Schwannoma of the ascending colon is an extremely rare neoplasm that is often discovered incidentally in the asymptomatic older population on surveillance colonoscopy. We present the case of a symptomatic 22-year-old male presenting with one month of discomfort in the right lower abdominal quadrant, abdominal bloating, and hematochezia. A sessile polyp measuring 0.5 cm was identified in the ascending colon on the colonoscopy. The polyp was completely resected using cold snare polypectomy. Histological examination of the resected polyp with hematoxylin-eosin staining revealed small nodules of bland spindle cells with focal nuclear condensation. The lesional cells tested positive for S-100 and SOX-10 on immunohistochemical analysis, establishing the diagnosis of benign schwannoma. Since this lesion was submucosal, its diagnosis required an endoscopic biopsy that could only be performed on the mucosa. It was difficult to distinguish it from other mesenchymal tumors , and this makes the differential diagnosis exceedingly challenging. If the immunohistochemistry is positive for S-100 and negative for C-KIT, CD-34, actin, and desmin, it aids in diagnosis. These tumors have non-specific radiological features and are asymptomatic. Schwannoma is a neoplasm arising from Schwann cells and can occur anywhere along the peripheral nerves in the body . HoweverA previously healthy 22-year-old male with a one-month history of abdominal bloating and mild discomfort in the right lower abdominal quadrant presented with hematochezia. Physical examination and laboratory evaluation showed no significant findings. Abdominal ultrasound revealed fatty liver and two gallstones, each measuring 3 mm. The family history of the patient was significant, with a diagnosis of colon cancer in the father (at 47 years of age) and celiac disease in the brother. Ultrasound and CT scan findings of the abdomen were normal. Esophagogastroduodenoscopy was also insignificant. Therefore, a colonoscopy was performed and revealed a sessile polyp measuring 0.5 cm on the ascending colon, approximately at a distance of 8.4 cm from the cecum Figure . The polThe polyp was removed completely with a cold snare polypectomy. Histopathological examination revealed small nodules of bland spindle cells with focal nuclear condensation suggestive of a verocay body Figure .Immunohistochemical analysis revealed that the lesional cells were strongly positive for SOX-10 Figure  and mildSchwannomas are exceedingly rare neoplasms that arise from Schwann cells in the nerve sheath. Gastrointestinal schwannomas (GIS) appear as spindle cell tumors in the gastrointestinal system and originate from Auerbach’s myenteric plexus rather than Meissner’s submucosal plexus. They account for 2-6% of all mesenchymal cancers . Even amSchwannoma rarely ulcerates into the mucosa and is frequently identified as a submucosal mass or polyp on a colonoscopy or CT scan . MucosalPostsurgical immunohistopathologic evaluation of the excised polyp yields a conclusive diagnosis . SchwannComplete surgical excision with free negative margins is the best treatment option for colonic schwannoma . RadicalColonic schwannomas are rare, slow-growing benign neoplasms that usually occur in the older population. This case report showed that colonic schwannoma may also present in younger populations and has a very vague clinical presentation. It is usually identified on a colonoscopy or CT scan. Postsurgical immunohistopathologic examination of the excised mass is used to establish a conclusive diagnosis. Endoscopic excision or wedge resection is the treatment of choice.


Ascites is defined as the accumulation of fluid in the peritoneal cavity, following an imbalance between production and reabsorption; it is detectable from 50 mL on ultrasound. Three mechanisms have been classically implicated, according to Starling's forces: an increase in the hydrostatic pressure gradient , a reduction in the oncotic pressure gradient , and an increase in peritoneal capillary permeability. This latter mechanism, plus the difference between lymph production and excretion (which favors the accumulation of exudate), explains some of the most notable causes of non-hypertensive ascites ), including peritoneal carcinomatosis and tuberculosis. We present the case of a young man, originally from a tuberculosis endemic area, in whom the study of ascitic fluid guided the workup and the definitive diagnosis, which was unexpected for his age. Finally, a practical approach to non-hypertensive ascites is provided. Treatment of the patient with ascites is based on the serum ascites albumin gradient (SAAG) in a simultaneous serum and paracentesis sample , which cWith the exception of ovarian neoplasms, ascitic fluid cytology could be erroneous, since it has a sensitivity of 60% and a specificity of 100%, being positive in less than 10% of primary liver neoplasms or liver metastases, which can also show a SAAG >1.1, given the intrahepatic venous stasis secondary to extrinsic compression of the sinusoids; therefore, when these causes are suspected, it is suggested to perform a biopsy whenever possible ,4. A comA 21-year-old African American male, who came from the Carmen de Atrato region , with no personal or family history, was admitted to the emergency department due to five months of abdominal pain. The pain was diffuse, intermittent, and started on epigastrium, and then it was associated with bloating, early satiety, and transient constipation periods of up to one week, with no associated