COVID-19 pneumonia represents the most severe pandemic of the twenty-first century and has crucial clinical, social and economical implications. The scientific community has focused attention and resources on clinical and radiological features of COVID-19 pneumonia. Few papers analysing the vast spectrum of differential diagnoses have been published.Complexity of differential diagnosis lays in the evidence of similar radiological findings as ground-glass opacities, crazy paving pattern and consolidations in COVID-19 pneumonia and a multitude of other lung diseases. Differential diagnosis is and will be extremely important during and after the pandemic peak, when there are fewer COVID-19 pneumonia cases. The aim of our pictorial essay is to schematically present COVID-19 pneumonia most frequent differential diagnoses to help the radiologist face the current COVID-19 pneumonia challenge.Clinical data, laboratory tests and imaging are pillars of a trident, which allows to reach a correct diagnosis in order to grant an excellent allocation of human and economical resources. The radiologist has a pivotal role in the early diagnosis of COVID-19 pneumonia because he may raise suspicion of the pathology and help to avoid COVID-19 virus spread. COVID-19 pneumonia generally passes through four stages characterised by different radiological features.Ground-glass opacities, crazy paving, consolidations and fibrotic striae are common to various pathologies.Radiological differential diagnosis roots in the localisation of these radiological features in the lung and in their timing of occurrence.Presence of pulmonary nodules, pleural effusions and adenopathies is suggestive of alternative diagnoses.Since December 2019, when a novel coronavirus has been identified in Wuhan .Since COVID-19 outbreak, the scientific community has focused on clinical and radiological features of COVID-19 pneumonia, while few papers have analysed the vast spectrum of COVID-19 pneumonia differential diagnoses –6.Complexity of differential diagnosis is high, especially in populations not drastically affected by COVID-19, and will be higher after the peak of pandemic, when there are few cases.The crucial and challenging role of the radiologist is and will be to raise suspicion of COVID-19 pneumonia, to guarantee a correct allocation of human and economic resources, and to prevent COVID-19 virus spread.The aim of our pictorial essay is to schematically present COVID-19 pneumonia most frequent differential diagnoses to help the radiologist face the current COVID-19 pneumonia challenge.COVID-19 pneumonia is a virulent pulmonary pathology, with high person-to-person transmission  through Chest radiography has been defined as insensitive in mild or early COVID-19 infection ; therefoCT is more sensitive for early COVID-19 pneumonia detection, disease progression monitoring and alternative diagnosis assessment [Early phase (0–4 days) or stage 1: ground-glass opacities   or stage 2: crazy paving pattern , 13, extPeak phase 9–13 days) or stage 3 : consoli3 days orAbsorption phase or stage 4 ≥ 14 days) : ground-4 days : Further characteristic and mostly constant findings in COVID-19 pneumonia are , 14, 15 subpleural, bilateral or less frequently unilateral, opacities, which are commonly located in the inferior lobes;possible evidence of peripheral pulmonary vessel widening;rarity of pleural effusions, pulmonary nodules and mediastinal lymphadenopathies.Feng Pan identified four stages of lung involvement on chest CT, even if semiological findings may coexist in phase transitions  Table 11:Early pAcute respiratory distress syndrome (ARDS) represents a dire complication of severe cases of COVID-19 pneumonia , 16 and Radiologically ARDS presents an anteroposterior gradient and dependent consolidations coexisting with widespread ground-glass attenuation  Fig. 1gg1g.There is a wide spectrum of possible differential diagnoses for COVID-19 pneumonia, and it is always necessary to consider a triptych of clinical, laboratory and radiological data to reach the correct diagnosis Table .Table 2RRespiratory infections are the most common illnesses occurring in humans , the mosStaphylococcus aureus, Streptococcus pneumoniae, Moraxella catarrhalis, Enterobacteriaceae) [single consolidation with air bronchogram, usually presenting as lobar pneumonia and not exceeding pleural cleavages  , 21, 22 Mycoplasma pneumoniae, Chlamydia pneumoniae) [multifocal pneumonia presents with ground-glass opacities or consolidations and usually coexists with centrilobular nodules and thickening of bronchovascular bundles    [additional findings as centrilobular nodules, cavitations and pneumatoceles (more common in eumonia) ;hilomediastinal lymphadenomegalies , 21, 22;pleural effusions , 21, 22.Radiological features for differential diagnosis:Viral pneumonia represents a various entity, and it is mainly the current epidemic context which suggests COVID-19 origin . Treatmepreferential centro-parenchymal involvement   Fig. 2cc2c;additional findings as centrilobular nodules and bronchial wall thickening  ;coexisting pulmonary oedema (Hantavirus) ;pleural effusions  ;hilomediastinal lymphadenomegalies (Influence type A) .Radiological features for differential diagnosis:It is an opportunistic fungal pneumonia, mostly affecting immunodeficient patients affected by AIDS or undergoing immunosuppressive therapies Fig. d, e.symmetrical, centroparenchymal and perihilar, confluent ground-glass opacities, generally with subpleural sparing  and a prrarity and late onset of crazy paving pattern;additional findings as nodules and pneumatoceles.Anamnesis and laboratory tests are of use, but often not sufficient for a differential diagnosis with COVID-19 pneumonia. Radiological features for differential diagnosis:Angioinvasive aspergillosis is caused by Aspergillus Fumigatus  and geneground-glass opacities and crazy paving pattern are not typical and do not precede consolidations, which frequently present a surrounding ground-glass halo   Fig. 2ff2f;in case of consolidations with no ground-glass halo and absence of other ground-glass opacities, COVID-19 pneumonia is unlikely;presence of air crescent sign , 27, 29 lymphadenomegalies and pleural effusions .Radiological features for differential diagnosis:Two pathophysiologic and radiologic phases are recognised in cardiogenic pulmonary oedema development : interstpossible coexistence of ground-glass opacities, crazy paving pattern and consolidations with different timing of occurrence respect to COVID-19 pneumonia, crazy paving being the first pattern to be appreciated in interstitial oedema;diffuse, bilateral, centro-parenchymal crazy paving and ground-glass opacities with subpleural preservation. Consolidations are late findings and generally coexist with pleural effusions;bilateral pleural effusions, more evident in the alveolar phase of oedema;mediastinal lymphadenomegalies;cardiomegaly.Apart from anamnesis, radiological features for differential diagnosis are , 30:possChen et al.  reportedAPE, which is is commonly secondary to a phlebothrombosis, most frequently of the lower limbs, has an acute clinical onset  and may different phases of infarction maturation, in correlation to the onset time;segmental shape of infarctions are located in the vascular territories of the occluded vessels;presence of vessel embolus, vessel occlusion and residual peripheral clot deposition.It is crucial to collect a proper anamnesis and perform radiological examinations as AngioCT to identify luminal defects of pulmonary vessels Fig. d. RadiolIt has been speculated on extensive pulmonary thromboembolism in severe cases of COVID-19 pneumonia , 32, 33,COVID-19 pneumonia and pulmonary embolism may coexist; in particular, a person might present with the symptoms of acute pulmonary embolism and actually suffer of fulminant pulmonary embolisation but be also infected with COVID-19 being asymptomatic from the infection . GGO witVasculitis is an inflammatory process in which immune effector cells infiltrate blood vessels and surrounding tissues , 36. ANCDAH, presenting as ground-glass opacities or consolidative foci if bleeding is massive, is more prominent in the perihilar region and in the inferior lobes , 37;DAH presents different timing of presentation, crazy paving being the last pattern to be appreciated , 38;symmetric, bilateral ground-glass opacities or consolidations, which tend to be migratory or transient (EGPA: eosinophilic granulomatosis with polyangiitis) , 37;typical vasculitis features, as pulmonary artery aneurysms and excavated nodules may be present and are more common in granulomatosis with polyangiitis, but rarely present in EGPA , 37;coexistence of bronchial and tracheal thickening in granulomatosis with polyangiitis;coexistence of bronchial and bronchiolar thickening or centrilobular nodules in EGPA , 38;pulmonary oedema findings, secondary to cardiac involvement in EGPA , 26;pleural effusions , 37.Anamnesis and laboratory tests may be useful to prove a clinical suspect. Radiological features for differential diagnosis are:It is an interstitial pathology caused by repetitive inhalation of and sensitization to a wide range of inorganic and organic antigens  in relatcentroparenchymal and centrilobular ground-glass opacities ;rarity of crazy paving pattern and consolidations , 42;coexistence of other radiological findings as mosaic oligemia, cysts, centrilobular emphysema and centrilobular micronodules , 43;“headcheese sign,” which represents the coexistence of patchy ground-glass opacities, preserved pulmonary regions, and air trapping on HRCT  Fig. 4bb4b;centrilobular fibrosis, architectural distorsions, traction bronchiectases and honeycombing in the chronic stage , 45;mediastinal lymphadenomegalies.Radiological features for differential diagnosis Fig. a:centropEosinophilic pneumonia represents a various group of pulmonary disorders associated with peripheral or tissue eosinophilia. Laboratory tests and anamnesis are crucial for a correct differential diagnosis –48. HRTCmigrant lesions with absence of crazy paving pattern in SPE  ;pleural effusions, centrilobular nodules and thickening of bronchovascular bundles in AEP (acute eosinophilic pneumonia) , 49, 50;centrilobular consolidations with rarity of ground-glass opacities and crazy paving pattern in CEP (chronic eosinophilic pneumonia) , 51, 52;presence of additional findings in CEP as nodules, atelectasis, band-like opacities and pleural effusions , 51.Radiological features for differential diagnosis are Fig. c, d:migrIt is caused by the aspiration of different substances into the airways and lungs . RadioloTo the purpose of our paper, we will focus on fluid aspiration pneumonia and on lipoid pneumonia.dependent ground-glass opacities;in the late phases, with recurrence of aspiration, fibrotic architectural distortions.Frequently, these patients are dysfagic and their meals are liquid. Radiological features for differential diagnosis are  Fig. 5aa5a:depenIt is an acute or chronic, reactive pneumonia resulting from endogenous lipid accumulation or from exogenous lipid aspiration , 56. ChrAnamnesis of lipid inhalation or fat storage disease is necessary to diagnose a lipoid pneumonia. Comparison with previous chest exams is suggested.ground-glass opacities and consolidations predominantly involve the middle and inferior lobes ;consolidations typically present very low CT attenuation in relation to their fat content  Fig. 5cc5c;fibrosis during chronic stage , 58.Radiological features for differential diagnosis are Fig. b:ground-It is a syndrome caused by progressive accumulation of surfactant in pulmonary alveoli and may be primary in most cases or secondary to toxic inhalation syndromes, haematologic neoplasms and immune deficiency , 60. Anamainly centro-parenchymal and perihilar crazy paving areas ;juxtaposition of severely affected secondary lobules and normal secondary lobules;rarely, consolidations with air bronchogram in severe forms ;fibrotic alterations in advanced stage;pleural effusions, cardiomegaly and lymphadenomegalies, which are features of complicated PAP.Radiological features for differential diagnosis are Fig. d, e:mainAnamnesis of drug taking or recreative drug abuse is crucial for diagnosis and institution of appropriate treatment .Among the most frequent causative drugs and the related most common pulmonary diseases we cite , 63: amiRadiological features for differential diagnosis of cited pathologies have already been analysed in the previous chapters.COVID-19 pneumonia early diagnosis is crucial to avoid the spread of the virus in a disease that may range from asymptomatic to extremely severe and to favour an optimal allocation of human and economic resources. In this context, the role of the radiologist is pivotal and challenging, because he filters patients and identifies possible cases of COVID-19 pneumonia.


