Small and large bowel obstructions are responsible for approximately 15% of hospital admissions for acute abdominal pain in the USA and ~ 20% of cases needing acute surgical care. Starting from the analysis of a common clinical problem, we want to guide primary care physicians in the initial management of a patient presenting with acute abdominal pain associated with intestinal obstruction. Bowel obstruction is an important cause of morbidity and mortality accounting for nearly 30,000 deaths and more than $3 billion per year in direct medical costs; it is responsible for approximately 15% of hospital admissions for acute abdominal pain in the USA and ~ 20% of cases needing acute surgical care [Bowel obstruction etiology is based on a mechanical intrinsic luminal obstruction or extrinsic compression Table . AdynamiIn 90% of cases, small bowel obstruction is caused by adhesions, hernias, and neoplasms . AdhesivAn 81-year-old woman with arterial hypertension and a laparotomic appendectomy when she was 12 years old presents to the emergency department with intermittent acute abdominal pain and vomiting. The last defecation was 2 days ago, and the bowel is closed to gas. Current medications only include valsartan 80 mg daily. The body temperature is 37.5 °C, and all vital parameters are normal. The remainder of the examination demonstrates pain and localized peritonism in the lower right quadrant. Laboratory tests are normal except white blood cells at 14,000 per microliter.Questions include the following: How should this patient be evaluated and treated? What is the working diagnosis? Options include stump appendicitis, right colon diverticulitis, pelvic inflammatory disease, bowel obstruction, gastroenteritis, right renal colic, right colon cancer, bowel ischemia, or inflammatory bowel disease Fig. .Fig. 1MaA complete history along with physical exam and laboratory tests should be performed upon presentation to the emergency unit. Patients should be asked about their last defecation/bowel gas passage. Having a history of previous abdominal surgery has 85% sensitivity and 78% specificity to predict adhesive small bowel obstruction . PreviouIn bowel obstruction, abdominal pain is classically a colic onset due to an increase in motility to overcome occlusion. This is later replaced by continuous pain attributable to reduced peristalsis and dilation. Pain can be intense and untreatable with analgesics in case of ischemia  or perforation.Nausea and emesis are earlier and more represented in small bowel obstruction. An abdominal examination can detect a strong predictive sign such as abdominal distension  with a positive likelihood ratio of 16.8 and negative likelihood ratio of 0.27 ; peritonEach hernia orifice  and all laparotomic/laparoscopic incision scars should be carefully examined. Digital rectal examination and rectoscopy can be useful in patients to detect blood or a rectal mass suggestive of colorectal malignancy.Anamnesis and clinical examination can be very difficult in elderly or unconscious patients. In these patients is fundamental the evaluation of vital signs with a cardiopulmonary examination: severe bowel obstruction can cause hypovolemic shock and in case of perforation, septic shock. Abnormal vital signs or the general appearance of the patient including facial expression, skin color and temperature, and altered mental activity should alert the clinician that a patient may be in critical conditions. The most common signs of shock include tachycardia, tachypnea, cool extremities, mottled or cyanotic skin, slow capillary refill, and oliguria.A complete blood count, renal function and electrolytes , and liver function tests are suggested as the first laboratory tests. Low serum bicarbonate levels, low arterial blood pH, high lactic acid level, marked leukocytosis, and hyperamylasemia may be useful in the diagnosis of intestinal ischemia. A coagulation profile should be also tested because of the potential need for emergency surgery.Supportive treatment must begin as soon as possible with intravenous crystalloids, anti-emetics, and bowel rest.Isotonic dextrose-saline crystalloid and balanced isotonic crystalloid replacement fluids containing supplemental potassium in an equivalent volume to the patient’s losses are recommended. Nasogastric suction can be diagnostically useful to analyze gastric contents . Nasogastric suction can be also therapeutically important to prevent aspiration pneumonia decompressing the proximal bowel . A FoleyAbdominal plain X-ray is the first level radiologic study. In small bowel obstruction, plain abdominal radiographic findings are diagnostic in 50–60%, inconclusive in 20–30%, and misleading in 10–20% of patients , 13.TablP < 0.01). However, the positive predictive value did not differ significantly between clinical assessment only and with plain radiographs [In one study after radiography, the sensitivity of bowel obstruction was significantly higher than after clinical evaluation only: 74% versus 57%, respectively .In case of perforation/bowel resection with contaminated surgical fields, suture repair is preferred due to the risk of mesh infection. Diagnostic laparoscopy may be a useful tool to assess bowel viability after reduction of complicated hernias . Repair Internal hernias are treated with prompt reduction, suture repair, and bowel resection anastomosis in case of intestinal necrosis.Historically, abdominal adhesiolysis through laparotomy has been the standard therapy for adhesive small bowel obstruction. In the case of emergent surgical exploration  or for conservative treatment failure, operative laparotomic surgery is the treatment of choice , 19, 52.Small bowel obstruction caused by small bowel tumors  is treated with resection and anastomosis. Oncologic management of these tumors must be obviously considered following the same schemes of tumors that arise outside the small bowel –58.For large bowel obstruction caused by sigmoid volvulus without ischemia or perforation, the best strategy is an endoscopic detorsion procedure followed by same admission surgery that includes a sigmoid colectomy with primary anastomosis. Exclusively endoscopic therapy without subsequent surgery must be reserved for high-surgical-risk patients. In case of ischemic volvulus or failed derotation, surgery has to be performed as soon as possible. In cecal volvulus, endoscopy has no role, and surgery (right hemicolectomy) is the only option . The rolResection and primary anastomosis are the desired procedure for diverticular large bowel obstruction, and it should be attempted regardless of bowel preparation after a successful conservative treatment in the same admission . ExclusiResection and primary anastomosis are the best options for malignant large bowel obstruction in the absence of significant risk factors or perforations. Patients with high surgical risk or perforations are better managed with staged procedure . Many prospective and retrospective studies into resection and primary anastomosis in malignant large bowel obstruction have reported percentages of anastomotic leaks ranging from 2.2 to 12% , 61 compCarcinomatosis, endometriosis, inflammatory bowel disease stenosis, intussusception, post-ischemic stenosis, radiation stenosis, postanastomotic stenosis, gallstones, foreign bodies, bezoars, and tuberculosis can cause bowel obstruction in a minority of cases . Here, a computed tomography scan is critical for diagnosis. Conservative treatment should be started in the absence of ischemia or perforation, but surgery is needed as a rescue therapy for failed conservative treatments or as elective therapy to prevent recurrence .Bowel obstruction is a common and challenging surgical emergency. Further studies are needed to evaluate more precisely the role of conservative treatment in adhesive small bowel obstruction and its length. Moreover, a great debate is underway about laparoscopic adhesiolysis potentialities against possible severe technical complications. Both issues are critical to decrease the well-known adhesive small bowel obstruction recurrence risk. Stents will probably become the mainstay of cancer large bowel obstruction transforming emergency operations in elective cases decreasing complications and stomas.The recommendations in this article are concordant with guidelines published by the World Society of Emergency Surgery  Table 33.Table 3Bowel obstruction is the most likely diagnosis in the patient described in the vignette. High white blood cells and peritonism could suggest adhesive small bowel obstruction with possible ischemia. A computed tomography scan with intravenous contrast must be performed as soon as possible. If computed tomography scan confirms adhesive small bowel obstruction with ischemia or perforation, then the patient must go to surgery as soon as possible: a laparoscopic approach should be attempted.If computed tomography shows adhesive small bowel obstruction without ischemia or perforation, then a conservative treatment should be initiated: nasogastric suction and fluid replacement therapy have to be performed with a scrupulous wait-and-see strategy. After gastric contents are cleared, a water-soluble contrast administration challenge should be performed. The patient has to be monitored with regard to ischemia . Surgery has to be performed immediately in the case of clinical deterioration—preferably beginning with a laparoscopic technique.After 24 h, a plain abdominal X-ray should be performed to determine if oral contrast reached the large bowel: if positive, then oral nutrition can be started. If negative, non-operative management could be continued for another 48 h: surgery should be performed after this limit—preferably starting with a laparoscopic approach.