rectal bleeding or hematochezia. No fever, chills, weight loss, functional class, paroxysmal nocturnal dyspnea, orthopnea, or bendopnea was present.On physical examination, the patient was in stable condition, no jaundice was observed, and the abdomen was swollen and tense, with painful palpation in the upper quadrants; furthermore, an ascitic wave was present, and dullness was heard on the flanks. No collateral circulation or masses were observed. There was no jugular ingurgitation and no relevant findings on rectal examination or genital examination. The patient denied recent trips or any drug intake. An abdominal ultrasonography was performed two weeks before admission, which showed perihepatic, perisplenic spaces, and paracolic gutters, with an estimated volume of 250 mL, suggestive of peritoneal carcinomatosis.On admission, hydration status was optimized, and analgesia was administered. Initial laboratory workup Table  reportedMycobacterium tuberculosis  was calculated: 28 points from 13 abdomen-pelvis regions . The preliminary report of peritoneum biopsy was compatible with moderately differentiated rectal adenocarcinoma, confirmed by immunohistochemistry (cytokeratin (CK) 7, CK20, and caudal homeobox protein 2 (CDX2)).As a PCI higher than 20 is a contraindication for curative surgical intervention, a permanent drainage catheter was implanted in the abdominal cavity for recurrent ascites, and pain management was optimized by the palliative care team. Complementary studies, including simple and contrast thorax CT, ruled out distant metastases. Chemotherapy with palliative purpose was started by clinical oncology, with a CAPEOX/CAPOX protocol: capecitabine and oxaliplatin.Colorectal cancer is the main gastrointestinal neoplasm, being the third in global incidence and the second cause of cancer death in the world. About 150,000 new cases are diagnosed annually in the United States, of which a minority  are adolescents and young adults. This figure has increased in the last 20 years, despite screening programs. The vast majority of these malignancies in this age group are sporadic .The estimated lifetime risk is between 5% and 6%, being absolutely rare in kids and adolescents, with an annual incidence of 1/1,000,000 in the US population younger than 20 years; only 1% of all cases present before 30 years and 18% before 50 years. Of the latter group, 44% of cases with rectal cancer were observed in patients younger than 30 years . In ColoA debut with ascites usually involves an adverse prognosis. For example, the apparition of ascites on cirrhotic patients is associated to a mortality of 15% and 44% on one- and five-year follow-up, respectively , which iThree main metastatic dissemination routes have been described for colorectal cancer: a lymphatic route, with dissemination from the tumor, with embolism which then deposits in lymph nodes; hematogenous route, which relates to venules involvement, generating distant deposits on liver and lung; and the third route is cell pass and peritoneal invasion, which may be due to tumor extension, with involvement of the whole wall, or peritoneal involvement after surgery due to trauma, blood loss, or lymphatic trauma. This explains that peritoneal carcinomatosis due to colon cancer may be the first step in the extension of the disease and not necessarily a consequence of established metastatic disease in the medium to long term ,10.In a retrospective study of 3,019 patients with colorectal cancer, 11% had peritoneal carcinomatosis. From this group, 61% had synchronous disease and 39% had metachronous disease. The most frequent site of appearance was the liver (15%). Generally, synchronous peritoneal metastatic presentation was 8%, and 25% for relapse .,20.19,20It has been observed that around 18% of patients with hepatocarcinoma may present with a neutrophil cell count on ascitic fluid >250/μL. In these cases, a high red blood cell count >10,000/μL, as well as an RBC/WBC index >100 and a percentage of polymorphonuclear cells >75%, reasonably rules out concomitant active infection ,20.The literature has described a few case reports such as ours. Of note is the report of a case in which a 27-year-old male from Uganda presented with severe abdominal distention, weight loss, and fever, clinical condition similar to that described in our case. Due to his place of provenance, tuberculosis, acute/chronic hepatitis, and lymphoproliferative disorders, as well as human immunodeficiency virus (HIV), were ruled out. Finally, a colorectal carcinoma was diagnosed from a cell block, showing atypical glands with an overabundance of extracellular mucin, suggestive of mucinous adenocarcinoma, which was confirmed by immunohistochemistry  . FinallySAAG may be useful to differentiate whether ascites is secondary or not to portal hypertension , with a sensitivity of 95%. This way, in those patients with peritoneal carcinomatosis, with no liver lesions, SAAG will usually be <1.1 g/dL; in those with hepatic lesions , SAAG will usually be >1.1 g/dL. The association of the clinical findings with the biochemical properties of the ascitic fluid, in addition to microbiological studies, will allow an adequate differential diagnosis, beyond the principle of parsimony, which holds that "all things being equal, the simplest of the competing explanations is the most likely to be correct"; it should not be forgotten that cancer is a rule-out diagnosis for non-hypertensive ascites, colorectal cancer being an increasing entity in the young population.