Intravenous pyogenic granuloma (IVPG) is a special type of pyogenic granuloma, and its preoperative diagnosis is difficult. We report a rare case of IVPG that develops in the lumen of the internal jugular vein (IJV). Here, we analyze the imaging characteristics of present case and summarize the imaging characteristics of previous reported cases.A 44-year-old man who presented with a growth in the IJV without any symptoms.A diagnosis of IVPG was made, based on the pathological examination after surgery.The patient underwent surgery to excise the vein segment containing the neoplasm.The patient did not present with any complications in the postoperative follow-up period.For clinician, IVPG's preoperative diagnosis is difficult. Although histopathology remains the gold standard for diagnosis, the combination of multiple types of imaging examinations is necessary to rule out the differential diagnoses of IVPG. IVPG arising from the internal jugular vein (IJV) is quite rare. We report a rare case of IVPG arising from the IJV in our center.Lobular capillary hemangioma (LCH) [pyogenic granuloma (PG) or granulation tissue-type hemangiomas] is a benign tumor that develops commonly on the skin or mucous membrane and is red or violaceous in color and shows polypoid growth. It is neither caused by infection nor a real inflammatory granuloma. Intravenous pyogenic granuloma (IVPG) is a special type of PG that usually develops in the veins of the neck and upper extremities, such as the jugular vein,2A 44-year-old man had a nodule on the left IJV without any symptoms based on a routine examination by ultrasonography. There were no abnormalities on medical history and physical examination. In addition, laboratory test results were normal.According to the doppler ultrasound examination, an abnormal echo (mainly with hypoecho) was probed in the middle section of the left IJV, with a clear boundary and slightly irregular shape. Its dimensions were 8.7 × 7.7 × 6.7 mm, connecting to the posterior lateral wall of the IJV, and swinging with the pulse of the vessel Fig. A, B. ColCT demonstrated that there was an isodense nodule in the proximal lumen of the left IJV, the size of which was approximately 8.5 × 5.9 mm, with a CT value of 40 HU. Thin-layer CT showed increased tortuosity and small vessels shadowing within the nodule during arterial phase enhancement Fig. B with a On MRI, a pedunculated nodule was observed protruding into the lumen at the initial segment of the IJV, with clear boundary and smooth edge without obvious invasion to the adjacent venous wall. Subsequently, it was significantly enhanced, and its dimension was approximately 8.2 × 5.9 mm. The nodule was isointense on T1-weighted imaging but hyperintense on T2-weighted imaging Fig. A--C.Therefore, the possibility of intracavitary thrombosis could be ruled out, and we suggested that the neoformation was a primitive endoluminal neoplasia. Subsequently, the patient underwent surgery with general anesthesia to excise the neoformation and explore the area around the left IJV. We found a pedunculated red neoformation with soft texture, which measured approximately 5 × 3 mm within the left IJV after cutting open the left IJV Fig. A. A reddHistological examination results showed a large number of proliferating capillaries, presenting with lobular distribution. Endothelial cells of the capillaries were flat, surrounded by fibrous tissue and myxoid stroma. Moreover, there was a small amount of chronic inflammatory cell infiltration. Immunohistochemical staining results showed CD34 (+), CD31 (+), ERG (+), VIM (+), KI67 5% (+), and SMA (−)  in CT. Tortuous small vascular shadows in the nodule were observed during the arterial phase of CT in the present case, which also suggests that the tumor is mainly composed of neonatal blood vessels. On MRI, the nodule was isointense on T1-weighted imaging but hyperintense on T2-weighted imaging. The nodule tends to be benign on a combination of imaging findings, and differential diagnoses included venous thrombosis, intravascular papillary endothelial hyperplasia (IPEH), or any other vascular tumor. IPEH is mainly characterized by sheet-like hyperintensity on T1-weighted imaging and polycystic nodule-like hypointensity on T2-weighted imaging, In the present case, we reconstructed the IJV after excising the neoplasm and the connected vein wall due to small neoplasm and localized coloboma of the IJV. Nevertheless, whether to reconstruct the vein mainly depends on the site and degree of neoplasm involvement. In some veins, such as the vena cava, venous reconstruction may be required to restore venous continuity.Surgical excision is the main treatment for IVPG arising from the IJV, which involves complete excision of the vein segment containing the neoplasm.Primary IVPG arising from the IJV is a rare subtype of vascular tumor without characteristic epidemiological and endocrinological features. Although histopathology remains the gold standard for diagnosis, preoperative imaging examination is necessary, and the combination of multiple types of imaging examinations is conducive to the differential diagnosis of IVPG. In the future, the detection rate of IVPG may increase with the improvement of imaging technique and people's health awareness. Therefore, it is indispensable to summarize the imaging characteristics of IVPG developing in various parts of the body.Data curation: Binjie Fu, Zhui Li, Yangyang Feng, Yu Zhao, Hong Liu.Investigation: Yuheng Yang, Hong Liu.Methodology: Yuheng Yang, Xiaoping Ye, Hong Liu.Project administration: Yuheng Yang.Resources: Binjie Fu, Zhui Li, Yangyang Feng, Yu Zhao, Hong Liu.Software: Xiaoping Ye.Supervision: Hong Liu.Validation: Xiaoping Ye.Visualization: Xiaoping Ye.Writing – original draft: Yuheng Yang.Writing – review & editing: Yuheng Yang, Xiaoping Ye.


Gonadoblastoma is a rare ovarian neoplasm which belongs to “germ cell-sex cord-stromal tumor” category. This tumor is frequently associated with invasive germ cell malignancy. It commonly arises in dysgenetic gonads of young individuals who are phenotypically females but possess 46XY karyotype. It has been rarely reported in females with normal phenotype and genotype.We herein describe a case of 10-year-old female who presented with abdominal pain, abdominal distention and fever. CT scan of the abdomen and pelvis revealed bilateral ovarian masses, ascites and pelvic and para-aortic lymphadenopathy. Serum lactate dehydrogenase levels were also elevated. She underwent left salpingo-oophorectomy, right ovarian biopsy, omentectomy and para-aortic lymphadenopathy.Microscopically, tumor showed in situ and invasive components. In situ component was arranged in nests and lobules formed by immature sertoli cells forming acini and encircling large polygonal primitive germ cells. Immature sertoli cells were positive for immunohitochemical (IHC) stains cytokeratin AE1/AE3, inhibin and calretinin, while germ cells were positive for SALL4, Oct 3/4, placental alkaline phosphatase (PLAP) and CD117. Invasive component was arranged in sheets of large-sized, polygonal-shaped primitive germ cells which were also positive for SALL4, Oct 3/4, PLAP and CD117 IHC stains. Hence, the diagnosis of “gonadoblastoma with dysgerminoma” was made. The tumor was limited to both ovaries. Cytogenetic analysis of peripheral blood revealed normal female 46XX karyotype. The patient received two cycles of adjuvant chemotherapy and was then lost to follow-up.We conclude that gonadoblastoma, although rare, should be considered as a differential diagnosis in ovarian tumors of young females. Invasive germ cell component should always be carefully searched for as it guides about treatment and predicts prognosis. Gonadoblastoma is a rare ovarian neoplasm that comprises a mixture of primitive germ cells and immature sex cord cells ,2. It isMost of the patients are phenotypic females with gonadal dysgenesis and 46XY karyotype. However, abnormal gonads may not the rule and rare cases of phenotypically and genotypically normal females, i.e. with 46XX karyotype and normal gonads, have also been reported ,6.Management of gonadoblastoma essentially consists of surgery that may be followed by chemotherapy in cases with malignant germ cell component. Prognosis of gonadoblastoma depends on the characteristics of malignant germ cell component. However, cases with dysgerminoma have also reported excellent outcome ,8.We herein report clinicopathological features of an extremely rare case of bilateral gonadoblastoma with dysgerminoma in a young female with phenotypically normal female genital tract and normal 46XX karyotype.A 10-year-old female presented to the gynecology clinic with complaints of abdominal pain, abdominal distention and fever for 15 days. On physical examination, generalized abdominal tenderness, distention and ascites were noted. Rest of the physical examination was unremarkable, and normal female external genitalia were noted. CT scan of the abdomen and pelvis revealed a large solid left adnexal mass with specks of calcifications measuring 16 x 10 x 9 cm. Right ovary was also mildly enlarged with calcifications and measured 3 x 1 x 0.5 cm. Both fallopian tubes, uterus and vagina were well formed and appeared unremarkable except for hydrometrocolpos. Ascites and multiple enlarged para-aortic and retroperitoneal lymph nodes were also noted.Rest of abdominal and pelvic viscera were unremarkable. No omental or peritoneal deposits were identified. Evaluation of serum tumor markers revealed raised levels of LDH and normal levels of AFP and β-HCG. Based on these findings, exploratory laparotomy, left salpingo-oophorectomy, right ovarian biopsy, para-aortic lymph node sampling and partial omentectomy were performed.Macroscopically, cut surface of the left ovarian mass was lobulated, firm, pale white, solid with few spongy and myxoid areas. Focal calcification was seen. Necrosis was absent. Cut surface of the smaller ovary was firm, pale white, solid and gritty. Microscopic examination of both ovarian masses revealed a neoplastic lesion comprising of germ cell and sex cord-stromal components arranged in sheets along with nests and lobules separated by fibrous septae Figures , 1B. CalThe sex cord component demonstrated positive expression for cytokeratin AE1/AE3, inhibin and calretinin immunohistochemical (IHC) stains Figure .SALL4, Oct 3/4, placental alkaline phosphatase (PLAP) and CD117 IHC stains were positive in germ cell component , and then unfortunately she was lost to follow-up.Gonadoblastoma commonly occurs in the second and third decades of life with a mean age of 18 years . BilaterMajority of the gonadoblastoma arise in patients genotypically having Y chromosome and phenotypically having dysgenetic gonads . The genCytogenetic analysis/karyotyping of peripheral blood determines the germline status of Y chromosome. In cases of mosaicism, tumor tissue shows the presence of Y chromosome while Y chromosome is absent on karyotyping of peripheral blood. McCuaig et al. performed cytogenetic analysis on peripheral blood, tumor tissue and adjacent ovarian tissue. This suggested that the Y chromosome status should be assessed in tumor tissue and peripheral blood using different techniques such as fluorescence in situ hybridization and quantitative fluorescence-polymerase chain reaction . One-third of these cases were bilateral ,6,9,10. Pure gonadoblastomas behave in a benign fashion. Cases with dysgerminoma have excellent prognosis. However, prognosis is unfavorable for other tumor types such as yolk sac tumor ,8.Surgery is the mainstay of treatment, and most of the patients undergo oophorectomy which can be accompanied by salpingectomy, hysterectomy, omentectomy and lymph node dissection ,6,10. GeThe lack of follow-up information about our patient is the main limitation of this case report. However, the clinical and histopathological features of our patient are quite characteristic and further endorse the features reported in the literature.Gonadoblastoma can occur in young females who are phenotypically and genotypically normal and therefore should be considered as a differential diagnosis in these patients. It is frequently associated with invasive malignancy which is dysgerminoma in most cases. The prognosis is generally excellent. However, careful search for invasive germ cell tumor is necessary as it determines the prognosis and subsequent treatment plan.