Nasopharyngeal carcinoma (NPC) with cystic liver metastases is so rarely observed that there are only three cases reported in the published literature.We present a case of NPC that received complete response after chemotherapy and definitive radiotherapy, but a liver cystic lesion was revealed on abdominal sonogram three months after the initial therapy. The cystic liver lesion initially resembled a simple liver cyst with fast growth, and then evolved into an abscess-like mass after a short term. Though abscess drainage was performed, and the mass shrank significantly, but it returned to previous size two months later.Surgical resection was administrated both for diagnosis and treatment, and eventually the lesion was histologically demonstrated to be a liver metastasis. Eight months after the partial hepatectomy, cystic liver metastases recurred on computed tomography (CT) scan.Though palliative systematic chemotherapy including paclitaxel, cisplatin, gemcitabine, navobine and anti-epidemal growth factor receptor (anti-EGFR) molecular-targeted therapy were performed, the cystic metastases still gradually progressed. Then Transcatheter Hepatic Artery Chemoembolization (TACE) was administrated for five times, and all the lesions were obviously decreased in size.After TACE treatment, the liver metastases maintained stable for six months, but lung metastases were noted. Finally, the patient died of liver failure.The rare cystic appearance may be a special form which exists for liver metastases of NPC, indicating poor prognosis. Oncologists need to enhance the recognition and diagnosis level of this type of metastases. Intense follow-up and early diagnosis are important. While emphasizing the importance of local therapy and personal principles for liver metastases, TACE may be a preferred method for unresectable cystic liver metastases from NPC. Liver is the third most frequent metastatic site, and liver metastases usually present as solitary or multiple solid masses on imaging, while cystic appearance is extremely rare.Nasopharyngeal carcinoma (NPC) is an epidemic in south-east Asia, especially in southern China, where nonkeratinized undifferentiated subtype accounts for approximately 95% of NPC cases and the propensity to distant metastasize is the greatest, ranging from 17% to 54%.We report a patient with NPC who presented a single cystic liver cyst at her first follow-up, the lesion evolved into an abscess-like mass afterward, then was histologically demonstrated to be a liver metastasis from NPC 7 months later. Ethics approval was gained from the Shaoxing People's Hospital Ethics Committee and the patient's consent was also obtained.2A 40-year-old female, with 3-month history of nasal obstruction and tinnitus was admitted in August 2012. Nasopharyngeal endoscopy and biopsy already had been performed in another hospital, showing nonkeratinizing undifferentiated NPC. This histopathologic diagnosis was confirmed in our center. Magnetic resonance imaging (MRI) of the nasopharynx and neck revealed the tumor was confined to the nasopharynx and the bilateral locoregional cervical lymph nodes enlarged with its greatest dimension of 2 cm. Chest computed tomography (CT) scan, ultrasound of abdomen, and whole-body bone scan ruled out distant metastases. So clinical staging was determined to be T1N2M0, IIIA according to American Joint Committee on Cancer TNM Staging System for NPC .The patient was treated with definitive IMRT to 7050 cGy for primary tumor and 6600 cGy for infiltrated regional lymph nodes. Concurrent chemotherapy based on cisplatin and 5-flurorouracil was administrated for 2 cycles and then 2-cycle chemotherapy was given subsequently to consolidate the efficiency with the same regimen. At the end of therapy, she obtained clinical complete response by nasopharynx and neck MRI.In the initial therapy, the patient had undergone abdominal ultrasonography for 4 times, and no hepatic lesions were noted during this period. Nevertheless, when she came to our hospital for 3-month conventional follow-up in April 2013, abdominal sonogram revealed a liver cystic lesion with thin wall and smooth margin of 18 × 16 mm in the right liver lobe, and the lesion was interpreted as a simple liver cyst , rare malignant tumors (biliary cystadenoma and cystadenocarcinoma) and malignant metastatic disease. The cystic nature of metastases is due to the rapid growth beyond hepatic arterial blood supply of the lesion, leading to central necrosis. Cystic liver metastases may have heterogeneous and ill-defined borders, irregular and incomplete septa, and the inner surfaces are typically ragged with mural nodules. In addition, cystic metastases present an enhanced rim on the arterial phase of CT and MRI. 18F-FDG positron emission tomography-CT (PET-CT) is much more sensitive and accurate than CT and MRI. It is recommended as the preferred modality to identify malignant tumor. However, our case denied the PET-CT check because of the high costs.It is usually easy to make the differential diagnosis between cystic liver metastases from benign hepatic cystic lesions based on imaging feature. Single cysts manifest as a well-defined homogeneous and hypodense mass, with thin wall and an absence of internal structures, when an intravenous contrast material is administrated, there is no enhancement of the wall and contents on CT imaging. Single cysts are usually asymptomatic, unless the lesions are big enough to produce pressure effect. Generally, pyogenic abscess exhibits a low-density mass and may have variable appearance on CT scans, “double target sign” which shows a central hypoattenuating lesion is surrounded by a ring of enhancing tissue and encircled by an outer rim of hypoattenuation, which is a characteristic of abscess cavity. In clinical practice, patients with liver abscess may present sepsis and space–occupying-related symptom. Most liver metastases are solid, and a small part of it is cystic. Cystic liver metastases usually come from colorectal cancer, ovarian cancer, pancreatic cancer, gastrointestinal stromal tumor, melanoma, sarcoma, neuroendocrine tumors, and so on.The cystic hepatic lesion of the mentioned patient resembled benign single cyst with fast growth at first. After a short term, it evolved into an abscess-like mass, but finally demonstrated to be a liver metastasis from NPC. Because of the atypical feature of the liver metastasis, it took 7 months to make the diagnosis. If the oncologist realized the cystic appearance of the hepatic metastases from NPC and asked the application of biopsy earlier, the patient would live longer. Another report noted a 50-year-old man with a huge hematoma in the liver which was derived from a diagnosed NPC. He was treated with intermittent drainage of the liver hemotoma while receiving neoadjuvant chemotherapy and subsequent concurrent chemoradiotherapy. Then biopsy of the cystic lesion was performed, and histologic examination revealed a metastasis carcinoma from NPC, and bone metastases were detected several months later. The third case described a pediatric patient aged 14 with NPC metastases to liver, spleen, lymph node, and bone marrow. The liver lesions resembled benign cystic disease, but showed uptake on PET-CT. She died when receiving the 5th cycle chemotherapy. The prognosis of all 3 cases was extremely poor.Cystic liver metastases from NPC are extremely rare. To our knowledge, there are only 3 cases reported in the published literature. One case reported by Kao et al was about a middle-aged woman diagnosed as NPC, who underwent standard local radiation therapy and chemotherapy with cisplatin and 5-flurorouracil. Only 1 year after initial therapy, multiple cystic hepatic lesions were found on sonogram and demonstrated to be liver metastases by aspiration. Though paclitaxel plus cisplatin and docetaxel were administrated, she died of live coma and failure of multiple organs in a short term after chemotherapy. Our case presented no liver lesions until the primary follow-up and the relative history of liver insult was denied. Most importantly, her liver tumor resection specimen was EBERs positive in situ hybridization, highly supporting the diagnosis of a hepatic metastasis. The EBV has been proved to be strongly related to NPC, and EBER in situ hybridization is a readily available method for determining whether a tumor was metastasized from NPC.Because primary squamous liver carcinoma and secondary squamous hepatic metastases are similar in morphologic and immunophenotypical characters by histology, and primary squamous liver carcinoma may also reveal cystic mass on medical imaging, in fact, to make the diagnosis of liver metastases from NPC of our case, another possibility as primary squamous liver carcinoma should be ruled out, although only 30 or so cases have been reported in the literature. Primary squamous liver carcinoma has been reported to originate from pre-exist liver cysts or be associated with teratomas or liver cirrhosis. The most common metastatic organs are lung, bone, liver, and lymph node. With the incidence of 29.3% to 36%, liver is reported as the third most frequent metastatic site with the worst prognosis, and the median survival time is 3 to 5 months. The management of metastatic NPC is essentially palliative. Retrospective studies have shown this proportion of patients obtain greatly variable clinical outcomes with different ages, anatomical sites, and numbers of metastases. Therefore, individualized strategies are recommended for metastatic NPC. Not only systematic chemotherapy and aggressive local therapy but also molecular-targeted therapy can be chosen to be performed to this group of patients.Almost half of the patients with NPC are at the advanced stage when the diagnosis is made, and 15% to 30% of patients in this group experience failure at distant sites. Paclitaxel, docetaxel, gemcitabine, and capecitabine can be chosen as the second line chemotherapy regimen, achieving 30% to 40% response.–16 However, systematic chemotherapy showed limited efforts on our patient. Epidermal growth factor receptor (EGFR) nearly expresses in all head and neck carcinoma. Anti-EGFR molecular therapy shows clinical efficiency in advanced head and neck cancer cases. NCCN guideline recommends the combination regimen as cisplatin/docetaxel/cetuximab, cisplatin/paclitaxel/cetuximab, and cisplatin/5-Fu/cetuximab for unresectable, recurrent and metastatic head and neck cancers. Nimotuzumab is a humanized IgG1 monoclonal antibody targeting EGFR. Nimotuzumab with radiotherapy has been recommended as a standard treatment for locally advanced NPC in Chinese NCCN guideline for head and neck cancers. Chinese oncologists reported that nimotuzumab plus gemcitabine treating metastatic NPC obtained objective response rate (ORR) of 61.4%, and the combination of nimotuzumab plus cisplatin and paclitaxel had ORR of 64.2% in the treatment of recurrent NPC cases. On the contrary, our patient underwent nimotuzumab plus gemcitabine and received little response.Platinum-based chemotherapy is considered to be the major systemic treatment and the most common combination of cisplatin and 5-Fu yields a response of 70% to 80%, while paclitaxel, docetaxel, gemcitabine, capecitabine combined with cisplatin achieve the similar response.,20 but of noncolorectal and noneuroendocrine carcinoma patients, the effort of hepatic resection remains controversial based on limited studies and small number of patients. Likewise, the management of liver metastases from NPC also has not been extensively investigated. Jun Huang et al reported a comparative study of the patients with NPC with hepatic metastases who were treated by partial hepatectomy or TACE, the progression-free survival and the median overall survival of the resection group was 45.2 and 21.2 months, respectively. However, in the control group, the progression-free survival and the median overall survival were 14.1 and 4.2 months, respectively. Hepatectomy has a significant advantage over TACE. Other studies were sporadic case reports regarding NPC with hepatic metastases. Weitz et al reported 2 patients with NPC with hepatic metastases who underwent partial hepatectomy had a long survival time. Another case report was about a patient with a solitary liver metastasis from NPC treated by segmentectomy, no signs of local or distant recurrence were noted during the 6-month follow-up. Compared with these reports, our case only obtained a recurrence-free interval of 3 months after the partial hepatectomy. The reasons that contributing to the short recurrence-free interval may include the relatively large size of the lesion and the absence of adjuvant chemotherapy. Adam et al analyzed 1452 patients from 41 centers who received hepatic resection for noncolorectal and nonendocrine carcinoma. He proposed that squamous histology was a poor diagnosis factor, patients with head and neck cancer with squamous histology experienced poor outcome, and the 5-year survival rate was <15%.In terms of local therapy to hepatic metastases, hepatic resection is considered to be the only local curative treatment. The role of hepatic resection has been well established in patients of colorectal and neuroendocrine carcinomas,The TACE is a widely accepted method of palliative chemotherapy in unresectable hepatocellular carcinoma and secondary liver neoplasms. Owing to hepatic arterial supply of the metastatic lesions and targeted delivery of drug in optimal doses, TACE is less invasive over systemic chemotherapy and has been proved effective in local tumor control and the improvement of life quality. Our patient had kept a relatively long period of stable status of hepatic metastases by TACE, though the liver lesions were insufficient in blood supply. It leads to a hypothesis that cystic lesions may be sensitive to TACE which can be chosen as a preferred method for unresectable cystic liver metastases from NPC, but it still needs further evidence.Despite systematic chemotherapy, anti-EGFR targeted therapy and local therapy were administrated. Our case experienced ex-hepatic distant metastases and hepatic metastases progression. Management of metastatic NPC is still a great challenge. In recent years, new systematic immunotherapeutic approaches for NPC are merging, like T-cell immunotherapy targeting EBV-infected carcinoma and anti-PD-1 therapy. However, these strategies remain experimental.4This report presents the forth case with cystic liver metastases from NPC in the published literature. This rare cystic appearance may be a special form, which does exist for liver metastases of NPC, indicating poor prognosis. Oncologists need to enhance the recognition and diagnosis level of this type of metastases. Intense follow-up and early diagnosis are important. While emphasizing the importance of local therapy and personal principles for liver metastases, TACE may be a preferred method for unresectable cystic liver metastases from NPC.Conceptualization: Qiuxia Liu.Data curation: Qiuxia Liu, Jianfang Wang, Caiping Sun, Jun Xu.Investigation: Qiuxia Liu, Jianfang Wang, Caiping Sun, Jun Xu.Resources: Qiuxia Liu.Supervision: Qiuxia Liu.