Echinococcus granulosus. The association between these two diseases is still not well-defined. A preoperative diagnosis may be challenging, especially if HCC and CE present as a single lesion and if atypical imaging features are present. Herein, we present a case of the patient that was initially diagnosed as an extensive necrotic tumor in the left liver lobe and highly suspicious of being HCC associated with peritumoral hematoma. Left hemihepatectomy was performed, and the histopathological findings showed the collision of two lesions: a hydatid cyst and HCC.A coexistance of liver cystic echinococcosis (CE) and hepatocellular carcinoma (HCC) is very rare. HCC is the leading cause of cancer-related mortality worldwide, while CE is a globally endemic zoonosis caused by the cestode tapeworm  Echinococcus granulosus [Hepatocellular carcinoma (HCC) is the most common primary liver cancer and the fourth-most frequent malignancy in the general population . Althouganulosus . The livanulosus . With thanulosus . Even thanulosus ,9,10,11.anulosus ,13,14. HA 58 year-old female patient was admitted to our hospital  because of persistent epigastric pain, which lasted one month. The accompanying symptoms included weight loss and nausea. Her previous medical history did not indicate chronic liver disease, nor were other comorbidities present. On admission, an abdominal ultrasound examination revealed a large, solid, cystic lesion occupying the left liver lobe. Routine laboratory tests, including liver function analyses and tumor markers, were within normal limits. Serological tests for the viral hepatitis were negative. The patient underwent computed tomography (CT) examination of the abdomen, which showed a large, predominantly cystic lesion measuring up to 14 cm in diameter occupying lateral part of the left liver lobe with extrahepatic growth in the gastrohepatic ligament, compressing the stomach. The walls of the cyst were thick, irregular with coarse calcifications A. AdjaceIn order to further characterize the lesion, the patient was refered to a magnetic resonance imaging (MRI) examination. During the MRI, a clearly demarcated heterogeneous cystic lesion located in liver segments II and III and in the gastrohepatic ligament was seen. The internal content of the cyst displayed a heterogeneous signal intensity on the T2-weighted image with a central hyperintensity. Moreover, irregular, thick areas of hypointensity in the periphery were noted, which showed a high signal intensity in the T1-weighted image, indicating necrotic debris, and suggested the presence of internal hemorrhaging A,B. NextThe patient underwent left hemihepatectomy, and the tumor was completely resected, with negative margins. The macroscopic findings clearly showed that the cystic portion of the tumor corresponded to a parasitic cyst adjacent to the malignant alteration of the liver parenchyma . A furthAlthough both HCC and CE are common liver lesions with high incidence rates in the general population, their synchronous occurrence is very rare in clinical practice, especially in a non-cirrhotic liver ,7,8,9. TWhen HCC and CE mimick a single liver lesion, a preoperative diagnosis might be very challenging. If typical imaging features of CE are present in the form of cystic lesions with multiple internal daughter cysts and the absence of postcontrast enhancement, the diagnosis of cystic echinococcosis is straightforward . HoweverThe treatment for HCC depends on the stage of the disease. The BCLC staging system is a currently widely used method to stage HCC and provides guidelines for treatment . AccordiEchinoccocus granulosus infection and malignant tumor development [Echinoccocus granulosus protoscoleces have a promoting effect on the growth, migration and invasion capacities of HCC cells [Echinococcus granulosus and some malignant tumors might be the possible mechanisms of echinococcal antitumor activity [There is an increasing number of studies indicating that there might be a possible connection between elopment ,12,13. Telopment ,12,13. ICC cells . If HCC CC cells ,12. HoweCC cells . NeverthCC cells . NonetheCC cells ,14. Antiactivity . In thisactivity ,26. Furtactivity . HoweverThe coexistence of HCC and CE as a solitary lesion on the grounds of a non-cirrhotic liver is extremely rare in clinical practice. If typical imaging features are absent, a preoperative diagnosis is very challenging. In such cases, the presence of calcifications inside the cystic lesion adjacent to the solid tumor should indicate the possibility of concomitant CE and HCC. Therefore, if there is any diagnostic dilemma, a serological test for hydatid disease should be performed. Based on the present case, it might be hypothesized that CE may promote the development of HCC in patients without underlying cirrhosis. Nevertheless, further studies are needed to clarify the possible relation among these diseases in patients without chronic liver disease.


Wandering spleen is a rare disease that is easily misdiagnosed. When combined with splenic pedicle torsion and even splenic infarction, wandering spleen is a rare and critical cause of surgical acute abdomen. We report an 18-year-old male patient with abdominal organ inversion diagnosed as acute appendicitis before operation. Laparoscopic exploration confirmed wandering spleen with splenic pedicle torsion led to splenic infarction and was complicated by appendicitis. He was treated with laparoscopic appendectomy and abdominal splenectomy. The patient recovered well after the operation and was discharged from the hospital in 7 days. During the 4-year follow-up, there was no report of complicated infections such as pneumonia or sepsis. An abdominal ultrasound showed abdominal organ inversion, splenomegaly, intestinal and lower abdominal effusion, and mesenteric lymph node enlargement. An abdominal computed tomography (CT) showed Abdominal organ inversion, Splenomegaly, and Intestinal effusion-labeled spleen.We report a young male,who was admitted to the hospital because of worsening acute abdominal pain on the third day. Physical examinations showed tenderness and rebound pain at McBurney’s point in the lower right quadrant. Laboratory test results are as follows: WBC: 12.46×109/L; PLT: 63×109/L neutrophils: 10.4×109/L; blood amylase: 34 U/L; and urinary amylase: 327 U/L. He underwent urgent radiological examination, and the results were received in the shortest possible time. An abdominal ultrasound showed abdominal organ inversion, splenomegaly, intestinal and lower abdominal effusion, and mesenteric lymph node enlargement. An abdominal computed tomography (CT) showed abdominal organ inversion, splenomegaly, and intestinal effusion-labeled spleen : significant congestion and bleeding in the splenic sinus, atrophy of the white pulp, and multiple focal infarcts under the touch that showed hemorrhagic infarct changes. The patient had a stable postoperative course with no abdominal bleeding and no peritonitis. The abdominal drainage tube was removed before discharge. His incision healed well, and his physical examination showed no abnormalities. At last, he was discharged 7 days after surgery.Wandering spleen is characterized by the lack of normal peritoneal attachment. It can cause splenic ectopia due to the relaxation of ligaments in the spleen, kidney, and stomach . The splThis patient was admitted to the hospital with an acute abdomen. According to the results of color Doppler ultrasound and spiral CT, it was considered that the abdominal organs were reversed. The abdominal signs