Adult hypertrophic pyloric stenosis (AHPS) is a rare disease and presents as pyloric obstruction. Double pylorus is also a rare condition due to a gastroduodenal fistula connecting from the gastric antrum to the duodenum. A 42‐year‐old woman without a history of vomiting in infancy presented with postprandial abdominal distension and repeated vomiting. Abdominal computed tomography showed gastric dilatation and wall thickening of the distal stomach. Endoscopy and contrast gastrography revealed gastric outlet obstruction due to stenosis and an ulcer in the antral and pyloric region. Endoscopic ultrasonography revealed circumferential thickening of the muscularis propria layer of the pylorus. Her symptoms improved with treatment consisting of drainage, fasting, and a proton pump inhibitor. Two weeks after onset, follow‐up endoscopy revealed a healing ulcer and double channel pylorus. Based on her clinical course and findings of clinical images, she was diagnosed with gastric outlet obstruction due to AHPS that was improved by double channel pylorus formation. In conclusion, AHPS that was improved by double channel pylorus formation is an extremely rare condition, and we should be aware of this disease entity. This article presents an adult‐onset case of hypertrophic pyloric stenosis (AHPS). In this case, long term (over six months) persisting thickening of the muscularis propria in the pyloric region proved the precise diagnosis of AHPS. Furthermore, it is very interesting that double pylorus due to possible penetrating gastric ulcer led to the improvement of her symptom. Adult hypertrophic pyloric stenosis (AHPS) is a rare disease and presents in adult life as pyloric obstruction. It is a benign disease resulting from hypertrophy of the circular fibers of the pyloric canal. The most common type of AHPS is due to peptic ulcer disease, malignancy, and certain inflammatory diseases.A 42‐year‐old woman without a history of vomiting in infancy presented to the emergency department with postprandial abdominal distension and repeated vomiting but no abdominal pain. Laboratory examination showed normal results, including a normal serum Helicobacter pylori antibody level, except for mild anemia. Abdominal computed tomography (CT) showed gastric dilatation and wall thickening of the distal stomach Fig. , and a dOur patient's clinical and imaging findings suggested that the double pylorus formed in our patient in the following manner. When we retrospectively reviewed the endoscopic images from the EGD examination that the patient had undergone 1 year prior to the current admission, antral narrowing was observed. She may have had asymptomatic AHPS at that time. When the patient came to the emergency room, endoscopy and contrast gastrography revealed gastric outlet obstruction due to stenosis and a deep ulcer in the stenotic antrum. During the hospital admission, the deep ulcer made a fistula with the duodenum that would eventually become a double pylorus. After double pylorus formation, the patient no longer had symptoms of AHPS and she was discharged from the hospital.AHPS can be classified into the following three types. The first type is the late stage of infantile hypertrophic pyloric stenosis (HPS). The second type is HPS commencing in adult life but secondary to other diseases such as hiatal hernia, duodenal ulcer, gastric ulcer, tumors, or inflammatory diseases. The third type is primary idiopathic HPS (IHPS) presenting in adult life without any apparent cause.Patients with AHPS who have persistent symptoms usually undergo surgical treatment such as partial gastrectomy, gastroenterostomy, pyloromyotomy, or pyloroplasty. Recently, endoscopic treatment methods including pyloroplastyOn another note, double pylorus due to a gastroduodenal fistula connecting from the gastric antrum to the duodenum is a rare condition.In conclusion, we presented an extremely rare patient with gastric outlet obstruction due to AHPS whose symptoms improved by double channel pylorus formation. We should be aware of this disease entity and this disease entity should be included in the differential diagnoses of spontaneous improvement of gastric outlet obstruction.


The diagnosis of hydatid cyst should be considered in children with seizure in endemic regions. The diagnosis of hydatid cyst should be considered in children with seizure in endemic regions. He had a history of 2 years headache and seizure. In MRI and computed tomography (CT) scan, a primary cerebral hydatid cyst was evident. The diagnosis of hydatid cyst should be considered in children with mentioned characters in endemic regions.Echinococcus granulosus.Hydatid disease, an important zoonotic disease, is caused by infestation of the larva of dog tapeworm Dogs are definite hosts for the parasite and pass eggs with their faces. Humans are accidentally infected through ingestion of food or water contaminated with dog faces or by direct contact with dogs.2A 13‐year‐old boy presented to our hospital with abdominal pain and fever. The pain was constant, nonradiation, worsened with light activity. He has any other gastrointestinal symptoms. The patient had no history of previous hospitalization and did not take any medication. The patient lived in a rural zone. In admitting time, the patient was febrile (body temperature 40°C), blood pressure 110/75 mm Hg, heart rate 92 beats/min, and breathing rate 18 bpm. A general physical examination revealed no abnormality.33; hemoglobin 12.4 g/dL; and platelets 275 000 cells/mm3. Laboratory examinations revealed alkaline phosphatase levels (548 IU/L) which was high. Also, blood and urine culture, serological tests, and C‐reactive protein (CRP) tests were performed. The results of all tests were negative. Urinalysis and other laboratory work‐ups were normal. Erythrocyte sedimentation rate (ESR) was 16 mm/h. Chest radiography, electrocardiogram, and ultrasonography (US) of the abdomen and pelvic were normal.The complete blood investigation performed with the following results: white blood cell count 7400 cell/mmCranial CT revealed a left parieto‐occipital hypodense, cystic, well‐ demarcated Figure . MagnetiBased on the cranial imaging findings, differential diagnosis of other cystic lesions such as abscesses, large granulomas, cystic gliomas, epidermal cysts, and arachnoid cysts, arising from the brain were considered. However, imaging and serological findings confirmed the diagnosis of hydatid cyst. A wide parieto‐occipital craniotomy was performed. The dura was dissected Figure  and the Treatment with albendazole was started when the patient was discharged from the hospital for prevention of further echinococcosis, and also, he received Dilantin (phenytoin sodium) (5 mg/kg/d in divided doses) for prevention of seizure. Postoperation MRI and CT scan images revealed no sign of mass in the operated area , Echinococcus multilocularis , Echinococcus oligarthus, and Echinococcus vogeli (which cause polycystic hydatid disease). After ingestion of eggs, they hatch in the small intestine submucosa and enter veins or lymphatic vesselsEchinococcosis is a parasitic disease caused by the larval stage of a tapeworm Central nervous system hydatidosis is rare and is usually diagnosed during childhood.Brain hydatid cyst also is classified as primary (single) or secondary (multiple). The primary cysts are formed as a result of direct infestation of the brain without involvement of other organs and the secondary multiple cysts result from spontaneous, traumatic, or surgical rupture of a solitary cranial cyst.Hydatid cyst see in anywhere of the brain and most commonly located supratentorially, in the middle cerebral artery territory.E granulosus are used for verifying the imaging results.Intracranial hydatid cysts are usually present with headache, vomiting, and seizure due to raised intracranial pressure and brain compression. In physical examination, papilledema and neurological deficit may be presented.Computed tomography scan of cranial hydatid cysts showed an intraparenchymal spherical cystic lesion with distinct borders. Also, the cyst fluid was isodense with CSF. MRI imaging showed a low signal intensity rim of the cyst wall, while the cyst content had signal intensities similar to CSF.In our case, CT scan revealed a left parieto‐occipital hypodense, cystic, well‐demarcated, round lesion causing shift to the right. MRI imaging showed a mass consists of membranous structures with CSF intensity in the paraventricular area of left hemisphere.Although in small or inoperable brain cysts, medical therapy has shown promising effects but surgery remains the golden treatment by which cysts can be removed without rupture and results in a complete cure. Chemotherapy with two benzimidazoles (ABZ or MBZ) is indicated for inoperable patients with primary liver/lung echinococcosis and for patients with multiple cysts in two or more organs.Dowling technique is the most effective surgical method for removing cerebral hydatid cysts without causing rupture.Albendazole has been used successfully for treatment of hydatid cyst in brain,In summary, intracranial hydatidosis is rare and more affects pediatric age group. It may misdiagnose as intracranial cyst, so in differential diagnosis of intracranial cyst especially in endemic areas, age of the patients will be helpful. Total surgical remove of the cysts without rupture is still the treatment of choice in cerebral hydatidosis.None declared.All authors, MGh, ASh, FRY, and SAH, are equally contributed to the design, analysis, and presentation and critically revise of this study. BG: is surgeon of patient. MGh: is specialist in infectious disease and involved in study design. ASh: involved in study design, writing, submission, and revision.Applicable.Written informed consent was obtained from the parents of patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor of this journal.


