Pancreatitis and pseudo-pancreatic cysts are frequently encountered entities; however, intrahepatic pseudocysts presenting as large number of liver cysts with absence of overt signs of pancreatitis has never been reported in literature. Here, we report an interesting case of multiple intrahepatic pancreatic pseudocysts (MIHPPs), a challenging diagnosis to be kept in mind while dealing with complex cystic lesions of liver. Pseudocysts are common complication of pancreatitis, often these are located within the vicinity of the pancreas in the lesser sac and the retroperitoneum. Extra pancreatic location of these cysts within the liver is a diagnosis often missed, with only 50 odd cases reported in literature till date. Most of these reported cases are either subcapsular in location or limited in number to one or two lesions. Although rare, possibility of MIHPPs is an important diagnosis that should be kept in mind while considering list of differentials for complex cystic lesions of the liver even in the absence of overt signs of pancreatitis. Pseudocyst formation is a very common complication of acute pancreatitis. Most pseudocysts are located around the pancreatic gland, but they have also been described at a site distant from the pancreas; from the mediastinum to the scrotum, as the fluid dissects through tissue planes , 2. A raA 68-year-old male with no past history of hepato-biliary or pancreatic diseases came to the emergency room with non-radiating right upper abdominal pain since 10 days. He was chronic alcoholic since 15 years. Physical examination revealed moderate right hypochondrial pain and tenderness. No evidence of jaundice was present. The bowel sounds were normal. Abdominal ultrasound demonstrated multiple intrahepatic well defined lesions of variable size involving both lobes of liver with few appearing almost solid while few of them solid –cystic appearance with low level internal echoes and debris. No color flow was noted in the solid component. The liver was also enlarged in size measuring approximately 17cm. Multiple calculi were also seen in gallbladder. The pancreas appeared normal with no evidence of intra-peritoneal free fluid. Initial diagnosis of multiple hepatic abscess or hydatid cyst was conveyed to the referring physician. However, in absence of clinical history of fever and classical US findings of hydatid cyst, further evaluation with CECT abdomen was suggested. A computed tomography (CT) scan revealed multiple variable sized well-defined, homogeneous, fluid attenuating lesions involving both lobes of liver  and 2. A Laboratory investigations revealed 13,500 white blood cells/mm3 (75% neutrophils), normal hemoglobin, 870 IU/L serum amylase , 920 IU/L serum lipase , 320 U/L AST , 276U/L ALT  and 251mU/mL alkaline phosphatase .Once the echinococcal antigen test was negative, US guided aspiration of fluid was done from one of the cysts. The aspirate revealed a clear fluid with raised level of 1222 U/L amylase and 6000 U/L lipase thus confirming the diagnosis of MIHPPs. The larger cysts were aspirated under US guidance while the smaller cysts were managed conservatively.Pancreatic pseudocysts, a well-known complication of pancreatitis has virtually been described in all organs of the body. However, intrahepatic location of pseudocyst has rarely been identified .Intrahepatic pseudocysts are usually single and most commonly involve the left lobe of liver, but multiple intrahepatic pseudocysts (MIHPPs) involving both lobes of liver have also been described -6.MIHPPs can be subcapsular or intraparenchymal in location. Two pathophysiological mechanisms have been proposed to explain formation of intrahepatic pseudocysts -10. AccoPoint in contention in our case is unusual appearance of the lesion on US, with lesion appearing as solid and solid - cystic on the background of normal looking pancreas causing diagnostic dilemma at initial evaluation and prompting further imaging work up of the lesion. Such a large number of IHPPs in a single case have never been previously described in literature.This atypical US appearance of the pseudocysts may be contributed due to debris making the US diagnosis of these lesions difficult. In acute settings, they may appear hyperdense on NCCT scans due to hemorrhage and debris within.Diagnosis of intrahepatic pseudocyst is a diagnostic challenge as it is not even considered in the differentials for cystic liver lesions in absence of pancreatic pathology. Definite diagnosis of MIHPPs can only be made if aspirate from the lesions reveal elevated amylase/lipase or direct communication can be demonstrated between intrahepatic pseudocysts and peripancreatic collection. ERCP also helps in confirming the diagnosis by demonstrating disrupted pancreatic duct with accumulation of contrast in intrahepatic collection.Management of intrahepatic pseudocyst tends to differ from pancreatic pseudocyst. No definitive guidelines have been provided regarding the management however USG guided percutaneous drainage is preferred over other options such as surgical resection or transpapillary stenting. Possibility of intrahepatic pseudocyst should always be kept in mind in case of acute or chronic pancreatitis presenting with atypical fluid collection in liver. Even in patients without overt signs of pancreatitis, MIHHPs should be kept in the list of differentials for multiple complex cystic lesions of liver, as the patient might have remote history of pancreatitis with complications manifesting at the time of presentation. However, confirmatory diagnosis can only be arrived at on demonstration of raised amylase levels in fluid aspirate or direct communication between cyst and peripancreatic fluid collection or pancreatic duct.The authors declare that they have no conflict of interest.


We report the case of a seventy-four-year-old man with a slow-growing 2 cm mass on the back that arose near the surgical scar of previously excised melanoma, invasive to a Breslow depth of 3 mm. Preoperative clinical diagnosis was “in-transit” melanoma metastasis. After surgical excision, histopathologic examination revealed a dermal nodular proliferation of spindle cells arranged in storiform pattern, with mild pleomorphism, infiltrating around appendages and into the subcutaneous tissue. Immunohistochemical investigation documented diffuse positivity for CD34 and vimentin of spindle cells. Scattered dendritic cells, containing dark pigment in varying proportion and positive for S100, Melan-A and HMB-45, were also observed. A final diagnosis of Bednar tumour was formulated. Subsequently, the patient developed numerous metastases from the primary melanoma and died after 18 months. Bednar tumour is a rare pigmented variant of dermatofibrosarcoma protuberans of intermediate malignant potential. The presence of pigmented cells in Bednar tumour requires careful differential diagnosis with malignant or benign pigmented skin tumours. The clinical history of a Bednar tumour developing close to the scar of a previous melanoma gives the opportunity of a critical and intriguing discussion about the potential origin of pigmented cells in this rare variant of dermatofibrosarcoma protuberans. Dermatofibrosarcoma protuberans (DFSP) is a neoplasm of intermediate malignant potential that grows slowly, but can be locally aggressive, with a high rate of local recurrence and a low rate of metastasis, mainly to lungs and bones , 2. ConsA seventy-four-year-old Caucasian man underwent the surgical excision of a cutaneous lesion of the back. The pathological diagnosis was superficial spreading MM, Clark level IV with a Breslow thickness 3 mm. MM cells appeared classically epithelioid and mitotic activity was less than 1 per 10 HPF. After two months, the patient underwent the left axillary satellite lymphadenectomy, which revealed the presence of metastasis from MM. Primary and metastatic tumours resulted diffusely positive to immunohistochemical staining for S100 and Melan-A (Ventana/Roche). Successive enlarged left axillary lymphadenectomy revealed an additional nodal metastasis. Two months later, a skin mass of 2 cm developed close to the previous surgical skin scar of the back. The clinical diagnosis was “in-transit” metastasis from MM. After surgical excision, macroscopic examination revealed a brownish-grey, multinodular, apparently circumscribed dermo-hypodermal mass, without haemorrhage or necrotic areas. Formalin-fixed paraffin sections, stained with haematoxylin-eosin, revealed a noncircumscribed, highly cellular dermal neoplasm, characterized by monotonous, slightly atypical spindle cells, arranged in storiform pattern, that deeply infiltrated subcutaneous tissue entrapping fat cells in a characteristic “honeycombing” pattern. A moderate mitotic activity (3 per 10 HPF) was recorded. Scattered heavily pigmented cells, with round to oval vesicular nuclei and dendritic cytoplasm, were also noted . Immunoh de novo neoplasm, as fascicular, rather than storiform, architecture, a greater cellularity with increased mitotic activity and necrosis. CD34 expression is generally reduced or lost in fibrosarcomatous areas of DFSP [α-actin, desmin and HHF-35 [BT is a rare variant of DFSP, which is a locally aggressive spindle-cell mesenchymal tumour. Several variants of DFSP have been described . Myxoid  of DFSP . The atr of DFSP . Myoid D of DFSP . Giant c of DFSP . As repo of DFSP . The mos of DFSP . Spindled HHF-35 , 2, 11. d HHF-35 . In spind HHF-35 . Synoviad HHF-35 . Extrapld HHF-35 . In addid HHF-35 , 17. Hisd HHF-35 , 18. Hypd HHF-35 , 20. Thed HHF-35 , 21. In


