Nerve root metastasis without extension from an adjacent process has been seldom reported in case of solid tumors. We describe a case of solitary nerve root metastasis of gastric adenocarcinoma, likely due to hematogenous spread.A 75-year-old man presented with radiculopathy refractory to medical treatment. MRI and CT demonstrated a right-sided S1 nerve root mass involving the spinal ganglion in its intra-foraminal region with avid enhancement, initial erosive bone changes on sacral foramina and focal hyperaccumulation on 18F – FDG CT-PET, suspicious for metastasis. The histopathological examination confirmed a metastasis of gastric adenocarcinoma.A review of the current literature revealed only ten cases of hematogenous metastases to spinal nerve root ganglia; the primary lesions in those cases were an oat cell carcinoma of the lung, two cases of colonic adenocarcinoma, a case of uterine adenocarcinoma, a ductal breast carcinoma, a Ewing’s sarcoma, a Renal Cell Carcinoma, a gastro-intestinal stromal tumor, a follicular thyroid carcinoma, a pulmonary adenocarcinoma.In the setting of a known malignancy, a nerve root metastasis should be considered in the differential diagnosis of a nerve root mass, although it occurs very rarely. Nerve2On May 2018, a 75-year-old man was referred to our Department due to an increasing low-back pain and right-sided radicular pain irradiating down to the right lower limb, along the posterior thigh and the postero-lateral aspect of the lower extremity up to the lateral aspect of the foot, numbness in the sole of his right foot, especially marked on the plantar surface of his toe (VAS 8); the patient’s pain was refractory to conventional medical treatment. He complained also with a subjective decrease in strength in his right lower limb; his walk was slow and difficult due to the pain.On clinical examination hypoesthesia at the right S1 and S2 dermatome areas and moderate muscle weakness in the right lower limb (3–4/5) were observed, reflexes were bilaterally normally elicitable and plantar response was flexor.2 and Capecitabine 825 mg\mq2. In clinical and radiological follow-up, the last on December 2017  and reconstruction by mean Roux-en-Y anastomosis 2 years before (on January 2016) due to a gastric adenocarcinoma in the subcardial region; the histopathological diagnosis was poorly-differentiated gastric adenocarcinoma of intestinal type and 4/14 lymphnodes were positive for metastatic disease . Before surgery (January 2016), he underwent a whole body CT scan using a first-generation 640-slice CT scanner , that showed the subcardial neoplasm measuring about 25 × 15 mm  without ber 2017 a, no locHis past medical history also included a L1 vertebral body fracture 3 years before (on July 2015), requiring a lumbar MRI (Magnetic Resonance Imaging) performed on a 1.5T General Electric Medical System a, with sOn May 2018, patient underwent a lumbar MRI using a 1.5T General Electric Medical System, that showed a significant enlargement of the intraforaminal right S1 nerve root due to the presence of a right-sided S1 nerve root mass involving the spinal ganglion in its intra-foraminal region and growing along the spinal nerve root, measuring about 25 × 20 × 25 mm; the mass showed homogeneously hypo-intensity signal on SE T1-weighted images  CT-PET h that shHe also underwent a whole body CT scan using a first-generation 640-slice CT scanner , that confirmed the presence of the slightly hypervascular right-sided S1 nerve root mass b–d and sA fine-needle aspiration biopsy (FNAB) was performed and the histologic examination confirmed a metastasis of gastric adenocarcinoma f.Following interdisciplinary discussion, the patient was referred for radiotherapy; radiation to the right S1 nerve root mass was performed to relieve the local pain and a total dose of 16 Gy in two fractions was given. The patient reported a reduction of pain intensity (VAS 6) and muscle weakness in the right lower limb (2+/5).3Although metastatic involvement of peripheral nerves by perineural invasion from an adjacent cancer is not uncommon, especially in the context of head and neck tumors , nerve rStomach cancer is the fifth most frequently diagnosed cancer and the third leading cause of cancer death, is responsible for over 1.000.000 new cases in 2018 and an estimated 783.000 deaths  . GastrecWe report the case of a patient with S1 nerve root metastasis of gastric adenocarcinoma. To our knowledge, only ten cases of metastases to spinal nerve root ganglia, arising from distant solid tumors, have been reported in the literature, our case is the eleventh reported case of spinal nerve root metastasis  and the Various routes of dissemination have been proposed, which include direct invasion , lymphohematogenous , spinal fluid and retrograde endoneurial spread . Our casA review of the current literature revealed only ten cases of hematogenous metastases to spinal nerve root ganglia; the primary lesions in those cases were an oat cell carcinoma of the lung, two cases of colonic adenocarcinoma, a case of uterine adenocarcinoma, a ductal breast carcinoma, a Ewing’s sarcoma, a RCC  ,14, a gaClinically, nerve root metastases mimic radiculopathy and/or cauda equina syndrome, with the earliest symptoms being pain and weakness, followed by sensory loss, and bowel and bladder dysfunction . AccordiNerve root metastases manifest on CT/MRI as thickening of involved nerve root with contrast enhancement and, sometimes, erosive changes in the adjacent bones ,14,15,18The radiological appearance of nerve root metastasis might mimic that of a peripheral nerve sheath tumor (PNST) , which rTumors of the peripheral nerves are relatively common; these tumors arise from either the neuronal tissue or from the cells of the neural sheath . PNST caRadiological appearance of MPNST is non - specific, however these lesions tend to be isointense to muscle in T1 weighted-sequences, while in our case the metastatic lesion was hypointense to muscle in T1 weighted-sequence. Both MPNST and metastases show hyperintensity on T2 weighted-sequences, avid contrast enhancement with increased metabolic activity on F-18 FDG-CT-PET .Although differential diagnosis among MPNST and benign PNST is often challenging, some MRI features can be helpful. Differently from benign PNST, MPNST usually tend to be larger, to show less-defined margins and a more inhomogeneous contrast enhancement ,20.Presence of cystic or hemorrhagic areas within the lesion and perilesional edema are more typical of malignant lesion; moreover benignant lesion are rarely larger than 5 cm and characterized by a more homogeneous signal on MRI sequences .A central hypointense region on T2-weighted images (target sign) is typical in neurofibromas, that show often focal central enhancement while widespread and-or peripheral enhancement is more typical of malignant neoplasms .Lastly, bone involvement, often with an erosive pattern, is highly suspicious for malignancy , as conf4Our case shows that, in the setting of a known malignancy, the possibility of a nerve root metastasis should be considered in patients with history of gastric cancer and increasing radicular symptoms, especially in case of refractory pain.No conflict of interest.No source of funding.This study was approved by the Research Ethics Committee of the University of L’Aquila.The authors obtained patient consent to use all the images presented.Alessandra Di Sibio, Lucia Romano, Antonio Giuliani, Marco Varrassi: Writing the paper.Maria Carmela De Donato, Antonio Iacopino, Marco Perri: Data collection and analysis.Mario Schietroma, Francesco Carlei, Ernesto Di Cesare, Carlo Masciocchi: Study concept.Alessandra Di Sibio, Lucia Romano, Antonio Giuliani, Marco Varrassi, Maria Carmela De Donato, Antonio Iacopino, Marco Perri, Mario Schietroma, Francesco Carlei, Ernesto Di Cesare, Carlo Masciocchi: Critical revision.N/A.Prof. Ernesto Di Cesare.Not commissioned, externally peer-reviewed.