Low-grade fibromyxoid sarcoma (LGFMS) is a rare tumor characterized by bland histological features and aggressive clinical course. The most common anatomic locations of occurrence are the lower extremities, thorax, inguinal area, and upper limbs. Primary mediastinal sarcomas are even rarer. To the best of our knowledge, only seven cases of primary mediastinal LGFMS have been reported in the literature. Here, we report a case of primary mediastinal LGFMS.A 26-year-old Pakistani man presented with fever and vomiting for the past 2 months. On a routine chest x-ray, a mediastinal mass was incidentally found. Computed tomography (CT) scan showed a large circumscribed lobulated soft tissue density mass lesion in an anterior mediastinum. Grossly, the resected mass measured 17.0 × 12.0 × 11.0 cm. The cut surface was gray white with a whorled-like appearance and foci of calcification and cystic changes. Histologically, a spindle cell lesion was seen with alternating myxoid and hyalinized areas. The shaped cells were arranged in bundles. Immunohistochemical staining showed positive reactivity patterns with MUC4 and focally for epithelial membrane antigen (EMA). The diagnosis was confirmed as LGFMS. The patient is free of symptoms and recurrence 22 months after the surgery.In conclusion, we report a rare case of primary mediastinal LGFMS in a young male patient that was discovered incidentally. Our patient is on regular follow-up to look for evidence of recurrence as these tumors are prone to recurrences. Low-grade fibromyxoid sarcoma (LGFMS) is a rare tumor with generic histologic characteristics and intense clinical progression. This tumor is found predominately in the extremities, both upper and lower, inguinal region, and thorax . On histSince this was a retrospective observational study and did not involve actual patients or patient’s images, ethical approval was not sought for this study.A 26-year-old Pakistani man presented with fever and vomiting (non-bilious and non-bloody). He had been under treatment in another hospital for the past 2 months. Due to progressive worsening of symptoms, he was kept as an inpatient with a working diagnosis of acute hepatitis A and jaundice and was managed accordingly. A mediastinal mass was incidentally found during a routine chest X-ray Fig. a, b. A cGrossly, the resected mass was encapsulated and measured 17.0 × 12.0 × 11.0 cm. The cut surface was gray white with a whorled-like appearance and foci of calcification and cystic areas Fig. . HistoloPostoperatively, the patient was kept nil per oral for 6 hours and started on intravenous analgesics and antibiotics. His chest x-ray showed no residual mass, consolidation, pneumothorax, or pleural effusion. He was sent home in a stable condition on the 4th day of surgery and followed in the clinic for 2 months. The patient is free of symptoms and recurrence 22 months after surgery.In 1987, Evans first recognized LGFMS as a unique entity seen in young and middle-aged adults, which was initially identified as a slow-growing, asymptomatic tumor of the soft tissue with a deceivingly mild histology but with a higher risk of recurrence and metastasis . Translocation of ‘FUS-CREB3L1’ fusion gene is reported in about 5% of the LGFMS cases. The genes CREB3L2 and CREB3L1 play a critical role in codifying transcription factors and contributing to the pathogenesis of the disease. In a study of 14 patients diagnosed with LGFMS, Cesne he tumor .It is possible to use combined radiotherapy and chemotherapy for locally advanced unrespectable tumors. As with other soft tissue sarcomas, pre- or postoperative radiotherapy with often infiltrative growth patterns can be considered to improve local cancer spread, particularly in the extremities. Some centers use ‘isolated limb perfusion’ (ILP) to salvage limbs during surgery and avoid radiotherapy in a select few patients. Among other localized treatments are cryoablation and radiofrequency ablation (RFA) and cryoablation to treat cancers with more than metastatic lesions , 15.In conclusion, we report a rare case of primary mediastinal LGFMS that was incidentally discovered in a young male patient. Our patient is regularly looking for evidence of recurrence as these tumors are prone to recurrence. This aggressive sarcoma with a benign appearance should always be present in the differential diagnosis of all benign-looking neoplasms of the spindle cells.


A 46-year-old male first visited the dermatology unit complaining of a fast-growing lesion on the ankle . DermoscMyopericytoma is a rare, benign, slow-growing soft-tissue tumor of perivascular cells. The most common location is on the distal extremities, and though the etiology is unknown, it has been associated with local trauma. Histologically, it is characterized by a well-circumscribed, non-encapsulated proliferation of spindle-shaped cells arranged in perivascular concentric rings. Therefore, it can be a differential diagnosis for hemangiopericytomas, myofibromas, and glomus tumors. Dermoscopy has been previously disclosed unfocused arborizing vessels, structureless light brown areas, and shiny white streaks ,2. In ou


Primary gastrointestinal lymphoma is very rare compared to gastrointestinal tract lymphoma arising from secondary to primary nodal disease. Extra nodal lymphoma can involve any part of the gastrointestinal tract, most commonly being the stomach followed by small intestine and ileocecal region. They are indistinguishable from other benign and malignant conditions and are clinically non-specific. While perforation is common among patients undergoing lymphoma treatment, presentation of primary gastrointestinal lymphoma as perforation is rare and needs proper evaluation and management. Here, we describe an interesting case in which a patient presenting with peritonitis was found to have perforation and mass of the jejunum. Resection and anastomosis were performed as intervention, with subsequent histopathological examination showing Malignant Non-Hodgkin lymphoma (NHL). Postoperative follow up was provided along with the appropriate chemotherapy regimen. •Perforated hollow viscus is the perforation of any hollow viscera resulting from inflammatory, infectious, traumatic causes, and neoplasms.•Lymphoma may cause peritonitis due to hollow viscus perforation.•Clinicians should be aware of peritonitis due to Non-Hodgkin Lymphoma because the clinical presentation of small intestinal lymphoma is non-specific. The cauLymphoma is a possible but uncommon cause of acute abdomen. The presentation of intestinal lymphoma with perforation occurs at reported frequencies of 1–25%, This perforation can be caused due to complications of chemotherapy .Here, we report the uncommon case of a male suffering peritonitis with Non-Hodgkin Lymphoma located in small intestine and reported the case in accordance to the SCARE 2018 guidelines .1.1A 36-year-old male patient presented to the hospital with a 1-day history of abdominal pain on the whole abdomen. Pain was initially located at the umbilical area, but then generalized and became severe every time, and was accompanied by nausea, vomiting and fever. The patient had no history of peptic disease or trauma, but he had of significant weight loss (9 Kg over 3-months) and loos of appetite. Clinical examination revealed a heart rate of 110, respiratory rate 24 times/minute, blood pressure 140/70 mmHg, temperature 38 °C. Upon abdominal exam, we found distention of the patient's abdomen, bowel sounds were reduced on auscultation, and a tender generalized pain of the abdomen accompanied with tympanic sounds on percussion. Rectal examination was normal. Other system were normal. Complete blood count with leukocytes 14.000 cell/ml with a 75,6% neutrophil composition. Patient was negative for hepatitis B and human immunodeficiency virus (HIV). An abdominal X-ray within normal limits. We diagnosed the patient with peritonitis suspected due to perforation of the hollow viscus.An exploratory laparotomy was then performed. Immediately after the midline incision and opening of the abdomen in layers, approximately 100 ml of bowel content was revealed in the cavity. Perforation was observed on two sites within the jejunum, approximately 30 cm and 40 cm from the Treitz ligament with a size of 0,5 mm × 1 mm and 0,6 mm × 2 mm . No abnoWe performed resection of the jejunum segment containing the mass and the mesentery, followed by end to end anastomosis. The resected jejunum specimen was sent to the anatomical pathology laboratory. Seven days after the operation, the histopathological examination of the specimens revealed features of Malignant Non-Hodgkin Lymphoma of the diffuse large B-cell type  and ImmuThe patient was discharged 8 days postoperative with a good condition, and was chemotherapy by oncology division with Cyclophosphamide-Hydroxyldaunorubicin (Doxorubicin)-VinCRIStine-Prednisone (CHOP) regimen for further management. This patients received a total of six cycles of CHOP regimen. At follow-up up to 6 months, there were no signs of recurrence on physical examination and CT scan with contrast of the chest, abdomen, and pelvis (C/A/P CT Scan) within normal limit. We doesn't performed Positron emission tomography (PET) because our institution doesn't have PET scan.2We report the case of a 36-year-old man with primary jejunal Non-Hodgkin lymphoma (NHL), whose first clinical symptomatology were signs of peritonitis. Laparotomy performed in emergency identified a perforation in the jejunum, which required segmental resection with anastomosis.Lymphomas of the digestive tract are uncommon, even though incidence has risen slightly in the last few decades, possibly due to improvements in immunohistochemistry which facilitates the diagnosis ,6. LymphThe incidence, distibution of NHL subtypes, and age of presentation across geographic regions varies, it seem to be related to environmental, racial, and host . Risk faThe clinical presentation of small intestinal lymphoma is non-specific and the patients have symptoms, such as non-specific abdominal pain (70–80%), weight loss (30%) , nausea,•Absence of palpable adenopathy in clinical examination•Absence of mediastinal lymphadenopathy in a chest x-ray•Normal range of total and differential white cell count;•Involvement of only regional lymph nodes, discovered on surgery;•Liver and spleen remain without disease.The definition of a primary gastrointestinal lymphoma was described by Dawson et al. (1961): ,12.•AbseHelicobacter pylori eradication therapy is the first-line treatment of gastric mucosa-associated lymphoid tissue (MALT) lymphoma; involved-field radiotherapy or surgery is recommended for patients of non-gastric MALT with an early stage (I/II E); and R–CHOP regimen  is recommended for gastric diffuse large B-cell lymphoma (DLBCL) [Staging of gastrointestinal lymphoma was performed according to modification the Ann Arbor staging System proposed by Musshoff ,12 was c (DLBCL) ,17,18.TaThe surveillance for NHL according NCCN is physical exam , and whole body PET/CT scans with contrast of the chest, abdomen, and pelvis (C/A/P) every 3–6 months for the first 5 years, then every 12 months for every subsequent year . For our3Morbidity and mortality increase significantly in primary gastrointestinal lymphomas presenting as perforation. However, as seen in our patient, proper understanding of the disease, timely intervention, and good clinical management will yield favourable results.Not commissioned, externally peer reviewed.Written informed consent was obtained from the patient for publication of this case report and accompanying images.WS, SD, PRI and MF researched the literature and wrote the manuscript. WS, SD, and MF operated on the patient and had the idea for this case report. WS, and PRI checked the manuscript and made corrections. PRI and MF provided the overall guidance and support. All authors read and approved the final manuscript.None.Warsinggih.No funding or sponsorship.The study is exempt from ethical approval in our institution.The authors declare that they have no conflict of interests.