were tenderness and rebound pain in the right lower abdomen. Combined with the patient’s medical history, symptoms, and auxiliary examination, the diagnosis was acute appendicitis and abdominal organ inversion. Retrospective analysis, because the patient had tenderness and rebound pain at McBurney’s point of the lower right abdomen and elevated leukocyte levels, was consistent with the initial diagnosis of appendicitis. What is more, the patient’s pain suddenly worsened on the third day, which we speculate may have been caused by torsion of the wandering spleen.There are two kinds of visceral inversion: full visceral inversion and partial visceral inversion with a totally reversed side. The latter has a population incidence rate of about 1 in 10,000 . Full visceral inversion is an extremely rare anatomical variation of human viscera. It refers to the 180° inversion of the position of all viscera, such as the heart, lung, diaphragm, liver, spleen, stomach, and intestine. Placement looks like a mirror image of normal organ placement, and the circulation, respiration, and digestion functions are normal. There is no conclusion on the cause of visceral inversion; it is mostly considered related to family genetics, and the distortion of the chromosome structure may be its basic cause.Acute appendicitis leads to acute peritonitis, which is in line with the indication of emergency surgical treatment. Laparoscopic exploration of the abdominal cavity found that the anatomical position of abdominal organs was the same as that of unaffected people, and there was no organ inversion. At the same time, it was found spleen wandering and, near the lower right abdomen, splenic pedicle torsion and splenic infarction. The appendix was in the anterior position of the cecum with inflammatory changes. After a laparoscopic appendectomy, the infarcted spleen was removed under laparoscopic localization. Combined with the pathological results, the diagnosis was wandering spleen and appendicitis. Reflecting on the patient's diagnosis and treatment process, it can be seen that in the early stage of treatment, noninvasive imaging examination, ultrasound, and CT suggested that the patient had abdominal organ inversion. This is mainly because, on the one hand, wandering spleen is a rare clinical condition, the doctor lacked experience recognizing the condition, and the patient’s splenic pedicle torsion and splenic infarction differed from the morphological and imaging features of the spleen in the physiological state, which increased the difficulty of identification. On the other hand, the signs of abdominal infection caused by appendicitis interfered with the correct anatomical location. After the noninvasive imaging examination, the correct diagnosis could be made through laparoscopic exploration, and timely treatment could be provided.Haemophilus influenzae, pneumococcus, and Neisseria meningitidis after splenectomy and complications such as sepsis, increased cancer incidence, and higher thrombotic rates. Because of these risks, it is recommended that young patients retain their spleen for as long as possible (Although the incorrect judgment of organ inversion does not affect the correct treatment of patients, the process of misdiagnosis offers us experiences worthy of reference. First, surgery is the first choice for the treatment of wandering spleen because conservative treatment will increase complications , and splpossible . HoweverAlthough the patient was only 18 years old, he had splenic pedicle torsion and splenic infarction, which eliminated the possibility of preserving the spleen. Moreover, he had a long infarction time and a large ischemic area. Doctors did not rule out the formation of local thrombosis and microthrombosis. In this case, they should not have chosen splenic fixation because splenectomy was the most appropriate treatment. Through strengthening publicity and education and perioperative nursing, prophylactic use of antibiotics, the 23-valent pneumococcal polysaccharide vaccine, was conducted as planned 2 weeks after the operation, and there was no postoperative infection .This was a rare case of wandering spleen with splenic pedicle torsion. Appendicitis was present as well. The main cause of acute abdomen was wandering spleen with splenic pedicle torsion rather than appendicitis. Therefore, laparoscopic abdominal exploration was the first choice for the final diagnosis of wandering spleen, and splenectomy was the best treatment.


Melanocytic lesions of the central nervous system (CNS) are an infrequent, broad and diverse group of entities, both benign and malignant, found in all age groups, with imaging findings ranging from well-circumscribed focal lesions to diffuse leptomeningeal involvement. On MRI, they are usually distinguished by a high signal on T1WI sequences, given the paramagnetic effect of melanin, thus making it difficult to differentiate among them. To describe the imaging and epidemiological characteristics of a retrospective series of CNS melanocytic lesions. MR images of 23 patients with CNS melanocytic lesions diagnosed between January 2012 and June 2018 were analyzed. Most patients were female , with a median age of 47 years (range: 3 weeks to 72 years). The primary melanocytic lesions accounted for 8/19 cases (42.1%), which included neurocutaneous melanosis, meningeal melanocytomas and primary malignant melanomas. Secondary melanocytic lesions (metastatic) accounted for 10/19 cases (52.6%). There was one case of a tumor with secondary melanization, from a melanocytic neuroectodermal tumor of infancy. There were also four cases of primary ocular melanomas. The most frequent findings were the cerebral location, high T1WI signal and marked contrast-enhancement. The present review describes the wide variety of melanocytic lesions that could affect the CNS, emphasizing the MRI characteristics. Knowledge of the imaging, clinical and epidemiological characteristics of CNS melanocytic lesions is essential for their correct interpretation, given the significant overlap between lesion features and the variable prognosis. Their imaging appearance will depend largely on their intrinsic melanin content. They are usually classified into primary, secondary (metastases) and tumors with secondary melanization ,Primary melanocytic lesions are very rare, with an estimated incidence of 0.9 per 10 million people/yearPrevious studies are scarce and generally limited to reports and case series, thus making diagnostic and management standardization difficult for these patientsThe purpose of our study was to review the epidemiological characteristics and MRI features of CNS melanocytic lesions, through a retrospective study on a case series.We conducted a descriptive retrospective study on patients with melanocytic lesions of the CNS who were identified through searching the imaging databases at our institution covering the period from January 2012 to June 2018. As this was a retrospective study, ethics committee approval and informed consent were waived.The search was carried out among MRI reports, and those in which it was concluded that some type of CNS melanocytic lesion was present were selected. The search terms used were: “melanocytosis”, “melanocytoma, “melanoma”, “melanomatosis”, “melanocytic”, “melanocytic lesions”, “melanosis” and “melanization”. Subsequently, clinical and pathological data were collected when available.Although primary ocular melanomas are not considered in the usual classifications of CNS melanocytic lesions, they were included here, given