Pseudoepitheliomatous keratotic and micaceous balanitis (PKMB) is an extremely rare disease. Herein, we report a case of PKMB in a patient who underwent two surgical procedures, since the 5-FU cream was not available.A 50 year-old Korean man undergoing circumcision in a local clinic presented with a tumor-like lesion on the glans penis. Peeling the mass was performed to remove the entire mass after an excisional biopsy. A pathologic finding of mass showed hyperkeratotic and papillomatous squamous epithelium without obvious cytologic atypia. Considering that the lesion recurred after 4 weeks, the patient underwent glansectomy with split-thickness skin graft (STSG). There had been no evidence of recurrence at the surgical site during the follow-up at 6 years postoperatively.If the 5-FU cream is not available, two surgical procedures can be performed for treatment and biopsy. Peeling the mass has the advantage of confirming the characteristics of the whole lesion, but it cannot confirm tumor invasion because it is unable to obtain the subepithelial layer. Glansectomy is able to accurately identify the tumor stage because it removes the tumor and total glans penis and has excellent outcome.PKMB is very rare and has a characteristic appearance, which is mica-like crusts and keratotic horny mass on the glans penis. Glansectomy with STSG is a good procedure when the 5-FU cream was not available. The distinctive clinical findings of this disease are mica-like crusts and hard keratotic mass on the glans penis. Since its first report in 1961, less than 30 case reports have been reported worldwide , surgicaThis work has been reported in accordance with the SCARE criteria .2A 50 year-old Korean man undergoing circumcision in a local clinic because of persistent urinary tract infection presented with a tumor-like lesion on the glans. He had no pain or signs of irritation. He had no history of sexually transmitted disease (STD) and prohibited drug use, but was 15 pack-year smoker. Physical examination a showed 2.1After administration of spinal anesthesia, the hard mass was dissected starting from the urethral meatus, which was easily separated from the glans penis b-c. It wEpithelialization of the peeled surface took 3 weeks, and the patient complained of severe pain during penile erection and contact. Recurrence was observed in the glans after 4 weeks, and the recurred mass was observed around the glans penis and circumcised scars until 8 weeks.2.2Glansectomy with STSG was performed using the method reported by Parnham et al. . During There had been no evidence of recurrence at the surgical site with a follow-up duration of 6 years . He comp3PKMB is a rare disease that typically occurs on the glans penis in older men . It has Considering the rarity of this disease, there is a lack of research on the appropriate treatment methods. However, studies to date have suggested that the treatment methods should depend on the stage of the disease ,7,10. KrMost previous studies recommend the topical therapy, but in countries where such agent is not available, wide excision may be considered such as partial penectomy . Among tIn this case, three methods were used to obtain tissues to confirm the pathological findings. First, excisional deep biopsy, which is performed to determine the direction of treatment; however, this cannot identify exactly the stage of the entire tumors, because only a part of the tumor is identified. Second, resurfacing has the advantage of confirming the characteristics of the whole lesion because it removes the entire tumors, but it cannot confirm tumor invasion because it is unable to obtain the subepithelial layer. Lastly, glansectomy is able to accurately identify the stage, including tumor invasion, because it removes the tumor and total glans penis. However, this procedure has several disadvantages. It may be more invasive and requires additional skin graft, and patients may be dissatisfied with the appearance of the penis after the surgery.PKMB is often resistant to topical treatments, such as 5-FU cream, radiotherapy and cryotherapy. In these cases, progression to verrucous carcinoma or squamous cell carcinoma has been reported in a considerable proportion of cases, and they are now considered tumors with a malignant potential . Conside4PKMB is very rare and has a characteristic appearance, which is mica-like crusts and keratotic horny mass on the glans penis. Deep biopsies are needed to obtain subepithelial tissues for the accurate diagnosis of PKMB. Glansectomy with STSG is a good procedure when the 5-FU cream was not available.The authors report no conflict of interests.There was no funding.This case report was approved by institutional review board in our institution (IRB No. 2017-12-008).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.Seong Cheol Kim: Operated the patient, Wrote the manuscript, Drafted the manuscript.Joo Yeon Kim, Ji Yeon Kim: Diagnosed the tumors pathologically.Cheol Kyu Oh, Jae-Seung Chung: Reviewed the manuscript.Myungchan Park, Sang Hyun Park: Collected the data.None.Seong Cheol Kim.Not commissioned, externally peer-reviewed.


Decompression sickness (DCS) induced by extravascular and intravascular gas bubbles during decompression can present with varying manifestations, such as joint pain, numbness, cutaneous symptoms, and cardiopulmonary dysfunction. However, mesenteric venous thrombosis (MVT) is a rare complication of DCS. To the best of our knowledge, only a few cases have been reported, and surgical cases of MVT secondary to DCS have not yet been reported.A 59-year-old man who was a fisherman and recreational diver dived to a depth of 100 feet. After diving, he noted abdominal and postcervical pain and visited a community hospital. Computed tomography (CT) revealed a large amount of intravenous gas, so he was diagnosed with DCS. He was then transferred to a previous hospital, where hyperbaric oxygen therapy (HBOT) was performed. HBOT reduced the amount of venous gas, but his abdominal pain worsened, so he was transferred to our hospital. CT showed pneumatosis cystoides intestinalis. Because of the possibility of intestinal necrosis, a laparoscopic examination was performed, which revealed necrosis of the transverse colon. We therefore performed a transverse colon resection. He was discharged 36 days after the surgery and followed an uneventful postoperative course.DCS is likely to cause MVT. If intestinal necrosis is suspected, a laparoscopic examination may be useful for determining the diagnosis and treatment. MVT should be included as a differential diagnosis of abdominal pain that persists after HBOT. Decompression sickness (DCS) is caused by extravascular and/or intravascular gas bubbles that develop during decompression  and showWe herein report a case of mesenteric venous thrombosis that occurred after diving and discuss the most appropriate treatment strategy.A 59-year-old Japanese man was transferred to our hospital because of aggravated abdominal pain after initial treatment for DCS at the previous hospital.The patient was a fisherman and recreational diver and previously had DCS twice, which was treated conservatively both times. In addition, he was taking aspirin 100 mg/day because he had a history of percutaneous cardiac intervention for angina pectoris. He dove to a depth of 100 feet 3 days in a row using a self-contained underwater breathing apparatus (SCUBA) to search for a lost item. After surfacing, he experienced sudden abdominal and postcervical pain, so he visited a community hospital.Computed tomography (CT) revealed a large amount of intravenous gas throughout his whole body, including in the portal vein (PV) Fig. a, superi2 administration. Mottling and cutis marmorata were noted on his stomach. A physical examination revealed a distended and mildly hardened abdomen, strong abdominal pain, tense muscles, and tenderness, suggesting peritonitis on palpation. The laboratory data revealed an elevated white blood cell (WBC) count  and C-reactive protein (CRP) level, hemoconcentration, acute kidney injury, acute hepatic injury, and coagulopathy .On arrival, he was oriented, and his vital signs were as follows: blood pressure, 123/69 mmHg; pulse rate, 120 bpm; and oxygen saturation, 93% with 3 L/min OBecause of his kidney dysfunction, contrast-enhanced CT was avoided, and plain CT was conducted. CT revealed pneumatosis intestinalis of the transverse colon Fig. c, suggesAfter surgery, the patient was admitted to the intensive care unit. He was on mechanical ventilation, and meropenem, a broad-spectrum antibiotic, was administered for 6 days for peritonitis and bacterial translocation. Ulinastatin, a protease inhibitor, was administered for 3 days for acute circulatory failure, and sivelestat sodium hydrate, a neutrophil elastase inhibitor, was also administered for 5 days for acute lung injury. On postoperative day 3, early nutrition was started with the feeding tube. The patient was extubated 6 days after surgery. On postoperative day 36, he was discharged in good condition. Two months later, surgery for colostomy closure was performed. He is following an uneventful course.A pathological examination revealed diffuse edema, congestion, hemorrhaging, and necrotic changes in the mucosal, submucosal, and muscle layers and part of the subserosa Fig. a. In somDCS is caused by intravascular and/or extravascular bubbles that form from dissolved inert gas during or after decompression. Decompression illness (DCI) includes DCS and arterial gas embolism (AGE) and causes pulmonary barotrauma, expands gas stretches and ruptures alveolar capillaries, allowing alveolar gas to enter the arterial circulation. DCS is distinguished from AGE by its etiology .It is difficult to accurately calculate the incidence of DCS, but it is estimated that approximately 0.03% (per dive) of recreational divers are affected, according to The Divers Alert Network . The mecThe manifestations of DCS range from trivial to fatal, and the most common manifestation is pain, especially joint pain and muscle pain, followed by peripheral neurologic symptoms, such as numbness and paresthesia . ApproxiThe diagnosis of DCS is made on a clinical basis, so an accurate history and physical examination of symptomatic individuals after diving are crucial. Imaging studies such as chest X-ray, abdominal X-ray, ultrasonography, and CT are not necessary to diagnose DCS; however, these imaging studies help determine the severity and may provide significant findings that will aid in the selection of treatment . The fisContrast-enhanced CT is the most useful imaging tool for diagnosing MVT. The sensitivity and specificity of CT angiography are 93% and 100%, respectively . The mosThe basic treatment for DCS is a rapid administration of 100% oxygen . HBOT isMVT can occur as a complication of DCS. In addition, in severe cases of MVT, in which peritonitis or mesenteric ischemia is suspected, exploratory laparoscopy seems to be effective for obtaining an accurate diagnosis and determining subsequent treatment. If abdominal pain persists after the initial treatment for DCS (HBOT and adequate hydration), we should consider MVT as a severe complication of DCS.


Eosinophilic cholangitis (EC) is a rare benign disease characterized by transmural eosinophilic infiltration of the biliary tree that may result in biliary stricture and obstructive jaundice. The pathophysiology remains unknown but is theorized to involve an allergic mechanism. EC is diagnostically challenging as it may mimic cholangiocarcinoma (CCA) clinically and radiographically and involves a broad differential diagnosis including benign and malignant pathologies. In addition to tumor markers, ultrasonography, contrast-enhanced computed tomography (CT), and magnetic resonance cholangiopancreatography (MRCP), proper evaluation of malignant processes may need biopsy through endoscopic retrograde cholangiopancreatography (ERCP) and surgical exploration. We present the case of a 47-year-old female diagnosed with EC upon exploratory laparotomy. Eosinophilic cholangitis (EC) is a rare disorder of the biliary tract . AlthougNot only can EC mimic CCA in clinical presentation, but is also difficult to differentiate radiographically . Tumor mThe pathogenesis of EC is poorly understood . FurtherThe patient is a 47-year-old Hispanic American female without significant past medical history who presented with progressive intermittent right upper quadrant (RUQ) abdominal pain of two months duration. The pain was associated with intermittent nausea and an unintentional 10-pound weight loss. She had no personal or family history of gastrointestinal, allergic, or atopic disease. Physical examination was significant for moderate RUQ tenderness. Laboratory testing revealed aspartate aminotransferase (AST) 325 IU/L, alanine aminotransferase (ALT) 130 IU/L, alkaline phosphatase (ALP) 180 IU/L, total bilirubin (TB) 0.7 mg/dl; lipase 38 IU/L; white blood cells (WBC) 11.7 × 103/mm3, with a differential of 55% neutrophils, 14% lymphocytes, and 21% eosinophils. A RUQ ultrasonography revealed evidence of acute cholecystitis without common bile duct (CBD) dilation or evidence of stones. She underwent an uncomplicated laparoscopic cholecystectomy. She improved clinically and was discharged home. Pathology later confirmed eosinophilic cholecystitis.The following week she presented with recurrent RUQ pain. Laboratory testing revealed AST 631 IU/L; ALT 383 IU/L; ALP 214 IU/L; TB 2.4 mg/dl, lipase 27 IU/L; WBC 15 × 103/mm3, with 53% eosinophils. MRCP revealed mild intrahepatic biliary ductal dilatation with focal soft tissue density of the proximal to middle CBD. Tumor and immunological markers were unremarkable. Given the possibility of a malignant CBD stricture, an ERCP was performed, but without success in passing the guidewire through the stricture. Hence, a percutaneous transhepatic cholangiography (PTC) was inserted for decompression. She improved clinically and was released home. The following week, the abdominal pain recurred. However, it was now associated with fever, chills, and food aversion. Laboratory testing revealed WBC 17.1× 103/mm3, with a differential cell count of 84.5% neutrophils, 4.4% lymphocytes, and 1.4% eosinophils.CT scan revealed a 3-cm focal collection adjacent to the liver Figure . She wasEC is a rare benign disease characterized by transmural eosinophilic infiltration of the biliary tree that may result in biliary stricture and obstructive jaundice . The epiDue to the broad differential diagnosis, it is important to be able to distinguish between benign and malignant causes of stricture. This differentiation may be accomplished through several laboratory findings such as elevated tumor markers, although CA19-9 may be unspecific to benign and malignant etiologies . DiagnosThere are no established diagnostic criteria for EC ; howeverIn addition to tumor markers, ultrasonography, contrast-enhanced CT and MRCP, proper evaluation of malignant processes may need biopsy through ERCP . Lastly,Eosinophilic cholangitis is a rare cause of biliary obstruction. The pathophysiology remains unknown and is theorized to involve an allergic mechanism. While a diagnostic trial of steroids can be attempted, surgical intervention with extrahepatic bile duct excision along with roux-en-y hepaticojejunostomy may be needed as histological confirmation of eosinophilia prior to surgery is often not possible. It should be considered in the differential diagnosis of patients with obstructive jaundice in the setting of peripheral eosinophilia. Despite no established diagnostic criteria, the triad of wall thickening or stenosis of the biliary system, histopathological findings of eosinophilic infiltration, and reversibility of biliary abnormalities without treatment or following steroid treatment has been proposed by Matsumoto et al. Although we were unable to assess reversibility of disease, other aspects of the triad were present in our case, and future cases and reviews are needed to strengthen these diagnostic criteria.