The peripheral ameloblastoma (PA), also known as extraosseous ameloblastoma, is a rare soft tissue tumor of odontogenic origin, accounting for 1–5% of all ameloblastoma. In some cases, saucerization of underlying bone is the only radiological evidence of this lesion, and PA has identical histological characteristics of intraosseous ameloblastoma. However, it is slow growing, less aggressive, and less invasive in nature. The present report describes a rare case of PA in the maxillary labial gingiva of a 37-year-old man with port-wine vascular malformation. PA was clinically diagnosed as a pyogenic granuloma, and following the surgical treatment of the lesion, its histological features were of ameloblastoma. This case illustrates the importance of including peripheral ameloblastoma in the differential diagnosis of painless exophytic gingival swelling. Ameloblastoma is by far the most common benign epithelial odontogenic tumor, occurring mainly in the jaw. According to the WHO, ameloblastoma is generally classified into four variants: solid/multicystic, desmoplastic, unicystic, and peripheral. The first three variants are of intraosseous type, and the fourth is extraosseous. The peripheral ameloblastoma (PA) is an uncommon extraosseous variant of ameloblastoma, and only less than 200 cases of PA are having proper recognition in the literature . PA is cPeripheral ameloblastoma is more commonly located in the mandible, especially the lingual gingiva in the premolar region, followed by the anterior region . In the A 37-year-old healthy man was referred to the University Dental Hospital of Sharjah for the management of a soft tissue mass on the left anterior part of maxillary gingiva, being there for a duration of six months. According to the patient, the swelling enlarged gradually during that period, with frequent episodes of recurrent bleeding without any history of trauma. The patient also presented with a congenital port-wine stain on the left facial skin along the distribution of the maxillary division of the trigeminal nerve .Intraoral clinical examination revealed a labial painless bluish red mass, about 3 × 4 cm in diameter, which was a firm, pedunculated, nontender, and nonpulsatile lesion, extending from the upper right central incisor to the upper left canine .The oral mucosa overlying the lesion showed some degree of keratinization with multiple superficial ulcerations that bleed easily on a slight touch. The neighboring upper left lateral incisor was nonvital, mobile, and crowned, whereas the upper left canine responded to the vitality test. Finally, the upper right central incisor was firm. The patient had poor oral hygiene and suffered from generalized chronic periodontitis. No regional lymphadenopathy was present, and the facial port-wine stain extended intraorally to involve the upper left labial mucosa, the buccal gingiva, and the whole left side of the hard and soft palates .A panoramic radiograph showed a well-demarcated periapical radiolucency in the alveolar region extending from the root of tooth number 11 till tooth number 23, with nonvital tooth number 22. A supernumerary tooth was detected distal to the root of tooth number 21 within the radiolucent lesion, and an impacted right maxillary canine was present, yet it was irrelevant to the lesion. The nasal cavity and maxillary sinus cavity appeared normal and free from the lesion .The clinical differential diagnosis included hemangioma, pyogenic granuloma, peripheral giant cell granuloma, and peripheral ossifying fibroma.A soft tissue incisional biopsy of the lesion was taken under local anesthesia, and then the specimen was sent for histopathological examination. A brisk bleeding episode was provoked, yet it was well controlled with gauze and finger pressure. H&E stained sections of the soft tissue component revealed islands of odontogenic epithelium, arranged in plexiform, as well as in the follicular pattern. The proliferating basaloid cells were organized in nests or islands, with several microcysts in the center. The nuclei of peripheral cells were located at the opposite pole of the basement membrane. These islands of the odontogenic epithelium were seen immediately underneath the epithelial layer in the lamina propria, and the histologic findings were consistent with features of peripheral ameloblastoma. The maxillofacial surgeon explained the treatment plan to the patient and addressed the need for a complete removal of the soft tissue lesion alongside the bone lesion, due to the high possibility of lesion extension to the radiolucent area that was previously detected on orthopantomogram (OPG). The patient refused excision under general anesthesia (GA) and preferred the removal of the soft tissue mass only. Then, based on clinical, radiological, and histological findings and the patient's preference, a decision was made to excise the lesion under local anesthesia. Wide excision of the tumor was done to achieve complete disease-free margins, and the underlying saucerized bone was curated, leaving a raw bleeding area. Hemostasis was achieved using bone wax, and wound closure was done using labial mucosa advancement flap. Sutures were removed on the tenth postoperative day, and a temporary denture was placed. The patient was on frequent review up to three years with no evidence of recurrence Figures .The whole mass was transferred to the histopathology lab for further evaluation. The superficial part of the lesion showed typical odontogenic epithelium with follicular and some plexiform patterns; however, the deepest portion of the lesion showed aggressive behavior of the tumor with marked hyper cellularity with basaloid components, resembling basal cell carcinoma . ImmunohThe first well-documented case of peripheral ameloblastoma is reported by Stanley and Krogh in 1959 . Since tThe potential source of PA includes the extraosseous remnant of dental lamina or basal cell layer of the surface epithelium. PA is typically present as a slow, painless, exophytic growth of gingiva or oral mucosa .Generally, the color of peripheral ameloblastoma varies between pink and red or dark red and is usually present as a painless, sessile mass, which is usually a firm growth with a smooth surface, occasionally exhibiting a papillary or warty appearance . BleedinThe PWS is a congenital facial capillary malformation that occurs in 3 per 1000 live births. Above 40% of all the cases of port-wine stain are limited to the cutaneous distribution of the trigeminal nerve. The pathogenesis of PWS remains controversial , and theDental examination revealed a missing upper left central incisor, and the presence of an unsatisfactory cantilever bridge, which harbors dental plaque leading to poor oral hygiene in this area. This phenomenon raises the suspicion of a large pyogenic granuloma resulting from plaque accumulation and continuous irritation from the mobile bridge. Incidentally, the panoramic view showed a supernumerary microdont lateral to the midline in the radiolucent area, which then suggests the possibility of presence of an odontogenic cyst like dentigerous cyst.The differential diagnosis for such presentation includes reactive lesions, such as pyogenic granuloma, peripheral giant cell granuloma, and neoplastic lesions like peripheral ameloblastoma and basal cell carcinoma. Immunohistochemical staining of tissue against Ki67, CD10, and BER-Ep-4 is done to confirm the disease entity and to study the behavior of the lesion.The histopathological findings demonstrate proliferation strands of the odontogenic epithelium, arranged in many patterns to resemble that seen in intraosseous ameloblastoma that is follicular, plexiform, basal cell, and acanthomatous. The lesion in this case depicted a follicular pattern with acanthomatous changes, while the islands usually exhibit palisading of columnar basal cells and a stellate reticulum is seldom noticeable. Literature reveals documented cases with clear cell differentiation and ghost cells as variants . The hisKi67 and CD10 are markers for aggressive behavior with high tendency to local recurrence after resection. CD10 is primarily employed in invasion of the extracellular matrix, and positive stromal cells may signify the aggressiveness of the tumor . The strThe recommended treatment of choice is conservative supraperiosteal surgical excision  togetherIn the majority of cases of peripheral ameloblastoma, the lesion is unlikely to present with bone involvement, and this may be due to a barrier created by fibrous tissue of the gingiva and the periosteum. Occasionally, a superficial cupping or saucerization of the cortical plate is seen radiographically or at surgery, and occurrence of cupping or saucerization was thought to be due to tumor pressure rather than direct invasion. In the present case, the tumor was abutting to oral mucosa indicating that the tumor originated from the basal layer of the gingival epithelia. However, the intraoperative findings surprisingly showed multiple scalloping indentations of the underlying alveolar bone with rugged borders, which created some concerns to the nature of the lesion. The surgeon then decided to perform some amount of bone curettage in the region of the bony defect, to ensure complete removal of the tumor.Although peripheral ameloblastoma is a benign neoplasm with no aggressive behavior or invasive potential, which is different from intraosseous ameloblastoma, some recurrent lesions have been reported with a rate of 19% and one case of metastasis has also been documented . AlthougCoexistence of port-wine stain with gingival ameloblastoma on the same side has not been reported in the literature. As per our best knowledge, this is the first case of this type. The present case has highlighted the coexistence of both lesions.


