Echinococcosis multilocularis (Hydatid Disease - HD) is a zoonotic disease caused by the larval form of Echinococcus multilocularis (EM). The main sites for this zoonosis are the Middle East, China, India, Alaska, and Siberia. It is rather rare in Europe. In Poland, the Warmian-Masurian Province is the endemic region for Echinococcus multilocularis. The clinical manifestation of the disease is dependent on the location, the size of the cyst and the development stage of the parasite. Considering the uncommon character of echinococcosis in Central Europe, especially such located in the areas outside the liver and lungs, the authors would like to present a case of coexistence in one patient of two EM foci in the liver and the head of the pancreas.We present a clinical case of a 32-year-old man who was diagnosed with a cystic lesion with septa and calcification in the sixth segment of the liver and a suspicious change in the head of the pancreas. ELISA Em 2 plus test was positive, Western Blot method - the P-5 pattern showed an image that is characteristic of an EM infection. The sixth liver segment with a tumour and a tumour from the head of pancreas were excised by means of laparotomy. On the 6th day after the surgery the patient was discharged from hospital without complications and in good condition. Currently, he is under the control of a parasitic and zoonotic clinic. He takes an 800 mg daily dosage of Albendazole.The presented clinical case shows that if we have a patient with cystic / tumour change in the pancreas and positive immunological tests, CT and MRI of the abdominal cavity are usually sufficient in order to fully diagnose and to qualify such a person for surgery. The most effective treatment is surgical treatment supplemented with pre- and postsurgical treatment with Albendazole. Echinococcosis (hydatid disease) is a zoonotic disease caused by the larvae Echinococcus multilocularis (EM). The infection usually occurs after accidentally ingesting the parasite’s eggs. The oncospheres which are released from the eggs migrate to the internal organs, mainly the liver (50–77%) and lungs 15–47%), where they develop larval forms that form structures composed of small cysts with a diameter of 0.5 to a few millimetres, containing thousands of protoscolices – hydatid cysts 5–47%, wh. It is rThe clinical manifestation of the disease depends on the location, size of the cyst and the development stage of the parasite. The main symptoms in the abdominal location are abdominal pain, epigastric discomfort, vomiting and gradual weight loss. Diagnosis is based on imaging studies, mainly ultrasonography and computed tomography of the abdominal cavity. However, the diagnosis is quite difficult because changes shown in imaging often resemble neoplastic tumours. The enzyme-linked immunosorbent assays  are very helpful in differentiating between them. However, laparotomy or laparoscopy becomes the final diagnostic and therapeutic method due to the chance of obtaining false-positive or false-negative results of these tests. Surgical procedures are the most effective treatment methods .Taking into account the rare nature of echinococcosis in Central Europe, especially such affecting areas outside the liver and lungs, the authors would like to present a case of coexistence in one patient of two Echinococcus multilocularis foci in the liver and the head of the pancreas.A 32-year-old man reported to the general practitioner with general weakness, weight loss (12% in 6 months), and worsening stomach aches for about a month. The patient has not been treated for chronic diseases so far, he has not been taking any medications permanently. He lives in a city agglomeration. There are no animals in the house. After carefully collecting the family history, including the history of his childhood, it turned out that as a child he went to the forest very often and consumed unwashed forest fruits. The patient told, that he had eaten lots of unwashed berries and blueberries in the forest, immediately after picking them up about 10–15 years before the diagnosis. Abdominal cavity ultrasound revealed a cystic lesion with a septum and calcification in the sixth segment of the liver and a suspected solid change in the head of the pancreas.In the physical examination no deviations from the normal state were found. In laboratory tests, the following deviations from the normal state were observed: Alat-61 U / i , IgE -182,1 IU / ml  CA - 19.9 – normal, CEA – normal.The CT scans showed that chest organs were normal. The scan of the chest, abdominal and pelvic regions was performed after intravenous contrast administration with a slice thickness of 5 mm. In the abdominal cavity in the 6th liver segment a cystic change was found with calcification in the centre and small septum with dimensions of 43x28x43mm and a solid change with calcified septa with dimensions of 35x29mm in the head of the pancreas, which models the duodenum and the wall of the inferior vena cava Fig. .Fig. 1ByThe diagnosis embraced also an MR of the abdominal cavity, in which the presence of a change in the liver was confirmed. It showed a weaker signal in T1 images and a heterogeneous T2 signal. The change did not enhance after contrast administration and did not show signs of diffusion limitation. In addition, there was a change of 44x31x42 mm in the head of the pancreas, which models the duodenum and the inferior vena cava  were within the normal range, family history of gastrointestinal tumours were negative; imaging tests were carried out: ELISA test, Western blot - confirmation of EM infection. Therefore, we decided to remove the pancreatic and liver changes at the same time.The sixth liver segment with a tumour and a tumour from the head of pancreas were excised by means of laparotomy.The macroscopic analysis showed that the liver tumour was in the shape of an irregular cone with a height of 65 mm, after cross-section it consisted of yellow, irregular masses and small cysts filled with a jellylike content.The tumour of the pancreas head after the excision was 43x30x45 mm and appeared to be surrounded by a thin bag. The microcysts present in cross section were filled with a jellylike content.Histopathological examination confirmed the presence of EM in the liver and in the pancreas Fig. .Fig. 3EMIn the PCR study, the genetic material of EM was confirmed. Postsurgical course without complications. On the 6th day after surgery the patient was discharged from hospital without complications and in good condition.Currently, he is under the control of a parasitic and zoonotic clinic. He takes an 800 mg daily dosage of Albendazole. The follow-up CT of the abdomen performed 2 months after the surgery showed no recurrence in the abdominal cavity Fig. .Fig. 4ThHydatid disease is most commonly located in the right lobe of the liver. Pancreatic localisation is described in the literature with a frequency of 0.2–2%  Most oftIn our case, the changes in the liver and pancreas were similar in size and probably developed independently at the same time. There are several paths of the development of the EM in the body of a human host. Larvae with a diameter of 0.3 mm and smaller that have escaped the hepatic filtering system (first Lemman’s filter) can migrate to the lungs, which are the most frequent place where EM is located after the liver. If the larvae are not stopped in the lungs (second Lemman’s filter), they can into the bloodstream and to other organs.The oncospheres released in the small intestine penetrate through the mucous membrane into the blood vessels and enter the liver with blood. There is also the possibility of them getting into the lymphatic vessels. This migration of EM larvae resembles the spread of digestive tract tumours.Due to their small size, EM larvae can get back to the pancreas from the liver through the bile duct , 8, 9. AData from various sources describe hydatid disease in the form of a cyst. Solid changes are described sporadically. The clinical manifestation of the disease depends on the location, size of the cyst and the development stage of the parasite. Pancreatic cysts in the human pancreas grow slowly (3-20 mm / year), which is why clinical symptoms may appear late, especially if the change grows outside the organ and does not pressure the relevant clinical and anatomical structures  . Cystic Sometimes the cyst undergoes secondary superinfection. This occurs in the form of an abscess with acute abdominal symptoms. The literature also describes fistulas of the alimentary tract . ComplicDiagnosis of pancreatic cyst / tumour is based on the use of all of the available imaging tests - mainly USG, CT and abdominal MR. However, it is quite difficult because changes shown in imaging studies often resemble benign and malignant changes observed in the pancreas. Different types of EM antigens are very helpful in differentiating. Patients with positive immunological tests have a more frequent location of the cyst in the liver than other organs, although seronegativity does not exclude the extrahepatic location .It should be remembered that in patients with pancreatic EM localisation, bilirubin, aminotransferases, alkaline phosphatase, lipase, Ca-19.9 and CEA should be determined. Casoni’s test, which is still being carried out in some centres, has low sensitivity and is often false negative. We rely on ELISA and Western Blot test. However, due to the possibility of obtaining false-positive or false-negative results of these tests, often the final diagnostic and therapeutic method becomes a laparotomy or laparoscopy, which in combination with histopathological examination of the acquired tissue material gives 100% certainty of the diagnosis. Some authors report that 40–49% of EM invasions in the pancreatic localisation  can be cDetection of EM DNA by means of PCR is also an accessible and recognised method, but the reservoir material is required before surgery. Fearful of echinococcosis and symptoms of anaphylaxis, some authors do not recommend an aspiration biopsy, although PAIR is one of the techniques for the treatment of hydatid cyst which is generally recognised .A patient with cystic / tumour change in the pancreas and positive immunological tests, CT and MRI abdominal cavity, which are generally available and non-invasive, are usually sufficient to obtain a full diagnosis and qualify the patient for surgery .In case of complicated cysts, invasive tests like EUS and ERCP must be used for the diagnosis .When during the surgery we are not sure if there is a connection between the cysts with the bile, pancreatic or digestive tract, one can use the contrast administration to the cyst or perform intrasurgical cholangiography. You can use ERCP to perform intrasurgical bile duct prosthesis .The most effective treatment is surgery. Percutaneous drainage of pancreatic echinococcal cyst is an alternative to surgical treatment in people with high intrasurgical risk . SurgicaPresurgical use of Albendazole drugs reduces the risk of dissemination, anaphylaxis and recurrence of echinococcosis in patients operated on or treated with the use of the PAIR procedure. They are used at least 1 month before surgery or PAIR, while the length of application after surgery depends on the treatment centre. A minimum 2-year treatment period with Albendazole is recommended . It shouComplications of surgical treatment can be early or late. Perioperative mortality ranges from 0.5 to 4.0% . The morIn the case of intra-abdominal abscess and postsurgical wound infection, you should follow a routine procedure.The main late complications are recurrent cysts, which are rather rare in case of radical surgical treatment with the use of Albendazole . In the Based on exposure, clinical picture, imaging tests, and serological tests results Echinococcosis multilocularis can be diagnosed with sensitivity of up to 90–100% and specificity of up to 95–100%. The pancreatic EM location is a rare form of HD. The most effective treatment is radical surgical treatment combined with Albendazole treatment.