Respiratory epithelial adenomatoid hamartomas (REAHs) are becoming a more commonly recognized otolaryngologic tumor and are often misdiagnosed as inverted papilloma. Here, we present such a case in a patient with history of previous radiation exposure. Otolaryngologists and pathologists should be aware of the mucinous histological appearance of REAH to help differentiate from other growths. Given our patient's history, an association between REAH and previous radiation exposure is worth consideration. Respiratory epithelial adenomatoid hamartomas (REAHs) are becoming a more commonly recognized otolaryngologic tumor. REAHs are often misdiagnosed as inverted papilloma, with accurate diagnosis limited by a low index of suspicion. While the malignant potential of inverted papilloma should not be underestimated, REAHs may require less extensive management. Thus, an accurate diagnosis is important in preventing unnecessary intervention.Case reports are exempt from institutional review board approval at our institution.A 75-year-old woman was evaluated for long standing right-sided nasal obstruction, dependent mouth breathing, clear rhinorrhea, congestion, and hyposmia not relieved by intranasal steroids or nasal irrigations. She initially presented with a history of previous endoscopic sinus surgery in Russia more than 20 years ago followed by nasal polyposis treated with ambulatory cauterization. She was also noted to have a history of nonmelanotic skin cancers of the nose treated with radiation in Russia.Physical exam findings included a fleshy intranasal lesion that, in the setting of previous nasal skin cancer treated with radiation, raised a concern for possible secondary carcinoma. Computed tomography showed complete opacification of the right maxillary sinus, obstruction of the right ostiomeatal complex, and soft tissue density in the right nasal passage .Initial biopsy revealed a nasal mass that originated in the right inferior meatus. Pathology showed multiple polypoid fragments lined by a respiratory type epithelium with underlying edematous stroma with mild chronic inflammation. There was invagination of the surface epithelium into the underlying stroma resulting in nested aggregates of bland glandular and mucinous cells and focally benign squamous epithelium . These fThe patient presented with continued nasal obstruction and was evaluated for definitive treatment. Given her diagnosis of inverted papilloma and chronic rhinosinusitis, complete excision and revision endoscopic sinus surgery was recommended. During endoscopic sinus surgery, an exophytic mass with abnormal maxillary mucosa was seen emanating from the left inferior meatus that was thought to originate from the right maxillary sinus, given that it was protruding through a bony dehiscence into the inferior meatus and nasal cavity. Right partial inferior turbinectomy was performed, along with right extended maxillary antrostomy and stripping of maxillary mucosa to remove the entirety of the presumed inverted papilloma base within the maxillary sinus. Intraoperative and postoperative pathology again showed inverted papilloma.Given her suspicious history, a second opinion was requested. This noted stromal hyalinization characterized by thickened, eosinophilic basement membrane-like material. The polypoid lesion had an inverted growth pattern concerning for inverted papilloma, but it had a prominent glandular lining , leadingMany REAHs are found in the olfactory groove and associated with olfactory groove widening , 2. OtheTypical presenting symptoms include the following: nasal obstruction, nasal stuffiness, deviated septum, epistaxis, and chronic rhinosinusitis occurring from a few months to many years in duration . DiffereThe etiology of REAH is unclear, with no link to any specific exposures. That said, it is interesting to note that our patient had previous radiation exposure in the same region as her eventual REAH presentation. REAHs are typically associated with a benign course with no reported cases of recurrence after complete surgical resection .Greater recognition of REAHs as distinct from inverted papilloma, inflammatory polyps, and low-grade adenocarcinoma can help determine appropriate management and treatment. As REAHs are often benign and self-limiting, they do not need to be resected in asymptomatic patients or as aggressively resected in symptomatic patients. Patients should be followed with serial endoscopies to evaluate for possible disease progression, and conservative management may be sufficient.In conclusion, REAH is becoming a more widely recognized tumor. Expertize in pathology is required for diagnosis, and conservative management is the current treatment approach in asymptomatic patients. Otolaryngologists and pathologists should be aware of the mucinous histological appearance of REAH to help differentiate from other growths. In our patient, the disease was focused in the inferior meatus. Our findings support surveillance and diagnosis of REAH in other locations. Given our patient's history, an association between REAH and previous radiation exposure is worth consideration.


The diagnosis of lung typical carcinoid tumors results challenging when limited size and unfavorable sampling location is associated. It has been reported that bronchoscopy with endobronchial ultrasound (EBUS) significantly increases the diagnostic yield of peripheral nodules smaller than 2 cm.A 70-year-old Caucasian male complained of persistent fever and cough despite several antibiotic courses and steroid treatment. Chest radiology revealed the presence of a small single nodular opacity in the left upper lobe, whose standardized maximum uptake value (SUV) at fluorodeoxyglucose positron emission tomography-computed tomography (FDG PET/CT) was significantly high (4.5). The patient underwent bronchial endoscopy but any appreciable sign of endobronchial or intramural involvement was detected. Only radial ultrasound-guided bronchoscopy (R-EBUS) allowed transbronchial sampling whose pathological analysis revealed a typical carcinoid tumor. The patients underwent surgical lobectomy and clinic-radiological follow was started.With this case we aim at stressing the importance of ultrasound in the diagnostic process of lung small peripheral carcinoid, especially if they present without mucosal or sub mucosal involvement. Carcinoid tumors are low-grade neuroendocrine malignancies that usually affect the gastrointestinal tract . AccordiA 70-year-old mild former smoker (3 packs/year) male was admitted to the Respiratory Ward of the Santa Maria Nuova Hospital of Reggio Emilia (IT) for persistent cough and fever despite prolonged antibiotic courses and steroid therapy. Past medical history revealed surgical excision of the right vocal cord for a benign vocal cords tumor performed 30 years before. At the time of hospital admission the chest X ray showed a single lung nodular opacity of 1.6 × 1.3 cm in the left upper lobe that was confirmed by a subsequent chest computed tomography (CT) scan Fig. . Thus a Pulmonary carcinoid tumors are low-grade malignant tumors of neuroendocrine origin  accountiIn conclusion, we report a case of a peripherally located carcinoid tumor whose diagnosis was made possible only through transbronchial biopsy performed with R-EBUS. This case emphasizes how ultrasound is more than useful in the diagnostic process of lung small peripheral tumors, particularly when they present as occult at endoscopic investigation.