Tracheal invasion is a poor prognostic factor in well-differentiated thyroid cancer. Appropriate resection can improve the prognosis and maintain the patient’s quality of life. Shaving resection for superficial tracheal invasion is minimally invasive because it does not involve the tracheal lumen, despite the problematic risk of local recurrence. Window resection for tracheal mucosal and luminal invasion provides good tumor control and does not cause postoperative airway obstruction; however, the need for surgical closure of the tracheocutaneous fistula is a disadvantage of this method. Circumferential (sleeve) resection and end-to-end anastomosis are highly curative, but the risk of fatal complications, such as anastomosis dehiscence, is a concern.Well-differentiated thyroid carcinoma (WDTC) is a slow-growing cancer with a good prognosis, but may show extraglandular progression involving the invasion of tumor-adjacent tissues, such as the trachea, esophagus, and recurrent laryngeal nerve. Tracheal invasion by WDTC is infrequent. Since this condition is rare, relevant high-level evidence about it is lacking. Tracheal invasion by a WDTC has a negative impact on survival, with intraluminal tumor development constituting a worse prognostic factor than superficial tracheal invasion. In WDTC, curative resection is often feasible with a small safety margin, and complete resection can ensure a good prognosis. Despite its resectability, accurate knowledge of the tracheal and peritracheal anatomy and proper selection of surgical techniques are essential for complete resection. However, there is no standard guideline on surgical indications and the recommended procedure in trachea-invading WDTC. This review discusses the indications for radical resection and the three currently available major resection methods: shaving, window resection, and sleeve resection with end-to-end anastomosis. The review shows that the decision for radical resection should be based on the patient’s general condition, tumor status, expected survival duration, and the treating facility’s strengths and weaknesses. Well-differentiated thyroid carcinoma (WDTC) is a slow-growing cancer that has a good prognosis. The trachea has posterior anatomical proximity to the thyroid gland and is affected by extraglandular extension of thyroid cancer. Tracheal invasion by WDTC is infrequent, exemplified by a rate of 3.4–13% ,3,4,5. SOn a case-by-case basis, the possibility of resection, expected postoperative functional disability, and patient’s background  should be considered before deciding on surgical intervention. This review discusses the indications for radical resection and the three major resection methods currently available: shaving, window resection, and sleeve resection with end-to-end anastomosis .The trachea extends from the inferior edge of the cricoid cartilage to the tracheal carina. The anatomy of the trachea has been described previously , and thePreoperative evaluation of tracheal invasion is important in determining the surgical procedure. Tracheal invasion may reach the adventitia, intra-cartilage, submucosa, or lumen. Therefore, it is important to evaluate not only the presence but also the degree of invasion. Modalities for evaluation of tracheal invasion include computed tomography (CT), magnetic resonance imaging (MRI), bronchoscopy, and endobronchial ultrasound (EBUS). In cases where ultrasonography (US) or CT show the extrathyroidal extension and tracheal invasion is suspected, further evaluation is recommended.US is the first examination to be performed because it is minimally invasive, relatively inexpensive, well-tolerated, and does not require contrast agents. Yamamura et al.  evaluateThe American Head and Neck Society consensus statement indicates  that CT Similar to CT and US, MRI has been shown to be useful in evaluating tracheal invasion in several reports. Wang et al.  evaluateBronchoscopy is useful for observing infiltration into the tracheal lumen and changes in the tracheal mucosa. Although it is invasive, laryngeal fiber can be used as a substitute, and it is a familiar examination for otorhinolaryngologists. In esophageal cancer, the usefulness of bronchoscopy for the evaluation of tracheal invasion has been demonstrated ,26,27,28EBUS is excellent for assessing the presence and degree of the tracheal invasion by visualizing the layered structure of the tracheal walls from the tracheal lumen’s side. Wakamatsu et al.  comparedSeveral studies have compared methods of evaluating tracheal invasion. According to Wakamatsu et al. , MRI andTangential tumor excision/shaving is used for thyroid tumors that superficially invade the trachea. This method involves sharp resection of the invading thyroid tumors, which are difficult to remove by blunt dissection, from the tracheal cartilage or membrane using a scalpel. Complete macroscopic resection should be performed, although the resection should not extend into the tracheal lumen. Occasionally, electrocauterization may be performed intraoperatively if a microscopic residual tumor can be identified.Tsukahara et al. reported that in cases where macroscopic examination reveals that the tumor invasion has not reached the tracheal mucosal surface, a shaving tracheal surface resection achieved local control in 21 (95%) of their 22 patients. Thus, the authors concluded that if the tumor did not show macroscopically evident extension to the tracheal mucosal surface, local control by tracheal shaving would be feasible . The facOne of the reasons underlying the utility of the shaving technique for superficial tumor invasion of the trachea in thyroid cancer is that superficial invasion is difficult to define and diagnose. This is supported by the classification of tracheal invasion developed by Shin et al. , which sp = 0.030). It appears that, although the presence of microscopic residual disease did not affect recurrence-free survival, microscopic tumor remnants had a detrimental impact on survival, which was associated with a significant difference in DSS between patients with R0 and R2 statuses. However, the data in their study did not demonstrate a statistically significant difference in survival between the R0 and R1 groups.In a recent report, Kim et al.  noted thIn the 2016 study by Su et al. , no statThere are two major surgical techniques for the resection of intraluminal tracheal invasion by WDTC: window resection and circumferential resection (sleeve resection) followed by end-to-end anastomosis. Each surgical technique has its advantages and disadvantages as well as limitations with regard to the indications.Window resection is a technique wherein the tracheal wall invaded by thyroid cancer is resected in all layers with a safety margin. In cases in which the resection range is limited or only the posterior membranous tracheal wall is preserved, it can be sutured primarily without requiring a tracheostomy or tracheocutaneous fistula. If the excision is larger, a tracheocutaneous fistula is constructed. Several operations will be required to achieve closure of the fistula. The advantages of a window resection include a stable postoperative airway, less invasive surgery, low rate of surgical complications, and avoidance of postoperative fixation of the neck region . This prIf the tracheal resection is not particularly extensive, primary suture is possible and tracheostomy or tracheocutaneous fistula creation are not necessary. Even if a large resection is performed, if only the posterior membranous tracheal wall is preserved, it can still be sutured, as reported by Tsukahara et al. . They peOnce a tracheocutaneous fistula is constructed, primary closure of the fistula may be difficult in some cases, requiring reconstruction using various techniques. Window resection often requires multi-staged surgeries for reconstruction, which prolongs hospital stay and can adversely affect the quality of life. In addition, the inner surface of the reconstructed trachea is covered by skin and lacks mucosal epithelium and mucus secretion. A dry surface and squamous debris may cause chronic cough, discomfort, and obstruction of the respiratory tract. Small tracheal defects, up to a length equivalent to seven rings, can be closed nonproblematically using local skin flaps . A much Circumferential resection is performed when the invading thyroid carcinoma extends into the tracheal mucosa or lumen. After resection, end-to-end anastomosis is considered the ideal airway reconstruction technique because airway reconstruction can be completed in one stage, and the airway lumen would be covered by normal tracheal mucosa ,62,63. TAfter standard thyroidectomy, the tracheal segments showing invasion, including the membranous portion, are resected circumferentially while observing the tracheal lumen. The advantages of this approach include a full-thickness specimen of the trachea for the precise determination of invasion depth and margin status and visualization of the trachea with the residual tumor . The resAnastomotic dehiscence is one of the most feared complications as it is a life-threatening condition that can occur in all types of tracheal resections. In a review article, postoperative complication rates after tracheal sleeve resection with end-to-end anastomosis ranged from 15% to 39%. Postoperative mortality is approximately 1.2% . Older rFinally, it is important to avoid anastomosis dehiscence and suture with less tension. One technique for reducing anastomotic tension is suprahyoid laryngeal release. This technique involves transection of the mylohyoid, geniohyoid, and genioglossus muscles and the lesser cornua of the hyoid bone resulting in a laryngeal drop of 2–3 cm ,77. SuprThyroidectomy associated with tracheal resection with end-to-end anastomosis is universally accepted to achieve local tumor control. Complete resection of local disease has been reported to provide the longest survival ,74,81,82There is a general consensus that the order of curability of radical surgical treatment for tracheal invasion is sleeve > window > shaving resection. However, shaving is the least invasive surgery. In the case of superficial invasion of the trachea up to the perichondrium or cartilage, there is consensus that shaving is generally sufficient if the preoperative evaluation is accurate, although it is sometimes difficult to determine the degree of invasion. In a much deeper invasion, many reports indicate that shaving may increase the risk of local recurrence because of microscopic tumor remnants; therefore, shaving may not be sufficient. Moreover, there is consensus that a full-laminar resection is necessary if the disease extends into the lumen or tracheal mucosa. In such cases, there are two possible resection methods: window resection and circumferential resection. Notably, the perioperative risk of sleeve resection is higher than that of window resection, and window resection is not less invasive due to the need for another surgery to close the tracheocutaneous fistula and the possibility of stenosis. However, apart from these complications, window resection is relatively safe, which is a great advantage. Nonetheless, the evidence is inconclusive and requires prospective review. The decision will be based on the patient’s general condition, tumor status, expected duration of prognosis, and the facility’s strengths and weaknesses . In case


The prognosis of atypical pulmonary carcinoid with liver metastases is extremely poor, and patients with multiple liver metastases are often treated using non-surgical therapies. We report a case with multiple liver metastases from atypical pulmonary carcinoid that was successfully treated using two-stage hepatectomy combined with embolization of portal vein branches.A 48-year-old man was referred to our department after multiple liver tumors were detected in both liver lobes on computed tomography. He had undergone right upper lobectomy of the lung for atypical pulmonary carcinoid  2 years previously. Positron emission tomography-computed tomography showed no extrahepatic tumor manifestations. The tumors were located in segment 2, 3, 5/8 and the right hepatic vein drainage area. We planned complete resection of metastases in a two-stage hepatectomy. The first stage comprised concomitant left lateral segmentectomy, partial hepatectomy of segment 5/8 and portal vein embolization of the posterior segmental branches. The second stage comprised resection of the right hepatic vein drainage area, performed 21 days after the first surgery. Histopathological diagnosis was liver metastases of atypical pulmonary carcinoid. Postoperative bile leak developed, which was treated with endoscopic retrograde biliary drainage and percutaneous bile leak drainage. He has been followed for 24 months postoperatively without tumor recurrence.Two-stage hepatectomy may represent an option for bilobar multiple liver metastases from atypical pulmonary carcinoid.We successfully treated a patient with multiple liver metastases of atypical pulmonary carcinoid using a two-stage hepatectomy combined with portal vein embolization of the posterior segmental branches. Patients with multiple liver metastases from AC are often treated using non-surgical therapies such as somatostatin analogue, chemotherapy, peptide receptor radionuclide therapy, radiofrequency ablation or transarterial embolization ,2. Two-s2((A 48-year-old man was referred to our department with multiple liver tumors detected on follow-up computed tomography (CT). He had undergone right upper lobectomy of the lung for AC 2 years previously. The pathological stage of the primary had been T2aN0M0 (stage IB). He was asymptomatic and had no relevant medical history. Contrast-enhanced CT and gadolinium ethoxybenzyl diethylenetriamine pentaacetic acid (Gd-EOB-DTPA)-enhanced magnetic resonance imaging (MRI) revealed multiple metastases in both lobes of the liver a–d. Thesa,b. Fibrc. Duringd. Hepati and AC, has been widely used in pulmonary disease, unlike digestive disease. At this stage, no move has been made toward World Health Organization terminologies for gastroenteropancreatic neuroendocrine tumors (GEP-NETs) .AC shows a higher rate of distant metastases and a poorer prognosis. The 5- and 10- year survival rate for AC ranges from 40 % to 59 %, and from 35 % to 59 % respectively . MetastaGlazer reported that early and aggressive surgical management of liver metastases from neuroendocrine tumors is associated with significantly improved long-term survival rates.  SurgicalTwo-stage hepatectomy is now an established strategy for patients with multiple bilobar colorectal liver metastases, due to the short- and long-term outcomes ,4. AccorIn the current case, sufficient FLRV was achieved by portal vein embolization of the posterior segmental branch during the first surgery, allowing subsequent complete hepatic resection to be performed.Little evidence is available to guide surgery for liver metastases of AC, and therefore the present strategy was based on ENETS consensus guidelines for gastrointestinal neuroendocrine tumors ,9.Ligation of the posterior branches of the portal vein is complicated and may make the second-stage hepatectomy technically difficult , so we pSeveral cases of AC with recurrence beyond 10 years from the initial resection have been reported . Long-te4We successfully treated a patient with multiple liver metastases of AC using a two-stage hepatectomy combined with portal vein embolization of the posterior segmental branches. Two-stage hepatectomy may be a good option for bilobar multiple liver metastases of AC.There are no conflicts of interest.We have no sponsors.This study has been exempted by our institution.Written informed consent was obtained from the patient for publication of this case report and any accompanying images.TK: study design, data collection, data analysis, writing.SO: critical revisionKY: final approval of the articleAny other authors: data collectionAll authors read and approved the final manuscript.This paper is case report. The authors don’t need to register this work.Teppei Kamada, the corresponding author of this manuscript accept full responsibility for the work and the conduct of the study, access to the data and controlled the decision to publish.Not commissioned, externally peer-reviewed.