Somatostatinoma is a rare neuroendocrine tumor with an incidence rate of 1 in 40 million people. It presents mostly as asymptomatic tumor diagnosed incidentally on imaging or surgery when evaluating or treating possible causes of abdominal pain. It also can present with vague symptoms, or as a clinical triad of glucose intolerance, steatorrhea, and achlorhydria. The majority of somatostatinomas are present in the pancreatic head, followed by the duodenum, the pancreatic tail, and rarely the ampulla of Vater. The prognosis is poor as more than 77% of cases present as advanced disease with local invasion or distant metastasis. Surgical resection is the main treatment for early stage disease. Other treatment options include somatostatin analogue, molecular targeted therapy, and cytotoxic chemotherapy. The scarcity of somatostatinoma cases led to the lack of fully formulated treatment options. Herein, we present a 43-year old male patient who was referred by his primary care physician to our gastroenterology clinic due to elevated liver function test and double-duct sign on CT scan. We performed an ERCP, which revealed 2 cm ampullary lesion with upstream obstruction. Biopsies were taken and histopathology was unrevealing. He underwent a laparoscopic pancreaticoduodenectomy with histopathology revealed stage IIb somatostatinoma. Treating physicians should hold a high index of suspicion and maintain a broad differential diagnosis of elevated liver enzymes. Somatostatinoma is a rare neuroendocrine tumor with an incidence rate of 1 in 40 million people. It presents mostly as asymptomatic tumor diagnosed incidentally on imaging or surgery when evaluating or treating possible causes of abdominal pain. It can also present with vague symptoms, or as a clinical triad of glucose intolerance, steatorrhea, and achlorhydria. Somatostatinoma mostly involves the pancreatic head followed by the duodenum.Due to vague symptomatology it presents in >77% of cases as advanced disease with local invasion or distant metastasis. The rarity of the disease led to lack of fully formulated treatment protocol, with surgical resection, somatostatin analogue, molecular targeted therapy, and cytotoxic chemotherapy being available options. The prognosis is poor in advanced diseases.This is a 43-year-old white male patient with past medical history of hypertension and obesity. He was referred to the gastroenterology clinic from his primary care physician due to elevated liver function test found on routine annual physical exam. At the time of evaluation he had no complaint, and he denied any abdominal pain, nausea, vomiting, jaundice, weight changes, skin lesions, or discoloration. He also denied any prior history of illicit drug use, high risk sexual behavior, sexually transmitted diseases, or family history of liver or autoimmune diseases. His physical examination did not reveal any skin lesions suspicious for neurofibromatosis-1.His initial workup revealed a random glucose level of 85 mg/dl, calcium level 9.5 mg/dl, total bilirubin 1.3 mg/dl, direct bilirubin 0.6 mg/dl, alkaline phosphatase 640 U/L, AST 220 U/L, ALT of 494 U/L, total protein 7.1 g/dl and albumin 4.6 g/dl. Further workup revealed negative viral hepatitis panel, antinuclear antibodies, anti-mitochondrial antibodies and anti-smooth muscle antibodies. Iron studies, ceruloplasmin and alpha-1 antitrypsin were within normal limits.Abdominal ultrasonography revealed normal liver size and echotexture with no lesions, intrahepatic biliary duct dilatation, common bile duct dilatation 18 mm, contracted gallbladder with no cholelithiasis and no discrete pancreatic mass or ductal dilatation were identified. Magnetic resonance image/magnetic resonance cholangiopancreatography (MRI/MRCP) was performed and revealed moderate to severe intrahepatic and extra hepatic biliary ductal dilatation with mild enhancement of the distal common bile duct without choledocholithiasis or discrete mass, and pancreatic duct dilation with normal pancreas .An endoscopic retrograde cholangiopancreatography (ERCP) was performed and revealed a large ampullary mass distorting the anatomy, which precluded cannulation of the common bile duct . During The patient underwent laparoscopic pancreaticoduodenectomy (Whipple's procedure) with regional lymphadenectomy. Histopathology was consistent with 1.3 cm ampullary neuroendocrine tumor infiltrating the mucosa, submucosa and muscularis propria with angiolymphatic invasion. Immunohistochemical stains were positive for somatostatin, and negative for gastrin, serotonin and pancreatic polypeptide . There wThe post resection surveillance at 3, 6, and 12 months revealed a normal history and physical examination, a fasting somatostatin level of 41, 28, and 19 pg/ml, respectively, and a CT scan abdomen and pelvis with no evidence of recurrence. He is still under yearly surveillance with normal history, physical examination and fasting somatostatin level for the last four years.D or delta cells are neuroendocrine cells located in the gastrointestinal (GI) tract and pancreatic islets of Langerhans that secret somatostatin . SomatosSomatostatin was initially analyzed from the hypothalamus of sheep in 1973 and was later found to be present in the pancreas, lung, adrenal and thymic tissue . SomatosThe size and location of the tumor has a major influence on the severity and nature of symptoms . The majSomatostatinoma D cells are located in the submucosa and therefore the yield of the endoscopic biopsies is low. Only 60 to 83% of the biopsies have a positive yield for diagnosis . HistopaDue to the ambiguity of symptoms at presentation of the somatostatinoma, many of the tumors are diagnosed late. The vast majority of the cases (77%) were diagnosed after distant metastases or local invasion, with the liver being the most common site of invasion 42%). Other areas of tumor invasion are accounted by the lymph nodes (39%), duodenum (13%), spleen (6%), common bile duct, colon, stomach, left kidney, bone and pancreas [%. Other The scarcity of somatostatinoma cases led to the lack of fully formulated treatment options. Surgery is still the preferred treatment for smaller tumors of less than 2x2 cm; however, 70-92% of cases present with advanced disease for which surgery is not an option . Larger Somatostatinoma is a very rare neuroendocrine tumor. We reported this case to emphasize the importance of holding a high index of suspicion and maintaining a broad differential diagnosis of elevated liver enzymes. Unfortunately, there is no standardized treatment regimen so far, and the overall survival is poor due to the advanced stage on presentation.


Benign lymphoid polyps are uncommon lesions of the small bowel and the colon to a lesser degree that are mostly found in children. There are only few reported cases in adults in which the lesions were predominantly polypoid and described as lymphonodular hyperplasia. We present a case of a large benign lymphoid polyp in the transverse colon of a 64-year-old lady who was referred to our care for a history of alteration in her bowel habit and anemia. Colonoscopy showed a 3 cm (Paris 1p) friable polyp which was excised and retrieved. Histopathology examination confirmed its benign nature supported by immunohistochemical studies. Benign lymphoid polyp is a rare condition posing a diagnostic challenge as it can be misinterpreted as a malignant lesion. Benign lymphoid hyperplasia in the colon is a rare condition. It can present as a single polyp or multiple polypoid lesions. In order to avoid unnecessary intervention or surgery, it is vital yet challenging to differentiate these lesions from malignant lymphoma. This requires careful histological examination and special staining.A 64-year-old lady was referred to the Gastroenterology Clinic for Colonoscopy. She presented with a history of altered bowel habit and iron deficiency anemia. There was no history of abdominal pain, bleeding, or constitutional symptoms. Her past medical and surgical history was unremarkable except for newly diagnosed diabetes. There was no family history of inflammatory bowel disease or gastrointestinal malignancy. Examination revealed an overweight patient but was otherwise noncontributory. Blood tests confirmed iron deficiency anemia.Colonoscopy showed a large friable pedunculated polyp (Paris 1p) approximately 3 cm in size in the transverse colon . The polHistopathology examination revealed a polyp partially covered by colonic mucosa with areas of erosions and granulation tissue formation. The body of the polyp was composed of hyperplastic lymphoid tissue with multiple enlarged lymphoid follicles and prominent germinal centers. These lymphoid follicles were well-spaced and variably sized and shaped Figures  and 4, a et al. documented eighteen cases of rectal lymphoid hyperplasia, eleven of which were polypoidal lesions, three were sessile polyps, and two were nodular lesions [ et al. presented a case of a 52-year-old lady who had a large cecal polypoidal mass, measuring 8 cm in size, with severe lymphocytic infiltration and lymphoid follicles. Patient underwent a right hemicolectomy due to potential malignancy; however, histopathological analysis postoperatively confirmed the diagnosis of benign lymphoid hyperplasia [Colonic lymphoid hyperplasia is a rare condition especially in adults. It can be localized or diffuse nodular hyperplasia or to a lesser extent a solitary polyp . Polyps  lesions . While s lesions –6. In Ho lesions . Furthererplasia . Helicobacter pylori infections were reported to be linked to lymphoid hyperplasia in the gastrointestinal tract [There is an association between lymphoid hyperplasia and immunodeficiency conditions such as common variable immunodeficiency disease, human immunodeficiency virus, and selective IgA deficiency. Also, Giardia andal tract .Differentiating between benign and malignant polyps is challenging and sometimes requires using ancillary studies such as immunohistochemical analysis and monoclonal immunoglobin gene rearrangement . In the Colonic lymphoid polyps are rare in adults and can be misdiagnosed as malignant lymphoma. Presentation is variable; patients can be asymptomatic or present with complications requiring surgical intervention. Histological examination with immunohistochemical analysis is essential to differentiate lymphoid hyperplasia from lymphoid malignancy.


The Calcifying Odontogenic Cyst (COC) is a simple cyst lined by ameloblastoma-like epithelium with ghost cells. The peripheral COC is a rare lesion and few reports have been published considering its clinical and histopathological features. This article aimed to report on a case of a peripheral COC, discussing its clinical, imaginological and histopathological features. A 9-year-old male patient presented a 10x5 mm painless nodule in the palatal mucosa of the left central incisor. Panoramic, occlusal and periapical radiographs did not show alterations. A computed tomography exam showed a slight soft tissue swelling located in the palatal mucosa of the left maxillary central incisor. An excisional biopsy was performed. The histopathological analysis showed a cystic lesion adhered to an oral mucosa fragment and lined by an ameloblastoma-like epithelium with ghost cells. The diagnosis of peripheral COC was established and the patient has been disease-free for 5 years. Although rare, peripheral COC is an important lesion that should be considered as a differential diagnosis of gingival hyperplastic lesions. Key words:Calcifying odontogenic cyst, odontogenic tumors, peripheral calcifying odontogenic cyst. The caset al.  affectedMost of the reported peripheral COC developed as painless, well-delimited and smooth-surface nodules on the gingiva or alveolar mucosa ,6. The let al. (The histopathological analysis of the reported case disclosed typical findings of COC such as the fibrous connective capsule lined by an ameloblastoma-like epithelium and the presence of ghost cells, as well as areas of dentinoid formation in the capsule -10. Moreet al. .Conservative surgical excision is the standard treatment for peripheral COC and recurrences are rare -7. This Although rare, peripheral COC is an important lesion that should be considered as a differential diagnosis of gingival hyperplastic lesions.