Duodenal diverticulum (DD) is a common incidental finding, which rarely causes complications. Perforation is one of the most feared and the least common complications. Surgery is the mainstay for complicated duodenal diverticulum, but with the advancement of medical treatment and intensive care, nonoperative management has been reported. We present a rare case of perforated DD that failed medical management and subsequently underwent surgical intervention.A 77-year-old, healthy female presented with right-sided abdominal pain with low-grade fever and leukocytosis. Computed tomography (CT) of the abdomen showed retroperitoneal fluid collection around the second part of the duodenum, which was not amenable to percutaneous drainage. Contrast studies showed no evidence of perforation or leak of the stomach or duodenum. The diagnosis was made via an upper endoscopy that showed a large periampullary duodenal diverticulum with purulent drainage and normal-looking ampulla. After failed conservative management with broad-spectrum antibiotics and worsening symptoms, she underwent excision and primary repair of the diverticulum with a jejunal serosal patch and exploration of the common bile duct (CBD). She had an uncomplicated postoperative course and was discharged home on postoperative day four.Although rare, the duodenal diverticular perforation can be a life-threatening complication. Combined subjective, clinical, and radiological assessment of the patient is crucial in deciding whether to operate or not. The duodenum is the second most common site for diverticula following the colon . The incA 77-year-old healthy female presented with right-sided abdominal pain, associated with low-grade fever to 38.2 °C and mild epigastric tenderness. She was hemodynamically stable, with no significant past medical or surgical history. An outpatient CT of the abdomen Figures -2 ordereThere are two types of DD, congenital and acquired. The majority of the diverticula are acquired extraluminal pseudodiverticula . DD commHistorically, perforated DD was treated surgically, but most recently, several reports have shown good outcomes with nonoperative management -3. The oSurgical management is challenging due to the proximity of the diverticulum to the papilla; hence, it is highly recommended to identify the papilla before surgery preferably via an upper endoscopy or intraoperatively by inserting a catheter by cholecystectomy or choledochotomy -6. PatieA recent case series by Thorson et al. showed that nonoperative management carries a lower morbidity and mortality rate than an operative approach . A classWe report a case of complicated DD who failed nonoperative management and subsequently underwent surgery. DD are often asymptomatic but may present with perforation with subsequent retroperitoneal inflammation and infection. Nonoperative management should be attempted for a clinically stable non-peritonitic patient. Surgery is a challenging approach given their location and close proximity to the ampulla of Vater. Various surgical approaches could be performed depending on the individual clinical status.


Myxomas account for approximately half of all primary cardiac neoplasms. Most occur in the left atrium and only rarely are attached to the mitral valve, with just over 30 such cases reported in the literature. These neoplasms can manifest with a combination of obstruction of blood flow, systemic embolization, and constitutional symptoms.We present a case of a 32-year-old African American man presenting at an emergency department with symptoms of a transient ischemic attack. Transesophageal echocardiography identified a mass originating from the posterior leaflet of the mitral valve. The mass was surgically resected and histologically classified as a myxoma. He remained asymptomatic during the course of 5-year surveillance.Few similar cases have been described in the literature. Here we present a review of the diagnosis and surgical management of this rare presentation for mitral valve myxoma. Myxomas are rare stromal tumors of multipotential mesenchymal cell origin, capable of neural and endothelial differentiation . As cardA 32-year-old, previously healthy, African American man presented to an emergency department 45 minutes after the acute onset of left facial droop and right-sided weakness Fig. . A thoroA median sternotomy was performed and cardiopulmonary bypass was employed via aortic and bicaval cannulation with full anticoagulation. His aorta was cross-clamped and his heart arrested with retrograde cardioplegia. The aortic valve was examined through an ascending aortotomy and all three valve leaflets appeared normal. A left atriotomy was made and the mass was easily identified on the posterior mitral valve leaflet adjacent to the mitral valve annulus Fig. . The maset al. performed a review in 1997 for all cases of mitral valve myxoma since they were first reported in 1871, including six postmortem and 21 clinical cases [et al. updated the discussion and added an additional 10 cases to the body of knowledge [Primary tumors of the heart are exceedingly rare; the incidence of primary tumors of the heart found at autopsy ranges from 0.0017 to 0.33% . Myxomasnowledge , 18. Thinowledge .The classic triad of obstructive symptoms, systemic embolization, and constitutional symptoms occur in conjunction infrequently. However, one of the three attributes may signify this entity on its own . Obstrucet al. reported that three-dimensional TEE can provide exact spatial information regarding the shape and surface of the mass, clarify the involvement of the mitral valve, and identify any obstruction of the mitral valve annulus or laceration of the mitral valve leaflets [Before the advent of angiocardiography in 1951, cardiac myxoma was only diagnosed at autopsy . A thoroleaflets . They fuleaflets . On macrleaflets . Histopaleaflets . These cleaflets . On immuleaflets .et al. reported the first surgical excision of a myxoma of the mitral valve in 1979 [et al. reviewed a 30-year experience, and in 1995 presented the largest series to date of operative approaches to cardiac myxoma [Prompt surgical excision of a mitral valve myxoma must be undertaken as the patient is at risk for additional embolic events. Sandrasagra  in 1979 . The app in 1979 . There ic myxoma . Their rc myxoma –31.Patients must continue to be followed with serial examinations for early detection of recurrent disease. There is no standard protocol for surveillance; however, it has been our practice to perform an annual physical examination and complete a TEE at that time. The presented patient has been followed for 5 years and has had no sign of recurrence and had normal functioning valves on serial TEE examinations.Although mitral valve myxoma is a rare clinical entity, the diagnosis should be considered in all young patients presenting with symptoms of stroke. These patients should undergo echocardiography early and prompt surgical resection must be undertaken once the diagnosis of mitral valve myxoma has been made. We present this review for further discussion of this rare cardiac neoplasm.


Introduction: The prognosis of metastatic colorectal cancer (mCRC) patients receiving multiple cytotoxic agents and targeted therapies (CATT) has improved, but a complete cure by CATT is still very rare.Presentation of case: We report the successful treatment of ascending colon cancer complicated by peritoneal disseminations (PDs) with panitumumab (Pmab) plus mFOLFOX6 therapy. A 67-year-old male patient was diagnosed with clinical stage IV cancer of the ascending colon with PDs, and underwent ileostomy. Eighteen courses of Pmab plus mFOLFOX6 caused remarkable tumor shrinkage and the disappearance of PDs on ECT. Laparotomy revealed tumor shrinkage and scarring at the PD sites. We performed right hemicolectomy, subtotal omentectomy, and ileostomy closure as curability B surgery. Seven months later, new PDs were detected by ECT so we resumed Pmab plus mFOLFOX6 therapy. After nine courses of treatment, the target lesion had completely disappeared. After a total of 20 courses, we changed to Pmab monotherapy as maintenance therapy because there was no recurrence. Forty months after the initiation of Pmab monotherapy, there has been no oncologic progression.Discussion: Pmab plus mFOLFOX6 treatment resulted in a complete response for PDs, which is extremely rare for CATT.Conclusion: We consider that Pmab therapy should be introduced for the treatment of mCRC complicated by PDs. As a result, patient prognosis has been improving, and the median survival time is now 20–30 months . However2A 67-year-old man was referred to our hospital to determine the cause of his diarrhea and nausea. Colonoscopy revealed a type II tumor located in the ascending colon. Histopathological findings of the biopsy specimen of the ascending colon tumor revealed well–moderately differentiated tubular adenocarcinoma. The K-ras status was wild-type and it was epidermal growth factor receptor (EGFR)-positive.Thoracic and abdominal enhanced computed tomography (ECT) showed the wall thickness of the ascending colon, ascites in the pelvic cavity and right upper abdomen, and suspected peritoneal disseminations (PDs) in the left upper abdomen a, b. Blo2; bolus injection of 5FU, 400 mg/m2; continuous injection of 5FU, 2400 mg/m2/48 h) every 2 weeks. After 18 courses of this chemotherapy, abdominal ECT showed that the tumor volume had shrunk to around 50% and PDs and ascites had disappeared  staining of the tumor revealed extensive fibrosis from the submucosa to subserosal tissue with ductal adenocarcinoma duct in the muscularis propria. Metastasis was confirmed in three out of 14 regional lymph nodes, although two of the three metastasic lymph nodes were burned-out by Pmab plus CAT treatment. HE staining of the omentum revealed almost burned-out lesions with some duct adenocarcinoma a–d. The 2; bolus injection of 5FU, 400 mg/m2; continuous injection of 5FU, 2400 mg/m2/48 h) for new PDs. After nine courses of this therapy, the target lesion had completely disappeared on ECT  or anti-EGFR antibodies. Pmab is one such anti-EGFR antibody, and Pmab plus CAT treatment previously resulted in a better prognosis for mCRC than CAT without target therapies. Indeed, anti-EGFR antibodies plus CAT tend to cause early tumor shrinkage which improves prognosis . The regAlthough CATT has been effective for prolonging life, very few patients have achieved a CR for mCRC ,11. Qi eNCCN guidelines recommend anti-EGFR antibody treatment as first line therapy for left-sided colon cancer but not for metastatic right-sided colon cancer . InsteadThe prognosis of CRC patients complicated by PDs is poor compared with other organ metastases such as those of the liver and lung. Moreover, PDs diminish the quality of life by ascites retention, malnutrition, and intestinal obstruction. Bevacizumab plus CAT was reported to be superior to cetuximab plus CAT for patients complicated by PDs as measured by progression-free survival (PFS) and overall survival (OS). Bevacizumab-based triplet chemotherapy was also superior to cetuximab-based triplet chemotherapy as measured by PFS  and OS , but not for patients without PDs  . Based o4We report a case of metastatic ascending colon cancer complicated by PDs that was successfully treated with the anti-EGFR antibody Pmab plus CAT. We consider that Pmab plus CAT could be introduced for mCRC complicated by PD.The authors declare that they have no conflict of interests.None.This paper was not a research study, so ethical approval not required.I got written informed consent from the patient for publication of this case report.KT made a substantial contribution to study conception, conducted a literature search, and drafted the manuscript. KT, NY, HH, and TY contributed to the acquisition of data. KT performed the operation. KT, NY, HH, TY, and KY reviewed the manuscript and gave final approval for publication. All authors read and approved the final manuscript.This literature is case report, not research study.The Guarantors of this manuscript are Katsuji Tokuhara and Prof. Kazuhiko Yoshioka.Not commissioned, externally peer-reviewed.