their relative high frequency in adults, with prognostic importance and distinctive imaging characteristicsThe MRI studies that were searched here had been performed in 3T equipment  and in 1.5T equipment . All MRI protocols included the following sequences: conventional T1/T2-weighted imaging (T1/T2WI); susceptibility-weighted (T2*/SWI); diffusion-weighted (DWI/ADC maps) and contrast-enhanced T1 (T1WI-Gd). The information on all available MRI studies was collected from the radiological reports and biopsies were interpreted by certified neuropathologists (C.T.G. and S.C.B).,The features studied were the location and imaging aspects of the lesions. The imaging appearance was analyzed in the T1WI, T2WI, T1WI-Gd and DWI/ADC sequences. Analyses on gradient (GRE) or magnetic susceptibility (SWI) sequences were excluded, given the different magnets used and the difficulty in differentiating imaging between melanin and methemoglobin, given their similar paramagnetic effectsHyperintense lesions in T1WI were considered to be present when they presented higher signal intensity than normal brain cortex in some component of the lesion. Similarly, in T2WI sequences, the lesions were considered to be hyperintense, isointense or hypointense, according to their predominant signal, in relation to the signal intensity of the cortex. Regarding T1WI-Gd sequences, contrast enhancement was characterized as present (“+”) or absent (“-”) and according to its distribution, as focal, meningeal or mixed. Similarly, restricted diffusion was considered to be present when the ratio of ADC values was lower than that of the pontine white matter, in the solid component of the lesion.A total of 32 patients were found in our report database. Nine patients were excluded because no preoperative MRI was available. Thus, 23 patients were included for the analysis: 14 females and 9 males, with a median age of 47 years (range: 3 weeks to 72 years). There were 18 adults and 5 pediatric patients. In 19 patients, the tumors were described as a CNS melanocytic lesion and in four as a primary ocular melanoma. The compartmental location of the lesions is described in Primary melanocytic lesions accounted for 8/19 cases 42.1%): 4 pediatric and 4 adult cases, with a median age of 43 years (range: 3 weeks to 67 years). Secondary melanocytic lesions accounted for 10/19 cases (52.6%), with a median age of 51 years (range: 34-72 years). The only case of a tumor with secondary melanization had an age at diagnosis of 2 months. The primary ocular melanomas comprised 4 cases, with ages ranging from 41 to 64 years, and a median of 42 years. The specific diagnoses, availability of histological confirmation, ages and imaging characteristics of the primary and secondary melanocytic lesions and primary ocular melanomas are described in 2.1%: 4 p,Neurocutaneous melanosis accounted for 50% (4/8) of all the primary melanocytic lesions , and mosThere were two cases of primary meningeal melanocytomas , both maThere were two cases of primary malignant melanomas : one intThe secondary melanocytic lesion group (metastatic melanomas) showed several lesions in most of the cases, ranging from two to six lesions. Brain location predominated , while there was a single case with spinal  involvement. In 60% there was an exclusive supratentorial location and in 20% an exclusive infratentorial location. One case was both supra and infratentorial and one was both supratentorial and vertebral. Most of the cases presented the classic melanocytic pattern of high T1WI signal intensity, while in a single one case a supratentorial amelanocytic lesion was observed . Focal cIn the group of “tumors with secondary melanization”, a single case of a two-month-old male infant with a mass over the left mastoid fontanelle was found. A biopsy on this mass showed it to be a melanocytic neuroectodermal tumor .Primary ocular melanomas  were locth and 10th weeks of embryonic development, they migrate to the skin, mucosa, uveal ocular layer, inner ear and CNS. In the latter, they are located in the leptomeninges, where they are preferentially distributed in the convexity of the skull base, ventral brainstem and cervical cord,Melanocytes are the cells that produce and store melanin. They originate from the neural crest and, between the 8Melanin has a paramagnetic effect, which induces shortening of the T1 and T2 relaxation times on MRI. This gives rise to its characteristic high signal intensity in T1WI, with corresponding hypointensity in T2WI,,,In our retrospective series, a total 23 cases were identified, with predominance of the female sex, in contrast to the slight male predominance previously reported,The location of the melanocytic lesions was consistent with what had been reported,Regarding the imaging characteristics, the classical melanocytic pattern of T1WI hyperintensity/T2WI hypointensity signal predominated. In three cases , an “amelanocytic” pattern was observed, with intermediate/low T1WI signals. Almost all the cases presented contrast enhancement, either focal, leptomeningeal or mixed, with the exception of one case of ocular melanoma with a highly melanocytic pattern. These characteristics were the most constant imaging markers described,,,,Primary malignant melanomas of the CNS are very infrequent, with estimated annual incidences of 0.7 per 10 million inhabitants,,,Meningeal melanocytomas, previously called melanocytic meningiomas, are very rare benign lesions, with an estimated annual incidence of one case per million inhabitants, with isolated reports of malignant transformation. They also occur predominantly in the fifth decade of life and they tend to be located in the posterior fossa, Meckel’s cavum and cervicothoracic spinal canal,,,,,,Neurocutaneous melanosis is a rare type of non-inherited phacomatosis, characterized by congenital cutaneous nevi and leptomeningeal melanocytic proliferation. Around 100 cases have been reported in the literature,Secondary melanocytic lesions (metastatic melanoma) were the most frequently found diagnosis, similar to what had previously been reported,,,,Regarding the third group of CNS melanocytic lesions, i.e. tumors with secondary melanization, we presented a single case of an infant with a melanocytic neuroectodermal tumor of infancy. These are very rare lesions that affect newborns and children under one year of age, with a certain male predominance,th to 8th decade of life. It may or may not be symptomatic . On MRI, it presents lentiform or mushroom-shaped morphology, with a broad base of choroidal implantation and protrusion to the vitreous chamber, usually associated with retinal detachmentLastly, primary ocular melanoma is the most frequent malignant intraocular tumor in adults, with an incidence of approximately six cases per million/yearIn conclusion, CNS melanocytic lesions are rare and can be found in all age groups. The imaging characteristics are miscellaneous, depending on each specific diagnosis, its location and the amount of melanin contained. Nevertheless, there are some MRI features that can suggest this kind of lesions, like the high signal intensity in T1WI, low signal intensity in T2WI, restricted diffusion and either nodular or leptomeningeal contrast enhancement. In many cases, the diagnostic approach will be challenging, given the low incidence and frequent overlap of patterns. When faced with a suspicious lesion, it is essential to know the wide differentials like those described here, especially the feared metastatic melanoma, given its greater frequency and poor prognosis.