Varicocele is characterized by the dilatation of the veins of the spermatic cord. Its prevalence in general male population is 15% while in the infertile population the prevalence rises up to 25%. The varicocele is considered an etiological factor for male infertility. Although different pathophysiological patterns have been proposed, there is no consensus in the urological society to date. In most of the cases varicocele is asymptomatic but sometimes gives mild symptoms as dull pain at the scrotal region. A rare complication of this condition is the spontaneous or traumatic rupture and hematoma formation, either as spermatic cord hematoma or as scrotal hematoma. We are presenting two cases of varicocele rupture, presented with acute painful swelling of the left inguinal and scrotal region during sexual intercourse. Imaging studies revealed a scrotal hematoma in the first case and a spermatic cord hematoma in the second case, without signs of active bleeding. Both patients were treated conservatively and recovered uneventfully. Subsequently, we reviewed the literature in an effort to find the key points for the diagnosis and treatment of this condition. Acute scrotal swelling is a potential emergency. A rare cause of scrotal swelling is the spontaneous or traumatic rupture of varicocele. Timely diagnosis of varicocele rupture is challenging as the symptoms are nonspecific and resemble other conditions such as torsion of the testis or one of the testicular appendages, hydrocele, trauma, tumor, idiopathic scrotal edema, and incarcerated inguinal hernia . AlthougA 22-year-old male presented to the emergency department with painful swelling of the left hemiscrotum during sexual intercourse. The pain had sudden onset and was located at the left inguinal region. The patient noticed an extending swelling from the left inguinal area through the left hemiscrotum. The medical history revealed bilateral varicocele but no comorbidities or coagulation disorders. On examination, he had a painful swelling starting from the inguinal canal and extending through the entire left scrotum. A soft mass was palpable around the left testis and the spermatic cord. Laboratory tests were within normal range.Doppler ultrasonography of the scrotum revealed a hematoma extending from the left superficial inguinal ring to the left hemiscrotum, partially surrounding the left testis. No active bleeding was observed . Both teThe patient was treated conservatively with bed rest, ice packs, antibiotics, and analgesics. During the hospitalization he was stable, the pain gradually decreased on the second day and the patient was discharged the third day. Ecchymosis of the scrotum was noticed at the first hospitalization day which gradually disappeared along with the swelling after 4 weeks. Bilateral varicocele repair was uneventfully performed three months later.A 24-year-old male presented to the emergency department due to left inguinal-scrotal pain which occurred acutely during sexual intercourse. The patient was otherwise healthy without comorbidities or hematological discrepancies. During the physical examination, a mild swelling of the left inguinal region expanding towards the left testis was observed. On palpation, a normal feeling testis but a thickened and painful spermatic cord was found. Laboratory tests were normal.Doppler ultrasonography of the scrotum revealed a spermatic cord hematoma expanding towards the upper pole of the left testis, with no active bleeding . Left vaThe medical term varicocele describes the dilatation of the scrotal portion of pampiniform plexus and the internal spermatic venous system . Its preSpontaneous or traumatic rupture of the varicocele is a rare complication. Most commonly, causes of scrotal or spermatic cord hematoma are blunt trauma , ValsalvThe clinical presentation of the cases reported in the literature varies considerably. In our cases the patients presented to the hospital immediately after the onset of the pain, while in other cases the patients seek medical care up to 7 days after the causative event . The ageThe symptoms also varied considerably. In our cases, the first patient had severe pain and discomfort while the second presented with only mild pain and moderate discomfort. In the cases reported in the literature, patients experienced different degrees of pain, which in some cases was quite mild. The leading cause for referral was the scrotal swelling .The clinical findings though were comparable. In the cases reported, as in our own experience, there was a noticeable enlargement of the hemiscrotum extending to the inguinal canal. In most cases there was a thickened spermatic cord and a palpable scrotal mass, but the consistency of the mass differed from fluctuant to firm , 24. TheIn our facility we routinely use Doppler ultrasonography to evaluate acute scrotal conditions. The same approach was used in the two cases. The examination showed normal blood flow to the testis, thus excluding ischemic conditions, successfully identifying the scrotal, spermatic cord hematoma. Bilateral varicocele was confirmed in the first case and left side varicocele was identified in the second. In the first case the diagnosis was confirmed the next day with an MRI study of pelvis and scrotum. Ultrasound of the retroperitoneal space was performed in both cases to exclude a retroperitoneal hemorrhage presenting as scrotal hematoma .In most cases reported to date, imaging techniques were used to identify and to differentiate the cause of the acute scrotum. The Doppler ultrasonography was the most frequently used technique, although some physicians used abdominopelvic CT study , 24 and The type of treatment depends on patient's status, exclusion of the acute surgical conditions  and improvement of the symptoms. In our cases, considering that there was no absolute indication for surgical management, the treatment was conservative. Both patients were treated with NSAIDs, empiric antibiotics, bed rest, and ice packs, during and after hospitalization. The treatment strategies in the reported cases in literature were conservative when the clinical and imaging findings suggested scrotal hematoma , 24, 26,In cases where testicular torsion or incarcerated inguinal hernia was suspected or in presence of hemodynamic instability the first treatment option was surgery , 20, 25.Varicocele rupture is a rare complication of a common condition. High suspicion and careful evaluation of the patient is needed. Due to limited number of cases reported in the literature, it is difficult to draw a conclusion. With respect to the sparse evidence, some comments can be made. Scrotal hematoma is a difficult clinical diagnosis with no specific signs or symptoms. Imaging has an irreplaceable role in both differential diagnosis and identification of the spermatic cord, scrotal hematoma. Doppler ultrasonography seems to be sufficient in most cases, but it can be completed with a CT or MRI study if needed. The treatment depends on the patient's condition and the diagnostic certainty. The scrotal hematoma seems to be easily treated conservatively, but surgical exploration is the safest approach in case of uncertain diagnosis.


The odontogenic keratocyst (OKC), previously known as keratocystic odontogenic tumor has been the most disputable pathologies of the maxillofacial region. Patients with OKC are often asymptomatic but may present with pain, swelling, or discharge. Despite the aggressive nature, previous literature as early as 1970s reported the fact that parakeratinized OKC can be treated by means of marsupialization alone.The patient had reported with a complaint of pain and swelling in relation with a tooth in mandibular right quadrant.This case report discusses features of a rare, extensive, panmandibular OKC that is only second of its kind mentioned in the literature.As a usual treatment protocol, marsupialization was attempted first. Immunohistochemical analysis revealed reduced expression of Ki-67 and B cell lymphoma 2 (bcl-2) markers after marsupialization from 2 separate sites. However, due to incomplete resolution in the lower right anterior region, an aggressive approach was taken by curetting it out surgically along with associated teeth and cortical plate followed by application of Carnoy's solution.Postsurgery uneventful healing of the lesion was noted on regular follow-up visits with complete resolution at 40 months. The case has been followed for 10 years with no sign of relapse and reoccurrence.Based on the expression of markers it can thus be concluded that Ki-67 and bcl-2 are site specific and bear strong relationship with the recurrence of OKCs. Initially it was mistaken for a primordial cyst. Later its aggressive clinical behavior and tendency for recurrence literally detached its association from the category of cysts and it came to be known as an intraosseous benign neoplasm, keratocystic odontogenic tumor (KCOT) . Ironically, there were reports of OKC responding to marsupialization and hence the revised classification by WHO, published in 2017, reclassified the OKC as a cystic lesion instead of benign intraosseous neoplasm, KCOT. In spite of extensive research, we have still not arrived at a consensus as to the behavior of this obscure entity. Approximately one-half of all keratocysts occurs at the angle of the mandible and extend for varying distances into the ascending ramus and forward into the body. In many instances, patients are remarkably free of symptoms until the cysts reached a large size and involved the maxillary sinus and the entire ascending ramus, including the condylar and coronoid processes.The odontogenic keratocyst (OKC) has been a standout amongst the most disputable pathological entities of the maxillofacial region since the mid of 20th century. characteristic palisaded pattern with uniform nuclei are seen in the basal cell layer. An important feature of OKCs being the daughter cysts which are formed by budding of basal layer into the surrounding connective tissue. The fibrous cyst wall is relatively thin and usually lacks inflammatory cell infiltrate.It's typical histological highlights incorporate a thin parakeratinized stratified squamous epithelium, roughly 5 to 8 cells thick, secured by a thin ridged layer of parakeratin These marked differences are believed to be identified with the distinctive duration of postoperative follow-up periods, surgical procedures utilized, or incorporation of cases with nevoid basal cell carcinoma disorder (NBCCS).There has been reported recurrences ranging from 0% to 100%. have found a recurrence rate of around 17% to 56% when treated by simple enucleation in their systematic review of 14 investigations. They have also suggested that addition of Carnoy's solution to the cystic cavity for 3 minutes after enucleation. This reduced the recurrence to 1.6% which is comparable to resection, without associated morbidity.Treatment strategies have been devised on the basis of molecular studies and extensive reviews of literature suggesting the modalities with reduced possibility of recurrence. Blanas et al has also proposed a treatment strategy based on the pattern of behavior of OKC. He has suggested careful cystic enucleation with excision of overlying mucosa and recommended electrocoagulation/Carnoy's solution application in selective areas where the cyst was attached to the soft tissues. On the other hand, marsupialization alone as the sole treatment modality for OKC was suggested by Pogrel with an average of 2.9 years follow-up. He even found uprighting and eruption of teeth in the cyst. His explanation to this was Immunohistochemistry evidence of higher interleukin-1 alpha levels in OKC that significantly reduced after marsupialization. In all his cases, histologic material taken after marsupialization showed normal epithelium with no daughter cysts or remnants or budding of basal epithelial layer. He found preoperative bcl-2 protein expression strictly limited to basal layer and postoperative bcl-2 negative normal oral mucosa specimen.Stoelinga The line of management in our case was influenced by the classic work of Pogrel who had demonstrated that marsupialization can be considered as a definitive treatment modality for OKCs.To the best of authors’ knowledge, this rare extensive nature of lesion has been reported only once in the literature by Gupta et al.2A 35-year-old female visited the Department of Oral and Maxillofacial Surgery with the complaint of pain on chewing and swelling on the right side of the face. Medical history was not contributory. On intraoral examination, the only positive finding was a grossly carious, tender, right mandibular first molar. Radiographic examination revealed an extensive, multilocular radiolucency bounded by a radiopaque (sclerotic) margin all around. Figure Marsupialization was considered as an appropriate treatment plan, as the lesion was quite extensive. So, bone windows were made by excising mucoperiosteum along with the bone in the left mandibular buccal vestibule and anterior mandibular labial vestibule. Since the extraction socket of mandibular right first molar provided a potential means of irrigation of the cystic cavity, it was enlarged by removal of interradicular bone by means of bone rongeur and rotary instruments with copious saline irrigation. The cystic contents were evacuated, and the cystic cavity was packed with tape gauze soaked (and squeezed) in 2% povidone-iodine for 3 days. This was followed by periodic irrigation and suction of the cystic cavity with 2% povidone-iodine and normal saline (1:1 proportion). The irrigation of the cystic cavity was initially done every alternate day for 15 days, then twice weekly for about 4 months followed by weekly irrigation. Acrylic plugs were prepared to prevent premature closure of the bony defects. Orthopantomograms were taken at regular intervals to monitor the progress. Histopathological examination was carried out from the base of the resolved lesion in the anterior mandibular area. The pretreatment biopsy specimen and the lesional tissue from the base of the resolved lesions were subjected to immunohistochemical analysis to assess the expression of Ki-67, a proliferative marker, and bcl-2, an antiapoptotic marker .The preoperative radiograph revealed that the cystic lesion involved whole of the mandible, sparing both the condyles. Progressive reduction in the radiolucency was noted in successive radiographs taken. The radiographs taken at an interval of 4 months after marsupialization depicted gradual resolution of the lesion (as evidenced by a radiographic reduction in the radiolucency accompanied simultaneously by replacement with radiopaque bone), except for a small area in the mandibular right quadrant (4th quadrant). Clinically the bone windows have largely filled up from within, leaving a 1.5 cm depth, self-cleansing bony defect in the mandibular left quadrant, and a 0.5 cm self-cleansing, bony defect in the mandibular anterior area. The immunohistochemical report of the pretreatment biopsy specimen from lower anterior and lower left tissues showed the characteristic parakeratinized epithelium of OKC Fig.  with an The timeline of patient follow-ups have been summarized in Table 3 marsupialization/decompression followed by cystectomy, and mandibular resection. However, in our case enucleation had the possibility of pathologic jaw fracture, whereas cystectomy and resection had their own share of morbidity including functional, psychologic, cosmetic, and financial implications. Marsupialization as a potential treatment modality for parakeratinized OKC has received a great deal of attention after the research work of Pogrel; however, the findings of this case suggests otherwise.Based on extensive research, recommended treatment modalities for OKC that are known to reduce/prevent recurrence include enucleation, excision of overlying mucosa followed by application of Carnoy's solution, Bcl-2 inhibits apoptosis to facilitate cellular proliferation in the basal and suprabasal layers, whereas apoptosis maintains the homeostasis of the thickness of the lining epithelium and allows the synthesis of large amounts of keratin in the surface layer of OKCs. There is a regulated balance between cell proliferation, cell differentiation, and cell death in this type of lesion, this may explain why OKCs do not transform into tumor masses, instead of having neoplastic behavior with an increased potential to proliferate.The reduced expression of Ki-67 after marsupialization indicate the reduced proliferative activity and potential for recurrence and that of bcl-2 indicates the conversion of the classic OKC epithelium to the stratified squamous epithelium. The bcl-2 gene, located at chromosome 18q21, is characterized by its ability to stop apoptosis (programmed cell death) without promoting cell proliferation. It was also observed that Ki-67 expression was found to be more common in odontogenic cysts and tumors that are more aggressive in nature.Ki-67 antigen is more specific marker of proliferating cells, maximally expressed during S-phase. The levels of Ki-67 in the epithelial linings were found to be double in syndrome-related OKCs as compared to the sporadic cysts, indicating a greater level of proliferative activity in the former. This was reflected in the multiplicity of cysts and the increased numbers of satellite cysts and epithelial islands in the OKCs of NBCCS patients. This suggested that it was a genetic factor, possibly related to defective tumor suppression functions, that was reflected by the higher proliferative activity in their epithelial linings. revealed strong expression of interleukin (IL)-1α, mRNA and protein in the epithelial cells of OKCs, those reduced significantly after marsupialization. In fact, Ki-67 labeling index of the epithelial cells diminishes proportionally with the grade of IL-1α, mRNA expression after the marsupialization. This finding suggested that, marsupialization may reduce the size of OKCs by inhibiting IL-1α expression and the epithelial cell proliferation. It was observed in this case that there was significant reduction in Ki-67 and Bcl-2 at the healing sites.The study done by Ninomiya et al, in that extraction of all the mandibular teeth including third molars was done and full bilateral buccal decortication and cystotomy was performed for the panmandibular OKC.At a 2-year follow-up radiolucency along with root divergence in 42 and 43 region was observed which was curetted out and chemical cauterization done. Six months post-operation, showed nonresolution of lesion that prompted the surgeons to adopt an aggressive approach by removing total labial cortex along with associated teeth. It was also observed that there was no reduction of Ki-67 and Bcl-2 from the above site where the lesion did not resolve by marsupialization alone. Our results counter the treatment plan of Gupta et al,In an attempt to cover the large denuded lingual cortical plate, we mobilized the tissue from the labial mucosa. However, this resulted in the loss of vestibule in the lower anterior region. Rehabilitation was done with a tooth supported prosthesis. After a period of 10 years, it was observed that the lesion had complete healing with no reported evidence of any recurrence or new lesion.4 in 2017 may represent true cystic tumors but they respond to simple treatment such as marsupialization.To conclude, the authors state that marsupialization must be considered as the first line of management, especially for an OKC of such an extensive size, but cannot be considered as a definitive treatment modality for OKC. Nonetheless, other aggressive surgical treatments like removal of cortical plate along with overlying mucoperiosteum should be considered when the lesions are nonresponsive. Our study also reinforces the paradox that OKC, reclassified by WHOWe would like to thank Dr Priti and Dr Atul Deshmukh for assistance in the Histopathologic diagnosis of the case. The authors extend their appreciation to the Deanship of Scientific Research at King Khalid University for funding this work through General Research Project under grant number (GRP-184-40).Abdul Ahad Khan orcid: 0000-0001-7078-2069.AAK, SAQ has been involved in drafting and writing the manuscript. AAK, AAD made substantial contributions for the conception of the manuscript. AAK, SSA made substantial contributions for acquisition of clinical data. MIb and MZ has been involved in revising the manuscript critically for important intellectual content. SMA made substantial contributions for acquisition of radiological data. MIs made substantial contributions for acquisition of clinical data, for the conception of the manuscript and gave final approval for the version of the manuscript to be published. Each author has participated sufficiently in the work to take public responsibility for appropriate portions of the content, and agreed to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved. All the authors have made significant contributions to the manuscript and have also read and approved the manuscript.Funding information has been added the acknowledgement section.


Lithopedion is a rare complication of pregnancy which occurs when a fetus dies and becomes too large to be reabsorbed by the body. This entity in rare circumstances can be challenging for physicians to diagnose since it has a range of clinical manifestations.Lithopedion is a word derived from the Greek words We present a case of a 55-year-old, gravida IV para III, Ethiopian woman from Ethiopia with a retained fetus and vesicovaginal fistula after an obstructed labor and a neglected intrauterine fetal demise of approximately 22 years. The diagnosis was confirmed by suggestive clinical history, physical examination findings, and an abdominopelvic computed tomography scan. Laparotomy and removal of the lithopedion was done and our patient was sent to a fistula hospital for vesicovaginal fistula repair.This case is a rare phenomenon in which the dead fetus remained in the uterus for a long time after a neglected obstructed labor and uterine rupture. The word lithopedion is derived from Greek words to refer to a fetus that has calcified or changed to bone. This condition was first described in the tenth century by Albucasis, a surgeon of the Arabic era of medicine .In the modern era of medicine, the occurrence of a lithopedion is extremely rare. Since the earliest case was discovered in 1582 in France, less than 300 cases of lithopedion have been reported. However, in places with limited access to health care facilities and poor health awareness, physicians may encounter this rare condition and its confusing clinical presentation [Here we report a case of an obstructed labor 22 years ago which led to a uterine rupture, formation of a lithopedion, and a vesicovaginal fistula (VVF).Our patient is a 55-year-old Ethiopian woman, gravida IV para III abortion 0, who gave birth to three children at home by spontaneous vaginal delivery. She presented to our hospital claiming she was carrying a dead fetus in her womb from a pregnancy that had been there for 22 years. The pregnancy lasted up to nine months uneventfully at which time the membrane ruptured and she went into labor. After 3 days of labor at home, she developed vaginal bleeding and visited a nearby hospital where she was informed that she had a uterine rupture which had to be operated on. However, she refused the surgery and went home. Over time she developed urinary incontinence.She lost all her three children from an unspecified medical illness before their first birthday, although she claimed all the pregnancies and the labor were uneventful. Since the incident, she is divorced and lives alone supported by her sister.At her current presentation, she had lower abdominal pain which had increased in severity, vaginal discharge, and urine per vagina since the onset of the condition. She had no other known medical illness.On physical examination there was a gravid uterus of 20-week size and a non-tender, fixed, and firm abdominopelvic mass with no signs of fluid collection in her peritoneal cavity. There was a continuous leakage of urine through her vagina but a leak point was unidentified. Her vaginal canal was filled with a 4 cm by 5 cm, oval and stony hard mass.Laboratory findings, complete blood count, and organ function tests were in the normal range. An abdominal ultrasound was difficult to perform as there was only shadowing in her pelvis from the bony structures and our patient was incontinent, therefore, she was unable to retain urine.Abdominopelvic computed tomography (CT) findings: On the scanogram, multiple calcified tubular fetal parts were demonstrated in the pelvis of our patient Fig. . On the In January 2019, our patient underwent exploratory laparotomy. It was difficult to enter the peritoneal cavity because of the extensive adhesion of her uterus to the anterior abdominal wall. Her uterus was completely covered by the omentum forming a complex mass. Therefore, the peritoneal cavity was entered at the level of the epigastrium. Lysis of the adhesion and omentectomy were done starting from above to free her uterus from the anterior abdominal wall and the omentum which had gone into the uterine cavity through the anterior uterine wall rupture site.in situ to drain urine but it could not prevent leakage through the fistula.Multiple pieces of old necrotic fetal bones Fig. a, b wereShe had a fairly stable postoperative course and was discharged with a referral to a fistula hospital for repair of the VVF.Lithopedion, or stone child, represents a condition in which a dead fetus is retained and calcified . The reaThis rare entity occurs with an unpredictable clinical course. Most of the cases may remain asymptomatic for a long period of time or present with persistent or recurrent abdominal pain and obstructive symptoms of the bowel and urinary system. It can also present with complications such as pelvic abscess, cephalopelvic disproportion in future pregnancies, extrusion of fetal parts through the abdominal wall, rectum or vagina fistula formation, tubal infertility, and lithopedion-induced malignancy , 6, 7.This case report might not represent a classic example of a lithopedion as it is not merely an extrauterine pregnancy, but there have been five cases of lithopedion reported in the horn of a bicornuate uterus . UterineSince most literature on this subject are case reports, it is not clear what the best diagnostic tools might be or the most appropriate management approach. However, surgery has been the chosen treatment in most of the reported cases.This case report is unusual in nature because our patient carried a dead fetus in her abdomen for more than two decades and presented with VVF which occurred after a uterine rupture.