Leishmaniasis is an emerging infectious disease. Due to human migration and tourism, visceral leishmaniasis may become more common in non-endemic areas. In the Mediterranean basin, visceral leishmaniasis typically occurs in rural regions.Leishmania amastigotes were observed in the liver biopsy specimens and a polymerase chain reaction confirmed the diagnosis. Only five pathological confirmed cases of acute hepatitis due to visceral leishmaniasis have been described so far, just two in adults and both from Barcelona. A revision of the literature is performed.We present an unusual urban case of acute liver failure due to visceral leishmaniasis, following a prolonged fever of unknown origin. After obtaining negative results from the bone marrow aspirate, we performed a liver biopsy that elucidated the diagnosis. The liver involvement in visceral leishmaniasis may appear as chronic granulomatous hepatitis. However diffuse hepatitis process, a necro-inflammatory pattern, without forming granulomas were observed in the liver biopsy specimens in this case. Intracytoplasmic Acute hepatitis is an uncommon debut of visceral leishmaniasis in immunocompetent patients. Furthermore there are only few cases in the literature that describe the histopathological changes that we found in this patient. In conclusion, in case of acute hepatitis leading to liver failure, leishmaniasis should be considered a differential diagnosis  and liver biopsy can elucidate the diagnosis. Leishmaniasis is an emerging infectious disease, caused by protozoa that live inside macrophages in mammals. Sandflies become infected after feeding on the reservoir animal  or infected humans and can then transmit the parasite to other humans .Leishmania and the immune response of the host. Many cases are asymptomatic, reflecting the ability of the host immune system to control the parasite. There are two main clinical forms: visceral leishmaniasis and cutaneous leishmaniasis. Cutaneous leishmaniasis presents as single or multiple papular, nodular or ulcerous skin lesions. Visceral leishmaniasis is the most serious clinical form of this disease. Persistent fever, splenomegaly and pancytopenia characterize visceral leishmaniasis . T. T7]. ThEl Hag et al has reported histopathology changes in the liver in visceral leishmaniasis [Leishmania amastigotes. The disease is rare in Spain and no common exposure or link could be traced between the two cases. Although a clear epidemiological exposure for our case was not identified, Leishmania infantum is endemic in the Mediterranean basin and therefore, exposure could have occurred even in Spain [Leishmania detection in bone marrow samples.The liver involvement in visceral leishmaniasis may appear as chronic granulomatous hepatitis . El Hag maniasis . All casmaniasis . Very smin Spain . InteresAcute hepatitis is an uncommon debut of visceral leishmaniasis in immunocompetent patients. Furthermore there are only few cases in the literature that describe the histopathological changes that we found in this patient. In conclusion, in case of acute hepatitis leading to liver failure, leishmaniasis should be considered a differential diagnosis  and liver biopsy can elucidate the diagnosis.


Massive localized lymphedema (MLL) is a non-neoplastic benign soft tissue lesion that may be confused with sarcomas or other neoplastic proliferations both clinically and morphologically. Most occur in morbidly obese adults on the lower extremities. The objective of this article is to document a case of MLL in the retroperitoneal cavity which is a previously unreported site for this lesion, and to highlight its unusual clinical features.The patient was a non-obese male who had undergone major abdominal surgery due to bladder extrophy 17 years ago. Abdominal ultrasonography detected a large incidental mass in the right renal sinus during his investigation for nephrolithiasis. The lesion extending from renal pelvis down to pelvis was resected and its histopathological findings were compatible with massive localized lymphedema.Retroperitoneum has to be added to the list of locations that MLL can be found. Liposarcoma will be a challenging differential diagnosis when the lesion is encountered in an unusual site. Massive localized lymphedema (MLL) is a rare pseudosarcomatous lesion afflicting predominantly morbidly obese or obese individuals, first described by Farshid and Weiss in 1998 . Althoug2. USG showed a hyperechogenic lesion at the fat intensity filling out right renal sinus completely. Computerized tomography scan confirmed the presence of a fatty mass that extended from the renal sinus down to the pelvis cuffing the right ureter throughout its entire length with severe hydronephrosis. Left kidney was unremarkable except a small scar at the upper pole. The presence of high density regions inside the lesion imparted a suspicion for liposarcoma. The resection of the mass with right radical nephroureterectomy was performed.53-year-old man underwent abdominal ultrasonography (USG) during investigation for bilateral renal nephrolithiasis. The patient had normal blood biochemistry, and had no cardiovascular or hormonal disorder. He had been operated 17 years earlier to repair his extrophic bladder by creating an Indiana pouch. His weight was 85 kg.s with a body mass index of 28.7 kg/mMDM2 amplification. The findings were found compatible with MLL. The most possible predisposing factor in this current case appears to be operational trauma which occurred 17 years ago. He did not have lymphedema in the scrotum, legs, abdominal wall, or in the other regions of the body. The patient has stayed recurrence-free for the past 5 years after diagnosis.Macroscopically, the tumor was 16x13x6 cm in size, fatty in appearance marbled with irregular whitish solid areas or fibrosis Fig.  or irreg2 [The vast majority of adults with MLL are morbidly obese or obese individuals. Kurt H, et al. have reported the largest series of MLL in the literature consisting of 46 patients and the mean body-mass index of their patients was 59.6 kg/m2 –5. Anter2 , 2, 6–102 . Trauma The histopathology of MLL is rather characteristic although its differential diagnosis may include a variety of other benign and more importantly a few malignant processes. The diagnosis can be straightforward in particular when the mass is located superficially and associated with classic skin alterations. If located in the deep sites of the body as in our case, atypical lipomatous tumor/well-differentiated liposarcoma (ALT/WDLS) will be the most significant differential diagnosis clinically and pathologically. This is especially true if atypical looking spindle cells or multinucleated fibroblasts are present in the fibrous septa similar to those seen in atypical lipomatous tumor/well-differentiated liposarcoma. ALT/WDLS is composed of a relatively mature-looking adipocytic proliferation although significant variation in cell size is easily appreciable. Focal adipocytic nuclear atypia and hyperchromasia contribute to the diagnosis of ALT/WDLS, and scattered hyperchromatic as well as multinucleated stromal cells are often identified within the fibrous septa. Bizarre polylobated hyperchromatic stromal cells of liposarcoma usually contrasts with multilobated reactive stromal fibroblasts in MLL that show open, relatively pale chromatin. Atypical reactive stromal cells in the perinephric soft tissue adjacent to the kidney have also been described in cases of renal cell carcinoma . Kurt H,Immunoglobulin G4-related disease (IgG4-RD) and idiopathic retroperitoneal fibrosis can be other considerations in the differential diagnosis of retroperitoneal MLL. IgG4-RD is associated with infiltration of the tissue by numerous IgG4-positive plasma cells as well as storiform fibrosis and obliterative phlebitis. Affected patients in IgG4-RD often have high levels of serum IgG4. Because of their good response to glucocorticoid, correct diagnosis is important in their treatment to avoid unnecessary primary surgery.Progression of untreated MLL to angiosarcoma has been observed in 10.3% of cases reported in the literature [Massive localized lymphedema has recently emerged as a separate clinical entity. It is characterized by a benign large mass that grows over the years and is usually found in the lower extremity of morbidly obese patients. However, it may develop in unusual sites such as retroperitoneum in non-obese individuals particularly after major surgery. Since gross appearance and histology of MLL are similar to well-differentiated liposarcoma, awareness of its existence is essential for correct diagnosis and patient management.