There are no previous reports of debulking of giant liver hemangioma. This report describes our experience with debulking surgery for a patient with bilateral giant liver hemangiomas with severe symptoms.We present a case of symptomatic giant liver hemangioma in a 41-year-old woman. She presented with abdominal pain and fullness at a local hospital and underwent trans-arterial embolization (TAE). TAE was not effective, and she was not able to walk a long distance. Giant liver hemangiomas, 30 cm and 15 cm in diameter, were located in the right liver and in the left lateral section, respectively, and normal liver parenchyma with tiny liver hemangiomas was present in segment 4. The liver function was normal. However, right hemi-hepatectomy with left lateral sectionectomy was considered to be risky according to the 3DCT volumetry. Therefore, we performed right hemi-hepatectomy in order to reduce her symptoms. The postoperative course was uneventful, and she was discharged on the 14th day after surgery. The abdominal fullness and abdominal pain disappeared immediately after surgery. The hemangiomas in the remnant liver, 15 cm in diameter, showed no change, and she is well without symptoms 7 years after surgery.Debulking surgery is one of the options for bilateral giant liver hemangiomas with severe symptoms. Surgery for liver hemangiomas is still controversial. However, surgical outcomes are reported to be good –3. Giant4/μL), normal serum total bilirubin (1.0 mg/dL), normal serum albumin (4.5 g/dL), normal serum prothrombin time (88%), mildly elevated D-dimer (9.78 μg/mL), and mildly elevated FDP (23.1 μg/mL). The Child-Pugh classification was A, and the indocyanine green retention rate at 15 min was 4%.A 41-year-old woman was referred to our hospital to have treatment for giant liver hemangioma. The liver hemangioma was detected 6 years previously, and the tumor had gradually increased in size starting 3 years before admission. She underwent trans-arterial embolization (TAE) two times for liver hemangioma at another hospital. However, TAE was not effective, and the liver hemangioma increased in size. She felt abdominal pain and abdominal fullness, which made her unable to walk a long distance. There was no past history of chronic hepatitis or blood transfusion. Admission laboratory tests revealed a mildly decreased hemoglobin (10.1 g/dL), normal platelet counts (14.7 × 10Plain computed tomography (CT) showed giant liver hemangiomas with lipiodol deposits Fig. a. MagnetThe abdominal fullness and abdominal pain disappeared immediately after surgery, and she completed a full marathon 3 years after surgery. The hemangioma in the remnant liver, 15 cm in diameter, shows no change, and she is well without symptoms 7 years after surgery (Fig. Abdominal pain is the most frequent complaint of liver hemangioma when the tumor is larger than 10 cm in diameter . The mecLiver function is normal in most cases of liver hemangioma; therefore, liver resection is considered one of the options if it is resectable. However, there are some unresectable cases due to the location of the tumors or coagulopathy. Liver transplantation should be considered at that time . HoweverMost reports of debulking surgery are for advanced ovarian cancer to improve survival after surgery. Very few reports on debulking surgery for benign tumors have been published. Debulking of a colon hemangioma with Kasabach-Meritt syndrome was reported in 1998. In that report, a 39-year-old man had a history of multiple hemangiomas since birth, with involvement of his left perineum and lower extremity, liver, spleen, descending colon, and rectum. He experienced eight episodes of major lower gastrointestinal bleeding since age 18. He underwent left colectomy because a technetium 99m pertechnetate-labeled red cell scan indicated the location of massive bleeding to be from the hemangioma of the sigmoid colon. The patient’s coagulation profile normalized 6 weeks after surgery, and he had not had any further episodes of bleeding 4 years after surgery . A searcLiver hemangioma is a benign disease; therefore, surgical intervention should be carefully considered when a tumor is increasing in size, larger than 10 cm in diameter, and symptomatic and has coagulopathy. However, liver resection is considered one of the options for a resectable liver hemangioma because short- and long-term surgical outcomes are good. Even if curative resection is not possible, debulking surgery should be considered because surgery improves symptoms and coagulopathy. However, this is a case report and there are no similar reports in the PubMed database. We need more evidence on whether debulking surgery could be acceptable for patients who cannot undergo complete resection of complicated liver hemangiomas.Debulking surgery is one of the options for bilateral giant liver hemangiomas with severe symptoms.


A 65-year-old woman with a long surgical history was referred to our hospital's Colorectal Unit for ileostomy management. The patient retained an ileostomy for almost a decade after a series of complicated operations she had undergone, which had several side effects such as electrolyte imbalances, high output, weight loss, and a parastomal hernia. Our hospital's colorectal surgeon proposed to replace the ileostomy with a permanent sigmoidostomy and asked for an imaging evaluation of the parastomal hernia content before the surgery. A computed tomography of the abdomen was performed using our Computed Tomography Department's 64-detector row CT scanner after oral administration of contrast media, without intravenous contrast media injection due to allergy. Concerning the parastomal ileostomal hernia, besides small bowel loops with intraluminal gastrografin, inside the parastomal hernial sac, there also was an almost rounded cystic lesion. Absence of the gallbladder at its typical position and no record of cholecystectomy raised suspicion for gallbladder projection inside the sac. Our suspicion was confirmed during the surgery. Nonexisting acute cholecystitis allowed easy reduction of the gallbladder along with the small bowel loops inside the peritoneal cavity, without proceeding to cholecystectomy at the same time. Finally, ileostomy was annulated and an end colostomy was established. Four days after the surgery, the patient was discharged from the hospital and was happy to live an almost normal life thereafter. We present the case of a 65-year-old female patient, whose parastomal hernia was complicated with the gallbladder being part of the content within the hernial sac. Unlike most of the reported cases in literature (8 reported worldwide till this moment), our patient did not present to our department with incarceration of the hernia or acute cholecystitis.A 65-year-old woman, who had already been operated several times, was referred to the Colorectal Unit of our hospital for consultation. Her first operation took place ten years ago for surgical management of a rectocele. More specifically, a gynecologist performed a rectopexy to the patient, but unfortunately, the operation was complicated with a rectovaginal fistula. A diverting colostomy at that time was the bridging treatment for a future effort for permanent therapy. This was attempted with a low anterior resection plus a prophylactic loop ileostomy and excision of the fistula. Once more, the postoperative course was not the proper one with rupture of the anastomosis and urgent reoperation in a septic field. Finally, a pull-through procedure was the salvage. The loop ileostomy remained intact, and that exactly was the problem. The patient had a remaining ileostomy for about 9 years, with all the side effects and complications this could have: electrolyte depletion, high output, severe weight loss, and lately, an existing parastomal hernia. So, the patient finally presented to our hospital's Colorectal Department for ileostomy annulation and with her consent, our colorectal surgeon decided to replace it with a permanent sigmoidostomy instead. Before the operation, an imaging evaluation of the parastomal hernia with an abdominal computed tomography (CT) was ordered. The examination was performed using our Computed Tomography Department's 64-detector row CT scanner  with CT slices of 3 mm after oral administration of contrast media. Intravenous iodinated contrast media was not used due to reported allergic reaction of the patient in the past. Coronal, sagittal, and oblique multiplanar reconstructions (MPR images) were also evaluated additionally to axial images. Inside the parastomal hernial sac, apart from small bowel loops, CT revealed a cystic lesion of 8 × 3.5 cm Figures . NonexisAs already mentioned, this is an unusual case of parastomal gallbladder herniation, unexpectedly found during preoperative CT abdominal imaging in a patient coming for surgical management of a remaining ileostomy.To our knowledge, this is an extremely rare condition since there are only 8 such reported cases around the world up to now –8. In faNoteworthy is also the fact that despite her long surgical history of about a decade and consequently despite lots of previous imaging examinations, our patient's parastomal gallbladder herniation was never mentioned until she came to our hospital.According to the existing literature, this rare type of parastomal organ projection seems to occur in elderly patients, aging 75-year average –8. ElderFinally, it should be pointed out that our clinical case has a lot in common with the case presented by Rogers et al. , as both patients were women with no signs of acute cholecystitis or incarceration of the herniated gallbladder and so, it did not require urgent intervention with cholecystectomy . Our maiTo conclude, parastomal gallbladder herniation is an interesting condition because of its rarity, occurring so far in elderly people and usually complicated with cholecystitis or incarceration. Absence of such situations and appearance at a younger age makes our case presentation particularly zestful.