Congenital adhesions are rare findings in adults. We present a case of appendicitis in a middle-aged male with extensive congenital adhesions of the terminal ileum to the right lateral abdominal wall. The small bowel mesentery completely obscured the inflamed appendix. Alternative techniques were required as a result of these intraoperative discoveries, and substantial adhesiolysis was carried out before a successful appendicectomy. Although the congenital adhesions described here are extremely uncommon, the authors suggest that practitioners should be aware of them because they could change typical clinical manifestations and surgical approaches. Adhesions between the small bowel and other intra-abdominal structures usually form after abdominal inflammation such as post-surgery, radiation, or pelvic inflammatory disease . AdhesioCongenital intra-abdominal adhesions are a rare phenomenon usually observed in children. Most reported congenital adhesions are discovered during surgery for small bowel obstruction where a congenital band causes incarceration and strangulation of the small bowel as it passes through .To the best of our knowledge, significant congenital adhesions of the small bowel itself to the abdominal wall have not been reported in the literature. This is an important entity for clinicians to be aware of, as it could alter the clinical presentation of common pathologies and increase the difficulty of an otherwise standard operation.We present the case of a 40-year-old male who presented with acute appendicitis. During the laparoscopic appendicectomy, extensive congenital small bowel adhesions were encountered, which increased the difficulty of surgical access.The patient was a 40-year-old male who presented to the emergency department with three days of migratory right iliac fossa pain. It was constant in nature and worse on movement. He did not have any nausea or vomiting, no changes in bowel habits, and no subjective fevers. Apart from the pain, he stated that he felt well, without other systemic features of infection.The patient’s only past medical history was diabetes and hyperlipidemia, which were both well-controlled on oral medications. He reported no past intra-abdominal surgery or trauma, and no past colonoscopies or gastroscopies. He did not smoke or drink alcohol.On examination, his vital signs were within normal limits. His abdomen was mildly distended, and he was guarding in the right iliac fossa. He had equivocal rebound tenderness. He did not have Rovsing’s sign or psoas's sign present. He had mild percussion tenderness at the right iliac fossa.A contrast-enhanced CT demonstrated a thickened and dilated appendix with associated fat stranding, consistent with appendicitis. At the time, no abnormal features of the small bowel were identified on imaging. Interestingly, the patient’s laboratory results showed very minimal inflammation, which was incongruent with the CT findings.He had a normal white cell count and a C-reactive protein (CRP) of only 16. All other laboratory parameters are within normal limits.A diagnosis of appendicitis was made, and the patient was started on intravenous broad-spectrum antibiotics. Arrangements were made to proceed to the operating theater for laparoscopic appendicectomy.Once pneumoperitoneum and visualization were established, it was noted that the ascending colon and the cecum were not visible. Upon further inspection, it was noted that a segment of the small bowel congenitally adhered to the right lateral abdominal wall Figure .The appendix, cecum, and ascending colon were completely obscured under the mesentery of this small bowel. It was clear that the infection and inflammation were well contained under the small bowel mesentery. Comparing intra-operative images with the preoperative CT scan, it can be determined that this segment of the small bowel is the terminal ileum. As the appendix and cecum were tucked far under the terminal ileum mesentery Figure , the decThe patient was discharged from the hospital two days after the operation when bowel function returned. The subsequent 30-day follow-up revealed no complications and he was discharged from the clinic.Acute appendicitis is one of the most common causes of acute abdomen and is one of the most common surgically treated pathologies . Intra-aThe congenital adhesions encountered could also explain the unusual preoperative biochemistry. It was noted the patient only had a small increase in inflammatory markers; this was despite significant stranding on CT and a three-day history. The authors believe that these atypical findings were a result of the small bowel mesentery containing the infection. Interestingly, there have been other cases reported where an inflamed appendix was found in unusual spaces in the abdomen, leading to unremarkable changes in inflammatory markers. Probert et al. describe a case of appendicitis in an Amyand hernia in which the patient had down-trending inflammatory markers without treatment . Sigley Our case also highlights a distinct advantage of a laparoscopic appendicectomy compared to open surgery. Traditionally, both open and laparoscopic appendicectomies are considered safe and effective in treating appendicitis . Both meAcute appendicitis is one of the most common emergency general surgery presentations to the emergency department. Patients mostly present with raised inflammatory markers and localized peritonism, especially if they have been symptomatic for more than 24 hours. In our case, the patient did not have these significant findings despite 72 hours of pain. This case highlights the importance of clinician awareness of the varied presentations in abdominal pathologies and the possibilities of congenital adhesions causing these variations. Although the congenital adhesions described in this case are extremely rare, clinicians should not be falsely reassured by benign inflammatory markers and incongruent examinations.