Hemangiopericytoma (HPC) is a rare pediatric neoplasm with a high risk of bleeding, aggressive growth and high early relapse rates. Surgical excision remains the mainstream treatment, while the functions of chemotherapy and radiotherapy remain controversial. In particular, an infantile giant extracranial HPC located in the forehead has never been reported.A 3-day-old girl was delivered normally with a giant tumor localized mainly in the right frontal region. The surface of the mass was filled with vascularity.According to the results of imaging and pathological examinations, the diagnosis was HPC grade II.Gross total resection of the tumor and the invading partial frontal bone followed by skin scalp reconstruction was carried out without any blood transfusion.No recurrence was identified during 5 years of follow-up. And better outcomes can be achieved without adjuvant therapy.Multimodality imaging and a collaborative multidisciplinary approach are indispensable for the successful surgical management of infantile HPC, especially for giant tumors and their potential risk of life-threatening bleeding. Gross total resection is the optimal option for infantile HPC, and even without adjuvant therapy, it achieves better outcomes. Only after NAB2-STAT6 fusion was discovered using whole-exome sequencing was HPC reclassified from SFT to SFT combined with conventional HPC, resulting in a unique entity, SFT/HPC.Hemangiopericytoma (HPC), which was first described by Stout and Murray in 1942, is a hypervascular neoplasm originating from the pericytes of Zimmeman that is surrounded by capillary and postcapillary venules.,4,5 and that intracranial HPC accounts for less than 1% of central nervous system (CNS) cases. Surgeries performed on infants remain a challenge due to surgery-related complications and outcomes. Here, we report a giant infantile head HPC patient who underwent gross total resection (GTR) of the tumor and had a beneficial outcome at a 5-year follow-up.Previous studies have shown that the incidence of head and neck HPC ranges from 4.5% to 10%2A 3-day-old girl was delivered normally with a giant tumor localized mainly in the right frontal region on July 22, 2013. On physical examination, the infant's weight was 4.7 kg, her height was 52 cm, and her head circumference was 39 cm. Additionally, a 9 × 7.1 × 5.1 cm soft, rounded and well-defined mass with a broad base occupied most of the forehead. The surface of the mass was filled with vascularity  scan revealed a large mass tumor located in the front of the head, and computed tomography angiography (CTA) demonstrated that the blood supply to the vascularized tumor originated mainly from the bilateral supratrochlear artery and partly from the distal part of the right superficial temporal artery Fig. A. MoreovAfter a thorough preoperative evaluation with a clinical collaborative multidisciplinary team that included members of the neurosurgery, pediatric, anesthesiology and intensive care units and a discussion of perioperative management, the surgery strategy and any potentially risky situations that could arise during the operation, the surgery was performed. During the operation, a reasonable incision was designed Fig. , and theHematoxylin and eosin (HE) staining showed that the tumor cells had polymorphic nuclei that were round and oval-shaped, were surrounded by typical staghorn-shaped blood vessels Fig. A, and shAfter the operation, the infant was sent to the intensive care unit to monitor her vital signs. There was no sign of bleeding in the surgery area on a CT performed on the 1st day after the operation, which revealed that a gross total resection of the tumor was achieved. Approximately one week later, the infant's wound became black. With careful wound nursing, she was discharged in good condition four weeks later. She did not receive chemotherapy or radiotherapy. No evidence of recurrence or metastasis was found during follow-ups performed 6 months after the operation and then annually and biannually once stability was confirmed. After a 5-year follow-up period, the wound could not be found without careful examination Fig. E.3–8 but the most common site is the head and neck.,10HPCs originating from capillary pericytes are extremely rare pediatric tumors with signs and symptoms indicating aggressive behavior. This disease includes two distinct clinical types: infantile (occurring at < 1 year old) and adult (occurring at > 1 year old). Infantile HPC can occur anywhere in the body, including primary soft tissues, deeper structures and the lower extremities,,12 (2 cases), tongue,14 (2 cases), maxillary sinus (1 case), rhinopharynx (1 case), orbit–19 (3 cases), neck,20–24 (6 cases), trunk and back–28 (7 cases), abdomen,12,29–32 (5 cases), arms and legs,26,33–36 (10 cases) and perianal region (1 case). The male to female ratio was 20 to 18  with spontaneous regression under careful monitoring. The lesion in our case was located in the front region near the orbit and was larger than the previous lesions located in the head that were reported by Perez et al,12 To the best of our knowledge, this is the first report to describe an infantile giant extracranial HPC located in the forehead.We identified 38 cases of infantile extracranial HPC that were reported in the literature Table . The loc,5,20 Perez et al reported a neonate with HPC that caused hypovolemic shock due to bleeding in the temporal lobe; the patient underwent emergency surgery and was transfused with more blood. Ogawa et al described a case of infantile HPC located on the tongue that showed sudden bleeding and required a total of 32 units of red cell concentrate due to severe tumor bleeding. The giant tumor described in our report had an extremely high risk of bleeding. If it began to bleed, the infant's life would be at risk. Therefore, imaging examinations were very useful for quickly detecting the extent of the tumor, which helped the doctors select the best methods for treating the HPC during the perioperative period, even though there were no specific features for diagnosing infantile HPC. In our case, both CT and MRI were very valuable for evaluating tumor invasiveness, whereas CTA was used for assessing the tumor blood supply and provided the surgeons with the ability to control bleeding during the operation.The clinical symptoms of infantile HPC can include hemorrhage, compression of local structures, and even sudden death. Han et al showed that 40% of metastatic and 85% of nonmetastatic tumors had positive CD34 immunostaining. Bertero et al demonstrated that in HPC, the mean rate of CD34 expression was 64%, while the rate for Bcl-2 was 63%. Our case showed typical pathological manifestations, including more tumor cells and a “staghorn” vasculature on hematoxylin and eosin staining (HE), and was CD34-positive for immunostaining.HPCs represent a diversity of neoplasms ranging from well- to poorly differentiated tumors . They are divided into three grades based on histological subtype, including WHO grade I (SFT); WHO grade II (HPC), which contains more pump cells and has a “staghorn” vasculature; and WHO grade III (anaplastic HPC), which shows > 5 mitoses per high-power field (HPF). Although detecting the expression of CD34, bcl-2, CD99, CD31, S100, and EMA by immunohistochemical staining can be used to better differentiate SFT/HPC from meningioma and myofibroma, it is not generally effective in diagnosing SFT/HPC.,33 treated with preoperative chemotherapy that achieved GTR of the tumor and one case treated with two-stage surgery including external carotid artery sacrifice without follow-up. Fourteen cases were treated with partial resection and chemotherapy, 2 with biopsy and chemotherapy, and another 2 with only biopsy. Additionally, Pushker et al reported one lesion in the orbit that was treated with only chemotherapy and was followed up for 14 months without recurrence. Two cases reported by Atkinson et al,24 were tumors located in the orbit that were treated by partial resection and radiotherapy, but both infants eventually died at 1 and 4 months old. In our case, we devised a comprehensive and scientific preoperative plan and carefully operated to resect the tumor and adjacent affected frontal bone, without any adjuvant therapy, the infant showed no recurrence at the most recent follow-up.In terms of treatment for infantile HPC, we identified 19 cases previously reported in the literature that were treated with complete resection; these included two cases,41 The risk of surgery-related morbidity and mortality in infantile HPCs increases with the amount of bleeding, and this may be the greatest barrier to complete resection. CTA or digital subtraction angiography (DSA) can be used to identify the main supplying blood arteries and is an indispensable adjuvant examination approach in HPCs. Hanak et al reported achieving GTR in 59% of adult HPCs with preoperative embolization, which was negatively correlated with intraoperative blood loss. It was only the case reported by Ogawa, which involved severe bleeding from a tumor in the tongue, in which embolization of the lingual artery with iodized oil was performed before partial resection. Chemotherapy was also considered in that case.According to the literature, the mainstay treatment for HPC is total tumor resection whenever possible.,43 have suggested that there is no need for chemotherapy. Bien et al showed that in cases of infantile-type HPC with unresectable, life-threatening tumors, chemotherapy could produce a favorable outcome, while macrotumors or unresectable adult-type HPCs should be considered for chemotherapy and radiotherapy. A systematic review by Spina et al revealed that Gamma Knife radiosurgery (KGS) could be a feasible and effective therapy for HPCs.Although chemotherapy and radiotherapy can reduce local recurrence and metastasis, their functions need to be prospectively validated. Some studies In their report, the tumors were located in the lung, forearm, thigh, perianal area and calf, but they did not report on tumor size. Perez et al followed up a case for 11 months after resection of a temporal lobe HPC. The follow-up time was 90 months after secondary surgery and chemotherapy for an HPC located in the head that was reported by Bien et al In our case, although the tumor was giant and had a risk of hemorrhage that threatened the infant's life, with a collaborative multidisciplinary approach, we determined that a complete resection without adjuvant therapy would achieve a better outcome.Overall, the outcomes of infantile HPC are different from those found in adult HPC due to differences in treatments and tumor locations. In the literature, the longest follow-up (2–27 years) for infantile HPC was reported by Fernandez, et alIn summary, multimodality imaging and a collaborative multidisciplinary approach are indispensable for the successful surgical management of infantile HPC. GTR, even without adjuvant therapy, is the better option for infantile HPC when there is a potential risk of threat to the infant's life. However, a longer follow-up period is essential due to the aggressive behavior of HPCs. We hope that this case report may be helpful in the efficient management of infantile HPC, especially in giant tumors with their potential risk of life-threatening bleeding.Conceptualization: AiJun Peng, LiBing ZhangMethodology: LiangXue Zhou, AiJun Peng, LiBing ZhangConceptualization: AiJun Peng, LiBing Zhang, LiangXue Zhou.Formal analysis: Hai Zhao, LiangXue Zhou.Methodology: LiBing Zhang, LiangXue Zhou.Supervision: LiangXue Zhou.Writing – original draft: AiJun Peng, LiBing Zhang.Writing – review & editing: Hai Zhao, LiangXue Zhou.