Idiopathic tracheal stenosis (ITS) is a rare condition, and diagnosis of exclusion should be suspected in patients with exercise intolerance, wheezing, and dyspnea on exertion with a flow-volume loop suggestive of fixed airway obstruction. We report a case of a 32-year-old asthmatic woman with an existing diagnosis of vocal cord dysfunction and previous normal CT scan of the neck. She continued to have fixed upper airway obstruction on repeated flow-volume loops with persistent wheezing and cough along with occasional stridor and hoarseness of voice despite appropriate management of her asthma. She was finally diagnosed with ITS on a repeat CT scan of the neck for which she underwent laser surgery, steroid injection, and controlled radial expansion balloon dilation with a successful reduction of stenosis. This case illustrates the importance of clinical suspicion for early diagnosis of ITS in poorly controlled asthmatic patients and the relevance of non-surgical management of this condition. Idiopathic tracheal stenosis (ITS) is usually misdiagnosed as asthma, which delays appropriate management and adds to the frustrations of patients. Diagnosis is based on history, neck imaging, and spirometry and confirmed with direct visualization of the stenotic segment during bronchoscopy. Treatment approaches include surgical and non-surgical options.A 32-year-old Caucasian woman, a lifelong non-smoker with a body mass index of 44.3, was referred to our outpatient pulmonary clinic for asthma and vocal cord dysfunction. She had complaints of cough, wheezing, and dyspnea on exertion with occasional stridor for many years for which she was on a steroid inhaler along with albuterol inhaler. She reported poor control of her symptoms and denied any history of recurrent infections, increased sputum production, hemoptysis, esophageal reflux, tracheal trauma, previous endotracheal intubation, or any inflammatory or infiltrative processes. Her erythrocyte sedimentation rate, antinuclear antibody, rheumatoid factor, anti-myeloperoxidase (MPO), anti-proteinase 3 (PR3), anti-Sjögren's syndrome A (SSA/Ro), anti-Sjögren Syndrome B (SSB/La), anti-Scl70, and circulating levels of alpha -1 antitrypsin were within the normal limits. She had no eosinophilia, and her IgE level was normal, along with normal immunoglobulin levels. She also had a sleep study, which was normal, and over 18 months, had four pulmonary function tests (PFTs), all of which showed an obstructive pattern with no bronchodilator response and normal diffusion capacity. Flow-volume loops showed flattening of inspiratory and expiratory maneuvers, suggestive of fixed upper airway obstruction.Three years prior to our initial evaluation, she had a CT scan of the neck, which showed only mild prominence of parapharyngeal mucosa that appeared reactive with no other abnormalities and underwent otorhinolaryngology evaluation and was diagnosed as vocal cord dysfunction. During one of her follow-up appointments in our clinic, she endorsed new onset hoarseness of voice. Giving her new onset hoarseness of voice and multiple previous PFTs suggestive of fixed upper airway obstruction, we decided to repeat her imaging. She underwent a CT scan of the neck, which confirmed an irregularly narrowed (60%) trachea 3 cm above the carina and 6 cm below the vocal cords with septation along the right posterior aspect of the trachea Figure .Subsequently, she underwent bronchoscopy that showed complex tracheal stenosis approximately 3 cm above the carina and extending for 4 cm into the trachea. It showed complex narrowing that caused significant scarring in the middle leading to a small airway on the right posterior wall of the trachea, giving an appearance of a tracheal bronchus Figure . The narITS represents a diagnosis of exclusion and occurs exclusively in middle-aged women, mostly postmenopausal -3. It prTracheal stenosis could be due to extrinsic  or intrinsic causes. Etiologies of intrinsic stenosis include autoimmune conditions , inhalational burn, infectious processes , or idiopathic as in our patient . The diaThere is some controversy regarding the most favored approach for the treatment of ITS, with balloon dilation being the most conservative approach . Other mITS is a rare condition, and a diagnosis of exclusion should be suspected in patients who present with unexplained wheezing, hoarseness, dyspnea on exertion, and cough along with flow-volume loops suggestive of fixed upper airway obstruction. It may mimic symptoms of asthma and, in our case, an existing diagnosis of vocal cord dysfunction, which in turn can lead to delay in diagnosis and definitive management. Despite growing evidence of lesser recurrence rates in favor of surgical correction, we believe that there is still a significant role of minimally invasive measures in the treatment of the stenosis. However, it is agreeable that repeated laser and CRE balloon dilations would render the patient an unsuitable candidate for definitive surgical management. Patients may continue to have flow-volume loops suggestive of fixed upper airway obstruction despite clinical improvement, which makes it more challenging to follow up these patients for relapse. More studies regarding the resolution of the fixed defect in the flow-volume loop after laser surgery and balloon dilation are needed.


Primary non-Hodgkin’s lymphoma of the bone remains an uncommon presentation of non-Hodgkin’s lymphoma. Primary lymphoma of the cranial vault is exceptionally rare. Here, we present a 62-year-old immunocompetent male presenting with the rapid growth of a left parietal scalp lesion and new-onset seizure. In addition to his imaging, which showed an extracranial, cranial, and intracranial mass with bony destruction, sagittal sinus involvement, and parenchymal invasion, his diagnostic angiogram demonstrated extensive vascular supply from both the right and left external carotid branches. Intraoperatively, we confirmed a frank invasion of the posterior sagittal sinus. After subtotal resection followed by R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone therapy, the patient continues to be disease-free at the 10-month follow-up. We report here a case of primary cranial vault lymphoma that very closely mimicked meningioma in many ways, with positive angiography and intraoperatively confirmed venous sinus invasion. Primary non-Hodgkin’s lymphoma (NHL) of the bone remains an uncommon presentation of NHL, accounting for 0.3% to 5% of all NHL -5. PrimaHistory and examinationA 62-year-old male on vacation presented to our emergency department after suffering a new, left-sided, simple partial seizure that became a generalized clonic seizure. He had been aware of a right parietal scalp mass that had rapidly progressed in size from a small nodule to approximately grapefruit-sized in four months. After resolution of his post-ictal state, he initially had upper and lower extremity paresis, which had resolved at presentation. Before his seizure, he had been completely asymptomatic other than noticing the physical features of the growing mass. He denied any previous neurological and constitutional symptoms, including night sweats, fever, chills, abnormal weight loss, or inguinal, axillary, or otherwise lymphadenopathy.On examination, the patient was alert, oriented, and without focal neurological deficits. No lymphadenopathy, splenomegaly, and hepatomegaly were appreciated. A roughly grapefruit-sized, hard, immobile, nontender right parietal mass under the normal-appearing scalp was appreciated. Computed tomography (CT) of the brain demonstrated a large subcutaneous mass with osteolytic infiltration of the right parieto-occipital bone with intracranial extension measuring 7.5 x 5 x 9.6cm. The mass was abutting the superior sagittal sinus (SSS). Bone windows showed frank bony destruction of the calvarium Figure .Due to the proximity and appearance of extension around the SSS, CT angiography (CTA) of the head and neck was obtained, which noted vascularity within the mass and the appearance of invasion into the posterior segment of the SSS.Magnetic resonance imaging (MRI) of the brain with and without contrast was obtained and showed a large right parietal mass with extension through the calvarium, crossing midline with the involvement of the posterior sagittal sinus, mass effect on the parietal lobe, and evidence of parenchymal invasion with associated cerebral edema. Also noted was extensive dural involvement and extension, consistent with possible atypical or malignant meningioma Figure .Angiography was performed demonstrating the right parietal branch of the superficial temporal artery, as well as the occipital artery, appeared to supply the scalp portion of the mass. In addition, the distal parietal branch of the right middle meningeal artery (MMA) appeared to also supply the mass. Significant stenosis in the posterior third of the SSS but with continued patency was illustrated Figure .The patient was medically stabilized, started on levetiracetam and decadron, and a complete metastatic workup with contrasted CT of the chest/abdomen/pelvis was performed, which was unremarkable. Upon work-up completion, the patient elected to return home for any treatment and/or intervention. However, he re-presented within 24 hours with another generalized clonic seizure and now had persistent left upper and lower extremity paresis, continued left lower extremity clonic movements of which he had gross neglect, prolonged post-ictal period, and continued numbness and tingling of the left upper extremity. His levetiracetam was increased and valproic acid was added, but after several days, he continued to have intermittent simple partial seizures involving the left upper and lower extremity and significant left-sided paresis. Given his now progressive neurological decline, the patient was taken for right frontal-parietal decompressive craniectomy and resection of the mass.OperationIntraoperatively, a large, highly vascular, firm, dense mass was encountered that had effectively destroyed a large portion of the involved calvarium. On careful dissection, extensive dural involvement was found, with the extension of the mass through the dura, forming a plaque upon the right motor and sensory cortices with intraparenchymal invasion. The mass was also found to have invaded the SSS, which was skeletonized and debulked as much as possible. Ultimately, the patient received a subtotal resection with titanium cranioplasty Figure .PathologyPostoperatively, initial pathology was reported as a possible World Health Organization (WHO) grade III meningioma. Stains on permanent showed positive CD45, positive BCL2, positive CD20, negative EMA, negative GFAP, and Ki-67 of 50%. Final pathology returned as diffuse large B-cell lymphoma (DLBCL) with fluorescence in situ hybridization (FISH) showing a 68% loss of BCL6. Additional cerebrospinal fluid (CSF) analysis was negative for any signs of atypical lymphocytes or lymphoid neoplasm.Postoperative course and treatmentThe patient underwent two cycles, one inpatient and one outpatient, of R-CHOP  chemotherapy as well as two rounds of intravenous (IV) rituximab and methotrexate for the noted intraoperative parenchymal invasion. While cerebrospinal fluid (CSF) studies were pending, he received a single round of intrathecal cytarabine given the dural invasion into and through the leptomeningeal space. After multiple CSF analyses demonstrated no abnormality, no further intrathecal therapy was administered. At discharge, he was neurologically intact, no further seizures, and at the time of this article, has completed his treatment and remains disease-free 10 months postoperatively.Primary lymphoma of the cranial vault remains an extremely rare disease -8. The rOur case is unique in several aspects: the patient presented with a history of relatively rapid growth and contrasted studies and angiography showed a large extensively vascular mass, with large feeding vessels, and aggressive sagittal sinus involvement. Initially, our differential diagnosis leaned towards a high-grade meningioma, though lymphoma and metastatic carcinoma were considered. The features of this mass, however, were seemingly inconsistent with meningioma, specifically osteolytic rather than hyperostotic bony invasion, and the intra-axial component Table  5,14]. ,14. 5,14On angiography, the right superficial temporal artery, the left occipital artery, and the right distal MMA were found as vascular supply to the mass, and we believe this to be the first case report to give such details on PCVL. Some authors report the findings on angiography of vascularity to be diagnostic of meningioma while the lack thereof more indicative of PCVL or PDL -18. SecoWe feel it is important to emphasize how closely this case resembled meningioma during the patient’s workup. His MRI showed a likely dural-based mass, there was aggressive bony destruction, and he had positive angiography with multiple feeding vessels. While typically extra-axial lesions, a review on invasive meningiomas by Brokinkel et al. found brain invasion in 5%-78%, with rates of invasion increasing with WHO grade . A revieGiven such a low incidence and few reports, it is difficult to definitively establish a prognosis for these patients. El Asri et al. reported that of 22 cases in which the outcome was available, 13 of 16 patients followed out to 12 months were still alive . In the In summary, PCVL continues to represent a rare pathology that will elude consideration in a patient’s diagnosis. The most common errant diagnosis, after a review of the available cases, will be that of meningioma and multiple features on radiographic imaging will continue to make diagnosis difficult. Here, we report a case where PCVL had extensive arterial vascular supply, as demonstrated in angiography, which we believe to be the first reported of its kind. In instances of patients presenting with such extensive extracranial, cranial, and intracranial involvement, the diagnosis of PCVL will need to be diligently considered even in the face of a positive angiography.