Hyperzincemia and hypercalprotectinemia is a rare inflammatory disease caused by a mutation in the PSTPIP1 gene, with a dysregulation of calprotectin metabolism. Calprotectin is a zinc-binding protein with antimicrobial properties and pro-inflammatory action. The authors report the case of a 20 year-old girl with cutaneous ulcers comparable with pyoderma gangrenosum, growth failure and chronic anemia, who was given the diagnosis of hyperzincemia and hypercalprotectinemia. Measurement of serum zinc and calprotectin concentrations are indicated in these cases. Hyperzincemia and hypercalprotectinemia (Hz/Hc) is an autoinflammatory disorder characterized by chronic systemic inflammation, cutaneous lesions, arthralgia/arthritis, hepatosplenomegaly, pancytopenia, and growth failure, first described in 1985.4A 20 year-old woman reported persistent ulcerated skin lesions with variable severity for five years, recurrent abdominal pain with episodes of diarrhea and joint pain in both knees accompanied by edema and local erythema since childhood. She reported chronic anemia that was unresponsive to several treatments. On the physical examination, the patient had short stature (percentile < 3%), cutaneous ulcerations with violaceous edges, interspersed by scar tissue and pustules with friable, bleeding and granular central area, together with the presence of purulent exudate and hematic crusts, affecting both lower eyelids, breast, and legs, in addition to a pronounced hepatosplenomegaly A–C. The Considering the diagnostic hypothesis of Hz/Hc, serum levels of zinc and calprotectin were measured: 869 μg/L  and 642 μg/L , respectively.Cyclosporin A (3.5 mg/kg/day) and prednisolone (1.5 mg/kg/day) were initiated, leading to wound healing, weight gain, and partial improvement of anemia, but the therapeutic response worsened after an attempt of waning, evolving with aggravation of preexisting lesions and development of an ulcerated lesion in the point of transcutaneous hepatic biopsy, compatible with pathergy D–F. ImmuIn the genetic assessment of the patient, the c.748G>A variant (Glu250Lys) was found in heterozygosis in the exon 1 of the PSTPIP1 gene. This variant is constituted by a substitution of glutamic acid for lysine at the codon 250 of the translated protein, which has been described as pathogenic in the Infervers and Clinvar databases (rs28939089) when associated with pyogenic arthritis, PG, and acne (PAPA) syndrome (MIM 604416).Hz/Hc is a rare autoinflammatory condition, clinically characterized by pustular and ulcerative inflammatory cutaneous lesions, recurrent arthritis, hepatosplenomegaly, pancytopenia, and growth failure, with no predilection for gender, and usually diagnosed during youth. The disease is characterized by laboratory findings, such as extremely high serum levels of zinc and mostly calprotectin .Calprotectin is formed by a complex of two MRP8/14 proteins that are endogenous activators of the toll-like receptor 4 (TLR-4) and belong to the family of the alarmins. This protein is found in the neutrophil cytosol and has intense pro-inflammatory properties.To date, no treatment has been fully effective, with reports of the use of corticosteroids, cyclosporine, tacrolimus, TNF, and IL-1 inhibitors. Hz/Hc is a chronic multisystem disease with nonspecific symptomatology and is present since childhood in most cases.The skin changes reported in Hz/Hc are vasculitis, eczemas, furuncle-like ulcers, and necrotic lesions. They tend to be inflammatory and ulcerated, sometimes pruritic, with clinical and histopathologic similarities to PG, usually affecting the lower limbs symmetrically, with reports of facial lesions, particularly on the eyelids. The observation of chronic lesions in young children and adults with hepatosplenomegaly, arthralgia/arthritis, high CRP, and microcytic anemia is suggestive of Hz/Hc.None declared.Ludimila Oliveira Resende: Elaboration and writing of the manuscript.Marilia Formentini Scotton Jorge: Elaboration and writing of the manuscript; intellectual participation in propaedeutic and/or therapeutic conduct of the cases studied.Juliano Vilaverde Schmitt: Effective participation in research orientation; intellectual participation in propaedeutic and/or therapeutic conduct of the cases studied.None declared.


Solitary fibrous tumors (SFTs) are rare soft-tissue tumors characterized with spindle-cell, which occur more common in the chest and rarely seen in the abdomen. So far as we knew, SFTs accompanied with venopathy of portal vein has rarely been reported.A 36-year-old male presented with left-sided abdominal mass and portal vein expansion on ultrasound.The post-operative histopathology confirmed the diagnosis of Solitary fibrous tumor.Laparotomy was performed and the mass was completely removed.Patients had no symptoms, recovered well without recurrence; the portal vein and splenic vein dilatation were alleviated and the symptoms of portal hypertension were relieved.SFTs presents with few symptoms in the early stage of the disease. A rich arteriovenous shunt is beneficial to the diagnosis of SFTs by B-ultrasound and computed tomography (CT) examinations. However, the diagnosis of SFTs must depend on histopathology. The onset of SFTs is insidious with no obvious symptoms at early stage. As a result, the diagnosis is somewhat difficult to ascertain at an early stage. For accurate results, the diagnosis of SFTs must always depend on histopathology. Early diagnosis of SFTs has proven to be helpful in both the treatment and prognosis of the condition. Currently, there are no effective methods to achieve an early diagnosis of SFTs. However, we found out from our study that a rich arteriovenous shunt is beneficial in diagnosing SFTs by b-ultrasound and computed tomography (CT) examinations. In our hospital, we recently admitted 1 case of abdominal SFTs with portal hypertension. Anatomical and pathological examination suggested that direct reflux of arterial blood draining into the vein through arteriovenous short circuit in the tumor contributed to portal vein disease. Subsequently, we retrospectively analyzed the clinical and pathological data of 12 patients with confirmed SFTs. Arteriovenous short circuit is a common pathological phenomenon in SFTs. We confirmed the manifestation on the basis of diversified literature and noticed that it presents with a series of symptoms related to hemodynamic changes.Solitary fibrous tumors (SFTs) were first proposed by Klemperer and Rabin in 1931.2A 36-year-old China male, who was previously healthy, was found to have a left-sided abdominal mass on physical examination. An abdominal ultrasound was preformed and revealed the presence of a large mass, The mass was located inferior to the kidney and measured, approximately 15.5 × 12.3 × 5.2 cm, The patient also had portal vein expansion (inner diameter of about 18 mm) a dilated the splenic vein (inner diameter about 16 mm). An abdominal CT examination confirmed the presence of a large and soft tissue mass in the left lower abdomen and spleen enlargement. CTA demonstrated that the tumor was located in the free abdomen below the left kidney with abundant blood supply and periphery in focus. The nourishing artery branched directly from the abdominal aorta under the celiac axis and the reflux vein was grossly distorted. The latter divided into 2 to 3 branches which drained into the portal vein posterior to the kidney. The portal vein was also widened. The branches of the splenic veins were distorted and expanded into the tumor.Abnormalities were not seen in blood routine examination, biochemistry indexes, and the examination of coagulation routines. There were also no obvious abnormalities in the examination of electrocardiogram and chest radiograph.Laparotomy was performed with general anesthesia and the mass was completely excised. In the operation, a solitary hemorrhagic tumor of 15.5 cm∗12.3 cm∗5.2 cm was found in the left lower abdomen. The surface of the mass was dark red and with a several large blood vessels. Most of the blood vessels were veins and the thickest one was about 1 cm in diameter. One of artery connecting to the abdominal aorta was considered as a nourishing artery according to CTA examination Several large distorted veins merged into the portal vein posterior of the kidney and spleen. The portal vein had a significantly wider lumen.Its macroscopic inspection showed that the cut surface of the tumor was dark red with calcified areas. The nuclei of the tumors were markedly heteromorphic, with thick chromatin. The markers BCL-2 (+), CD34 (+), CD31 (+), and Vimentin (+), were positive, and the mitotic count was low. The proliferative index counted by Ki67 was weakly positive. Furthermore, elastic fiber staining demonstrated an arteriovenous short circuit inside the tumor. The final diagnosis for these manifestations was solitary fibrous tumor. Recovery was complete without a need for further treatment. The patient underwent abdominal ultrasound 10 months post-operation without recurrence, and the portal vein and splenic vein dilatation were alleviated . The symptoms of portal hypertension were also relieved.3 The tumor usually becomes apparent when compression symptoms become apparent. There was no gender difference in SFTs. SFTs can occur almost anywhere in the body but is common in areas such as the pleura, pericardium, meninges, peritoneum, and so on. This patient had no clinical symptoms and was admitted for the mass and the wider portal vein (increased pressure) identified on physical examination. In this case, the feeder vessel of the tumor originated from the independent branch of the abdominal aorta, where pressure was higher; The vein directly returns to the portal vein. This anatomy contributed significantly to the increased portal vein pressure in this patient. Moreover, the capillary network connecting the artery and the vein could balance the difference in arteriovenous pressure. However, the blood supply of the case was not sufficient to explain the increased portal pressure (width