Primary serous peritoneal carcinoma (PPC) is a rare malignancy often presenting with a significant disease burden and a poor prognosis. A 65-year-old female was seen in the surgical outpatient clinic with a two-month history of weight loss, altered bowel habits and a CT scan characterizing a heterogenous right paracolic gutter mass and suspicious liver lesions. At colonoscopy, a prominent appendiceal orifice was biopsied to be poorly differentiated carcinoma favouring gynaecological tract origin. The patient was admitted with an acute small bowel obstruction secondary to progression of metastases and underwent neoadjuvant chemotherapy with a near complete response on repeat staging. A debulking hysterectomy, bilateral salpingo-oophorectomy, pelvic peritonectomy and small bowel nodule excision were performed. The diagnosis of PPC was confirmed when no malignancy was found in the pelvic organs. The presence of intraluminal colonic metastasis with PPC is exceedingly rare with this being only the third such case in the literature. Primary peritoneal carcinoma (PPC) is a rare epithelial cancer closely associated histologically and in clinical behavior with epithelial ovarian carcinoma. As such they are classified and treated together as one clinical entity by the International Federation of Gynaecology and Obstetrics . High-grUnfortunately, high-grade serous carcinoma carries a poor overall prognosis and due to its aggressive nature, most patients present in a subacute fashion with 75% of patients presenting with Stage III or Stage IV disease ,3. AcuteA 65-year-old female was seen in the surgical outpatient clinic with two months of weight loss, altered bowel habits and bloating. Her medical history included hyperlipidemia, hypertension and a previous appendicectomy. She had early menarche, was nulliparous and an active smoker. She did not have any history of hormonal therapy or a family history of malignancy. The only pertinent physical examination finding was mild generalized abdominal tenderness. Computed tomography (CT) scan of the abdomen showed a heterogenous mesenteric mass in the right paracolic gutter with associated lymphadenopathy and multiple suspicious parenchymal liver lesions. The patient had an elevated CA125 level of 245U/mL and a transvaginal ultrasound was normal. At colonoscopy, a slightly prominent appendiceal orifice was biopsied -positron emission tomography and CT scan (FDG PET/CT) showed only two areas of residual omental deposits (<15mm) compared to the pre-chemotherapy imaging Figure . She undThis case is remarkable for both the rare occurrence of an intraluminal bowel metastasis secondary to PPC and for its demonstration of the varied presentations and aggressive clinical behaviour of PPC. Intraluminal bowel metastasis of PPC is exceedingly rare. The pathway of dissemination is thought to be via the transcoelomic route - spreading through the peritoneum . Often pThe preferred treatment for Stage III and IV epithelial ovarian cancers including PPC is primary surgical cytoreduction followed by systemic chemotherapy - ideally a platinum-plus-taxane combination. The aim of surgical treatment is to achieve <10mm of residual disease macroscopically. Neoadjuvant chemotherapy is offered to patients with unresectable disease, patients unlikely to be optimally cytoreduced if taken to theatre at the time of presentation or those who are poor initial operative candidates ,9. ThosePPC is a rare and aggressive cancer and should be considered as a differential in patients with peritoneal disease and relevant risk factors, particularly to allow for counselling regarding the associated poor prognosis. In addition to the unusual presence of a large bowel metastasis, the rapid progression of symptomatology in this case demonstrates both the subacute and acute presentation of PPC and the appropriate change in management adapting to the changing clinical scenario as per the guidelines and literature.


Anatomic variations can be an additional challenge for any surgeon, especially in complicated surgical procedures, and they could lead to complications if not identified timely.We present a case report of an incidental intraoperative finding of unilateral duplicated ureter, identified during pelvic lymphadenectomy for a patient who underwent surgical treatment for ovarian cancer. Also, we include an update on this anatomic variation following the literature review.The importance of a systematic approach in the identification of the relevant anatomy and landmarks, taking into consideration the variation of the duplicated ureter, is emphasized in this paper and may contribute beneficially to the experience of surgeons operating in this field. Ureters are muscle-walled cylindrical tubes with stratified, transitional epithelium which play a significant role in draining urine from the kidney to the bladder in the human body through regular contractions called peristalsis . TypicalDuplicated collecting system is defined as one kidney with two separate renal pelves (duplex kidney) which do not communicate.The prevalence of unilateral duplicated ureter has been reported at 0.7% to 4%, and the incidence in autopsy studies is 0.5% , affectiDuplication of the ureter may be complete or incomplete. Incomplete duplication (known as bifid ureter) involves two separate ureters at the proximal aspect, and they join at any point below the uretero-pelvic junction but before entering into the bladder. This happens due to the bifurcation of the ureteric bud prior to its invasion into the metanephric blastema. On the other hand, a ureteral bud that arises twice separately, results in a complete duplication of the ureter (double ureter), which is continuous and leads to two openings into the bladder. The ureter draining from the lower pole of the kidney is the equal to the single-system ureter and hence is called orthotopic, while the upper pole ureter is called ectopic (Weigert-Meyer rule) and is inserted inferior and medial to the orthotopic ureteral orifice ,6. In coWe present a rare case of unilateral duplicated collecting system identified during pelvic lymphadenectomy with a review of the current literature.Informed written consent form has been obtained by the patient.A 67-years-old lady was referred to the suspected cancer pathway of our department for postmenopausal bleeding. From previous medical and gynaecological history, she is