Central pancreatectomy (CP) is a well-described procedure done for neck and proximal body tumors of the pancreas. It can be done for benign lesions where an adequate length of normal distal pancreas will be left leading to organ preservation. The currently described benefit of the procedure is decreased long-term morbidity due to retention of both the spleen and the preservation of functioning pancreas. This is usually dependent on the preservation of distal pancreatic vascularity by splenic artery preservation. Many studies have described splenic preservation by Warshaw technique by safeguarding the short gastric (SGA) and left gastroepiploic (LGEA) vessels in case of distal pancreatectomy. However, distal pancreatic preservation during CP with splenic vessels ligation is not given a significant mention in the current literature in relation to Warshaw technique. Here, we present a 19-year-old girl diagnosed with an exophytic solid pseudopapillary tumor of the pancreatic body that was selected for central pancreatectomy. In view of splenic vessels involvement, she underwent ligation of the splenic vessels and splenic preservation was based on the LGEA and SGA. Distal pancreas was anastomosed with a roux en loop of jejunum and intra-operatively, we were able to demonstrate the back flow in the splenic vessels. Postoperative computed tomography showed adequate enhancement of the spleen along with retrograde blood flow into the distal splenic artery with enhancement of the distal pancreas. Her postoperative period went uneventful. Thus CP with extended Warshaw technique is a safe and feasible procedure where indicated. Central pancreatectomy (CP) is a parenchyma preserving pancreatic resection done in benign and low malignant lesions of pancreas. Splenic perfusion occurs by the retrograde blood supply via the short gastric and left gastroepiploic vessels and is well documented in Warshaw technique which is done for distal pancreatectomy . HoweverA 19-year-old girl was presented with complaints of non-radiating localised pain in the epigastrium for four months, not associated with vomiting, fever or bowel habit irregularities. Her past medical history was insignificant except for a minor trauma to her upper abdomen three years back when she was evaluated for an incidentally detected retroperitoneal solid cystic mass lesion. Her abdominal examination showed an ill-defined mass in the epigastric region which was firm and non-tender. Ultrasonography of the abdomen showed a solid mass lesion with heterogeneous echo texture in the lesser sac arising from the pancreas. Further imaging evaluation with the contrast-enhanced computed tomography (CECT) abdomen showed an exophytic, well defined and encapsulated solid heterogeneously enhancing mass lesion arising from the body of pancreas pushing the stomach  at the confluence and few perisplenic collaterals were noted. A clinical diagnosis of solid pseudo papillary neoplasm was made and we proceeded for laparotomy with the intent of curative resection.The abdomen was opened by a midline laparotomy instead of the rooftop incision for cosmetic benefit. Intraoperatively, we found a highly vascular 10 × 10 cm lobular mass of variegated consistency arising from the proximal body of pancreas in the lesser sac with dense adhesions to the greater omentum and posteriorly to the Toldt’s fascia. The mass was densely adherent to the splenic vein near the confluence. Figure The splenic artery was found to be coursing through the mass. The proximal pancreas was normal and distally more than 5 cm of the tail region of pancreas was un-involved. No metastatic deposit or regional nodal disease was evident.Decision was taken to proceed with central pancreatectomy. The lesser sac was opened and the gastroepiploic vessels along the greater curvature of stomach meticulously preserved. A gentle blunt adhesiolysis was done from the posterior wall of stomach. The part of pancreas containing the tumor was carefully dissected from SMV-PV axis and we found that the splenic artery was coursing through it. The tumor was densely adherent with the splenic vein at the confluence and both artery and vein needed ligation for tumor resection. Splenic artery was ligated proximally and distally to the tumor in view of encasement. Further mobilisation of middle segment of pancreas was done distally to facilitate anastomosis. A margin of 1 cm on either side of tumor was ensured and the tumor was resected. Distally normal pancreas more than 5 cm was preserved and the splenic hilar blood vessels were undisturbed.Central pancreatectomy was done and a handsewn, two-layered, end-to-side, duct-to-mucosa type anastomosis was fashioned between the distal pancreas and a retrocolic Roux loop of jejunum. The proximal end of the duct margin was doubly ligated. The maintenance of adequate splenic and distal pancreatic blood supply was achieved by preserving the short gastric and the left gastroepiploic vessels. At the end of the procedure, we were able to demonstrate adequate blood flow into the preserved distal splenic vessels within the distal pancreatic stump. The spleen was burgundy-colored and there was enlargement of spleen, both of which suggested adequate retrograde blood supply.The postoperative period went uneventful. A check CECT angiography was done on third post-operative day which highlighted a fully enhancing spleen and distal pancreas. Figure There was contrast flow into the terminal splenic artery stump up to the point of ligation via the left gastroepiploic vessels and in turn to the right gastroepiploic vessels suggesting adequate retrograde vascularity. The short gastric artery was enhanced adequately. The patient was discharged safe and well. At follow-up, after a month and three months, she was doing well.The histopathology revealed a well-encapsulated lesion composed of tumor cells arranged in sheets and pseudopapillary pattern with fibrovascular cores Figure .Tumor cells were small, round and uniform with moderate to abundant cytoplasm, fine chromatin and inconspicuous nucleoli. Immunohistochemistry was positive for CD56 and progesterone receptors. Hence, a final pathological diagnosis of solid pseudopapillary neoplasm was made. Aggressive features were identified including large tumor size (9 × 5 × 5 cm), focal capsular infiltration and perineural invasion.A parenchyma preserving pancreatic resection is possible in benign or low malignant tumors of the neck and proximal body as an alternative to distal pancreatectomy (DP). In 1908, Oskar Ehrhardt first wrote about segmental neck resection . Baka anSolid pseudopapillary neoplasm, previously called papillary cystic neoplasm, also known as Gruber-Frantz tumor, is a rare variety of pancreatic carcinoma of unclear histogenesis. They have characteristic predilection for young adult females in the second or third decade. Most often SPN presents as an abdominal mass lesion and may present with non-specific abdominal pain. The most common site of occurrence is the distal pancreas but they can present anywhere throughout the pancreas. On computed tomography, they appear as well delineated and isodense mass lesions. The CECT shows solid-cystic lesions with hypoenhancing areas and heterogeneous appearance. There may be features of calcification, pseudo capsule formation and invasion of adjacent vessels like splenic vessels .The indications for CP usually include benign and low-grade malignant tumors including neuroendocrine tumors, benign mucinous or serous cystadenomas and SPN, isolated pancreatic secondaries, commonly renal; focal chronic pancreatitis; and pancreatic trauma ,3. In alSPN has a low malignant potential and good prognosis despite locally advanced or metastatic disease and is amenable for segmental resection. Distal pancreatectomy, central pancreatectomy and pancreaticoduodenectomy are all viable treatment options for benign or malignant tumors arising from the middle segment of the pancreas .Warshaw technique involves preservation of retrograde perfusion to spleen through the short gastric and left gastroepiploic vessels in distal pancreatectomy . The WarWarshaw reviewed the progression of left-sided portal hypertension in Warshaw technique on long-term follow-up and reported a significant risk of developing gastric varices and perisplenic collaterals, but they mentioned a low risk of bleeding from them . Long-teIacono et al. conducted a meta-analysis with systematic review of 94 studies that included 963 patients who had undergone central pancreatectomy and noted that in the short term, CP had higher risk of postoperative morbidity with rates being around 46.0% in CP vs 28.6% in DP [Central pancreatectomy along with splenic artery ligation with distal pancreas and spleen preservation can be safely employed for locally aggressive SPN. This novel technique – Extended Warshaw Technique – is an efficient procedure for benign and low malignant potential tumors of the neck or body of pancreas. This procedure is relatively safe in long term with a slightly increased risk of fistula formation which usually is amenable to conservative management but more importantly with maintained long-term endocrine and exocrine functions.


Thoracic outlet syndrome (TOS) is a condition that results from the compression of the neurovascular bundle of the upper extremities. It has generally been divided into neurogenic and vascular TOS with vascular further being divided into venous and arterial. This case report is of a healthy 24-year-old female who was misdiagnosed initially in an emergency department only to return two days later for reevaluation. Here, the patient's admission and workup are discussed after she presented to the family medicine clinic for a referral for further evaluation by a cardiothoracic surgeon. Physical exam provocative tests which are often overlooked are reviewed, and osteopathic manipulative techniques and principles are mentioned. Anatomically, the thoracic outlet is the area containing the brachial plexus, subclavian artery, and subclavian vein as it passes from the neck into the upper extremity . The bonNeurogenic TOS comprises approximately 95% of cases while venous TOS is the second-most common with about 4% of cases . NeurogeThe deep-venous thrombosis is secondary to stasis of the blood or occlusion of the vessel which is part of Virchow’s Triad along with endothelial injury and hypercoagulability. This is considered a spontaneous upper-extremity DVT which has also been termed Paget Schroetter syndrome or an “effort” thrombosis -4. It isA 24-year-old Caucasian female presented to the emergency department (ED) with the chief complaint of right arm pain for the past four days. The pain started in her neck and radiated to the right trapezius and down to her elbow. She said she was seen at a different ED two days prior and was treated for a pinched nerve by being given dexamethasone, cyclobenzaprine, and ibuprofen. However, the pain worsened and she noticed color changes of her arm that were dependent on position. She said her hand was turning blue unless she held it above her head. She had a past medical history significant only for menstrual migraines and had been on oral contraception since she was a teenager. She denied tobacco or recreational drug use and drinks alcohol socially. She exercises regularly but the type of exercise was not specified. She admitted to a family history of cervical cancer in her mother, a grandfather with polycythemia and a grandmother with diabetes, hypertension and biliary duct cancer. Her recorded allergies included penicillin, latex, and adhesive tape.3 and neutrophils of 12.1 k/mm3. She had a chest X-ray without acute cardiopulmonary process. A venous Doppler ultrasound was reported as significant for an acute right axillary/subclavian deep venous thrombosis (DVT). She was admitted and seen by vascular surgery, cardiology, and hematology. She underwent catheter-directed thrombolysis and was started on anticoagulation therapy. She had a hypercoagulability workup which included phosphatidylserine/prothrombin antibody IgG/IgM, factor 5 mutation, antithrombin 3, antinuclear antibody, rheumatoid factor, factor 3 mutation, anti-Xa heparin, protein C, and protein S, all of which was negative. She was discharged on warfarin after a therapeutic international normalized ratio (INR) was achieved. Obstetrics and gynecology recommended discontinuing the oral contraceptive pills. She continued to follow up with her primary care physician and hematologist who was managing her warfarin and the diagnosis of venous thoracic outlet syndrome was made. She now presented to the office after moving to the area and requested a referral to cardiothoracic surgery to complete her evaluation for possible thoracic outlet decompression surgery.Her workup in the ED included labs and imaging. The labs appeared normal except for a leukocytosis of 14.4 k/mmInitial physical exams are important as they can help identify the area of compression. Adson’s test indicates compression between the scalene muscles; the costoclavicular test, also known as the military posture test, indicates compression between the clavicle and first rib; and the hyperabduction test, also known as Wright’s test, indicates compression between the pectoralis minor and chest wall . For eacThe treatment of neurogenic and vascular TOS differ . NeurogeOsteopathic manipulations have also been shown to improve symptoms of thoracic outlet syndrome and are more commonly utilized in neurogenic TOS. Targeted areas include the cervical spine levels C2-C7, the thoracic spine level T1, rib 1, thoracic inlet, clavicle, and scalenes if somatic dysfunction is present . A combiThis case reviewed a less common presentation of thoracic outlet syndrome. It also demonstrates the importance of physical exam. While there is often pressure to see many patients in a short amount of time in addition to proper documentation it is important not to forget the basics with history and a thorough physical exam. A simple review of physical exam provocative tests may have helped make a correct diagnosis upon initial evaluation. Additionally, as an osteopathic family medicine resident, understanding additional osteopathic manipulative treatment techniques that may benefit a patient is useful, and continual practice is necessary to develop and refine the skill of manipulation.