widening). For this reason, we speculated that there may exist particular structures which could be responsible for the increased pressure of portal vein in the SFTs.There are few special symptoms in the early stage of SFTs. On a general scale, enhanced scanning is significantly enhanced. However, certain differences can be noticed with different sites on the body. MRI can indicate the nature of lesions through signal characteristics and enhanced features.Ultrasound and X-ray can show the site of the lesion and morphological characteristics, but it is difficult to make a clear diagnosis. CT examination often reveals as isolated masses with clear boundaries and generally uniform density. If necrotic areas are present in the tumor, the masses may be uneven. The cytoplasm is eosinophilic, the nucleus is irregular, and the chromatin distribution is uniform. The morphology of SFTs varies with the location. Tumor color is mostly grayish white the number of blood vessels. Texture of the tumor varies by the amount of collagen fibers present. The typical morphological feature of SFTs is the presence of interlaced dense and loose cells. Tumor cells in dense areas are arranged in bunches, vortexes, or irregular patterns, with obvious atypia, thick chromatin and uneven distribution of mitotic figures. The loose area is highly vascularized and is mainly composed of collagen.A large number of studies have confirmed that SFTs can be classified as mesenchymal tumors. They are mainly composed of spindle cells and have a complex tissue structure, diverse cell morphology, and unclear boundaries.After a careful examination of routine sections, we found suspicious indication of arteriovenous shunts. To confirm the arteriovenous shunts, we used elastic fiber staining to distinguish arteries and veins, This helped us decipher whether or not an arteriovenous shunt exists in the solitary fibrous tumor. By considering the morphology of the tumor, radiographic findings, pathological results, and post-operative follow-up, it sufficed to conclude that:1)the diagnosis of SFTs, in this case, is correct;2)the pressure of feeder vessel is higher from the abdominal aorta.3)the presence of arteriovenous shunts in the tumor leads to the increased pressure of the reflux vein, and the venous flow directly returns into the portal vein leading to an increased pressure in the portal vein.4)the higher-pressure portal vein presents as wider inner diameter of the portal vein and slightly enlarged spleen on radiographic examination. Existing literature reports that solitary fibrous tumors have abundant blood supply.However, abnormal communication branches in SFTs have not been reported. The arteriovenous traffic of the tumor under microscope is shown in Figure Large, distorted blood vessels often present in SFTs, which is considered as a diagnostic criteria. Inspired by this case, we suspected that the wide and distorted vascular shadow in radiographic examination may cause increased intravascular pressure. Therefore, we retrospectively analyzed the other 12 patients (including 2 outpatients) who were diagnosed with solitary fibrous tumor by pathology in the past 3 years. We found arteriovenous shunts in 7/12 in the patients through the elastic fiber sections stained by paraffin. However, due to the incompleteness of clinical data, especially radiographic imaging in these cases, performing CTA reconstruction of tumor vessels was impossible. Therefore, relationship between large, distorted tumor blood vessels and arteriovenous shunts in tumor is still not clear. We proposed that in most cases arteriovenous shunts occur in solitary fibrous tumors, and can cause hemodynamic abnormalities such as portal hypertension. In addition, since not all the gross specimen is cut into sections for microscopic observation, arteriovenous shunts in the mass may be missed. Therefore, the actual incidence of arteriovenous shunts in SFTs may be higher than detected. As SFTs is derived from CD34 positive dendritic mesenchymal cells, CD34 is recognized as a relatively specific and accurate marker for SFTs. The CD34 positive detection rate of SFTs is close to 100%. However, positive CD34 is not unique to SFTs, and the diagnosis of SFTs should be combined with pathological morphological characteristics. The expression of p53, ki-67 was positively correlated with tumor malignancy. In this case, the pathological examination results showed typical morphological characteristics of SFTs, with obvious cellular atypia. Immunohistochemical results showed that CD34, CD31, and bcl-2 were strongly expressed and ki-67 was weakly positive. Therefore, it only requires combined analysis of with typical morphological and pathological examination as well as immunohistochemical detection to confirm SFTs.Currently, the markers commonly used to diagnose SFTs include CD34, CD31, cytokeratin, bcl-2, Calretinin, ki-67, p53, s-100, SMA, Vimentin, and wt-1 protein. Most benign tumors and some malignant tumors can be cured after surgical resection. Complete and/or incomplete resection of tumors closely determines the prognosis of SFTs. Adjuvant radiotherapy and chemotherapy can reduce the rate of postoperative recurrence and metastasis to a certain extent, which is often used for SFTs that is difficult to remove or has metastasized.The preferred treatment method for SFTs is surgery.4SFTs are relatively rare diseases. When patients present with this disease, the arteriovenous shunts in the tumor is consider contributing significantly to the increased reflux venous pressure as well as portal and splenic venous pressure. We confirmed that arteriovenous shunts are a common phenomenon in the SFTs which could lead to a series of symptoms such as portal hypertension A rich arteriovenous shunt is a clue of SFTs diagnosis detected by B-ultrasound and CT examinations. But most importantly, the identification of SFTs must always depend on histopathological examination.XF designed the work; XQW and HQY contributed to acquisition, analysis, or interpretation of data for the work; ZFM drafted the work; all authors contributed to the final approval of the version. We wish to thank our patient and his family for consenting to the publication of this case report.Conceptualization: Xu-Qing Wang, Xin Fan.Data curation: He Han, Gong Chen.Formal analysis: Han-Qing Yang, Xin Fan.Funding acquisition: Ji-Xiang Chen.Writing – original draft: Han-Qing Yang, Zhen-Fa Mao.


The occurrence of medulloblastoma (MB) in the elderly is an absolutely rare event. Concerning this issue we report on two MB patients beyond the 70th year of life. Two patients older than 70 years presented with a mass in the posterior fossa without evidence of a preexisting malignant tumor. After careful radiological work-up the suspected diagnosis was metastasis of an unknown primary tumor. Both patients underwent surgery and histopathological analysis revealed MB in both cases . The two cases presented here represent also one classical MB and one additional desmoplastic MB. To our knowledge we report for the first time that there are different molecular subtypes of MB in the elderly patients that seem to be consistent with those subtypes mainly occurring in young adults. Unfortunately the patients died within one week after surgery due to respiratory insufficiency and an unclear cause. The presented cases show that MB can occur in the elderly. Although this constellation is absolutely rare, MB should be considered in the differential diagnosis, especially when a primary tumor is not known or detected. Medulloblastomas (MB) are small, round, blue cell, neuroectodermal tumors with neuronal differentiation in the cerebellum. These tumors usually occur in childhood. The incidence of MB in young adults is estimated to be less than 1% of all intracranial neoplasms . The meaCase 1 concerned a 71-year-old male presenting with gait difficulties and vertigo. Cranial MRI revealed a low and focal contrast-enhancing nodular tumor in the left cerebellar hemisphere and upper vermis . The lesCase 2 was a 72-year-old male who was referred to the hospital because of change of personality and loss of weight. Cranial MRI showed a large low contrast-enhancing mass in the right cerebellar hemisphere consisting of a lateral solid component and a small medial cystic. The tumor caused occlusive hydrocephalus but no surrounding edema . MR reveUp to now there are four different molecular subtypes of MBs recognized, including activation of WNT-signaling pathway, SHH-signaling pathway, and the Non-WNT-/Non-SHH-subtype, which can be subdivided into group 3 (mostly MYC-amplified) and group 4 (often MYCN-amplified). Most of the desmoplastic MBs are of the SHH-activated subtype, whereas classic MBs are of the WNT- or the Non-WNT-/Non-SHH-subtype.In contrast to children, in young adults the most common molecular subtypes are the SHH-activated/p53-wildtype and group 4 MB , 7. All The two cases presented here represent also one classical MB and one additional desmoplastic MB. To our knowledge we report for the first time that there are different molecular subtypes of MB in the elderly patients that seem to be consistent with those subtypes mainly occurring in young adults.Unfortunately the presented cases had a very unsatisfactory postoperative course, so that we cannot report any response of the patients to the planned radiotherapy in case 1 and combined radiochemotherapy in case 2.Regarding the radiologic findings, we could not detect any differences to MB in younger ages. A moderate contrast-enhancing mass in T1-weighted sequences in the posterior fossa with surrounding brain edema and anatomical contact to the vermis and the fourth ventricle were typical findings. Histologic examination did not show any differences compared to the findings of MB occurring in younger patients.The presented cases show that MB can occur in the elderly. Although this constellation is absolutely rare, MB should be considered in the differential diagnosis, especially when a primary tumor is not known or detected.