Hemangiomas are common benign vascular tumors that often present in childhood. Diagnosis is based on clinical history, physical examination, and, when unclear, assisted with ultrasound or MRI. While the majority are small, nonproblematic, and can be managed conservatively, some hemangiomas may be associated with underlying syndromes or concerning for visceral involvement. Symptomatic lesions may develop ulceration, bleeding, vision disturbances, functional limitations, or disfigurement. The ideal treatment for a symptomatic hemangioma is often multimodal and may vary depending on the size, location, and proximity to critical structures. Medical treatments include topical beta blockers, oral propranolol, or steroid injections. Surgical resection and laser therapies may be necessary to optimize long term outcomes. While most IHs are sporadic, some familial clustering does occur2. Other risk factors include first trimester bleeding, preeclampsia, prematurity, advanced maternal age, placental abnormalities, female gender, and low birth weight2.Infantile hemangiomas (IHs) are benign vascular tumors that are common in children. They are present in an estimated 5% of the population and are characterized by abnormal proliferation of endothelial cells and abnormal blood vessel structure3. IHs are absent, subtle, or very small at birth, become clinically evident around 1 month of age, and have rapid growth during the first 6 months of life. They stabilize in growth at around 7–12 months of age and subsequently, and uniquely, undergo involution in early childhood. The rate of involution varies from one hemangioma to another, even in the same patient with more than one. IHs express GLUT1, a receptor also found on placental blood vessels, which has led to a theory that they may be caused by abnormal implantation of progenitor cells disrupted from the placenta during fetal development4.Hemangiomas are a tumor subtype of vascular anomalies as classified by the International Society for the Study of Vascular Anomalies (ISSVA), shown in5. They are categorized as rapidly involuting CH (RICH), non-involuting CH (NICH), or partially involuting CH (PICH) depending on their clinical behavior. RICHs have been reported to develop transient mild-to-moderate thrombocytopenia and consumptive coagulopathy as they involute6. CHs do not exhibit GLUT1 staining7.Congenital hemangiomas (CHs) are present at birth at their maximal size and lack a growth phaseIHs are further named based on the depth of growth as superficial, deep, or mixed  or may exhibit reticular, abortive, or minimal growth. IHs can also be focal, multifocal, or segmental based on their anatomic distribution. Focal, or localized, hemangiomas are discrete, solitary lesions with well-defined borders. Multifocal hemangiomas are discrete lesions that occur at multiple sites. Segmental hemangiomas are larger plaque-like lesions that may follow dermatomal distributions, often develop ulcerative complications, and demonstrate prolonged proliferation and involution phases. They are also associated with syndromes that require additional work-up.A thorough clinical history and physical examination leads to the accurate diagnosis of hemangiomas. Key aspects of the history include onset, timing, and progression of lesion growth, ulceration, bleeding, vision disturbance, and impact on function. Routine imaging is not necessary but can be useful in deeper lesions and diagnostic challenges.8. MRI can be used to provide further information regarding the extent of involvement in local tissue for deep disease. IHs are hyperintense on T2 and isointense on T1, with intense post-contrast enhancement on MRI. RICH and NICH may have more variability and heterogeneity in their imaging characteristics compared to IH on both ultrasound and MRI8.Ultrasound of IH is inexpensive, fast, and convenient. IH will demonstrate a well-defined mass with high vessel density, without abnormality of the surrounding fat, with uniform, pulsatile, fast-flow vascularity on Doppler with arterial and venous waveforms9. Multifocal and diffuse hepatic hemangiomas can lead to serious and life-threatening complications, so early identification is important and, when present, examination of thyroid function should be considered.Imaging with a screening abdominal ultrasound is recommended for children with five or more multifocal CHs because of an increased risk of concurrent liver hemangioma10. Ultrasound is useful to confirm diagnosis and is often sufficient as a singular imaging modality. MRI or immunohistology can be used if diagnostic uncertainty remains10.While superficial cutaneous lesions can be easily diagnosed on exam and history, deep hemangiomas may pose more of a challenge. Parotid hemangiomas are the most common parotid neoplasm in children and can present in patients younger than 4 months of age with a deep palpable cheek mass without cutaneous manifestations11. PHACE syndrome, the constellation of posterior fossa anomalies, hemangioma, arterial lesions, cardiac abnormalities or coarctation of the aorta, and eye abnormalities, is associated with large segmental IH of the face, scalp, and neck12. Complete screening should be considered in patients with large segmental hemangiomas of the head or central trunk. Patients with PHACE may be at increased risk of arterial ischemic stroke depending on their individual vascular anatomy13. Screening for PHACE should include physical exam, echocardiogram, MRI/MRA of the head and neck, and an ophthalmology exam12. LUMBAR syndrome is a rare grouping of lower body IH, urogenital anomalies, ulceration, myelopathy, bony deformities, anorectal malformations, arterial anomalies, and renal anomalies14. This also is described using the acronyms SACRAL, for spinal dysraphism, anogenital anomalies, cutaneous anomalies, renal anomalies, and lumbar hemangioma, or PELVIS, for perineal hemangioma, external genital malformation, lipomyelomeningocele, vesicorenal abnormalities, imperforate anus, and skin tag16.Rarely, hemangiomas can be associated with other congenital anomalies. Segmental hemangiomas in a beard distribution over the chin and neck have increased risk (60%) of concurrent subglottic hemangiomaOwing to their natural tendency towards involution, most inconspicuous IHs can be managed with observation alone. IHs that require intervention include those that become symptomatic during the growth phase, with ulceration, bleeding, vision disturbance, and functional limitations (breathing), or when imminent disfigurement is expected. Historic and current treatment options include medical therapy (steroids/propranolol), surgical resection, laser therapy, or direct intralesional steroid injections. A combination of more than one treatment modality is often performed in larger or resistant lesions.5. This is generally well-tolerated but may be associated with sleep disturbance, diarrhea, bronchial hyper-reactivity, and hypoglycemia. Dosing with feeds is recommended. It is contraindicated in patients with bradycardia, heart block, hypotension, and asthma5. EKG prior to starting therapy should be considered. Infants younger than 8 weeks of age should be admitted for inpatient initiation of treatment for close monitoring17. Early treatment during the proliferative phase is thought to be associated with improved outcomes18. Over 90% of IHs respond to propranolol with reduction in size and color. Deeper components respond better than superficial ones. Residual disease may require laser or intralesional therapy or surgical interventions. Topical timolol is an alternative option to oral propranolol for smaller lesions and has shown promise in halting proliferation and inducing early involution when used for thin, superficial IH19.Propranolol is the mainstay of treatment for large or symptomatic IH, including subglottic and parotid disease. Typical dosing is 1–3 mg/kg/day divided into two to three doses5. Typical dosing is prednisone at 2–3 mg/kg daily and monitoring for side effects including adrenal axis suppression, cushingoid facies, irritability, and stomach irritation is recommended. For localized IH, intralesional steroid injection can be considered and can be used in conjunction with the favorable responses obtained with propranolol. Sirolimus may be a last-resort treatment in refractory cases.Corticosteroid therapy is an alternative option for patients who have contraindications or inadequate response to propranolol20.Laser therapy is a treatment option typically used for persistent telangiectasia or residual lesions during or after hemangioma involution. Pulse dye laser is commonly used owing to its preferential absorption by hemoglobin; Nd:YAG is sometimes also used in lesions with significant venous drainage. Laser treatments during the growth phase of IH are controversial and generally not pursued because of the possibility of blistering, ulceration, and long-term pigmentation changes21. Scarring from ulcerative hemangiomas often requires revision in combination with resection of residual disease. Delaying surgery until after involution allows for the excision of smaller and less vascular lesions. Hemangiomas with a significant vertical growth pattern are at higher risk of leaving undesirable fibrofatty residuum after involution, which can be addressed with surgical excision22. This is particularly common in scalp IHs, which may develop alopecia during the involution phase and are easily excised with primary closure23.Surgical excision is reserved for ulcerative, bleeding, and significantly protruding hemangiomas. This can be performed alone or in combination with other treatments, especially when response to other treatments is limited or ineffective24.Hepatic hemangioma may require multimodal therapy, including treatment with steroids, propranolol, and embolization. Propranolol is currently favored owing to its low risk and high response rate. While not every hepatic hemangioma needs treatment, screening in these high-risk children has been shown to significantly reduce the risk of congestive heart failure, hypothyroidism, abdominal compartment syndrome, and mortalityHemangiomas are congenital vascular lesions divided into congenital and infantile subtypes. CHs are rare and fully present at birth; usually treatment is not required. IHs are common childhood tumors that undergo a characteristic pattern of proliferative growth phase followed by slow involution. Symptomatic IHs can be treated with low-dose beta-blocker medication, with the best results occurring from initiation of treatment in the early phase. Multimodal therapy may be required in some cases involving a combination of laser, intralesional, medical, and surgical interventions. Residuum from IHs can be managed with laser treatment or surgical excision when needed.