Peritonitis secondary to bowel perforation is a rare and potentially fatal complication in peritoneal dialysis (PD) patients. However, the early diagnosis of bowel perforation is difficult in PD patients because the initial symptoms and signs of bowel perforation are similar to those of PD-associated peritonitis. Furthermore, the risk of bowel perforation in PD patients is unclear. Here, we present a case of intestinal perforation located at the site of adhesive intestinal obstruction in a PD patient.A 73-year-old man on PD presented with progressive worsening of abdominal pain and cloudy peritoneal fluid. The peritoneal fluid cell count was increased to 980/ml and peritoneal dialysis-associated peritonitis was diagnosed. Computed tomography showed local adhesions causing agglomeration of the dilated intestine. He initially responded to antibiotic treatment; however, his abdominal pain was rapidly worsened after resumption of oral intake. On hospital day 23, computed tomography showed loss of contents from the dilated intestine and discharge of fecal material from the PD tube was noted. Thus, small bowel perforation was diagnosed, and he underwent ileocecal resection with colostomy creation. As indicators of EPS was not evident, PD catheter was removed. Since then, he has been on maintenance of hemodialysis since then.The findings of the present case suggest that adhesive intestinal obstruction in PD patients can increase the risk of intestinal perforation. Careful monitoring for the early detection of intestinal perforation is required in such cases. Peritonitis secondary to bowel perforation is a serious complication with a high mortality rate of 46.3% . DespiteThe causes and risks of intestinal perforation in PD patients are unclear. Diverticulosis is known to cause large bowel perforation in PD and non-PD patients . ApproxiHere, we present a case of small intestinal perforation located at the site of intestinal obstruction in a PD patient. An adhesive intestine caused by PD-associated peritonitis is uncommon; however, it can be a risk factor for intestinal perforation. Additionally, careful monitoring for the early detection of bowel perforation is considered important in such cases.A 73-year-old Japanese man on PD presented with progressive worsening of abdominal pain and cloudy peritoneal fluid. He had high blood pressure, and he started continuous ambulatory peritoneal dialysis (CAPD) because of hypertensive nephrosclerosis 8 years previously. A PD catheter was primarily inserted at the right abdomen, but it was removed and inserted at the left abdomen because of exit site and tunnel infection 5 years previously. He had no past medical history of diabetes mellitus and major abdominal surgery. In the peritoneal equilibration test, his result was high. Bloody ascites was not evident. One year previously, he had been hospitalized for PD-associated peritonitis caused by touch contamination that was treated with intraperitoneal cephazoline and cephtazidime. Bowel adhesion was not noted 5 years previously; however, local bowel adhesions and agglomeration of the intestine were detected by computed tomography (CT) after the identification of PD-associated peritonitis Fig. , b. The On physical examination, his blood pressure was 134/74 mmHg, pulse rate was 76 beats/min, and temperature was 99.7 ° F. He complained of severe pain in the right upper quadrant of the abdomen, and this area was tender on palpation. The exit site was clear. Laboratory tests revealed mild inflammation, with a white blood cell count of 10,100 /μL and C-reactive protein level of 0.9 mg/dL. The peritoneal fluid cell count was increased at 980 /mL. Based on these findings, PD-associated peritonitis was diagnosed. CT showed localized dilation of the intestine, which suggested adhesive small bowel obstruction Fig. . As we sEnterococcus faecalis and Bacteroides caccae. On CT, the intestinal contents disappeared and the dilated intestine collapsed, indicating that the intestinal contents had leaked into the abdominal cavity (Fig. After resumption of oral intake, his abdominal pain worsened and his peritoneal fluid cell count dramatically increased to 9600/mL on day 15. The peritoneal fluid became cloudy with a high amount of fibrin and white blood cells Fig. . Althougity Fig. . Consideity Fig. . As indiity Fig. , b. Althity Fig. -f. The pAlthough the early diagnosis of intestinal perforation is obviously important for a better outcome, definitive signs of intestinal perforation are often lacking in PD patients. The initial symptoms of intestinal perforation are often similar to PD-associated peritonitis. According to the ISPD guidelines, anaerobic growth in peritoneal fluid or polymicrobial peritonitis is frequently associated with intra-abdominal events requiring surgical attention; however culture is often false-negative –7. FurthDiverticulosis is one of the most common cause of colonic perforation ; howeverIntestinal perforation in the present case was secondary to adhesive bowel obstruction, which is generally a major complication of intraperitoneal surgery . Bowel aIn the present case, antibiotic therapy and fasting initially improved peritonitis, indicating that the patient’s peritonitis on admission was caused by bacterial translocation and not intestinal perforation. However, improvement of adhesive intestinal obstruction was insufficient and persistent bowel obstruction led to intestinal perforation when the intraluminal pressure increased after restarting oral food intake. In conclusion, the combination of adhesive intestinal obstruction and peritonitis in PD patients can increase the risk of intestinal perforation. Close monitoring for the early detection of intestinal perforation is required in such cases, and the timing of oral food intake resumption should be considered carefully.


Solitary fibrous tumors are tumors of mesenchymal origin that occur in the extremities and occasionally in pleura, meninges, and so on, but are extremely rare in the adrenal gland. Their biological behavior is variable but mostly benign.A 13-year-old Oromo girl presented with a progressively increasing right upper abdominal mass of 3 years’ duration. She had dull dragging pain and an occasional low-grade fever. On examination she had 20 × 20 cm mass with well-defined medial and inferior border. Both ultrasound and computed tomography scan showed a highly vascularized mass arising from her right adrenal gland but she had neither the constitutional symptoms of a functional adrenal tumor nor an abnormal biochemical test. Surgical resection showed a vascularized mass with attachments to the right lobe of the liver with a weight of 1900 g. It was found to be a giant solitary fibrous tumor of her right adrenal gland with infrequent mitosis. She stayed for 5 days after surgery and was discharged. She showed remarkable recovery at follow-up at 3 months.Although very rare, solitary fibrous tumor of the adrenal gland should be considered in differential diagnosis of adrenal masses. Solitary fibrous tumors (SFTs) are tumors of mesenchymal origin occurring mainly in the extremities but also in the pleura, meninges, and so on  . SFTs aThe presence of nuclear atypia, hypercellularity, greater than 4 mitosis/10 high-power field (HPF), and necrosis may be associated with, but not predictive of, aggressive clinical behavior. The current treatment is complete surgical resection , 3.A 13-year-old Oromo girl presented with a progressively increasing right-sided abdominal mass, low-grade intermittent fever, and a dull right upper abdominal pain of 3 years’ duration with no other associated symptoms. There were no known past illnesses and there was no family history of similar illness. She was given pain medications and antibiotics on various occasions but there was no improvement.Her general appearance was not acutely sick looking. Her vital signs were within normal limits. The pertinent abnormal finding was right-sided abdominal mass with well-defined medial and inferior border extending to right subcostal region. Complete blood count (CBC), urine analysis, and organ function tests were all normal. Ultrasound and a computed tomography (CT) scan demonstrated a huge vascular suprarenal mass displacing her right kidney caudally; it measurements were 16 × 19 cm and it contained multiple internal calcifications. There were no enlarged regional nodes and no vascular invasion.Laboratory tests for functional adrenal tumors including serum and 24-hour urine metanephrines were all normal.A working diagnosis of huge nonfunctional adrenal tumor was made and our patient underwent exploratory surgery through a bilateral subcostal incision. The operative findings were a well-capsulated and highly vascularized mass arising from the superior aspect of her right kidney, which got a significant blood supply from the right lobe of the liver. The mass was successfully and completely resected and the specimen subjected to histopathology.The histopathology report showed 18 × 15 × 12 cm white solid mass with necrotic center arising from right adrenal gland. There was a patternless proliferation of spindle cells and ovoid cells that had mild pleomorphic nuclei and focally hyalinized stroma containing blood vessels. Mitosis was seen infrequently. These findings were consistent with a SFT of the adrenal gland.Our patient was followed-up for 3 months and is doing remarkable well and members of her family were very grateful.This is the sixth or seventh case of its kind in the world to the best of our knowledge Figs. , and 4.FThis patient presented with a huge (18 × 15 × 12 cm) slow-growing right adrenal mass with no symptoms of a functional adrenal tumor. Adrenal cortical tumors are common but tumors greater than 20 cm in diameter are unusual. A larger nonfunctional adrenal mass measuring 32 cm in diameter with a weight of 1700 gram was reported by Li and colleagues from Department of Oncosurgery at Anyang Tumor Hospital, Henan, China .et al. on a patient who underwent investigation for fever of unknown origin and was later found to have SFT of adrenal gland [There was a report by Treglia al gland . Our patThe intraoperative finding of a very highly vascularized mass and histopathology finding of patternless proliferation of spindle cells fit with the expected finding of SFT .SFTs have a variable biological behavior but are mostly benign. A long history and histopathology report of infrequent mitosis as well as absence of invasion or metastasis on imaging and surgery suggest that it belongs to the benign end of the spectrum in SFT , 5. A poComplete surgical excision is the recommended treatment in SFT and that was done for our patient .SFT arising in an endocrine gland in general or adrenal gland in particular is rare and a Medline® search showed only a few case reports –8. All tet al. reported a very rare bilateral SFT of the adrenal gland in a 54-year-old patient with a bilateral adrenal incidentaloma [et al. reported SFT in 42 years old women discovered accidentally on imaging and the mass remained unchanged for 5 years before the she agreed for surgery [et al. reported a SFT incidentally discovered in the left adrenal gland of a 22 weeks pregnant 23-year-old woman [et al. also reported SFT in a 71-year-old Arab man [Most of the reported cases of SFTs of the adrenal gland were discovered incidentally. Toniato entaloma . Prevot  surgery . Bongiovld woman . The masld woman . Ho et aArab man , 11.Our patient is not only the youngest but also had the biggest SFT ever reported. This is the sixth or seventh case of its kind in the world to the best of our knowledge.Although rare, SFT should be considered in the differential diagnosis of nonfunctional adrenal mass.