Spontaneous esophageal rupture (Boerhaave syndrome) is a rare, though frequently fatal, event. It is generally caused by a sudden increase in pressure inside the esophagus. In some cases, full-thickness perforations of the esophagus may develop from previous lesions that initially involve only the esophageal mucosa  and which, following further triggering events, give rise to a transmural lesion.Here, we present the case of a 45-year-old subject who suddenly died of acute cardio-respiratory failure, an autopsy was performed to identify the cause of death.The autopsy examination revealed a full-thickness rupture of the esophageal wall. Through the integration of necroscopy findings, anamnestic data, and histopathological examination, it has been possible to establish that complete esophageal rupture resulted from the evolution of a previous partial lesion of the esophageal wall, and that an untreated Mallory-Weiss syndrome evolved into a rapidly fatal Boerhaave syndrome.This case shows that distal esophageal tears, rather than constituting a distinct entity, may be part of a spectrum of diseases and that a partial lesion of the esophageal wall caused by barogenic injury may evolve into a full-thickness rupture following further barotraumas. Traumatic perforations result from penetrating wounds that tear the cervical and/or thoracic esophagus, and may be caused by road accidents. The ingestion of foreign bodies may cause erosion of the esophageal wall in areas of physiological narrowing, causing fistulization and sepsis. Disease-related perforations may occur in patients with esophageal diverticula, cancer, or infective esophagitis. Spontaneous lesions may involve only a part of the esophageal wall  or constitute a full-thickness rupture of the organ, giving rise to Boerhaave syndrome, which accounts for 8 to 56% of all esophageal perforations.Esophageal perforations may be iatrogenic, traumatic, due to foreign bodies, related to other pathologies or spontaneous. The main symptoms may vary in severity according to the depth of the lesion, and are constituted by hematemesis (seen in about 40% of cases), melena (the onset symptom in about 30% of cases), hematochezia, abdominal pain, chest pain, tachycardia, syncope, hypotension, or hemorrhagic shock.Mallory-Weiss syndrome is characterized by longitudinal lacerations of the esophageal mucosa and is a common cause of gastrointestinal bleeding, accounting for 15% of hemorrhages of the upper digestive tract. These symptoms, however, are not pathognomonic for Boerhaave syndrome, and may therefore be confused with the symptoms of other diseases, including pneumonia, acute myocardial infarction, pericarditis, and pancreatitis; the consequent, potential delay in reaching a diagnosis explains the high mortality rate of this pathology .Boerhaave syndrome, which involves transmural esophageal rupture, is a clinical emergency, as it carries high mortality. The main symptoms include vomiting, chest pain , dyspnea, dysphagia, subcutaneous emphysema, tachycardia, fever, tachypnea, and epigastric pain. In rare cases, these esophageal perforations may be manifested by hematemesis or other signs of gastrointestinal bleeding, including melena. These pathologies are usually associated to episodes of vomiting or retching after the consumption of alcoholic beverages or a heavy meal. Other precipitating factors include: severe coughing, acute asthma, epilepsy, constipation, childbirth, lifting weights, thoraco-abdominal traumas, and endoscopy maneuvers.,10Both syndromes seem to share the same pathogenic mechanisms; both are linked to increased pressure inside the esophagus, and therefore to a barotrauma, and occur more commonly in men with a history of alcohol abuse.Here, we describe the case of a 45-year-old man who died as a result of a spontaneous rupture of the esophagus. Integrated analysis of autopsy, anamnestic, and histopathological findings revealed that the fatal esophageal perforation was not manifested suddenly, but had developed from a previous partial wall lesion, which had been present for some days, thus indicating a transition from an initial Mallory-Weiss lesion to a Boerhaave syndrome.23 of coffee-colored material was found in the left pleural cavity, and 350 cm3 in the right cavity .Experimental data obtained on fresh cadavers with the medial portion of the esophagus clamped have revealed that a pressure of 150 mmHg is able to produce a tear at the level of the gastro-esophageal junction. described the case of a 19-year-old subject who died in his sleep as a result of an esophageal rupture which caused mediastinitis, septic shock, and multi-organ failure. Kimura-Katakota et al reported a case of sudden death due to perforation of the inferior third of the esophagus in a 45-year-old man who was asthmatic and alcoholic; the cause of death was ascribed to acute cardio-respiratory failure. In both of these cases, the esophageal tear was found in the left lateral wall of the distal third of the esophagus, which, according to the literature, is the most frequent site of Boerhaave syndrome.Few cases of spontaneous esophageal perforation have been reported in the medical-forensic literature. Clément et alUnlike the above-mentioned cases, in the case reported here we hypothesize that the esophageal rupture was not of sudden onset in a previously healthy esophageal wall; rather, the full-thickness laceration developed from a previous Mallory-Weiss lesion (therefore involving only the mucosa) that had not been diagnosed or treated. This hypothesis stems from the integrated analysis of a set of data gathered from the autopsy, the patient's anamnesis, and the histopathlogical examination. In addition, the hematic material found both in the intestine and at the death scene was not made up of live red blood that could have resulted from a recent hemorrhage . Rather, this abundant material consisted of digested blackish blood (the hemoglobin had undergone a process of degradation) which was indicative of a previous hemorrhage. This finding enabled us to establish that, before the fatal perforation, bleeding had occurred in the upper digestive tract, probably not copiously but intermittently, producing a gradual loss of blood due to a tear in the esophageal mucosa. The bleeding was probably due to a lesion of the mucosa and of the underlying congested venous plexus. Furthermore, the origin of this bleeding could not be explained by the possible coexistence of other pathologies, in that the autopsy and histopathological examination did not reveal the coexistence of any other conditions able to cause digestive hemorrhage . Secondly, analysis of the anamnesis, as reported by the subject's family members, revealed that the subject, who had a history of alcohol abuse, had been suffering from malaise and thoracic and epigastric pain for about a week without consulting a doctor; only on the day of his death, when the pain symptoms due to the full-thickness esophageal perforation probably worsened, did he call the emergency services, 20 minutes before he was found dead.First of all, the autopsy examination revealed a full-thickness, longitudinal, esophageal tear, measuring about 3 cm in length, in the right lateral wall of the esophagus; as mentioned above, this is statistically the most common site of Mallory-Weiss lesions, while full-thickness ruptures more frequently occur in the left wall. Moreover, intestinal section also revealed the presence of abundant digested hematic material. The sudden rupture of the esophagus does not generally give rise to copious hemorrhage.On the basis of these findings, it was therefore hypothesized that the subject was suffering from a Mallory-Weiss lesion  which had been causing symptoms  for a week. This lesion probably did not elicit massive bleeding, which would have caused the subject's condition to precipitate rapidly; rather, the hemorrhage, which was probably mild and intermittent , had given rise to obscure, aspecific symptoms. On the day of his death, a further barotrauma caused by vomiting (as observed at the death scene) appears to have deepened the pre-existing wall lesion, causing a full-thickness rupture of the esophageal wall, which rapidly led to death. Finally, on the days prior to death, any possible alcohol intoxication and its anesthetizing effect could have masked or attenuated the underlying symptoms of an esophageal lesion that had been ongoing for at least a week.Toxicological tests carried out on the body fluids proved negative, indicating that the subject had not consumed alcohol shortly before death. Nevertheless, in this case too, a history of alcohol abuse emerged (confirmed by the marked hepatic steatosis observed during autopsy), which could have facilitated the formation of the esophageal tear. The effect of alcohol consumption in the pathogenesis of esophageal lesions lies in the fact that alcohol can, directly or indirectly, cause vomiting. Moreover, alcohol acts on the gastric and esophageal mucosa to increase the retrodiffusion of hydrogen ions, reducing their protective effect; in addition, intoxication by alcohol can alter motor activity and pressure at the level of the inferior esophageal sphincter. As a result of negative intrathoracic pressure, the contents of the stomach and bacteria rapidly enter the pleural cavity, causing a state of sepsis. The septic shock due to mediastinitis leads to multi-organ failure and death. The time-lag between the onset of spontaneous perforation of the esophagus and death has been estimated to be 20 to 24 hours, if medical treatment is not undertaken. In the case reported here, death occurred very rapidly. Indeed, the subject was found dead only 20 minutes after he had called the emergency services to report intense thoracic-abdominal pain and vomiting. It can therefore be hypothesized that no more than an hour had elapsed between the onset of vomiting (which triggered the rupture and the acute symptoms) and death. Furthermore, it is likely that the subject's state of general malaise and the anemia due to persistent slow bleeding contributed to accelerating the fatal evolution of the disease. In this subject, no signs of mediastinitis or multiorgan failure, nor macroscopic or microscopic signs of sepsis, were observed. Thus, on the basis of the autopsy and histopathlogical findings, the cause of death was ascribed to acute cardio-respiratory failure due to pleural effusion following esophageal rupture. Indeed, in agreement with Kimura-Kataoka et al, it may be hypothesized that the time-lag between perforation and death was sufficient to allow the gastric contents to enter the pleural cavities, thereby causing respiratory failure due to pulmonary compression, but not sufficient to cause sepsis and multi-organ failure.Further confirmation of the presence of a wall lesion prior to the complete laceration emerged from the histopathlogical examination of esophageal samples taken from the site of the tear. Firstly, microscopic examination confirmed the vitality of the lesion, revealing the presence of a neutrophil infiltrate in the esophageal submucosa and aspecific signs of injury and inflammation. Moreover, digested alimentary material was found at the site of the tear, and the presence of accumulations of hemosiderin was detected (by means of Perl stain) in the mucosa and submucosa, presumably indicating bleeding prior to complete perforation. In general, owing to the presence of hydrochloric acid and the remains of food, esophageal perforation may give rise to chemical mediastinitis and hemorrhagic necrosis. and that a partial lesion of the esophageal wall caused by barogenic injury may evolve into a full-thickness rupture following further barotraumas. Although esophageal pathologies are rarely identified on autopsy as the cause of death, in the event of the finding of an esophageal perforation, it is essential for the forensic pathologist to be able to construct a correct picture of the fatal pathology through careful and combined analysis of the autopsy findings and the anamnestic and histopathological data.The case reported here is the first in which the integrated study of anamnestic, necroscopic, and autopsy data has revealed the possible transition of a Mallory-Weiss syndrome to a Boerhaave syndrome. This observation supports the concept that distal esophageal tears, rather than constituting a distinct entity, may be part of a spectrum of diseases,Conceptualization: Maria Cuccì.Data curation: Anna Roncallo.Investigation: Giulio Fraternali Orcioni.Methodology: Fiorella Caputo.Supervision: Francesco Ventura.