Spontaneous tumor lysis syndrome is a rare oncological emergency associated with multiorgan failure. It is characterized by an elevation of uric acid, hyperphosphatemia, hypocalcemia, hyperkalemia and renal failure in the setting of no active chemotherapy as a result of lysis of massive tumor burden. Early recognition of the disease and prompt management would affect morbidity and mortality. We present the case of an 80-year-old Caucasian male with a history of recently diagnosed diffuse large B-cell lymphoma who had worsening fatigue, weakness and decreased appetite for three days. On admission, laboratory investigations were significant for elevated creatinine, uric acid, and phosphorous. He was started on hemodialysis and rasburicase in view of hyperuricemia. Subsequently, chemotherapy was started. He tolerated chemotherapy initially but later developed multiorgan failure. His family then opted for comfort measures and the patient passed away soon after. In conclusion, spontaneous tumor lysis syndrome is a common association with hematological cancers. Prophylaxis with allopurinol and rasburicase is recommended in all patients who are at an increased risk for tumor lysis syndrome. Tumor lysis syndrome (TLS) is considered an emergency in the field of oncology. In 1929, Bedrna and Polcák reported the first case of TLS in patients with chronic leukemia who underwent irradiation .Hyperkalemia can lead to life-threatening arrhythmias. Hyperphosphatemia can lead to tetany and seizure precipitation due to secondary hypocalcemia. Hyperuricemia can lead to acute kidney injury. A systemic inflammatory response syndrome may result due to the release of cytokines when tumor lysis occurs. All these manifestations can eventually lead to multiple organ failure .Spontaneous tumor lysis syndrome (SPTLS) can be described when TLS is manifested in the absence of active chemotherapy . There iIt has been reported that SPTLS occurs in malignancies including Burkitt’s lymphoma , acute mWe report the presentation and management of spontaneous tumor lysis syndrome in a case of diffuse large B-cell lymphoma.An 80-year-old Caucasian male with a past medical history of hypertension and hyperthyroidism was evaluated for increasing fatigue and weakness with loss of appetite for three days.The patient had complaints of an enlarging left neck mass that started a couple of months ago. Computed tomography of the neck revealed the presence of an 8.7 x 6 cm soft tissue mass in the mid-left cervical region extending caudally in the left supraclavicular and infraclavicular regions Figure . He undeOn admission, his vital signs were a pulse of 83 beats per minute, respiratory rate of 24 breaths per minute, blood pressure of 122/63 mm of Hg, temperature of 36.3 °C and oxygen saturation of 100% on room air. Physical exam showed diffuse cervical lymphadenopathy with a left neck mass measuring 9 x 6 cm. Laboratory investigations were significant for elevated creatinine of 2.74 mg/dl (baseline of 0.7 mg/dl), uric acid of 15.9 mg/dl and phosphorous of 5 mg/dl with lactate dehydrogenase (LDH) of 800 U/l. His potassium levels were 3.7 mmol/l, well within the normal range. He was started on aggressive hydration and was given a dose of rasburicase with improvement in uric acid levels from 15.9 mg/dl to 6.0 mg/dl the following day. Despite aggressive resuscitation, he had a decrease in urine output and subsequently developed acute renal failure requiring hemodialysis. The trends of serum phosphorus  and acute renal failure that required immediate hemodialysis, even before the start of chemotherapy [ μmol/L aThe risk factors for SPTLS can be divided into the intrinsic nature of the tumor and extrinsic factors associated with the host. Tumor-related intrinsic factors include an increased proliferation rate  with a rIt has been proposed that SPTLS can present with hyperuricemia in the absence of hyperphosphatemia. The rapid growth of the tumor due to increased cellular proliferation results in a rise in the serum uric acid levels due to a rapid proliferation of nuclear proteins. However, the phosphorus that is released is reutilized for the formation of new tumor cells . On the In SPTLS, at times no electrolyte abnormality may be seen until overt TLS develops. Two separate theories have been suggested for such an acute occurrence, cells undergoing necrosis result in the release of intracellular contents that are then reabsorbed into the proliferating cells  and thatProphylactic therapy is crucial for TLS management Figure . CoiffieStudies have revealed an increased efficacy of rasburicase when compared to allopurinol for the treatment of hyperuricemia in patients with hematologic cancers. Coiffier et al. conducted a study which revealed that initiation of rasburicase to control hyperuricemia helped in the prevention of TLS, in patients who were diagnosed with aggressive non-Hodgkin's lymphomas .Thus, in conclusion, SPTLS occurs rarely but has fatal consequences. Clinicians should be wary about the intrinsic and extrinsic risk factors with special attention to malignancies with a high proliferation rate. Our case reiterates the need for early recognition of this syndrome and initiation of appropriate therapy.


Various typical and atypical imaging findings for pulmonary sarcoidosis have been described in the literature. Ground-glass opacities are one of the atypical manifestations, reported as diffuse or patchy ill-defined opacities frequently associated with additional findings and interstitial nodules. We performed a literature review to determine if our case had previously been described. The literature describes cases of mass-like consolidations, but there are no reports of mass-like ground-glass opacities. The appearance of the ground-glass opacities in our case is unique, appearing as discrete well-defined mass-like ground-glass opacities in a peribronchovascular distribution without additional parenchymal findings typically seen in sarcoidosis. Sarcoidosis can present with a myriad of imaging findings in the chest. Typical and atypical manifestations have been described within the literature. Finding ground-glass opacities within the lungs on imaging is one of the atypical imaging features. These ground-glass opacities are typically diffuse or patchy and ill-defined, and frequently associated with additional imaging findings such as interstitial nodules , 2. GrouAn African American woman in her mid-30s presented to the emergency room with neurologic-type symptoms. Initial work-up included a chest X-ray which demonstrated multiple bilateral pulmonary masses and right paratracheal lymphadenopathy . FurtherThe patient did not report any significant respiratory symptoms. An extensive work-up was performed as the clinical differential included inflammatory, infectious, vasculitic, embolic, and neoplastic etiologies. Rheumatologic, serologic, and infectious work-up were negative. Her angiotensin converting enzyme (ACE) was elevated at 57 (reference range 8–52 U/L).She went on to have bronchoscopy. Cultures from bronchoalveolar lavage were negative for bacterial, fungal, mycobacterial, and viral etiologies. There was no evidence of malignancy. Fine-needle aspiration of an enlarged right paratracheal lymph node and biopsy of the right lower lobe  both revGiven the histopathologic findings with the elevated ACE level, the diagnosis was consistent with sarcoidosis and she was started on treatment with corticosteroids.To the best of our knowledge, this is the first case reporting mass-like ground-glass opacities in a peribronchovascular distribution as a presentation of sarcoidosis. Typical manifestations of thoracic sarcoidosis include bilateral symmetric hilar lymphadenopathy and interstitial lung disease . AtypicaWhen presented with the thoracic findings of mass-like ground-glass opacities in a peribronchovascular distribution and lymphadenopathy, one may include sarcoidosis in the differential diagnosis if provided with the appropriate clinical picture.


DEAR EDITOR1-3 We present a two year old child with a 3×3 centimeters mass over the nasal dorsum. As her parents said, the mass was present from her birth and had constant size and characteristics from then. Previously they had been referred to some physicians and had been diagnosed as hemangioma. Congenital midline nasal masses, although rare, have some differential diagnoses. Dermoidor epidermoid tumors, encephaloceles, vascular anomalies, and neuroglial heterotopia are the most common diagnoses. Nasal neuroglial heterotopia  is a rare benign congenital lesion which can be at intranasal, extranasal, or mixed anatomic location.With such a diagnosis and because of its size and location and probable future visual problems oral corticosteroids had been used, which was ineffective . She wasSmall defect in right nasal bone below the mass was also detected . We deci1 One group of these lesions are nasal glial heterotopias, previously known as nasal glioma. This lesion was first described in 18522 and a total number of about 250 cases had been reported up to 2001.3 In 1950 by presenting two case of nasal glioma, Black and Smith defined nasal glioma as a mass composed of glial tissue at or near nasal root which may be connected to brain by a pedicle of same tissue and there was no fluid filled space within the mass.4Congenital midline front nasal lesions are very rare benign lesions with an incidence of one in 20000 to 40000 births.5 Nasal glial heterotopia can be seen in different anatomic locations. Sixty percent are extra nasal, 30% are intranasal , and 10% are mixed.6 A fibrous band connecting them to the intracranial space was seen in 15-20% of cases.5 They can cause problems, especially the intranasal mass by its obstructing effect. The extranasal mass except its visibility and aesthetic concerns, are usually asymptomatic, although rare cases of visual problems has been reported.7The exact pathogenesis is not known and there are different theories for development of these lesions. Inappropriate closure of the anterior neuropore, ectopic neural tissue cells, and encephaloceles with lost intracranial connection, are some of these theories.8 Complete excision of the mass is the treatment of choice and inadequate resection may cause recurrence in 4-10% of cases.9 Intranasal lesions resection by a transnasal endoscopic approach is the treatment of choice. Extranasal glial heterotopias can be treated by external rhinoplasty approach, or by lateral or medial rhinotomy.10 Although congenital nasofrontal masses are rare lesions and nasal glial heterotopias are only a small part of this category, appropriate evaluation can lead to the correct diagnosis and help in choosing the best treatment option. These masses can simply get resected and medical therapies such as systemic corticosteroids should be avoided.Preoperative para clinical evaluations are necessary for more reliable diagnosis and better finding the probable lesions’ extensions. Vascular anomalies may be identified by Doppler ultrasonography scan. CT scan and/or MRI should be used, although MRI seems to be the imaging of choice.The authors declare no conflict of interest.