We report the case of a 35-year-old male patient who complained of right anteromedial leg pain, after an intensive sport exercise. At physical examination, internal pretibial soft tissue swelling containing prominent painful varices was found. Color Doppler ultrasound, radiographic examinations, followed by CT and MR complementary investigation, were performed. We report the case of a 35-year-old male patient who complained of right, anteromedial leg pain after an intensive sport exercise. At physical examination, internal pretibial soft tissue swelling containing prominent painful varices was found. Color-Doppler ultrasound and radiographic examinations, followed by computed tomography (CT) and MR complementary investigations, were performed.Color-Doppler sonography revealed a plot of thrombosed pretibial varices. A venous course was found through a focal cortical defect of the anteromedial tibial cortex Figure .Conventional radiography showed a small subcortical osteolytic defect immediately beneath the pretibial varices, and an enlarged nutrient canal in the posterior tibial diaphysis Figure .CT images Figure  and 4 coThese findings were also present on the asymptomatic contralateral tibia. MR examination showed no signal intensity alteration within the adjacent muscular structures Figure .Varicose veins in lower extremities affect about 15% of world population.The anomalous communication between pretibial varices and an intraosseous tibial vein presented by our patient is a rare condition that has been reported a few times in the literature, and to our knowledge, the first reported case was bilateral. The physiopathology is not fully understood. Some authors hypothesize that intraosseous venous drainage anomaly may be the cause of varices or deep venous thrombosis and conversely the consequence of venous insufficiency .The diagnosis can be done using imaging alone. Color-Doppler ultrasound confirms the presence of the characteristic venous flow pattern in the varices through a cortical impression defect. CT and MRI are used to confirm the diagnosis, to exclude the presence of soft tissue or osseous mass and to rule out other vascular anomalies . DiffereDiagnosis of pretibial varices with intraosseous venous drainage anomaly can be made by using imaging alone. Recognition of this rare anomaly can prevent misdiagnosis, and it is pivotal in the planning of appropriate treatment.The authors declare that they have no competing interests.


Advanced imaging has led to an increase in the incidental diagnosis of pancreatic cysts. Serous cystadenomas (SCAs) account for nearly 30% of cases. These are typically considered benign lesions but up to 16% of cases are resected for aggressive behavior and symptoms.A 64 year old female presented with a large incidental cyst in the pancreatic body. Pre-operative imaging conferred a diagnosis of SCA. After 2 years the cyst grew resulting in abdominal pain. Enlargement was associated with splenic vein occlusion and varices of the short gastric vessels. This change in behavior was concerning for malignant transformation. The cyst was resected by distal pancreatectomy and splenectomy. The patient recovered with minimal morbidity. Final pathology revealed a 15.5 × 10.3 × 8.5 cm SCA with negative margins.In this case a patient presents with aggressive radiographic features and new symptoms suggesting malignant transformation of a previously diagnosed SCA on imaging. Although malignant variants can be diagnosed by findings of metastatic deposits at the time of surgery or as recurrence years later, histologic findings cannot discriminate benign from malignant potential. Preoperative imaging is challenging with erroneous characterization in nearly 60% of cases.Based on the low risk of malignancy, selective surgical resection for SCA appears warranted. Accepted indications for surgery include development of symptoms or concern for correct diagnosis in a medically fit individual. Routine resection for cysts >4 cm has been suggested however, prospective studies are needed to determine benefit over risk rationale. An extensive classification of cystic neoplasms of the pancreas and lesions that resemble them exists . Many of2A 64 year old female was found to have an incidental pancreatic body cyst measuring 10 × 8 × 9 cm on computed tomography (CT) during work-up for pneumonia. An endoscopic ultrasound (EUS) described a honeycombed appearance with multiple small cysts suggestive of a SCA . No aspiThe patient’s case was presented at the multidisciplinary tumor board. Based on increased growth, aggressive radiographic findings, and new symptoms, surgical resection was recommended. The patient was taken to the operating room by a surgical oncologist who specializes in pancreatic resections. Upon midline laparotomy, the gastrocolic ligament was divided and the lesser sac was entered to expose the left upper quadrant mass. The mass extended from the diaphragm superiorly to the inferior mesenteric vein at the ligament of treitz inferiorly. The mass abutted the portal vein- splenic vein confluence and displaced the splenic flexure of the colon caudad. An antegrade pancreaticosplenectomy was performed. Retroperitoneal dissection revealed significant varices as well as infiltration of the colonic mesentery requiring resection. No metastatic disease was noted. The patient’s post-operative course was complicated by a grade A pancreatic leak which resolved within seven days post discharge. At nine months, the patient has no evidence for recurrent disease and her symptoms have completely resolved.Grossly the tumor measured 15.5 × 10.3 x 8.5 cm. It was encapsulated with fluid filled cysts ranging from 0.1 to 1.3 cm in dimension. Histologic findings confirmed a microcystic SCA . Targete35SCA constitute up to 30% of pancreatic cystic neoplasms . These lHistologically, microcystic SCA are round, well circumscribed lesions most often identified in the tail of the pancreas. They contain multiple, small, thin-walled cysts filled with clear serous fluid which do not communicate with the pancreatic duct. The epithelium lining the cystic spaces is composed of uniform cells with pale cytoplasm rich in glycogen, without atypia or mitotic activity. Macrocystic and solid variants also exist. Histologically benign tumors are indistinguishable from malignant ones. The majority of cystadenocarcinoma are defined by findings of perineural or vascular invasion, or metastatic disease to the lymph nodes or liver at the time of resection, or as recurrence several years after the initial diagnosis . The metNon-operative management with or without serial imaging is dependent on having a secure diagnosis. The radiographic diagnosis of SCA can prove challenging. This can lead to erroneous characterization and needless surgery in upwards of 60% of cases ,10. SCA As SCAs are generally believed to be a benign entity, surgery has been historically reserved for symptomatic patients, and management has been largely observation. Symptoms associated with SCAs are mostly related to mass effect or to infiltration of adjacent structures with abdominal pain and presence of a palpable mass most commonly described . More coThe prevalence of serous cystadenocarcinoma reported since 1989 is 3% . MalignaGreater use of radiography and advances in techniques has led to an increase in the incidental diagnosis of pancreatic cysts . The dia4In conclusion, based on the low risk of malignancy, selective surgical resection for SCA appears warranted. Clear and universally accepted indications for surgery include associated symptoms, and/or a concern for the correct diagnosis in a medically fit individual . ModeratThis research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors.The authors do not have any conflicts of interest to disclose related to this work.This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors.does meet the definition of research, and such research requires IRB approval.” Given this is a case report (that does not report on more than 3 patient cases), IRB approval was not needed.According to the University of South Florida’s Institutional Review Board Policy and Procedure Manual “The USF IRB regards case reports or a limited case series as an educational activity, and therefore it is permissible under the Health Insurance Portability and Accountability Act (HIPAA) as a part of health care operations (45 CFR 164.501) when reviewing medical records. However, from both the Common Rule and the Privacy Rule perspective, a case series involving more than three (3) cases Written informed consent from the patient has been obtained.Pamela Hodul MD FACS: Conceptualization, review & editing, supervision.Lauren Pointer MD: Data curation, writing.Luke Rothermiel MD: Data curation, review & editing.Carolina Strosberg MD: Data curation, writing.Daniel Anaya MD FACS: Review & editing.N/A- this study did not require submission for approval from our local Institutional Review Board at the University of South Florida. Since, this met the definition of a case report as outlined in USF IRB’s policy and procedure manual as referenced above.Pamela J. Hodul MD, FACS.Not commissioned, externally peer-reviewed.


The genital system and skin involvement of diffuse B-cell lymphomas are quite rare. The appearance of these rare types in the same patient and the same period makes the treatment of the disease difficult. But both types respond well to anthracyclines and immunotherapies. A 74-year-old woman was treated with R-CHOP  without surgery and/or radiotherapy, and no recurrence at 2 years follow-up. Despite the poor prognosis of these types of lymphomas, treatment responses are quite good as they are in other subtypes. Malignant lymphoma accounts for 0.05% of uterine malignancies in women .We report the case of a woman who developed diffuse large B-cell lymphoma (DLBCL) on endometrium and on skin of lower extremity. Patient received six courses of R-CHOP and complete remission was achieved. It was observed that complete response was achieved for two fast-growing lymphomas.A 74-year-old postmenopausal female presented to dermatology outpatient clinic with the complaint of a mass on dorsum of the left foot and a mass on the middle part of the medial surface of the left lower extremity for a month. A tru-cut biopsy was obtained from malignant necrotic ecchymosis under antibiotic prophylaxis. Magnetic resonance imaging (MRI) of the pelvic and MRI of the lower extremity were performed. The results showed a mass obliterating the endometrium and a pathologic lymphadenopathy, measuring 30 × 15 mm, on the left inguinal region. Vaginal ultrasound was performed and adnexal atrophy and cysts in uterus were detected. Dilatation and curettage was performed on endometrium and patient was diagnosed with high-grade cancer. MRI of the lower extremity showed a heterogeneous mass, which is measuring 56 × 40 mm and is infiltrating proximal parts of third and fourth metatarsal bones and muscle and fat tissue on dorsal part of left foot. Positron emission tomography–computed tomography (PET-CT) was performed for stating. The results demonstrated a lesion, measuring 10.5 × 6.5 × 5.5 cm, extending from skin to extensor muscles and infiltrating proximal fourth and fifth phalanges in intermetatarsal spaces on plantar surface of the foot (SUVmax: 13.6). A primary lesion, measuring 25 mm in diameter, that is extending from medial calcaneus to subcutaneous tissue on proximal and one-third part of the middle part of left lower extremity (SUVmax: 26.5) and a second primary mass, measuring 45 × 28 mm, that is filling corpus of the uterus (SUVmax: 45.8) are noted. A metastatic lymph node, measuring 2 cm in diameter, is noted (SUVmax: 9.1) and2. Primary lymphoma of the endometrium is often detected by vaginal hemorrhage in elderly postmenopausal women without constitutional symptoms. Patients are treated with radical hysterectomy and lymph node dissection, followed by R-CHOP therapy. The 5-year overall survival rate is 39.3 %. In some cases, the complete remission rate of immunochemotherapy without surgery is 75%.Primary cutaneous B-cell lymphoma is often observed in elderly women. Lesions are present on head, neck, trunk, or lower extremity. Regardless of skin localization, MUM1/IRF4, bcl-2, and FOX-P1 are strongly expressed.i-67 in endometrial involvement were 60% positive.Two rare types of lymphoma detected in the same patient were treated with R-CHOP chemotherapy. A very good partial response was achieved at the end of three courses, and complete remission was achieved at the end of six courses. Considering that there was no residual disease and that sequelae effects of RT administered to the foot would adversely affect the quality of life, involved-field RT was not administered to the patient. In the literature, cutaneous or extracutaneous relapses have been reported frequently, despite good response to immunochemotherapy. In this case, there was no recurrence of both endometrium and skin for 2 years. Immunologically, B surface markers such as CD20, CD79a, Pax-5, and bcl-2 are frequently positive in diffuse cutaneous B-cell lymphomas.It is very rare to detect uterine corpus and cutaneous B-cell lymphoma, leg type, simultaneously in the same patient. During diagnosis, PET-CT and MRI examinations demonstrated involvements other than primary focus. Although both foci are fast growing histologically, it was observed that complete remission could be achieved with immunochemotherapy.