A 51-year-old male presented with yellow discoloration of skin, previously healthy, back pain, and weight loss for 1 month. He had no history of asbestos exposure. Laboratory tests of liver function revealed . The patient’s serum carcinoembryonic antigen (CEA) level and CA19-9 level was normal. No abnormality was observed on chest X-ray examination. Abdominal post-contrast CT scan revealed an irregular, lobulated mass  in the head of the pancreas. The tumor enhancement was heterogeneous with indistinct boundaries, and the common bile duct and duodenum were involved. The tumor was adjacent to the portal vein, the superior mesenteric vein, the inferior vena cava, and the right renal vein. No enlarged lymph nodes were observed in the pelvic cavity, retroperitoneum, or bilateral inguinal region (Fig. The tumor was not amenable to radical surgery because of its location and size. The patient was treated with palliative chemotherapy. After two cycles of pemetrexed plus cisplatin, efficacy evaluation revealed progressive disease. The patient decided to cease chemotherapy and received the best supportive care. The patient died of cachexia 4 months post-diagnosis.1 and the spleen.2 The therapy for pancreatic mesothelioma is primarily surgical. Radiotherapy and chemotherapy have very limited effect in pancreatic malignant mesothelioma, and non-surgical therapy has only been suggested in a few cases with very extensive and inoperable lesions. The prognosis of pancreatic mesothelioma is different depending on the pathological type and stage. Rosanny et al.3 reported a case of intra-pancreatic epithelioid malignant mesothelioma. Follow-up revealed no evidence of residual or recurrent disease 32 months after pancreatoduodenectomy without adjuvant radiotherapy and chemotherapy. However, in our reported case, the tumor was not amenable to radical surgery because of its late stage. Although the patient was treated using palliative chemotherapy, the patient did not benefit as an efficacy evaluation revealed progressive disease after two treatment cycles, and the survival period was approximately 4 months after diagnosis. The prognosis of intra-pancreatic SMM may be poor because of its highly invasive behavior, local recurrence, and metastasis.Primary malignant mesotheliomas rarely develop in solid organs, and little is known about the behavior of such tumors. Here, we provide the first report of a case of intra-pancreatic SMM. Clinical diagnosis of pancreatic mesothelioma is difficult because of a lack of specific clinical symptoms and CT/MRI imaging findings. Histopathological and immunohistochemical examinations play an important role in the diagnosis of mesothelioma. Unlike conventional peritoneal mesothelioma, peritoneum metastasis and cavity effusion are rare at the time of initial diagnosis in solid organ cases, such as the liver


Anterior lumbar interbody fusion (ALIF) is commonly utilized for surgical management of degenerative lumbar pathology. Although it is a reasonably safe procedure, it can potentially lead to major complications in case of neurovascular injuries. Occurrence of lymphocele after an ALIF is however rare.We present a case of a rare abdominal lymphocele in a 56-year-old man who underwent L3-S1 ALIF and subsequently developed an abdominal lymphocele. A lymphocele can manifest in numerous ways which can affect and possibly delay diagnoses. In addition to a high index of suspicion, numerous tests such as imaging studies, fluid analysis, gram stain and culture are used to confirm the diagnosis.Various options exist for the treatment of lymphoceles, including laparoscopic marsupialization, ultrasound-guided aspiration, sclerotherapy, peritoneal window, and external drainage. Timely diagnosis and treatment of a lymphocele results in a successful resolution in most cases. Anterior lumbar interbody fusion (ALIF) is a commonly utilized modality for fusion of a wide array of lumbar degenerative pathology such as intervertebral disc degeneration, degenerative scoliosis and spondylolisthesis. It is an attractive option for the surgeon because it provides access to the entire disc space and allows a large interbody spacer that can restore lordosis and achieve high fusion rates. On account of the anterior approach it has the potential for major complications such as vascular injury, visceral damage, ureteral damage, and retrograde ejaculation. An abdominal lymphocele is a rare complication associated with this procedure -14. We rA 56-year-old man presented to us with chronic, progressive midline low back pain associated with right lower extremity radiculopathy. The patient described the pain as radiating and achy. On the physical exam, the patient had decreased sensation in the lateral aspect of the right lower extremity. Radiological findings demonstrated multilevel central stenosis and neuroforaminal narrowing with a degenerative scoliotic deformity of the lumbar spine Figures , 2. A coVarious surgical options were considered. Eventually the patient underwent an ALIF at the L3-S1 levels using a retroperitoneal approach followed by a T10-pelvis posterior spinal fusion and a right L4-L5 laminotomy and decompression in two stages (Figure The fluid was serosangionous in nature with a high output of greater than 1500 cc/day. Fluid gram stain showed no organisms and cultures also revealed no growth. He was discharged home with the drain in place for a total of 22 days after which the drain was discontinued upon clinic follow-up. The lymphocele recurred four days after the drain was removed, and the patient presented back to the emergency room with recurrence of abdominal distension. A new drain was placed again in the fluid collection, and was finally removed for paucity of drainage three weeks later. The patient’s symptoms as well as the lymphocele resolved and did not recur.An abdominal lymphocele is a rare complication of anterior lumbar interbody fusion -15. PresLymphocele accumulation in the retroperitoneal area can be asymptomatic, but may also manifest in abdominal symptoms. If leakage is constant it can cause nutritional deficiencies and can ultimately result in death with a mortality rate approaching 50% ,14. It iThe low incidence of symptomatic lymphoceles post ALIF can be attributed to the absorption of the leaked lymphatic fluid by collateral lymphatic vessels . ConsequAbdominal lymphocele is a rare yet significant complication that can present after an ALIF. Utilizing fluid analysis and imaging studies correctly are vital in a timely diagnosis of such a complication. While most retroperitoneal lymphoceles are asymptomatic and resolve spontaneously, some present with significant symptoms and require drainage or surgical intervention. There are many ways to approach treatment of an abdominal lymphocele that vary due to a surgeon’s preference. While this does not manifest in any long-term complications, it is a rare acute complication post ALIF that surgeons should be aware of.


The standard of care for benign pheochromocytomas and paragangliomas is surgical resection; however, there is no definitive curative or standard therapy for the treatment of malignant pheochromocytomas or paragangliomas. Current therapeutic options include surgical resection, chemotherapy (CVD therapy), MIBG-radiotherapy, somatostatin analogues, and combination targeted therapies such as temozolomide and thalidomide. Although some patients demonstrate short-lived responses to these various therapies, there has been no statistically significant survival benefit with any of the current regimens and therefore the aim is palliation and symptom control. Here, we present a 38-year-old man found to have an unresectable metastatic paraganglioma located in the Organ of Zuckerkandl who has been treated with single-agent thalidomide for over 15 years with minimal growth and stabilization of metastatic lesions. The management of our patient with single-agent therapy for this extended period of time challenges the efficacy and role of chemotherapy and other accepted therapies for malignant pheochromocytoma and paraganglioma. Pheochromocytomas are rare catecholamine-secreting tumors, the majority of which are benign and located within the adrenal medulla. Approximately 10% of these tumors are malignant. According to the World Health Organization, malignancy in a pheochromocytoma is defined by presence of regional or distant metastases associated with the primary pheochromocytoma . There aExtra-adrenal pheochromocytomas, or paragangliomas, have a much higher probability of being malignant , 2, 4, 5Standard treatment for benign pheochromocytomas and paragangliomas is surgical resection. There is, however, no current definitive curative or standard therapy for malignant pheochromocytomas or paragangliomas partly due to the rarity of these tumors. Current therapeutic options include attempts at surgical resection; chemotherapy consisting of cyclophosphamide, vincristine, and dacarbazine (CVD); MIBG-radiotherapy; somatostatin analogues; and new antineoplastic therapies including temozolomide and thalidomide –5. TheseDespite multiple treatment options, the 5-year mortality rate in patients with malignant pheochromocytomas and paragangliomas is greater than 50% in some reports . Here, wAn otherwise healthy 38-year-old man was found on CT to have a large 20 cm × 9 cm mass near the aortic bifurcation with associated areas of calcifications and low attenuated areas concerning for necrosis. In addition, bilateral lung masses, the largest measuring 2 cm, and multiple liver lesions with the largest measuring 3 cm, consistent with metastases, were identified. The patient underwent a CT-guided biopsy of the abdominal mass at an outside community hospital. Pathology and immunohistochemistry staining of synaptophysin and chromogranin established that the mass was consistent with a paraganglioma of the Organ of Zuckerkandl and the patient was subsequently referred to our center.Full laboratory workup was performed. Twenty-four-hour urine-free catecholamine evaluation revealed elevated epinephrine and norepinephrine levels , norepinephrine: 567 mcg/24 hours , dopamine: 231 mcg/24 hours ). Twenty-four-hour metanephrine levels were obtained and demonstrated elevated metanephrine and normetanephrine levels , normetanephrine: 2082 mcg/24 hours ). SDH gene testing was not performed and DOTATE PET CT imaging was unavailable.After discussion with the patient, he was started on single-agent thalidomide 200 mcg daily based on the lack of effective chemotherapy. The patient was followed closely with repeat imaging every 3 months. Throughout the initial portion of his treatment he remained asymptomatic; however, as his treatment course progressed, he became increasingly hypertensive which was easily controlled with doxazosin. CT scans demonstrated stability in lung and liver metastases as well as significant regression of the paraganglioma to nearly 50% of the original size. Despite stability of disease, after approximately 18 months of thalidomide treatment, he became increasingly symptomatic with mild palpitations. Subsequently, he underwent an exploratory laparotomy in attempt for surgical resection but intraoperatively the tumor was deemed unresectable due to encasement of the aorta and inferior vena cava. With these operative findings, the patient was continued on thalidomide and continues to be closely followed.At the current time, over 15 years since his initial diagnosis, the patient remains on single-agent thalidomide for tumor control and single antihypertensive therapy without adverse effects. Over the past 15 years, the primary tumor at the Organ of Zuckerkandl and one of the multiple liver metastases have both slowly enlarged by approximately 5 cm and 1 cm, respectively . The remHere, we presented a patient with a large, malignant paraganglioma of the Organ of Zuckerkandl with metastases to the liver and bilateral lungs treated with single-agent thalidomide for over 15 years. Our patient presented with three known risk factors for a malignant pheochromocytoma: size greater than 5 centimeters, extra-adrenal origin, and local invasiveness.There is currently no curative or standard therapy regimen for malignant pheochromocytomas or paragangliomas. Most treatments do not show any survival benefit, and surgical resection is not curative –5. The tOne treatment regimen that is tolerated relatively well is the combination antineoplastic therapy of temozolomide and thalidomide, which is considered a targeted therapy for neuroendocrine tumors . TemozolMost patients regardless of the therapy administered for treatment of malignant pheochromocytomas or paragangliomas have a poor prognosis with a historical five-year survival rate of less than 50% . We presMalignant pheochromocytomas and paragangliomas are rare and challenging tumors to manage for many reasons as there are no identifiable histological or biochemical differences to predict benign and malignant disease. The determination of benign versus malignant disease lies solely in the presence or absence of local or distant metastatic disease. These tumors are rare enough that the biology of the disease is still being investigated and is not fully understood. Due to this lack of full understanding of the disease, the treatment options for malignant pheochromocytomas or paragangliomas are directed toward symptom control and no standard of care treatment regimen has been elucidated. Here was presented a case of a single patient who after diagnosis of a malignant paraganglioma of the Organ of Zuckerkandl was managed on single-agent thalidomide for over 15 years with initial partial response and now stability of disease. The question remains as to whether his response is due to his treatment, disease biology, or a combination of both.