We report a rare case of sudden bilateral vision loss due to third ventricular cavernous angioma with intratumoral hemorrhage.A 45-year-old woman presented decreased visual acuity in both eyes. Her best corrected visual acuity was 0.1 in the right eye and 0.15 in the left eye. Goldmann perimetry showed bilateral central scotomas and bitemporal visual field defects. MRI demonstrated a lesion with mixed hypo- and hyperintensity at the optic chiasm, which was thought to be an intratumoral hemorrhage. The patient underwent bifrontal craniotomy. The tumor was exposed via an anterior interhemispheric approach, and histological evaluation of the mass led to a diagnosis of cavernous angioma. Six months after the surgery, her best corrected visual acuity was 0.9 in the right eye and 0.9 in the left, with slight bitemporal visual field defects..Third ventricular cavernous angioma is considered in the differential diagnosis of chiasmal syndrome. Contrast-enhanced MRI and FDG-PET might be useful for differential diagnosis of cavernous angioma from other chiasmal tumors including glioblastoma Chiasmal syndrome is associated with lesions of the optic chiasm, manifesting as various impairments of the visual field such as central scotoma, bitemporal paracentral scotoma, junction scotoma and bitemporal hemianopsia . PituitaWe report a rare case of chiasmal syndrome with sudden bilateral vision loss due to third ventricular cavernous angioma presenting with intratumoral hemorrhage.A 45-year-old woman presented decreased visual acuity in both eyes for three days. She visited an ophthalmic clinic where she was diagnosed as having bilateral optic neuritis with central scotoma and was referred to our hospital. A week earlier, she had complained of headaches and visited a neurologist, but no abnormality was found on brain magnetic resonance imaging (MRI) and neurological tests. She had no history of ocular or systemic disease.in both eyes. Slit-lamp examination revealed no signs of ocular disease. Fundus examination was normal. Goldmann perimetry showed bilateral central scotomas and bitemporal visual field defects  performed for differential diagnostic purposes did not show an increased FDG accumulation in the tumor. Her visual acuity had spontaneously improved to 0.9 in the right eye and 0.8 in the left with a disappearance of bilateral central scotoma since her first visit to our clinic. Spinal fluid examination showed laboratory data within normal limits. Pituitary function test showed that TSH was high, at 13.0μIU/ml, but other values were within normal limits.Despite spontaneous improvement in visual acuity, the patient underwent bifrontal craniotomy to prevent rebleeding 48 days after onset, and the tumor was exposed via an anterior interhemispheric approach. A yellow mass, developed from the anterior wall of the third ventricle to the lamina terminalis on the chiasm, was seen intraoperatively Fig. a. After Six months after the surgery, her best corrected visual acuity was 0.9 in the right eye and 0.9 in the left, with slight bitemporal visual field defects Fig. .Fig. 4GCavernous angioma (CA) of the central nervous system accounts for 8 to 15% of cerebrovascular malformations in postmortem studies –8, with CAs located at the FoM were the most likely to cause hydrocephalus (83.3%) followed by CAs located at the pineal region/posterior third ventricle (80%). On the other hand, CAs located in the suprachiasmatic region were most likely to cause endocrine dysfunction (33%), because of the close to the hypothalamus. Visual disturbances were also seen in CAs located in the suprachiasmatic region (100%).Our patient also developed headaches and presented increasing TSH. As mentioned, suprachiasmatic CAs sometimes cause endocrine dysfunction, because they are closed to hypothalamus which controls the pituitary gland. Further, the hemorrhage in the CA had compressed the chiasm and caused sudden bilateral vision loss in this case. The hemorrhage was absorbed, which may explain the spontaneous improvement in visual function before the surgery. A important issue with CAs is their potential to bleed –8. CA ma. These findings are different from previous reports of malignant tumors including glioblastoma. Because of these preoperative imaging results and the spontaneous visual recovery before the operation, we supposed the lesion might be CA rather than glioblastoma. Although MRI could not distinguish the tumor from the chiasm, they were actually separable at the surgery and her visual function recovered further after the surgery.In this present case, MRI detected mixed hypo- and hyperintensity at the chiasm, so the sudden vision loss of both eyes would have been cause by chiasmal compression due to acute intratumoral hemorrhage. Previous cases have shown that intratumoral hemorrhage is a common finding in glioblastoma . In this.Third ventricular CA is considered in the differential diagnosis of chiasmal syndrome. Contrast-enhanced MRI and FDG-PET might be useful for differential diagnosis of CA from other chiasmal tumors including glioblastoma


Streptococcus thoraltensis has mainly been reported to cause infections in animals. Its clinical significance as a human pathogen has not yet been fully elucidated and needs further investigation. We describe here a case of bacteremia attributed to S. thoraltensis in a 55-year-old female patient admitted to our department due to fever of unknown origin. To the best of our knowledge, this is the first reported case of S. thoraltensis bacteremia in a human and the first reported case of S. thoraltensis as a cause of fever of unknown origin in human. Streptococcus thoraltensis is a recently described species that has been isolated from several animal species [S. thoraltensis as a predominant colonizing isolate in the nasal cavity and oropharynx in 29 fuel workers [S. thoraltensis isolation from both the maternal placenta and newborn tracheal aspirate cultures and four cases from the subgingival plaque in patients undergoing tooth extraction have been reported in the literature [ species . Accorditerature –5.A 55-year-old female patient with past medical history of osteoporosis and hyperlipidemia was admitted to our department for a 2-month history of high-grade fever, up to 39°C, mainly occurring in the evening, which was responding to acetaminophen. She denied night sweats, anorexia, fatigue, weight loss, chest pain, shortness of breath, other joint pain, abdominal pain, recent travel, or a family history of autoimmune disease, although she reported traumatic dental procedure 6 months prior to admission.μL platelets, white blood cell count of 4.3 × 109 L with neutrophils as the predominant type, and mild normocytic anemia . The C-reactive protein (CRP) was 1.43 mg/dL (nl: <0.5), the erythrocyte sedimentation rate (ESR) was 32 mm/h, and creatine phosphokinase (CPK) was also elevated as 335 U/L (reference range: <180 U/L).On presentation, her vital signs revealed a temperature of 36.7°C, heart rate of 74 bpm, and blood pressure of 100/70 mmHg. Her physical examination was unremarkable, except for a systolic heart murmur, heard best over the aortic, pulmonic, and mitral area with radiation into the neck. Laboratory studies revealed thrombocytopenia with 123.000 Streptococcus thoraltensis was identified, while infection caused by Brucella, Mycobacterium tuberculosis, Coxiella, and Leishmania was excluded. The suspicion of an infective endocarditis (IE) was raised, and a transthoracic and transesophageal echo was performed. However, there was no evidence of IE, and only mild aortic stenosis was revealed. A panoramic X-ray showed no lesions. Additionally, as part of the diagnostic procedure, a chest, abdomen, and pelvis computed tomography (CT) scan was performed and revealed no abscesses and masses.From three blood cultures obtained on the 1st, 2nd, and 3rd day of her hospitalization, when she was febrile, Due to the fact that the patient continued to have high-grade fevers and the systolic heart murmur, empirical antibiotic treatment with ampicillin/sulbactam and gentamicin was initiated on day 4. Susceptibility testing was performed on the initial isolate and revealed intermediate sensitivity to these agents, as determined by the E-test method . The patS. thoraltensis isolation from human samples. To the best of our knowledge, this is the first reported case of S. thoraltensis bacteremia.We searched the literature including PubMed and individual references for publications of review articles, single cases, or case series with the following keywords: “Streptococcus thoraltensis” and “human.” There have been documented only five cases of S. thoraltensis from three individual cultures presents a strong case to implicate this organism.A significant limitation to this report is the method used to identify the pathogen due to the fact that the automated VITEK 2 system lacks the specificity of the 16S rRNA gene sequence, although a proper gold standard for the identification of streptococci is not available . NeverthS. thoraltensis cases and the unknown proper duration of treatment in this isolate's bacteremia. Regarding the source of the infection, it remains unknown, considering the long period between the dental procedure and the initiation of her symptoms (>4 months). Hence, her bacteremia can be considered community acquired of unknown origin.The characteristic of our patient's infection was high fever mainly occurring in the evening and relatively low levels of inflammation markers which nevertheless were decreased after the treatment initiation. Although our patient had a good clinical outcome, the case was clinically challenging regarding the lack of S. thoraltensis has mainly been reported to cause infections in animals. Its clinical significance as a human pathogen has not yet been fully elucidated and needs further investigation. Whether this case represents an isolated and rare bacteremia or an emerging infection will be established by novel possible cases.