Angiosarcomas are mesenchymal neoplasms of vascular origin that represent approximately 2% of soft tissue sarcomas. We discuss the case of a 75-year-old female who had presented with a purple nodular rash along the bilateral nasolabial folds. Upon further work-up, she was diagnosed with angiosarcoma, with the confirmed involvement of multi-focal sites. These included biopsy proven sites of the face and duodenum along with the radiographic involvement of the lungs, liver, and osseous tissue. We report this unique presentation of a rare malignancy and the treatment course with radiation, paclitaxel, and bevacizumab. We also discuss the implications of her co-morbid liver cirrhosis and gastric antral vascular ectasia (GAVE) in terms of its influence on the development of the angiosarcoma and treatment response. Angiosarcomas are rare mesenchymal neoplasms of endothelial cells which fall under the umbrella of soft tissue sarcomas. The annual incidence of soft tissue sarcomas in the United States is estimated to be 10,000 cases per year . AngiosaHere, we report a unique case of metastatic angiosarcoma with the involvement of the skin and small bowel, and have discussed the implications of comorbidities including cirrhosis and gastric antral vascular ectasia (GAVE).A 75-year-old Native American female presented to dermatology with a ‘port wine’ purple nodular rash on her nasolabial folds, of 12 months duration  imaging demonstrated right face superficial angiosarcoma without the invasion of deep tissues Figure , bilaterThese findings confirmed the angiosarcoma of the face with multiple synchronous sites, including the duodenum. The patient was presented at the multidisciplinary tumor board where it was recommended that she receive palliative radiation for local control of the lesion on the face/nasolabial folds. She was also started on systemic therapy with intravenous paclitaxel as per the Phase II Trial of Weekly Paclitaxel for Unresectable Angiosarcoma (ANGIOTAX) study .She experienced some treatment delay in between chemotherapy cycles due to radiation-induced myelosuppression. Side effects were monitored closely, given her history of cirrhosis and baseline bicytopenia. She also experienced grade 2 cutaneous toxicity from radiation therapy including painful erythematous lesions in the mouth and over the lips as well as blepharitis. Post-radiation skin changes on the face eventually healed well, following completion of radiation. She did not experience any paresthesia with paclitaxel.Imaging following two months of systemic chemotherapy revealed interval progression of hepatic and vertebral metastases Figure .Due to disease progression, paclitaxel was discontinued and treatment was changed to second-line bevacizumab based on the available data from a phase II trial . FollowiAngiosarcomas are tumors arising in either the blood vessels or lymphatics. The scalp and face are the most common sites of origin . They arThe involvement of the small bowel is even more uncommon and is associated with worse outcomes as compared to cutaneous angiosarcomas. A recent literature review of previously published data examining small bowel angiosarcomas concluded that survival with intestinal involvement is less than one year and confers an extremely poor prognosis . A retroIn our patient, due to synchronous presentation, it is not possible to determine whether the cutaneous lesion or the duodenal lesion was the primary tumor. A retrospective review of the published literature suggests that a majority of small bowel angiosarcomas are primary tumors. It was also noted that the likelihood of metastases to the intestine was the highest when the primary sites of the tumor were the skin or aorta . The preSome data have suggested that the benign vascular lesions may be a risk factor for malignant transformation into angiosarcoma . In thisOur patient also had cirrhosis deemed secondary to NASH or cryptogenic cirrhosis upon prior investigation. Exposure to vinyl chloride has been implicated as a risk factor for hepatic angiosarcoma and cirrhosis -15. It iIn the context of treatment, complete excision with wide margins is the treatment of choice for locally invasive disease. However, cutaneous angiosarcomas have been associated with high rates of local recurrence . Due to Though the incidence is low, their aggressive behavior and poor prognosis make angiosarcomas a threatening diagnosis that should be identified and treated as soon as possible. The risk factors that should alert a clinician to look for an angiosarcoma are many. They include radiation therapy, chronic lymphedema, environmental exposures, ultraviolet light, arsenic, anabolic steroids, foreign bodies, benign vascular lesions, and arteriovenous fistulas. This malignancy is also commonly associated with xeroderma pigmentosum, familial syndromes, retinoblastoma, and previous primary malignancy. The prognosis for a metastatic disease remains in evolution with the availability of multiple agents for targeted therapy such as imatinib, sorafenib, and bevacizumab.


This case reminds us that congenital abnormalities of the genito-urinary tract should be considered in case of urinary incontinence and recurrent urinary tract infections.The presence of an ectopic ureter may be indicated by continuous wetting, despite a normal voiding pattern, especially in girls. In most cases, an ectopic ureter is associated with a duplex collecting system and complete ureteral duplication. A 5-year-old girl presented with urinary incontinence regardless of the successful toilet training and a suspicion of left duplex kidney on a previous ultrasound. Contrast-enhanced computed tomography revealed a double left kidney with double ureters, both inserting together into the vagina. The surgical treatment consisted in the “en block” reimplantation of the ectopic ureters into the bladder, with complete resolution of the symptoms. The reported case does not represent just a typical presentation of a single ectopic ureter, as the duplex kidney system had  A constant urinary leakage is a common problem during childhood, but generally there is no underlying abnormality of the urinary tract. Urinary incontinence is the main symptom of an ectopic ureter, especially in females, while in both sexes, an ectopic ureter may present as a prenatal diagnosis because of a urinary tract infection (UTI), or congenital obstructive uropathy . These tIn most cases, the ectopic ureter originates from the upper renal pole of the duplex-system (80%) while single-system ureter ectopia is exceptional 20%). Compared to the ectopic ureter of the duplicated-system, diagnosis of the single-system ectopia can be delayed because it usually drains small dysplastic and hypofunctional kidneys, which may be difficult to identify with conventional imaging, like intravenous urography and urethrocystoscopy  since her infancy. At the age of 3 she underwent an abdominal ultrasound that suspected a double left kidney. A voiding cystourethrogram (VCUG) was performed and a vesicoureteral reflux (VUR) grade III on the right kidney was found. She was given chronic chemoprophylaxis without any UTI recurrence. She had a good toilet training and gave up daytime diapers around the age of 3. At this moment parents noticed the urinary incontinence, but this didn't bother them. At the age of 4 parents asked a urologist for help, who considered the symptoms to be the result of an overactive bladder and anticholinergic treatment was recommended. No improvement was noticed, and for this reason the little girl was sent to our department for further investigation.Complete blood count, biochemical tests, and urinalysis were all normal and the urine culture was negative.An abdominal ultrasound was performed and both kidneys had normal parenchyma and size, with a duplex-system suspicion on left side; the bladder was normal in appearance. The VCUG was repeated but no VUR was visualized. As we had a high suspicion of an ectopic ureter a contrast-enhanced computed tomography (CT) of the abdomen and pelvis was performed to visualize the entire urinary tract. The CT scan was performed with a 64 row MDCT system (Siemens Somatom Definition AS). The 3D images revealed important data about the entire collecting system and the ureters, namely a duplicated collecting system on the left side and subsequently both ureters extending from the kidney to the point of extravesical insertion into the vagina. The ectopic ureters were in very close vicinity Figures –3.In the evaluation of urinary incontinence in children of this age a differential diagnosis should be considered with the following: UTI, chronic kidney disease, diabetes, overactive bladder, ectopic ureter and vesico-vaginal fistula in case of a history of pelvic surgery.This case represents a rare congenital anomaly of the genito-urinary system. An ectopic ureter is defined as a ureter that does not insert into its normal anatomical position. The occurrence of ectopic ureters is 1/2,000 in newborns and 1/2,000–4,000 in general population. Gender ratio is 2–6:1 in favor of females  but may drain medially and superiorly to it (violating Weigert-Meyer law).Sometimes, other abnormalities like renal dysplasia and non-genitourinary anomalies  can be associated with an ectopic ureter , 8. In oIn assessing any child, particularly girls, with continuous urinary incontinence, accurate personal medical history and physical examination are mandatory.In all cases imaging studies are mandatory to confirm the diagnosis , 11. In When ectopic ureter insertion is located in the vestibule or vagina, it causes incontinence and rarely obstruction, making its detection difficult . In a reSymptoms, renal function, patient's age and life quality will be the ones deciding the management of an ectopic ureter. In adults, conservative treatment represents an appropriate option, but does not resolve the incontinence problem and has possible long-term risks. It consists in using different types of pads according to the leakage volume . The besWhen there is a preserved kidney function or a functioning upper pole in duplex system kidney, the surgery technique consists on ureteroneocystostomy or distal and proximal ureteroureterostomy respectively . In a reExcellent results are reported in pediatric urology using robot-assisted laparoscopic ureteroureterostomy, that provides a safe and efficient approach to manage ectopic ureters in duplicated collecting system .The surgical management of ectopic ureter depends on surgeon experience and preference, laparoscopic experience, pediatric material investments. Since the two ureters could not be separated, an “en bloc” ureteral reimplantation using the open “Cohen ureteroneocystostomy” was performed in this case. The urinary dribbling resolved postoperatively. On follow-up our patient had no UTI episode.In conclusion, the ectopic ureter should be keep in mind in case of persistent wetting and reccurent urinary tract infections.The exceptionality of this case, of which there are not many published, consists in the duplicated collecting system having ectopic both ureters. To the best of our knowledge, this is the first report of ipsilateral double ectopic ureter openings in children with concomitant preserved kidney function.A history of continuous wetting can be difficult to apprise in children.A complete physical exam may be the diagnostic key for urinary incontinence.The differential diagnosis of urinary incontinence in toddlers, especially girls, should include ectopic ureter.This case report does not require a committee approval. This report does not include any identifiers of the patient to protect patient confidentiality. Written informed consent was obtained from the patient's parents with their agreement to the publication of this case report.CD and IS drafted the article and acquisition and interpretation of data; EK revised it critically for important intellectual content; CA critical revision and final approval of the version to be published.The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.