A 30-year-old Caucasian man presented with an 18-month history of an asymptomatic calcified mass, located on the buccal side of the alveolar ridge. Medical records did not present any underlying conditions. On intraoral examination, the lesion was located on the right side of the maxilla, showing mucosal fenestration with mineralized tissue measuring approximately 1 cm in diameter. Radiographic examination showed multiple radiopaque masses. Incisional biopsy was performed, and histological analysis revealed a presence of enamel matrix, dentin, and cementum, resembling tooth-like structures. Surgical removal was offered after the diagnostic confirmation of peripheral odontoma, but the patient refused because of the asymptomatic nature of the lesion. Odontomas are the most common type of odontogenic tumors, being considered hamartomas when associated with dental development failure . Two typPO is extremely uncommon, with less than 25 cases reported in the literature , 5. PO aThe purpose of this article is to present a new case of erupted peripheral odontoma of the maxilla in a 30-year-old patient and to review and discuss the characteristics of the cases of PO in the craniofacial region described in the literature.A 30-year-old male patient was referred with a diagnosis of an asymptomatic calcified mass located on the right side of the anterior maxilla, lasting 18 months. On the intraoral examination, a mucosal cutaneous fenestration with a mineralized tissue measuring approximately 1 cm in diameter was observed . A periaIncisional biopsy was performed for the removal of the fragment, which then was placed in 10% neutral-buffered formalin and sent for histopathological analysis. The gross examination of the calcified specimen revealed a yellowish tumor with 4 × 4 × 5 mm.The histopathological analysis revealed structures composed of enamel, dentin, pulp chamber, and cement in the same order of arrangement as that of a normal tooth. A mature tubular dentin and an enamel matrix were also observed .After the diagnostic confirmation of erupted peripheral odontoma, surgical removal was performed to reduce the lesion. The patient was free of symptoms after the procedure, and no complications were recorded.Odontomas, by definition, refer to any tumor of odontogenic origin, although these entities are truly considered hamartomas . OdontomPO is usually asymptomatic and detected during routine radiographic examinations or once there is a delay in tooth eruption . The differential diagnosis of OP includes other tumors of odontogenic origin, exostoses, and osteomas , with thThe histogenesis of odontomas is primarily associated with remnants of soft tissues of the odontogenic epithelium, such as the gingival rests of Serres, which could lead to the production of mineralized structures similar to teeth as a result of odontoblastic hyperactivity and changes in the genetic component responsible for controlling dental development, including a reduction of epithelial-mesenchymal interactions .This condition can also be attributed to some pathological conditions, such as local trauma, inflammatory process, and infectious and genetic anomalies , 5.Some of the peripheral odontomas reported in the literature might be erupted odontomas because of intraosseous lesions, often related to unerupted or spontaneously erupted teeth . HoweverIn the present case, the patient was referred to an evaluation due to exposure of the mineralized tissue in the oral cavity, suggesting a growth of the lesion and the presence of mild discomfort in the adjacent periodontal area. The type of force that may have led to mucosal fenestration was probably caused by physiological bone resorption, since there was no report of delayed dental eruption.Histologically, PO resembles intraosseous odontomas, which can be classified as compound and complex. However, the absence of bone tissue is a finding also observed in the present case. This fact occurs due to the absence of bone erosion under the tumor, supporting the hypothesis of the development of this odontoma in the gingival tissue , 8.Clinically, some authors have described odontomas that erupted in older patients and whose lesions were histologically characterized as complex odontomas associated with noneruption of posterior teeth . ConversPeripheral odontoma is a rare benign odontogenic lesion that can be treated by local excision with good results. Both young and adult patients can present these alterations. A comprehensive evaluation with radiographic and histological examinations is important to establish the differential diagnosis and prevent unnecessary extensive resections.


Congenital infantile fibrosarcoma is a rare mesenchymal tumor seen in children as well as adults. The congenital variety is rare and out of the reported cases only one case sited in the neck has been reported so far. Another such case is presented here who was successfully managed.A 3-month-old Hindu baby boy presented with a congenital neck swelling. The apparent clinical diagnosis was lympho-venous malformation. With a remote possibility of malignancy, an excisional biopsy was done. Histopathology revealed congenital infantile fibrosarcoma.A successful excision of cervical congenital infantile fibrosarcoma has not been reported. This diagnosis should be kept as a possibility in all congenital cervical swellings. These are commonly misdiagnosed as lympho-venous malformations and histopathology is confirmatory. Congenital infantile fibrosarcoma (CIFS) is a rare mesenchymal tumor which, contrary to its name, is seen both in children as well as adults. The congenital variety is even rarer with only a few reported cases. Among the sites of origin of these congenital lesions, occurrence in the neck has been reported only once before . The casA 3-month-old Hindu baby boy presented with a congenital neck swelling on the right side of his neck. There was no history of birth trauma or breech delivery. Initially a small midline swelling, it progressively increased in size with age. It was soft and compressible with an overlying bluish hue at places. With a working diagnosis of a low flow lymphovascular malformation at another hospital, intralesional bleomycin was injected once after which the swelling became a little firm without any change in its size. One month after the bleomycin injection, it was a 5.5 × 7.5 cm firm, non-tender, well-defined swelling in the midline and extending into the right supraclavicular region Fig. . There wCIFS is one of the common non-rhabdomyosarcoma soft tissue tumors in children with an incidence of around 5 per million cases in the pediatric age group . AlthougThe differential diagnoses include a wide range of fibroblastic and myofibroblastic tumors. Pediatric fibroblastic and myofibroblastic tumors on the basis of their biologic behavior have been divided into various entities . These hComposite infantile myofibromatosis of the scalp with several distinct histopathological features including myofibroma, hemangiopericytoma, and fibrosarcoma have been described in newborns . An infaCIFSs, being vascular lesions, have been notoriously misdiagnosed as hemangiomas and lympho-venous malformations at birth and earlier, as was seen in the present case , 13. SusWide local excision, without any mutilating surgery, is the mainstay of management . In caseThe European Pediatric Soft Tissue Sarcoma Study Group  has developed conservative treatment recommendations according to initial resectability of the tumor . Initialin situ hybridization (FISH) in four additional fibrosarcomas occurring in patients aged 6–17 years. One of three cellular fibromatoses was characterized by extra copies of chromosome 8, 11, 17, and 20. Similar findings were not observed in any of the noncellular fibromatoses or in myofibromatoses [ETV6-NTRK3) gene fusion product identified by RT-PCR has been recognized as diagnostic of infantile fibrosarcoma. ETV6-NTRK3 transcript was present in 87.2% of patients where the investigation was performed by the European Pediatric Soft Tissue Sarcoma Study Group [et al. found that 9 out of 2031 advanced cancers from patients less than 21-years old (0.44%) harbored NTRK fusions [ETV6-NTRK3 [NTRK fusions occur in a subset of young patients with mesenchymal or sarcoma-like tumors at a low frequency, and are potential good targets for drugs. A case of refractory infantile fibrosarcoma (IFS) with constitutive activation of the tropomyosin-related kinase (TRK) signaling pathway from an ETV6-NTRK3 gene fusion experienced a rapid, radiographic response, thus depicting the potential for LOXO-101  to provide benefit for IFS harboring NTRK gene fusions [Although CIFSs are biologically more benign than their counterparts occurring in older patients, they are histologically similar. CIFSs belong to one end of a spectrum of fibrous proliferations occurring in children. Gains of chromosomes 8, 11, 17, and 20 (in various combinations) were observed in 11 of 12 fibrosarcomas occurring in infants under 2 years of age [dy Group . Pavlick fusions .ETV6-NTRK3 gene fusion due to technical resource constraints.Histopathologic characteristics include a solid, dense proliferation of spindle cells in interlacing bundles; positive for vimentin, and occasionally for desmin, SMA, and cytokeratin . SimilarThe incidence of metastatic spread of disease is 5–8% . The orgTo conclude, CIFSs should be kept in the differential diagnoses of soft tissue tumors in infants, even in congenital cases. The clinical picture is similar to lymphovascular malformations which might lead to misdiagnosis of these tumors. The mainstay of treatment is complete excision. However, chemotherapy does have a good response and can be a preferred option if surgery is not possible without major anatomical compromise. Overall survival in these tumors is excellent.