Aspergillus Penicillioides as the involved pathogen.In this case-report, the Authors show the case of a sudden death occurred in a 38-year-old woman submitted to surgical excision of a right acoustic neurinoma. At the autopsy, was detected a cerebral hemorrhage with multifocal localization by a ruptured rare fungal aneurysm of the Posterior Inferior Cerebellar Arthery (PICA). The PCR analysis, carried out on formalin-fixed paraffin-embedded tissue, identified the We discuss the main points of infectious aneurysms, being a potential neurosurgical complication. •Lethal intracranial hemorrhage by rupture of mycotic aneurysm.•Mycotic aneurysm of PICA.•Aspergillus in cadaver. The posterior inferior cerebellar artery (PICA) results to be involved in a low percentage of cases 0.49% - 3%) and, in this context, infectious aneurysms are typically observed deep in the posterior cranial fossa, in close anatomical relationship with the brainstem, the caudal cranial nerves and the skull base structures 9% - 3% a.3 and in immunosuppressed subjects [Intracranial infectious aneurysms arise in 65% of prosthetic heart valve carriers, who have developed bacterial endocarditis, especially a left one , in 6.3%subjects .In the infectious aneurysms pathogenesis, an arterial wall or a preexisting intracranial aneurysm are infected by different pathogens , i.e. prIn this report, we present a case of unexpected death occurred to a 38-year-old woman, due to the rupture of a fungal aneurysms involving the PICA, detected only in the post-mortem investigations. The lady had previously been subjected to acoustic neurinoma exeresis, with no evidence of preexisting aneurysms at pre-operative investigation by means of cerebral nuclear magnetic resonance (NMR) with gadolinium enhancement.1.1A 38-year-old woman underwent to neurosurgical surgery consisting in the exeresis of a right acoustic neurinoma (vestibular Schwannoma) by retromastoid access. In view of good post-operative clinical conditions, the patient was discharged on the fourth post-operative day. After an initial period of well-being, the woman began to experience worsening fatigue and fever (37.8 °C), reason why she went to the A&E of the same hospital where she had been operated 15 days before. The lady died while clinical exams were still being carried out.The autopsy performed to clarify the cause of death, identified massive cerebral hemorrhage with multifocal localization  A from a Aspergillus  did not show any infectious focus due to aspergillomas or invasive aspergillosis. Subsequently, we performed the molecular identification of the Aspergillus species involved. For extraction and purification of DNA from formalin-fixed paraffin-embedded tissues (FFPE) was used QIAamp DNA FFPE Tissue Kit (Qiagen). Amplification primers 5′-TCCGTAGGTGAACCTGCGG-3′ and 5′-GCTGCGTTCTTCATCGAT GC-3′ (Sigma-Aldrich) were used in a standard polymerase chain reaction (PCR) to amplify ITS1 region, located between the 18S and 5.8S rRNA genes [Aspergillus Penicillioides sequences.The subsequent histological examinations clarified the uncommon etiology of this aneurysm, highlighting a diffuse arterial wall colonization by septate mycotic hyphae, with a dichotomous 45° bifurcation, typical for ergillus . The hisNA genes . The obtTherefore, a post-mortem diagnosis of a lethal rupture of PICA fungal aneurysms was formulated. Fungal aneurysms have to be considered rare and occasional events, which are secondary to a mycotic infection.2Aspergillus is a ubiquitous fungus, that can be found as a commensal in paranasal sinuses, and more than 180 species have been described [Aspergillus is due to the production of the elastase enzyme, with which it progressively degrades the elastin present in the vessel walls, also causing the arising of inflammatory infiltrate. The development of the aneurysm is due to the proliferation of mycetes in the context of elastic lamina with its subsequent collapse and dissection from the media tunica [escribed . Aspergiescribed . The inva tunica . FurtherIn general, intracranial infectious aneurysms have a mainly fusiform and irregular morphology; the saccular aspect is less represented, being observed in 41% of cases . These aCurrently, the diagnosis of these pathological entities is based on the documentation of an intracranial aneurysm by vascular imaging in the presence of predisposing infectious conditions. For this purpose CT scans, both with and without contrast, are extremely useful, even if the gold standard test is the conventional angiography. Classic angiographic features of infectious aneurysms are multiplicity, distal location, fusiform shape, and a change in the size or the appearance of a new aneurysm on follow-up angiogram. Finally, positive cultures from peripheral blood or the infected aneurysm wall itself can confirm the diagnosis .Nowadays, the therapeutic approach of these pathological entities is not univocal and it results to be complex, due to the close topographic relationship of the neurovascular structures involved, the contiguity with the lower cranial nerves, the deep localization in the posterior cranial fossa and, finally, the high prevalence of desiccant fusiform aneurysms .However, there is a wide agreement on the need for a prolonged antibiotic therapy, moving towards the selective endovascular embolization or trapping with soft and ultra-soft electrolytically detachable coils only in the cases of dynamic unruptured infectious aneurysms, carrying out repeated angiographies during the follow-up. Conventional surgical repair is restricted for ruptured aneurysms associated with hematoma and high intracranial pressure .In our case, the patient developed non-specific symptoms, not immediately attributable to an intracranial aneurysm. Unfortunately, she deceased before the clinical-pathological diagnosis was performed by means of instrumental investigations. Certainly, the woman did not present preexisting aneurysms, which would have been observed at pre-operative cerebral NMR. This crucial aspect allows to place the onset of the PICA fungal aneurysm in the days after the surgery and to consider it as a rare neurosurgical complication of acoustic neurinoma exeresis. Moreover, the absence of microscopic signs of fungal infection in all of the other organs supports the hypothesis of an acute onset of this fungal aneurysm. In this case, therefore, both the topographical aspect (proximity of the aneurysm onset to the site of surgery) and temporal one   are coheThere are none.


Salivary gland tumors are rare among all head and neck tumors. Pleomorphic adenoma (PA) is the most commonly seen subtype, and 85% of the cases are located in the parotid gland. PA may very rarely be seen in minor salivary glands. Minor salivary gland PAs are mostly located in the hard and soft palates. Nasopharyngeal PA is very rare, and a total of 8 cases have been published to date. In this case report, a 51-year-old female patient who had nasopharyngeal PA with chondroid metaplasia is presented, and we review the relevant literature. Salivary gland tumors are rare and constitute less than 5% of all head and neck tumors . PleomorA 51-year-old female patient came to our clinic with postnasal drainage and intermittent nasal obstruction. Oropharynx examination was normal. There were no features on anterior rhinoscopic examination. On nasopharyngeal endoscopic examination, there was an approximately 1 × 1 cm mass with a smooth surface on the posterior wall of the nasopharynx . There wWhen a nasopharyngeal mass is detected in an adult patient, nasopharyngeal malignancy is usually suspected. Most common types of nasopharyngeal cancer are keratinising squamous cell carcinoma, nonkeratinising carcinoma, and basaloid squamous cell carcinomas , 13. MalPAs are the most common salivary gland tumors and comprise 45–70% of all salivary gland tumors , 17. ThePA is typically located in the parotid lower pole or the superficial lobe. Deep lobe localization is rare . PA is uNasopharyngeal PA is seen very rarely. According to the sources available to us, there have been only 8 cases reported in the literature –11. DetaPleomorphic adenoma-related squamous cell carcinoma (SCC) has been detected in 3 of 8 cases published in the literature , 7. In tSurgical procedures such as lateral rhinotomy and transnasal endoscopic approaches used for the other nasopharyngeal benign tumors can be used for this mass . In a caIn conclusion, very rarely seen nasopharyngeal PAs should be kept in mind in the differential diagnosis of nasopharyngeal masses. The gold standard for treatment of PA is total surgical excision of the mass.


Internal hernias and the appendicular tourniquet are two rare pathologies.We report here a case of a 68-year-old obese patient, who has acute small bowel obstruction due to strangulated internal hernia through an appendicular tourniquet. This appendicular tourniquet results from the adhesion between the tip of the appendix and its body. This obstruction was complicated by plugged perforation. Resection of the small bowel segment was performed, in addition to appendectomy, lavage, and drainage. The postoperative recovery was uneventful.Acute bowel obstruction secondary to strangulation of an internal hernia through an appendicular tourniquet is exceptional. Some forms of appendicitis can cause bowel obstruction , 2. Amon2, who had emergency consultation for an occlusive syndrome that had been evolving for three days prior to admission. He was complaining of abdominal pain, emesis, and cessation of passage of gas and stool. The pain had begun in the epigastrium. On examination, there was a normal temperature of 37.3 °C and a distended and flexible abdomen. The umbilical and inguinal hernia orifices were free and the rectal bulb empty. A gastroscopy performed the day before the hospitalization had concluded to an extrinsic mass compressing the gastric body associated with nodular congestive bulbopathy. An occlusion on a probable tumor of the transverse colon is evoked and a laparoscopy is first decided to locate the tumor and decide of a colectomy in one or two stages. Anesthesia was general with orotracheal intubation. We used one 10-mm optical port into the umbilical and one five-mm trocar into the right hypochondrium. At the laparoscopy, there is no colonic tumor but a significant distention of the small bowel leading to a perilous exploration. We decide to convert. At laparotomy, we discover an internal hernia of the strangulated small bowel in an orifice newly formed by the ileocecal appendix, with a plugged perforation in the throttling groove (Fig. It was a 68-year-old obese patient with a body mass index of 35 kg/mThe first three cases of small bowel obstruction due to appendicitis were described in 1901 by Hotchkiss . Since tThe appendicular tourniquet is an extremely rare complication of appendicitis. It is most often revealed by an internal hernia and its diagnosis is almost always intraoperative.


Extrahepatic bile duct duplication is an extremely rare congenital anomaly in which two common bile ducts exist. There are five different types of this anomaly and we present an unusual variant of duplication of an extrahepatic biliary system of type Va variety.This case report describes a 63-year-old women from rural Nepal who presented with type Va of duplicated extrahepatic bile duct, with chronic calculous cholecystitis and choledocholithiasis. She was managed with cholecystectomy with hepatic ductoplasty and hepaticojejunostomy.A rare case of double common bile duct (type Va) complicated by choledocholithiasis, cholangitis, and chronic cholecystitis is reported here. Rare cases are sometimes overlooked by modern diagnostic techniques. Correct diagnosis helps appropriate surgical intervention. An abdominal ultrasonogram showed multiple calculi in her gallbladder (largest 6 mm), dilated common bile duct (CBD) measuring 13 mm with dilated intrahepatic biliary ducts (IHBDs), and suspected calculi/mass in distal CBD. A contrast-enhanced computed tomography (CECT) scan of her abdomen revealed similar findings and could not differentiate mass or stone in distal CBD. Endoscopic retrograde cholangiopancreatography (ERCP) was planned with the aim of extracting the stone and, if present, a biopsy from the mass. ERCP was unsuccessful on account of difficult anatomy. Magnetic resonance cholangiopancreatography (MRCP) reported the following: IHBDs from the right lobe of the liver drained into the right hepatic duct that formed a separate CBD with the cystic duct opening into it; the IHBDs from the left lobe of the liver drained into the left hepatic duct that formed a separate CBD. Both CBDs appeared mildly dilated proximally and descended separately until a point where a stone was noted. Below this point, the CBD could not be traced so the type of anomaly was inconclusive , our patient was then planned for exploratory laparotomy to assess and confirm the radiological findings, and for definitive diagnosis and treatment of the condition. Intraoperative findings were contracted gall bladder with multiple tiny calculi, two separate draining bile ducts, which were fused just before opening to the ampulla, common duct with a calculus of 1 × 1 cmuct Fig. . So the Histologic examination of the resected specimens revealed chronic inflammatory changes in the gallbladder and common bile duct with no evidence of malignancy.et al. reviewed Japanese literature from 1968 to 2002 and found 47 patients with this anomaly [et al. (1988) [et al. (2007) [In the literature “common bile duct” is defined as a duct that directly drains into the gastrointestinal tract irrespective of its proximal anatomy . Our cas anomaly , which w. 1988)  and rece988  and . 2007) . The lea . The le. (2007) . This an. (2007) . Gastric. (2007) –15. It iA rare case of DCBD (type Va) complicated by choledocholithiasis, cholangitis, and chronic cholecystitis is reported here. Precise preoperative recognition is crucial. MRCP and multidetector CT are helpful in making the diagnosis. The case exemplifies how rare cases are overlooked by modern diagnostic techniques. Correct diagnosis helps appropriate surgical intervention.