suggested that GIT involvement with leukemia might parallel leukemic disease activity, as acceleration of the patient's WBC coincided with a worsening of his symptoms. Appropriate treatment of the underlying leukemia addressed his symptoms.Leukemic infiltration of the colon although rare should be considered whenever there is suspicion of AML on the blood film in a patient presenting with GIT symptoms. Appropriate examination and histological diagnosis by tissue sampling should always be considered in the management of patients presenting with perianal pain where the diagnosis is unclear.