Hair thread tourniquet syndrome (HTTS) is a rare condition where fibres constrict around appendages causing ischaemia and necrosis. It is a sporadically reported condition, where almost all reported cases showed involvement of fingers, toes or genitalia. A significant number of the cases are infants aged two weeks to six months where it is attributed to the mother’s excessive hair fall due to hormonal changes after delivery. We present a two-month-old infant who was irritable for the past two days with her left ring finger exhibiting an ischaemic constriction with no apparent insulting agent. She successfully treated surgically after we suspected an incomplete removal of hair thread in the emergency department. We would like to highlight the importance of a high index of suspicion in cases as such as early intervention saves the appendage.  Hair thread tourniquet syndrome (HTTS) is an uncommon condition in which an appendage is constricted by a fibre leading to ischaemia and subsequently necrosis. The annual incidence of HTTS is 0.02%, and it presents as an emergency . AttendiOne of the possible insulting agents to cause HTTS is human hair, which is known to have a tensile strength greater than 29,000 pounds per square inch. Given its nature that expands when wet and tightens when dry, the human hair is capable of constricting any appendage of the body constricting blood vessels that may lead to a deadly appendage . A two-month-old infant presented with persistent irritability despite all efforts to comfort her after one day, when the mother noted there was a swelling and constriction of her left ring finger upon removing her mitten. Physical examination revealed marked oedema, redness and congestion distal to the metacarpophalangeal joint of the ring finger Figure . There wWe explored the finger in the emergency department using loupes (3x magnification) and a pen torch. A few fine hair strands were seen strangulating just distal to the metacarpophalangeal joint and cut free Figure . AlthougUsing the microscope in the operating theatre, we confirmed that there were no more hair threads detected. As the hair strand had strangulated the finger, and since we wanted to reduce the oedema, we decided to undermine the constricting mark and close it with a vertical mattress suture Figure , 2B. TheThe aetiology of HTTS is currently not well known. However, the risk of getting HTTS is increased in an infant aged two weeks to six months due to telogen effluvium. Telogen effluvium is pronounced following pregnancy due to the endocrine flux, which leads to an excessive hair loss experienced by mothers in the post-partum period. This condition poses a higher chance of the hair strangulating the appendage as in the case above . AnotherAlthough most cases are accidental, child abuse cannot be excluded in cases that involved multiple appendages and ecchymoses noted on the infant's body . Other dThe outcome of the affected appendage largely depends on the prompt detection, correct diagnosis and early intervention to completely remove any encircling fibres to restore the circulation. This can be done with the aid of a magnifying glass in the emergency setting or in the operating theatre. When the appendages are severely edematous, it is difficult to determine whether the constriction has been removed completely. This is exemplified in our case, where we had difficulty confirming complete removal of all hair strands due to the oedema. Surgical exploration is mandatory if a complete removal cannot be achieved or satisfactorily confirmed and should be done under general anaesthesia to allow optimal exploration. In this case, the Z-plasty approach was used in the exploration to prevent potential risk of scar contracture which may occur.If the constricting agent is hair, the use of a depilatory agent is possible with minimal discomfort to the child . HoweverEducation is to be targeted to both parents and attending doctors to raise awareness of this uncommon diagnosis. Parents should be advised to launder their children clothes inside and to avoid using coverings over their children extremities for a long period of time without supervision .HTTS is an uncommon occurrence. We should have a high index of suspicion as early diagnosis is vital in determining the outcome of the appendage. Early intervention either by non-surgical or surgical method to release the strangulation of the affected appendage is important to prevent ischaemic necrosis.


Study Design Present study is a case report.Objective This study was to present a rare case of acute postpartum paraplegia due to spontaneous spinal epidural hematoma (SSEH).Background SSEH with incidence rate of 0.1 per 100,000 per year is an extremely rare cause of sudden onset neurological deficit in postpartum patients with no predisposing factors or intrapartum factor causing SSEH resulting in paraplegia.Material and Methods We hereby present our case of acute onset postpartum paraplegia which on magnetic resonance imaging (MRI) shows epidural hematoma around thoracic 12 to lumbar 2 (T12–L2) vertebral regions with adjacent cord changes. MRI is the investigation of choice in such cases which helps in proper timely management of patient. We planned the patient for surgical decompression of hematoma.Results Patient showed rapid reversal of neurological symptoms in postoperative period.Conclusions Acute onset postpartum paraplegia in a healthy female with no significant past history, predisposing factors or intrapartum factors may be caused by SSEH and it should be managed on emergency basis as early and proper treatment has an excellent prognosis as seen in our case. Spontaneous spinal epidural hematoma (SSEH) is an extremely rare entity with incidence of 0.1 per 100,000 every year.A 29-year-old female patient was referred to our department with acute onset postpartum paraplegia with bowel bladder involvement after delivery of her third child by normal vaginal delivery. Her antenatal course had no significant history with any history of antepartum low-back pain, trauma, drug use, or any physical exertion. On examination, she was conscious, well oriented, and afebrile. Neurological examination showed complete paraplegia (power 0/5 with decreased tone), associated bowel bladder involvement, and absent deep tendon reflexes at knee and ankle joint with complete sensory deficit below the level of L1. All necessary blood investigations including coagulation profile came out to be normal. Patient was planned for magnetic resonance imaging (MRI) of dorsolumbar spine with screening whole spine, which revealed extradural broad based ellipsoid lesion, measuring 50 × 9 mm along T12 to L2 vertebra displacing the dura anteriorly and T1 hyperintensity , T2 hypOn the basis of clinical evaluation and MRI report, the patient was planned for urgent decompression in emergency operation theater. After preoperative marking of D12–L2 vertebral levels under fluoroscopic guidance, midline exposure done and paraspinal muscles elevated upto medial edge of transverse processes on both sides with the help of cobb's and broad osteotome. Intraoperatively, bilateral complete laminectomy of L1 vertebrae was done with removal of ligamentum flavum, a well-defined firm organized hematoma dark reddish black in color (5 × 1 cm) was seen over the dura which was removed  and cheIn 1869, first case of SSEH was reported by Jackson , acute spinal ischemia, spondylitis, transverse myelitis, and dissecting aortic aneurysm.MRI is the best modality to evaluate and diagnose SSEH. It helps in early diagnosis, accurate determination of haematoma size, level of involvement, adjacent spinal cord changes, and amount of cord compression so that patient can be managed in time and properly with surgical intervention if requiredCommonly done procedure for SSEH includes complete laminectomy with hematoma decompression. We have done the same in our patient with excellent follow-up result. When SSEH occurs due to coagulation abnormalities or associated with mild neurological deficit or neurological deficit improving on its own, such cases can be managed conservatively with success.Acute onset postpartum paraplegia in a healthy female with no significant past history, predisposing factors or intrapartum factors may be caused by SSEH which is an extremely rare pathology during pregnancy/postpartum. Such cases must be managed on emergency basis as early and proper treatment has an excellent prognosis.


Spontaneous intra-parotid pseudoaneurysm of the external carotid artery are very rare, only one case has been reported in the literature until 2019. The authors describe the second rare case of a spontaneous pseudoaneurysm of the external carotid artery which mimicked a parotid neoplasm in a young patient with pulsatile and non-fixed mass. The clinical presentation, diagnosis, and management of this condition are discussed. We report a new etiology of this rare entity that should be considered in the differential diagnosis of an atypical parotid mass. •Vascular lesions are a rare cause of parotid masses.•Only one case of spontaneous intra-parotid pseudoaneurysm of the external carotid artery has been reported in the literature until 2019.•The etiology of this spontaneous lesion remains unknown, this is the first time that kimura disease has been described like an etiology of this entity.•Management of this lesion inclurdes observation, surgical excision, pressure dressings and embolization. The SCARE 2018 statement: Updating consensus Surgical CAse REport (SCARE) guidelines. International Journal of Surgery 2018; 60:132–136.Parotid masses are frequent in general practice of an otorlaryngologist, with benign tumors the most common cause in adult patients. Other causes, such as malignant lesions, are less frequent. Vascular lesions are a rare but important to be known by clinicians.The authors describe the second case in the literature of spontaneous intra-parotid pseudoaneurysm of the external carotid artery and the first due to a rare etiology.2We report a case of 22-years-old man, without past parotid inflammation, trauma or history of surgery, presented with slowly progressive and a palpable mass over the left parotid since 4 years.Initial clinical examination demonstrated a palpable, pulsatile and non-fixed mass, measuring 3 cm in diameter, with small neck masses. He had no weakness of her facial nerves.Fig. 1ClWe refer patient for ultrasound examination with Doppler of the lesion that strongly suggested the vascular nature of the mass. A contrast MRI study was requested owing to the finding of clinical and ultrasound examination. MRI demonstrated a well encapsulated lesion, 20 mm in diameter, in the superficial lobe of the left parotid gland. This lesion was hyperintense T1 and T2 confirming the diagnosis of pseudoaneurysm mimicking an intra-parotid mass , Fig. 3.Following discussion with the patient, the decision was to perform a surgical resection of the pseudoaneurysm starting by a superficial parotidectomy with identification and dissection of facial nerve after ligation of the facial artery. The patient was operated without incident, with a good postoperative warning .Fig. 4PeDefinitive histological examination confirmed the diagnosis of pseudoaneurysm of the external carotid artery with Angiolymphoid hyperplasia and eosinophilia compatible with Kimura diseases.The patient is undergoing regular review in the outpatient clinic at 3-month intervals during one year, and has been advised to contact the department of internal medicine for more investigations especially renal function tests, revealed without anomalies.3Pseudoaneurysms of the extra-cranial carotid arteries are uncommon and are most commonly post-traumatic, involving the internal carotid artery . Only 14The etiology of this spontaneous lesion remains unknown . WoodhouIn this present case, the vascular origin of parotid mass was suspected clinically, owing to the pulsatile nature of the mass, this was helpful in suggesting the diagnosis and requesting special radiological investigations. In the case of Fernandez et al. this sign was absent and the diagnosis of pseudoaneurysm of the external carotid artery was established intraoperatively .Duplex ultrasound (Doppler) offers a rapid noninvasive method for detecting carotid arterial injuries, but cannot be recommended as a sole diagnostic method . For LevManagement of this lesion includes observation, surgical excision, pressure dressings and embolization . The decClinical presentation and histopathologic examination of the vascular, lymph nodes and skin lesions confirmed the diagnosis of Kimura disease (KD) responsible for the intra parotid pseudoaneurysm.Kimura disease is chronic inflammatory and rare disorder usually seen in young adults that most commonly presents as painless lymphadenopathy or subcutaneous masses in the head and neck region, associated with pruritus . Renal dThe optimal treatment for KD is not well established, surgical excisions have been considered as the gold standard, especially for located lesion. The use of steroids, cytotoxic agents, cyclosporin, pentoxifylline, and radiotherapy, has been documented with variable results .4Although rare, spontaneous pseudoaneurism of parotid should be concedered in differencial diagnostic of parotid masses. Our patient is the first case reported in the literature of an intraparotid pseudoaneurism of the external carotid artery due to the Kimura disease.Written and signed consent has been obtained from the patient to publish this case.No sources of funding to declare.Omar Iziki: Corresponding author writing the paper.Redallah Larbi Abada: study concept.Sami Rouadi: study concept.Mohammed Roubal: correction of the paper.Mohammed Mahtar: correction of the paper.All the authors have no personal or financial conflicts of interest regard this case report.None.Omar Iziki.Written informed consent was obtained from the patient for publication of this case report and accompanying image.Not commissioned, externally peer reviewed.