Undifferentiated high‐grade pleomorphic sarcoma is a slow‐growing tumor rarely localized in the head and neck region. The treatment of UHPS should be based on large surgical resections in free margins associated with neck dissection. Postoperative radiotherapy improves local control of the disease and the prognosis quod vitam. The prePhysical examination revealed a solid mass with a maximum diameter of 5 cm without palpable regional lymph nodes and Grade 3 facial palsy according to the House‐Brackmann classification. Neck MRI showed a soft tissue mass of the parotid gland measuring 3.8 × 4.3 cm. The lesion infiltrated the residual part of salivary gland, the common branch of the facial nerve, the skin of the face, and the masseter muscle. Total body CT excluded macroscopic distant metastases.A radical parotidectomy with sacrifice of the facial nerve, extended to the skin and masseter muscle, and selective neck dissection (level Ib‐II‐III) were performed. Reconstruction of the facial region was performed using a radial fasciocutaneous free flap. No primary reconstruction of facial nerve was performed considering the poor prognosis and advanced age of the patient.The surgical specimen showed an ulcerated 5 cm large neoplasm. Soft tissues were infiltrated with a thickness of 1.5 cm. The tumor was composed of short interlacing bundles of large spindle and round cells exhibiting severe nuclear pleomorphism with scattered anaplastic cells and frequent mitoses (more than 10 mitoses/10 HPF). The tumor extended into the parotid gland, subcutaneous fat, and skeletal muscle with infiltrating borders. At immunohistochemistry, tumor cells were positive for vimentin and CD68 (both KP1 and PG‐M1 clones). Desmin, human caldesmon, smooth muscle actin, S100 protein, and cytokeratins (clone AE1/AE3) were negative Figure . SurgicaThe postoperative course was uneventful. Adjuvant radiotherapy was administered on the facial region starting 4 weeks after surgery for a total dosage of 60 Gy. The patient is free of disease 5 years later Figure . Informe3..Undifferentiated high‐grade pleomorphic sarcoma is a soft tissue sarcoma without a definable line of differentiation. It was first described by O'Brien and Stout with the name of malignant fibrous xanthoma.The etiology of UHPS is unknown but genetic background, previous radiotherapy, and traumatic burn injuries have been reported to be involved in the pathogenesis of this malignant tumor.The management of UHPS of the head and neck is guided by stage, location, size, and patient age.MRI is essential for preoperative staging and surgical planning, while total body CT or PET‐CT can be used to rule out distant metastases.In the present case, an incisional biopsy was performed because the lesion had already ulcerated the skin. The procedure was carried out after MRI evaluation to obtain a tissue core and not only superficial tumor. Although in the literature incisional biopsy is reported to have an accuracy of 94% with no false positive diagnoses for UHPS,Until now, a standard therapeutic strategy has not been established, although surgical excision is usually considered the mainstay of treatment.Modern reconstruction surgery with microvascular free flaps enables wider excisions with good functionality and aesthetic results. Microvascular reconstruction in the elderly can be performed with a high rate of success. A recent review of the literature showed that there is no difference in terms of free flap success, surgical complications, and mortality rate between older and younger patients.In this case, a latissimus dorsi free flap harvesting also the thoracodorsal nerve, which would have permitted to reconstruct the sacrificed facial nerve, could have been used for the reconstruction of the wide defect of parotid region. We preferred, however, to use as fasciocutaneus flap, a radial free flap in order to reconstruct the soft tissue defect without considering the reconstruction of facial nerve. The main reason for this choice was the possibility of working with a double team (demolitive/reconstructive) without the need to change the patient's surgical position therefore reducing duration of surgery. Furthermore, we choose not to proceed with the simultaneous reconstruction of the facial nerve considering the age of the patient and the severe prognosis of the lesion.As an alternative to a simultaneous facial nerve reconstruction, a static suspension of the soft tissue of the middle third of the face and a static technique to help the eyelid closure is recommended in literature. This procedure was initially considered in this case but it was not performed because the patient's hemodynamic conditions did not allow the anesthesiological time to be further prolonged.Adjuvant radiation therapy is generally recommended if margins are positive or close, if the lesion is high‐grade, in large tumors (more than 5 cm of diameter), or in the presence of metastatic lymph nodes.The use of chemotherapy and/or radiotherapy as the primary treatment modality has been less successful; chemoresistance is not uncommon, and at present, there is no specific trial showing significant improvement of survival after adjuvant systemic chemotherapy.(9). Positive or close surgical margins, after excision, correlate with an increased local recurrence rate and reduce overall survival.Overall survival rates for UHPS range from 30% to 74%4Undifferentiated high‐grade pleomorphic sarcoma is a slow‐growing tumor rarely localized in the head and neck region with potential nodal and distant metastases at the time of diagnosis. No specific guidelines for the management of this tumor have been established, but surgical excision with free margins associated with neck dissection in advanced cases in a multimodality regimen that currently represents the best chance of disease control. Postoperative radiotherapy improves local control, although neo‐adjuvant chemotherapy has been less investigated because it is limited by systemic toxicity. Free flap reconstruction makes more extensive resection possible and may improve local tumor control, while providing acceptable cosmetic and functional results.None declared.DT, MS, LORdZ, AZ: wrote this article.DC, MS and DT: made operation.


Introduction: With the rising worldwide obesity epidemic, bariatric surgeries are gaining popularity as the most effective modality for achieving long term results in weight loss and reducing its metabolic sequels.Case presentation: A 36-year-old female presented with a sudden onset of severe abdominal pain for 6 h. The patient underwent Mini gastric bypass (MGB) 2 years prior to presentation. Computed tomography (CT) scan revealed a 14-cm dilatation of the cecum occupying the left upper quadrant of the abdomen with the swirling appearance of the mesentery. These findings were confirmed through emergency exploratory laparotomy to be cecal volvulus. a right hemicolectomy and conversion of MGB to a conventional Roux-en-Y gastric bypass.Discussion: a modification of the classical gastric bypass surgery the MGB considered by its advocates to be a safer, simpler, and more effective option. However, serious complications can take place especially in the early learning curve of the procedure.We present a unexpected acute in top of chronic cecal volvulus in patient two years post MGB. The uncommon serious complication should be considered one of the differential diagnosis of acute surgical abdomen in those patients. Single The presented case details a patient with cecal volvulus and an internal hernia post MGB procedure who presented with acute abdominal pain to the emergency department of King Fahad Hospital of the University in Khobar, Saudi Arabia. Written consent was obtained from the patient for publication of this case report. Our case has been reported in line with the SCARE criteria .2A 36-year-old female presented to the emergency department two months post caesarean section with a sudden onset of severe abdominal pain for 6 h. The pain was associated with two episodes of vomiting, abdominal distension and constipation. The patient underwent MGB 2 years prior to presentation. In addition, she was complaining of severe dyspepsia and unsatisfactory weight loss following her MGB. She has no known chronic medical illnesses. She had undergone open cholecystectomy 12 years ago.On physical examination, she was afebrile, tachycardic with normal blood pressure. Abdominal examination showed right-sided abdominal fullness and tenderness and empty rectum on digital rectal examination.Radiological studies including abdominal X-ray  and comp3With bariatric surgeries being widely performed, the incidence of complications related to these procedures continues to rise. Bowel obstruction is a life threatening complication that may occur with bariatric surgeries . The difAlthough internal hernias are very rare, Petersen’s hernia (PH) accounts for more than 50% of the cases . PH occuAbdominal pain was the main clinical presentation in this case of cecal volvulus, which can be either chronic constant or intermittent upper quadrant pain as reported in a case series . In compUpon reviewing reported gastric bypass cases, CT was found to be the preferred modality to investigate abdominal pain and suspected bowel obstruction, and radiological findings were sufficient to confirm the presence of midgut malrotation ,18. SurpCecal volvulus could present in a considerably critical stage, when emergency surgical intervention is needed. When proceeding to exploratory laparotomy, the type of surgical management is dependent on the degree of ischemia and bowel gangrene. Resection and anastomosis, the surgical treatment of gangrenous bowel, is also equally recommended in viable bowel since it is associated with less recurrence rate . CecopexIn some cases, laparoscopic cecopexy has been sufficient for the treatment of non-gangrenous cecal volvulus post RYGB . If PeteIn conclusion, differential diagnoses of bowel obstruction after bariatric surgery should remain wide with the rising number of cases performed. Cecal volvulus should be considered as a potential cause of abdominal pain post-MGB that warrants urgent diagnosis and management to avail fatal sequels.None.This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors.Case reports are exempted from ethical approval according to policies of Imam Abdulrahman Bin Faisal University.Written informed consent was obtained from the patient for publication of this case report.Hanan AlGhamdi: design the study, data collection, writing the manuscript, performed the operation, reviewing the final manuscript of the case report, final approval.Wafa Aldhafeeri: study design, data collection, reviewing.Noor Allababidi: study design, data collection, writing the paper.Shadi Alshammary: study concept, reviewing article, correction and editing of the case report.Haitham Lardhi: study concept, reviewing article, correction and editing of the case report.N/A.Dr. Hanan Alghamdi.Not commissioned externally peer reviewed.


Langerhans cell histiocytosis previously known as histiocytosis X is a rare disease of children and young adults with a very broad clinical spectrum. In children, its annual incidence is estimated between 0.2–0.5 per 100,000.An 8-year-old Moroccan girl with no known personal or family history presented to our institution with painful swelling of both forearms. An X-ray and magnetic resonance imaging were inconclusive. We then performed a biopsy curettage (of her left forearm). Microscopic analysis followed by immunohistochemical analysis disclosed a diagnosis of Langerhans cell histiocytosis. No chemotherapy was necessary. Clinical and radiological improvement was achieved after 6 months.The particularity of this observation is the bilaterality of the lesion on both forearms and it has not previously been reported. Langerhans cell histiocytosis should be included in the differential diagnosis of osteomyelitis and Ewing’s sarcoma. Langerhans cell histiocytosis (LCH) is a rare disorder characterized by a proliferation of cells causing local or systemic effects . LCH incAn 8-year-old Moroccan girl presented to our institution with painful swelling of both forearms which initially appeared on her left forearm and 6 months later on her right forearm. Her family history and medical history were unremarkable. Given the exacerbation of the pain she consulted a doctor who obtained plain radiographs and noted a lesion in both forearms. She was then referred to us for further evaluation. She reported that despite daily use of nonsteroidal anti-inflammatory medications and narcotic analgesics, the pain in her forearms continued to progress. On physical examination she had no fever and had a good general condition. She presented a swelling in the upper third of her right forearm and the upper two-thirds of her left forearm with inflammatory signs Fig. . LaboratLCH is a rare disease with an incidence of 0.2–0.5 per 100,000 children per year . The disLCH is a rare disease in children with a very broad clinical spectrum. The particularity of this observation is the bilaterality of the lesion on both forearms and it has not previously been reported to the best of our knowledge. LCH should be included in the differential diagnosis of osteomyelitis and Ewing’s sarcoma.