Scleroderma is a rare disease of unknown etiology, which is characterized by thickening and hardening of skin due to an increased collagen production. A 44-year-old female patient with a scleroderma on the scalp known by our department, also presented an ipsilateral brain lesion since 2015, which was showing growth without any clinical symptomatology and the patient wanted the lesion to be removed. This atypical lesion underneath the scleroderma shows that diagnosis can be missed without brain imaging and biopsy. By definition, localized scleroderma involves the skin and the underlying tissue . The mosng (MRI) . Cerebrang (MRI) , 5, 6, eA 44-year-old of Asian origin female was followed up in our department since December 2015. She was referred by her family doctor due to a left frontal lobe cystic lesion. The patient has been following herbal treatment for her scleroderma since she was 3 years old in China. The patient had an MRI of her brain done in China, because a Chinese medicine practitioner in Toronto told her that she might have something in the brain due to scleroderma. The scleroderma was localised in the left frontal side of her scalp . She didet al. reported 2 cases in children [Borrelia Burgdorferi, has been implicated in Europe and Japan, but not confirmed in the United States [et al. [The patient had a mild increase in the lesion without any neurological deficiency and she asked for surgical treatment. Surgical indication was probably controversial at that time, although there was an increase in tumour size. Considering that the lesion was growing and she is a young patient, we carried forward with the surgery. This present case is remarkable for several reasons, but the main one is because of the uncommon mixed presentation. To our knowledge, this is the third case report presenting with a cavernoma ipsilateral to the localized scleroderma. Fain children . The locchildren . Cavernochildren . The patchildren -11. Therd States , 13. Gend States  and famid States , 15. Howd States , 17. Int [et al.  and K. T [et al. , showingThis case report highlights that patients diagnosed of a Linear Scleroderma should have an MRI of the brain to exclude any underlying pathology and to be monitored yearly to check the lesion´s behaviour.The authors declare no competing interests.


Pheochromocytoma is a rare tumor usually arising from the adrenal medulla . We report a case of pheochromocytoma presenting as orthostatic hypotension and electrolyte imbalance. A 51-year-old woman was admitted because of vomiting and chest pain. She had fluctuating blood pressure (BP) with episodes of orthostatic hypotension. Computed tomography pulmonary angiogram was performed to rule out pulmonary embolism; it showed a clear chest, but an incidental right suprarenal mass. The biochemical analysis supports the diagnosis of pheochromocytoma. Her electrolyte panel revealed persistently low potassium, calcium, and magnesium levels despite aggressive replacement. We speculated that hypotension was mainly due to vasodilatation caused by excess plasma epinephrine and prescribed doxazosin and a nonselective beta-adrenergic blocker which stabilized BP. The right adrenal tumor excised, and postoperatively she remained hemodynamically stable with no hypotensive episode. Laboratory data taken six weeks after surgery show normal 24-hour urine metanephrine and normetanephrine and normal serum magnesium and calcium levels. This case report highlights the variable presentation of pheochromocytoma. We also discuss the probable mechanisms of electrolyte imbalance in our case. It usually presents with palpitations, headache, and sweating along with hypertension. The diagnosis is confirmed by increased serum catecholamine levels, and by anatomical localization. The mainstay of definitive therapy is surgical resection . We repoA 51-year-old female with a past medical history of type 2 diabetes mellitus presented with complaints of chest pain for three days and vomiting for one day. She had episodes of palpitation, sweating, and weakness for the last three years. These episodes were self-resolving and last for 10-15 minutes. On admission, her blood pressure (BP) was 130/80 mmHg, pulse was 117/min, respiration was 24/min, and oxygen saturation (SpO2) was 100% in room air. While in the hospital she had episodes of orthostatic hypotension with systolic BP ranging from 60 to 130 mmHg and diastolic BP 30-90 mmHg. These episodes were associated with sweating, palpitation, and anxiety and resolved spontaneously. Her pulse remained high ranging between 110 and 120/min. Her electrolyte panel revealed persistently low magnesium, calcium, and potassium levels despite aggressive replacement. She also had persistent hyperglycemia requiring an insulin drip. Computed tomography (CT) angiogram was performed to rule out pulmonary embolism. Incidentally, a large suprarenal hypoattenuating mass was seen pressing on the right kidney. The CT scan of the abdomen with contrast and magnetic resonance imaging (MRI) confirmed it as 11 cm x 11 cm right suprarenal heterogeneous mass as shown in Figure Further workup along with a normal blood urea nitrogen (BUN), serum creatinine, and parathyroid hormone levels is listed in Table We made a diagnosis of pheochromocytoma and scheduled her for surgery. Preoperative management consisted of doxazosin 1 mg once daily, propranolol, and IV fluids. Orthostatic hypotension was abated. Three weeks later right adrenalectomy was performed. The pathology revealed a large tumor and a diagnosis of pheochromocytoma. The gross appearance and histology are shown in Figures Postoperatively, she remained hemodynamically stable with no hypotensive episode. Her electrolytes and blood glucose remained normal. Laboratory data taken six weeks after surgery show normal 24-hour urine metanephrine and normetanephrine and normal serum potassium, magnesium, and calcium levels.123I-labeled meta-iodobenzylguanide (MIBG) scintigraphy [Pheochromocytomas are rare, catecholamine-secreting, neuroendocrine tumors usually arising from chromaffin cells of the adrenal medulla. The classic symptoms of pheochromocytoma are headaches, palpitations, anxiety, diaphoresis, and paroxysmal or persistent hypertension . The diaIn patients with predominantly epinephrine-secreting tumors, symptoms of orthostatic hypotension such as lightheadedness, pre-syncope, and syncope may be more prevalent . The posPheochromocytoma should receive appropriate preoperative medical management to block the effects of released catecholamines. For preoperative blockade, there is no specific recommendation on the preferred drugs; alpha-adrenoceptor antagonists, calcium-channel blockers, or angiotensin-receptor blockers have all been recommended and appear useful. We recommend beta-adrenoceptor or calcium-channel blockers for tachyarrhythmias. Beta-adrenoceptor blockers have to be used only after adequate pretreatment with alpha-adrenoceptor antagonists. Adequate volume expansion before and after surgery is essential. The use of laparoscopy as the surgical method of choice for most abdominal pheochromocytomas is recommended for primary or multiple smaller tumors . There sAlthough most pheochromocytomas present with episodes of hypertension, rarely they can present with hypotensive episodes. Alpha-receptors antagonists have to be always used before beta-blockers for BP control and correction of hypovolemia before and after surgery is essential. The clinician should be aware of the possibility that hyperglycemia, hypokalemia, hypomagnesemia, and hypocalcemia can be part of the presentation of pheochromocytoma in the absence of hypertension.


Abnormal deposition of calcium in the skin or subcutaneous tissue is termed calcinosis cutis. Idiopathic calcinosis cutis of the scrotum is an uncommon entity. The pathogenesis of idiopathic calcinosis cutis of the scrotum is debatable. The condition presents as several brown to yellowish nodules on the scrotum, gradually progressive, and mostly asymptomatic. Here we report a case of idiopathic calcinosis cutis of the scrotum with a brief review of the literature and a discussion on pathogenesis.A healthy looking, 50-year-old Nepali man presented with multiple growths on his scrotum for 15 years, which were mostly asymptomatic with an occasional complaint of itching. On physical examination, multiple pink to brown nodules ranging in size from 0.5 × 0.5 × 0.5 cm to 3 × 3 × 1 cm, which were painless and firm in consistency, were noted. On laboratory examinations the following were found to be within normal limits: serum calcium, phosphorus, parathyroid hormone, and vitamin D hormone levels; uric acid; alkaline phosphatase; and lipid profile. Based on clinical features and laboratory reports, a diagnosis of idiopathic calcinosis cutis of the scrotum was made. The nodules were excised under local anesthesia in several sittings, which gave a good cosmetic result with no evidence of recurrence in 1-year follow-up period. A histopathological examination revealed dermis with areas of fibrosis and calcification along with numerous multinucleated giant cells and an absence of any cystic structure.Idiopathic calcinosis cutis of the scrotum is a benign condition, which remains mostly asymptomatic. It presents as progressive multiple nodules of varying numbers and sizes. A histopathological evaluation reveals areas of calcification. The cause is either dystrophic calcification of cysts or idiopathic. Excision is the treatment of choice. Abnormal deposition of calcium in the skin or subcutaneous tissue is termed calcinosis cutis. Skin is not a site for collection of calcium and is always a pathological phenomenon. Calcinosis cutis can involve any part of the skin. Depending on the cause, calcinosis cutis is classified into four types: dystrophic, metastatic, idiopathic, and iatrogenic . IdiopatA healthy looking, 50-year-old Nepali man came to our clinic with a complaint of multiple growths on his scrotum for 15 years. The growths started as a single lesion on the right side of his scrotum, with the gradual appearance of similar lesions on other parts. Several of these lesions coalesced at various places to form large-sized nodules. The condition was mostly asymptomatic with an occasional complaint of itching. There was no history of pain, burning sensation, trauma, ulceration, or discharge. The lesions did not interfere with urination or sexual activities. He was worried because of the increasing size of the growth and hence came to us for advice. He did not give a history of any systemic illness including metabolic, autoimmune, or malignant disorders. There was also no history of a similar complaint in his family.On physical examination, multiple pink to brown nodules ranging in size from 0.5 × 0.5 × 0.5 cm to 3 × 3 × 1 cm involving almost half of his scrotum were noticed . His postoperative period was unremarkable with good cosmetic result and no evidence of recurrence in a 1-year follow-up period. The cut section of nodules showed solid white to yellow homogenous areas. Histopathological examination revealed skin tissue lined by keratinized stratified squamous epithelium. The underlying dermis had areas of fibrosis and calcification Fig. . Numerouet al. in 1970 [ICCS is a benign, mostly asymptomatic, condition; although it appears early, patients generally present during the third to fourth decade of life. The symptom-free nature of the disease may be the reason for delayed presentation. The disease presents as slow-growing yellowish nodules in the scrotum, single to multiple in numbers, with varying sizes. It may sometimes be associated with itching or the nodule may break down to discharge chalky material. Infection of the nodules is uncommon. Although the first case was described by Lewinski in 1883 , the fir in 1970 . LewinskICCS is a clinical and histological diagnosis. The size, number, skin surface, and age of onset hint toward ICCS, while confirmation is always done on histopathology. A nodular lesion in the scrotum has several differential diagnoses which include steatocystoma multiforme, angiokeratoma, lipomata, fibromata, and lymphangioma circumscriptum . Howeveret al. examined 15 nodules from each patient and concluded that these nodules were calcified epidermoid cysts [et al. had earlier challenged this theory by using antikeratin monoclonal antibodies to find deposits of keratin in or around the calcium aggregates [et al. further reiterated the dystrophic theory by demonstrating the presence of keratin fibers and calcium granules in the surrounding dermis [As the condition is idiopathic, the exact etiology is not known. However, there is an intense debate going on to challenge the idiopathic nature of the disease. Several authors feel that ICCS is actually a misnomer and the underlying cause is the calcification of the epidermoid or epidermal cysts of the scrotum –9. In a id cysts . In anotid cysts . The folid cysts . Wright gregates . They fag dermis . It is ng dermis  and dartg dermis  are alsoTo label the condition as idiopathic, the presence of calcification elsewhere must be ruled out. A thorough biochemical and hormonal profile would help delineate the cause. If the condition is truly idiopathic, the laboratory investigations unequivocally fall within normal limits. Not one particular diagnostic modality is helpful. Fine-needle aspiration cytology, ultrasonography, and X-ray have been ordered with limited usefulness . As the ICCS is a benign condition, which remains mostly asymptomatic. It presents as progressive multiple nodules of varying numbers and sizes. A histopathological evaluation reveals areas of calcification. The cause is either dystrophic calcification of cysts or idiopathic. Excision is the treatment of choice.


Merkel cell carcinoma (MCC) is a highly aggressive neuroendocrine malignancy of skin origin. It is most commonly managed with upfront surgical resection which is then followed by radiotherapy as soon as possible postoperatively. Radiotherapy alone has been used in inoperable cases and in cases of patient preference to omit surgical management. MCC is a very radiosensitive disease, and an ideal location for definitive radiotherapy is the head and neck region (which represents the majority of cases), where resection often leads to reduced outcomes given the number of critical structures in the region and the inability of surgery to achieve the excision margins required for the aggressive nature of the disease without leading to severely impacted cosmesis and function. This case study looks at an elderly woman who was treated with definitive dose fractionation  radiotherapy to the head and neck region for Stage III, T1N2M0 disease, following rapid loco-regional relapse after surgical wide local excision. Two years postdefinitive radiotherapy, she has had complete loco-regional control both clinically and radiologically. Merkel cell carcinoma (MCC) is a highly aggressive neuroendocrine malignancy of skin origin . It is uAn 82-year-old Australian female was referred by her general practitioner to a general surgeon for biopsy +/- excision of a clinically up to 15 mm lesion of the mid-nose, which had been rapidly enlarging in size over the previous two to three weeks. The lesion was subsequently managed surgically by wide local excision and closed with an advancement flap. The histopathology report described an ulcerated tumor composed of undifferentiated small cells invading into the superficial subcutis. The cells stained strongly positive for cytokeratin-20 (CK-20), chromogranin, synaptophysin, and CAM 5.2; and stained negative for both thyroid transcription factor-1 (TTF-1) and CK-7. The immunohistochemical profile was therefore consistent with diagnosis of MCC  reported by the patient included Grade I xerostomia with reduced saliva without significant dietary alteration, Grade I dermatitis with mild erythema in the treatment field, Grade I fatigue, and Grade I facial oedema with localized edema. Late toxicities, defined by CTCAE as occurring more than three months after the completion of radiotherapy, included the persisting or consequential Grade I xerostomia; and Grade I facial edema with localized edema. The relatively mild reported xerostomia by the patient both in the acute and late stages was anomalous given that there was no sparing of either parotid gland from the target volumes. However, approximately 18 months postcompletion of radiotherapy, a lower right premolar tooth was extracted by the patient’s local dentist. The cause was thought by the treating dentist to be in the context of xerostomia and dose received to the mandible.The PET to assess treatment response three months postcompletion of radiotherapy demonstrated a complete metabolic response. All subsequent imaging have demonstrated complete response with no evidence of recurrence or distant disease. This has correlated with findings on clinical examination on three-six monthly reviews Figure . The patIn this case report, an elderly female with MCC of the head and neck region was treated to definitive dose radiotherapy following rapid loco-regional recurrence after initial management with surgery. The patient remains well, more than two years postcompletion of radiotherapy, and with no Grade II or higher late toxicities as per CTCAE criteria. This case report also highlights the importance of rapid treatment with radiotherapy of MCC following biopsy  proven diagnosis.An Australian retrospective study reported on records of 43 patients treated with definitive radiotherapy, either at diagnosis or in the relapse setting, between 1993 and 2007; again, the head and neck region was the most commonly treated site [The use of adjuvant or prophylactic nodal irradiation and the role of sentinel lymph node biopsy and mapping have been discussed and described in the literature in Stage I disease . In thisThere is an established relationship between MCC and the Merkel cell polyomavirus (MCPyV), thought to be an agent in MCC development, and which may be further potentiated in immunocompromise, including organ transplantation, HIV infection/AIDS, autoimmune diseases, and lymphoproliferative disorders, particularly B cell lymphoma . ImmunocThis case should hopefully provide further evidence that in some or perhaps most cases of MCC, and particularly in the head and neck region, (a) radiotherapy alone can provide definitive management, based on patient, tumour, and treatment characteristics, (b) and that radiotherapy can be delivered with tolerable side effects even in an extended treatment field.It is conceivable that in the not distant future, following discussion at an MDT, a patient could potentially be referred directly to both a radiation oncologist and to a surgeon following diagnosis of a biopsy-proven MCC, to discuss the options of either definitive radiotherapy or of upfront surgery followed by postoperative radiotherapy, should a patient wish to pursue active management.This report presents a case of an elderly woman who was treated with definitive dose fractionation  radiotherapy to the head and neck region for Stage III, T1N2M0 disease, following rapid loco-regional relapse after surgical wide local excision. Two years postdefinitive radiotherapy, she has had complete loco-regional control both clinically and radiologically. This case will hopefully add to a body of evidence that MCC of the head and neck region managed with radiotherapy alone can provide strong loco-regional control and that radiotherapy can be delivered with tolerable side effects in an extended treatment field in this setting. In addition, the case has highlighted the known radiosensitivity of MCC as well as necessity for rapid referral to radiation oncology following a diagnosis of MCC.


Rhinosporidium seeberi, which is classified under Mesomycetozoea.1 The disease is endemic in India, Sri Lanka, and tropical areas of Africa and South America.2 Most commonly it affects the nose, although mucous membrane of the nasopharynx, oropharynx, conjunctiva, rectum, and external genitalia can also be involved.3 Transmission occurs by bathing in stagnant ponds where animals also bathe. Patients usually present with a reddish nasal mass which bleeds profusely.2 The diagnosis is confirmed with excision biopsy of the lesion showing the typical sporangia in the tissue. The treatment is surgical excision followed by cauterization of the base to prevent recurrence. Dapsone has been tried in few cases to reduce the recurrence rate. Most patients require multiple surgeries as the recurrence rate is high.3A 52-year-old man presented with recurrent throat clearing and irritation since 2 months associated with occasional blood-tinged saliva. He was a known case of nasal rhinosporidiosis and had been operated 2 years ago. Examination revealed a reddish pedunculated fleshy mass with whitish spots posterior to the faucial pillar . Diagnos


Large sphenoid ridge meningiomas are surgically challenging because of their extension to adjacent structures. Extracranial meningiomas are very rare; most are of the secondary type and have an intracranial origin. Improved surgical methods are required for treatment of this entity.Here, we describe a case of a huge sphenoid ridge meningioma extending into the sphenoid sinus that was resected by staged transcranial and endoscopic endonasal resection via a transsphenoidal and transpterygoid approach. It was possible to excise the tumor from the sphenoid sinus in a minimally invasive manner via this approach.The transsphenoidal and transpterygoid approach was useful for providing a surgical field that allowed adequate visualization for removal of a sphenoid tumor that had expanded well into the lateral fossa of the sphenoid bone. Most exThe only curative treatment for meningioma is complete surgical extirpation. However, because of the tendency of this tumor to extend into adjacent structures, such as the cavernous sinus, the anterior clinoid process, the middle fossa, and greater and lesser sphenoid wings, resection of these tumors is challenging .Technological advances in recent years mean that surgical procedures for various pathological conditions, including tumors situated in the paranasal sinuses or the skull base, can be performed via a minimally invasive endoscopic endonasal approach (EEA).　Here we report the case of a patient in whom a sphenoid ridge meningioma in the sphenoid sinus was excised by staged surgery that consisted of a craniotomy for the intracranial portion and an endoscopic endonasal resection via a transsphenoidal and transpterygoid approach for the extracranial portion in the sphenoid sinus. The work has been reported in line with the SCARE criteria .2A 54-year-old man presented with a grade 4/5 right-sided hemiplegia. Neuroimaging studies revealed a left-sided sphenoid ridge meningioma; the tumor was exerting pressure on the brain parenchyma and extended further from the temporal lobe into the sphenoid sinus A. The paN-butyl cyanoacrylate was performed on the day before the first surgical resection. Subtotal tumor excision via craniotomy was performed by the neurosurgeons, but the portion of the tumor extending anterior to the tip of the temporal bone to the sphenoid sinus was not removed .Our manuscript included a statement at the end of the manuscript, as follows: "Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal on request”.Hidenori Yokoi: writing - preparation of the original draft, operating surgeon, investigation, methodology.Satoru Kodama: operating surgeon.Keisuke Maruyama: operating surgeon.Masachika Fujiwara: pathologic analysis.Yoshiaki Shiokawa: supervision.Koichiro Saito: supervision.Our manuscript is a case report and therefore, does not require registration.Dr. Hidenori Yokoi is the guarantor for this study.Not commissioned, externally peer-reviewed.