Not only diffuse large B‐cell lymphoma is a malignancy, but also is initially and orally diagnosed in early stages. However, it could be misdiagnosed with other oral pathologies. However yet, early diagnosis is still crucial for the prognosis, morbidity, and mortality in such cases. Additionally, whole‐body scanning with positron emission tomography/computed tomography should be performed for diagnosis and treatment process. Early diagnosis is crucial for the prognosis, morbidity, and mortality. Here, we report a patient with DLBCL presenting as the first sign of oral localization and its management and emphasize the importance of diagnostic biopsy.Hodgkin's lymphoma (HL) develops as a nodal disease whereas Non‐Hodgkin's Lymphoma (NHL) may be evolved extranodally. Salivary glands and gastrointestinal tract are another anatomic areas where NHL is seen. It is very unusual that this lesion will be seen orally and elsewhere in the body simultaneously.Diffuse large B‐cell lymphoma is the most common type of NHLs. It presents as a fast‐growing lesion/tumor, characteristically in lymph nodes, spleen, liver, bone marrow and rarely, in other organs.Primary malignant NHLs of mandible represent about 0.8% of all the tumors in this bone and about 0.6% of all malignant NHLs.Additionally, it is indicated to take a bone scan, after detection of clinical signs of osseous involvement through the mandible CT.22.1A 72‐year‐old Caucasian man was admitted to our department with a pain in the left posterior mandible and periodontal hyperplasia associated with the left mandibular second molar tooth. He also complained about the ongoing pain for 2 months and spontaneous hemorrhage within the lesion region.2.2According to his medical history, the patient had suffered from coronary angioplasty 6 years ago. He also suffered from malaise and fatigue for last 1 year and also inappetence for last 6 months.2.3Extraoral examination of the patient showed no visible swelling, tenderness or pus discharge. Skin color and temperature were normal. In the intraoral examination of the relevant region, oral hygiene level was not good and gingival tissue around the second molar was hyperplastic and had a tendency to spontaneous bleeding.2.4On the other hand, the panoramic radiograph (PANO) showed a radiolucent lesion with irregular margins located the periapical area and also extended coronally that led into the serious mobility of mandibular left second molar tooth Figure .2.5Considering the patient's medical history and after an exhaustively clinical and radiographical examination, we decided to perform an incisional biopsy under local anesthesia. As expected, the result of the pathological examination was peripheral giant cell granuloma (PGCG). We did not consider to take an initial photography before the surgical procedures, however, with regard to the pathological results, we decided to perform another surgery under local anesthesia 1 week later, including extraction of the tooth and a wide curettage of the lesion in the left posterior mandible. Despite the anesthetic procedures were performed properly and adequately, the patient was still suffering from pain but no severe hemorrhage during the curettage was observed from the surgical area. After pathological assessment of the second biopsy, the lesion was diagnosed as DLBCL Figure .2.6Even though the patient was relieved and healing was uneventful, we did several consultations and asked for PET/CT scan of entire body because of metastatic nature of DLBCL. After all of these scanning procedures, we doubted the patient may also have had DLBCL in his thyroid gland, gastric system, and prostate either.On the other hand, 2 weeks later from the second biopsy, our patient had satisfactory outcomes, such as; no pain, hemorrhage, or swelling in the operated area. He stated that he is totally relieved and he is ready for the prosthetic procedures.2.7Mandibular biopsy results showed sheets of medium to large lymphoid cells with hyperchromatic nuclei and scanty cytoplasm. These cells stained positive for CD3, CD20, LCA1, and LCA2 for Cyclin D1 . Ki67 proliferation index was %90. The tumor was also positive for MUM‐1 and BCL 2 and BCL 6  and he passed away within 2 years after diagnosis due to DLBCL.3Diffuse large B‐cell lymphoma is the most common type of NHL rating 30%‐40% of all cases.Swelling in mandible with or without pain is one of the nonspecific and frequently observed symptoms of extranodal lymphomas in mandible where neuropathic disorders, loose teeth, and cervical adenopathy are less common.B‐cell included concurrent granulomas of the NHLs are NOS; “not otherwise specified”, FCCL: “low‐grade lymphoma, follicular center cell lymphoma”, SLL; “small lymphocytic lymphoma”, NOS : “large cell lymphoma”, and other types of T‐cell NHL.LBCL development is about 30%‐40% of adult NHLs whose median age is the sixth decade. However, a wide range of age is likely as lesions may be seen in children either. LCLs’ nature is aggressive but fortunately curable with aggressive therapy.The follicular, small cleaved‐cell type are considered low grade with a 5‐year survival rate of 70%, however, DLC types of NHL are considered high grade and 5‐year survival of DLBCL is about 30%, as our patient passed away in the second year of diagnosis.It is challenging to detect primary DLBCL when it affects more than one organ of a human body. Even though it is rarely seen in the oral region, clinicians should keep in mind that differential diagnosis of these lesions is very difficult when other lesions are involved or related. However, early diagnosis is still crucial for the diagnosis, prognosis, morbidity, and mortality in such metastasis cases. Scanning whole‐body with PET/CT is highly recommended for diagnosis and treatment process.None declared.TY: Wrote the main part of the manuscript according to collected data, performed the surgical procedures and followed up with the patient. EK: Helped to data interpretation and manuscript evaluation, also helped to follow up with the patient. HO: Helped to evaluate and edit the manuscript. AA: Supervised development of work, helped in data interpretation and manuscript evaluation. KD: Performed histopathological evaluations and wrote “Pathological Assessments” part of the manuscript.


Objective: To report a case of congenital simple hamartoma of the retinal pigment epithelium (CSHRPE) with depigmentation at the margin.Method: Case report.Result: A 40-year-old Indian female was noted to have a small pigmented lesion with a depigmented margin toward the fovea in the right eye. The best-corrected vision was 6/6 in either eye. The optical coherence tomography revealed a highly reflective lesion at the retinal surface causing a shadow effect, suggestive of CSHRPE.Conclusion: Imaging features of a patient with CSHRPE with a crescent-like depigmented area at the margin are presented. However, these search results did not include the earliest description of the entity in 2 cases by Laqua [Congenital simple hamartoma of the retinal pigment epithelium (CSHRPE) is a rare benign pigmented lesion of the ocular fundus. A PubMed search on 21A 40-year-old Indian female presented to us for a routine check-up. The best corrected visual acuity was 6/6 and N6 in both eyes (+0.50/–2.00 x 90º in both eyes). The anterior segment was unremarkable in both eyes. Intraocular pressure was 12 mmHg in both eyes. The left fundus was normal. The right fundus showed a small pigmented flat lesion very near and superotemporal to the foveola. It had a crescent-like depigmented margin towards the foveola  revealed a minimally elevated highly reflective lesion at the retinal surface causing a shadow effect, suggestive of CSHRPE (Figure 2a Gass  defined The fluorescein angiogram shows hypofluorescence of the black lesion and hyperfluorescence at the depigmentation within the lesion  or at thMarginal depigmentation has been reported in case 2 of the series by Baskaran and colleagues  and caseIn conclusion, we describe a case of CSHRPE in an Indian female, who also had a depigmented crescentic area at the margin of the lesion.The authors declare that they have no competing interests.


Angiomyxomas are rare benign neoplasms of mesenchymal origin arising from the soft tissues of the perineum and pelvis.To our knowledge, we report the first case of ureteral angiomyxoma arising de novo in a fifty-four-year-old female patient who presented with macroscopic hematuria.The pathological findings, radiological features and operative management will be discussed.Angiomyxomas are notorious for their locally infiltrative nature and their propensity to recur. Clinical suspicion is capital to reach the diagnosis and a holistic care is key to a good outcome. Angiomyxomas are rare benign mesenchymal stromal tumors that are remarkably slow growing. These tumors have a myxoid pattern and contain prominent angiomatous components characterized by large thick walled vessels without arborization. Due to their aggressive infiltrative nature and frequent recurrence behavior they are referred to as aggressive angiomyxomas or AAMs. They are generally found in the pelvis of adult females [Steeper and Rosai  first de females .2We report a fifty-four-year-old overweight female patient (BMI 28.9) who came to the emergency department with a second episode of macroscopic hematuria. The first episode happened earlier after she fell on her back. Both episodes were spontaneously resolved after increasing oral intake of fluids. Clinical examination and blood work were unremarkable. She is known to have an idiopathic fascicular left ventricular tachycardia and a hypothyroidism that are well controlled by medication. The patient was referred to the urology outpatient clinic at our academic medical center with a uroCT (Computerized tomography) showing an obstructive worrisome lesion mimicking an upper tract urothelial carcinoma (UTUC) of the right pelvic ureter . Further3Initially, these tumors were described as occurring only in women, although the female to male ratio is now estimated to be 6:1 . The med4AAMs are rare benign neoplasms that are more predominant in females. Though rare, clinical suspicion is required in order to reach the diagnosis, and a multidisciplinary care is key to obtaining a good outcome. A careful primary surgical extirpation, as complete as technically feasible, should be the main primary surgical approach. The importance of pre-operative biopsy in this case should be mentioned. Local recurrence is common. The role of GnRH agonist treatment is unclear. There is no standard follow up regimen for AMMs though it should include an imaging modality.None.None.Not applicable. The study is exempt from ethical approval in our institution.Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal on request.Ahmed S. Zugail: Study concept, data collection and interpretation, writing the paper.Faris Baowaidan: Study concept, data collection and interpretation, writing the paper.Eva-Maria Comperat: Study concept, data collection and interpretation, writing the paper and supervision.Bertrand Guillonneau: Study concept, data collection and interpretation, writing the paper and supervision.Alexandre Colau: Study concept, data collection and interpretation, writing the paper and supervision.Not applicable.Ahmed S. Zugail.Not commissioned, externally peer reviewed.


The present case report describes imaging findings (CT and MRI features) of aprimary malignant fibrous hystiocitoma, presenting as a dual stage lesion,completely exophytic along liver surface with surgical and histologicalcorrelations. Imaging characteristics suggested the nature of the lesion and the behavior (expansile growth pattern) which addressedsurgeons to a conservative excision. We present a case of primary malignant fibrous hystiocitoma (MFH) arising from liverglisson capsule, surgically confirmed.To the best of our knowledge, this is the first reported case that shows MFH arisingfrom glisson capsule, with a growth pattern completely exophytic along liversurface.Imaging characteristics on CT and MRI suggested the encapsulated mesenchymalglissonian nature of the tumor, addressing the therapeutical conservative managementwith surgical excission of the mass. The resected specimen revealed a malignantfibrous hystiocitoma of 9 × 7 × 4 cm with a double component, as adual-stage lesion.A 70-year-old patient was admitted to our Institute for further examinations, afterincidental ultrasound finding of a huge lesion in gallbladder fossa of uncertainorigin.Patient was asymptomatic, laboratory tests as hepatitis B surface antigen andantibodies for the hepatitis C virus were negative, alpha-fetoprotein was withinnormal limits.Patient underwent CT examination with triphasic study, and then underwent MRIexamination after GD-DTPA e.v. administration.Both the exams showed a large encapsulated lesion, between gallbladder fossa, Vth andIVth liver segments with a peduncolated cranial nodule in the liver hilum.Nonenhanced CT scan showed a hypoattenuating lesion with different areas of markedhypoattenuation . ContrasT1 sequences, weakly hyperintense in T2, with areas of cystic changes after e.v. contrast administration.In regard to MRI characteristics ,lesion T2 and T1 sequences.Nodule in the liver hilum was conversely strongly hypointense both inNo lipidic areas were detected in the lesion on opposed phase and STIR images.According to imaging characteristics and laboratory markers radiologist suggesteddiagnosis of a huge liver encapsulated mesenchimal tumor arising from glissoncapsule. Interestingly this lesion showed a “two face” appearance, asthe peduncolated nodule in the hilum, seemed to be the same lesion in an earlierstage of evolution, due to a more conspicuous enhancement and homogeneoushypointensity signal respect to the bigger more heterogeneous lesion .Patient was candidated to surgery and a preoperative evaluation of the future liverremnant volume (FLRV) was performed, using the open-source OsiriX PAC softwaresystem with a stand-alone Apple computer. Liver volumes according to Couinaudsegmentation were manually performed, and the result showed sufficient remnant livervolume. Surgeons chose a conservative management consisting in the excision of thelonely lesions with a subsegmentectomy approach as they appeared encapsulated atimaging.At exploratory laparotomy the mass measured 9 × 7 × 4 centimeters,yellow in color, was tender, encapsulated and located next to gallbladder, with apeduncolated nodule in hepatic hilum . No otheHepatic subsegmentectomy of S5, S4b and cholecystectomy “en bloc” wasperformed.Microscopically the tumor revealed a high hypercellular proliferation of little andmedium cells with round or fusiform nucleus and infiltrative pattern of growth.Immunohistochemical study results showed that cells were strongly positive forvimentin, desmin and CD 34, negative for cytokeratin, LCA, CD 10, CD 31m actin,HMB-45 and S-100 . The patThe postoperative period was uneventful and the patient was discharged from thehospital 6 days after surgery. After surgery no chemotherapy was administered. Thepatient is alive and in good health, he received regular follow-up CT examinationsat our Institute for 18 months, laboratory tests as AST, ALT and alpha-fetoproteinat the last follow up were 14 (U/L), 16 (U/L) and 6 (ng/ml) respectively.1 Clinical presentation is variable as patients may present abdominal pain,nausea, vomiting and weight loss, due to the mass effect of these lesions thatusually are discovered when they reach a significant volume (mean diameter atdiagnosis ranges from 7 to 24 cm).2 Liver presentation is very unusual,3 generally hepatic MFH is discovered in middle aged patients (50–60years old) with a slight male predominance and prognosis is very poor, with a 2-yearsurvival rate approximately of 60%, with 20% suffering from local recurrence.4 Five histological subtypes have been described: storiform pleomorphic, giantcells, myxoid, inflammatory, and angiomatoid.7 Laboratory tests may be variable, some patients presented leukocytosis,abnormal liver transaminases and alkaline phosphatase, others may present laboratoryresults completely negative.4 This variable clinical presentation and biologic behavior often make accuratediagnosis really challenging. A variable spectrum of imaging appearances have indeedbeen described, however some characteristic imaging CT and MRI features may suggestthis difficult diagnosis. According to different studies8,9 liver MFH presents an inhomogeneous low density at precontrast CT, usuallywith heterogeneous enhancement because of necrotic areas at contrast-enhanced CT andMRI, in some cases a pseudocapsule of delayed enhancement may be depicted. Localrecurrence is a common finding and correlates with the depth and size of the primary tumor.9 No infiltrative behavior has been described with absence of portal veininvasion, bile duct obstruction or lymph node metastasis.8Li et al10 described four cases of inflammatory MFH of head and neck showing a similarpattern at imaging, with intense enhancement during arterial phase which persistedin the portal and delayed phases. At MRI primary hepatic MFH show an intermediatelow signal on 1T weighted images and heterogeneous high signal intensity onT2 weighed images.3 The imaging findings of our case were partially in accord with theliterature, indeed lesion was large and encapsulated, hypoattenuating at precontrastCT with heterogeneous enhancement in arterial phase, and slow centripetalenhancement in equilibrium phase. The cranial peduncolated nodule showed a moreconspicuous and homogeneous enhancement, with a little central cystic area. At MRIthe larger lesion was hypointese on T1 sequences, weakly hyperintense on T2, with areas of cystic changes after e.v. contrast administrationwhereas the nodule in the liver hilum was strongly hypointense both onT2 and T1 sequences. The marked hypointensity of the cranial nodule on2T weighted images has not yet been described in literature, and probably itis correlated to the high vascular matrix of the pedunculated lesion. Neither signsof infiltration of adjacent structures nor lymphadenopathies were detected. To thebest of our knowledge no other cases of liver MFH presenting as completely exophyticmass with a dual stage behavior have been discussed in literature.Malignant fibrous hystiocitoma represents a relatively ”young”pathological entity, as it was first described by Orien and Stout in 1964. It can beconsidered a sarcomatous lesion usually involving the deep fascia, extremities, or retroperitoneum.The present case illustrates CT and MRI findings of a liver primary MFHcompletely exophytic along liver capsule, imaging allowed the correctattribution of the mass to the liver thanks to the identification of thefeeding vessels.Contrast enhancement characteristics as MRI features correctly suggested themesenchymal nature of the lesion appearing as a dual stage tumor.At imaging the mass showed an expansile behavior, so surgeons chose aconservative subsegmentectomy approach according to CT evaluation of thefuture liver remnant volume (FLRV).


The great majority of cases of cluster headache (CH) are primary, but there are several reported cases of CH secondary to underlying structural lesions. The identification of these lesions is crucial for the achievement of an effective treatment and favorable outcome, although the determination of a cause-effect relationship between the two entities may be challenging. We present the first case of CH secondary to sphenoid sinus mucocele. This case reinforces the need to perform neuroimaging studies in CH patients in order to identify lesions that can constitute its cause, especially if atypical features are present. Activation of the trigeminovascular system due to direct contact between the lesion and the trigeminal nerve or by local edema and inflammation possibly plays a role in the pathophysiology of this CH secondary to sphenoid sinus mucocele. Although the great majority of cases of cluster headache (CH) are primary, a small number of patients present an underlying structural lesion . The disSphenoid sinus mucocele is a rare condition, comprising 1–2% of all paranasal sinuses mucoceles. Despite its benignity, it may displace or compress several vital contiguous neurological and vascular structures: cranial nerves II to VI, cavernous sinus, carotid artery, sphenopalatine artery and nerve, pterygoid canal and nerve, dura and pituitary gland .A 62-year-old man presented at the Neurology consultation with a six-week history of a severe, strictly left orbitotemporal headache, with a frequency of three attacks per week, occasionally more than one at the same day. Most of them occurred in the first half of the night, waking him up, and lasted between thirty minutes and one hour. He used to take ibuprofen as acute treatment, with unsatisfactory response, since he did not notice a significant difference between treated and untreated attacks in terms of duration and pain intensity. To relief the pain, he used to open the window to get some fresh air. The headache was always associated with ipsilateral conjunctival injection and lacrimation. Pain triggers were not identified by the patient. He had no personal or familial history of headaches. His medical history was remarkable for hypertension and asthma, with a past surgical history including septoplasty and bilateral middle turbinectomy and uncinectomy due to nasal respiratory insufficiency. By the time of medical evaluation he was asymptomatic and neurological exploration was unremarkable. The clinical picture was suggestive of a CH and the patient was medicated with verapamil 120 mg daily. A MRI scan was performed, which revealed a sphenoid sinus mucocele, without secure expansion of the sinus. Two weeks later the patient came to the Emergency Department with complaints of horizontal diplopia that he noted when he woke up in that morning. He maintained the headache attacks, with similar characteristics, despite prophylactic therapy. Neurological examination revealed left eye adduction palsy and ptosis. A brain CT scan was performed and excluded lesions other than the mucocele. Paranasal sinus MRI revealed molding of the medial wall of left cavernous sinus by the sphenoid mass . A paranThere are several reported cases of CH in the context of a structural lesion . HoweverThe clinical picture of our patient fulfilled the diagnostic criteria of cluster headache . The ageThe finding of a sphenoid sinus mucocele in the first MRI raised the suspicion of a secondary CH, but we could not establish an unequivocal cause-effect relationship between the two entities at that time. The development of an oculomotor palsy short after the onset of the headache, which was a direct complication of the sphenoid sinus lesion, implied an urgent surgical intervention, after which a sustained remission of the pain was observed, allowing the determination of the mucocele as the cause of the CH.The mechanism of pain and development of trigeminal-autonomic symptoms in CH is still controversial, but it is believed that the trigeminovascular system may play a role in its pathophysiology . In our This case shows that CH may be one of the multiple neurological manifestations of sphenoid sinus mucocele and reinforces the importance of neuroimaging studies in trigeminal-autonomic headaches. We suggest that, in the concomitant and ipsilaterally presence of these two entities, a cause-effect relationship should be strongly considered and surgical removal of the lesion should be performed as soon as possible, as it can be the only way to treat this disabling type of pain and prevent other serious neurological complications.