parity three, with three previous normal vaginal deliveries and a WHO performance status of zero (0). She had a breast cancer diagnosis more than 15 years ago and was treated with surgery, radiotherapy, and chemotherapy. The patient had regular smear tests, as per the National Cervical Screening programme, which were always normal. Hence, she had no previous large loop excision of the transformation zone (LLETZ) procedures. She is medicated for benign palpitations (secondary to anxiety) and hypercholesterolemia. There was no previous history of any persistent urinary tract symptoms like recurrent infections.Following the cancer pathway referral, the patient had a transvaginal ultrasound scan of the pelvis, performed by the local primary health care service. On the sonographic examination, the uterus was found to be anteverted of 8 cm size, an endometrial thickness of 10 mm, with a fundal 8 mm calcified fibroid and a 7.2 cm left ovarian complex cyst containing septae, irregular masses, and mural nodular thickening. No free fluid in the pouch of Douglas was seen. The tumour marker of Ca-125 was 24 and hence the risk of malignancy index (RMI) was calculated to be more than 200. Subsequently, an MRI abdomen and pelvis was booked, and the patient was referred to the gynaecological oncology (gynae-oncology) multi-disciplinary team (MDT). A Pipelle endometrial biopsy was obtained, which showed no hyperplasia or malignancy.The patient’s case was discussed at the gynae-oncology MDT meeting in view of the whole clinical picture. The MRI demonstrated a left ovarian thin-walled multilocular cystic lesion measuring 80 mm x 60 mm. The cyst was diffusely heterogeneous with different locules containing T2 bright serous fluid and T2 intermediate mucin Figure . There wFollowing the MDT discussion, the patient was consented for total abdominal hysterectomy (TAH), bilateral salpingo-oophorectomy (BSO), frozen section and/or omentectomy, and/or bilateral pelvic lymphadenectomy. The procedure was done under general anaesthesia; she was placed in Lloyd Davis position, cleaned, draped, and catheterized. A midline incision was made to gain access to the intra-abdominal cavity, peritoneal washings were taken for cytology analysis, and a full assessment was done to check for any metastasis. No obvious disease was noted in the abdomen or in the pelvis apart from the known left ovarian cystic lesion. A left salpingo-oophorectomy was performed, and the specimen was sent for frozen section, which suggested endometrioid adenocarcinoma leading to a total omentectomy and bilateral pelvic lymphadenectomy in addition to the TAH and BSO. During the dissection of the right pelvic sidewall, duplicated ureter was seen Figure  as perisThe procedure was completed with no complications. Post-operatively, the patient failed her trial without a catheter and had to be re-catheterized due to urine retention and developed subacute bowel obstruction, confirmed by CT abdomen-pelvis with contrast, which was managed conservatively and resolved. Following the urine retention, urine investigations were done showing white blood cells (WBC) 701 x106/L, red blood cells (RBC) 311 x106/L, with no epithelial, hyaline casts, granular casts, nor cellular casts. The culture which followed found >10^5 cfu/ml of Klebsiella pneumoniae sensitive to co-amoxiclav, which was prescribed for one week.The patient was discharged with a urine catheter in situ and on oral antibiotics. Follow-up appointments were scheduled in one weeks time to be seen by a urology nurse specialist for catheter removal, which was successful, and in four weeks time for surgical follow-up.The histology confirmed stage 1A/grade 2 left ovarian endometrioid adenocarcinoma with no lymph-vascular space invasion (LVSI). All of the 14 lymph nodes removed were negative. There was no disease within the omentum; the endometrium was found to be proliferative, with a benign endometrial polyp present. The myometrium contained multiple benign leiomyomas, and the cervix showed no evidence of cervical intraepithelial neoplasia (CIN) or cervical glandular intraepithelial neoplasia (CGIN).On gynae-oncology follow up, the patient had recovered well from the surgery and was completely asymptomatic with no abnormal urinary symptoms. Histology results were explained to the patient as well as the MDT recommendation for only surgical follow-up and no adjuvant treatment.Considering all of the above facts, with regards to the unilateral duplication of the ureter, this patient had no previous urinary symptoms in her life in order to undergo investigations, which could diagnose earlier this congenital variant. Also, there were no obvious abnormalities in the urinary tract system identified during the pre-operative to raise any suspicion of potential variation of the anatomy. Thankfully, intraoperatively, the surgical team performed a systematic approach in the identification of the landmarks and the anatomy, including the variation, before the dissection of the right sidewall lymph nodes, which prevented any ureteral injury.A retrospective review of the available pre-operative MRI abdomen-pelvis and post-operative CT abdomen-pelvis with contrast could not result in a safe conclusion of the type of duplication in this case. The only finding, which was noted during the retrospective review of the post-operative CT abdomen-pelvis was the right duplex kidney Figure . HoweverThe ureter develops during the fifth week of embryological development. The ureteric bud arises as a diverticulum from the caudal end of the mesonephric (Wolffian) duct and invades the metanephric blastema . The gliCongenital anomalies of urinary tracts, double ureter, and kidneys comprise 20% to 30% of prenatal anomalies . UreteriFrom data derived from autopsy studies, it has been suggested that the unilateral bifid ureter’s incidence is found 1 in 125 cases (0.8%) . Also, iThe majority of patients with duplicated ureter remain asymptomatic, but some complications can be persistent. The vast majority of these cases are diagnosed in early life with recurrent urinary tract infections . In adulAdditionally, the congenital duplex collecting system can potentially increase the risk of ureteral injury during surgery. Iatrogenic ureteral injuries are not a rare complication of any open or laparoscopic surgical procedure involving the pelvis, especially in gynaecological procedures . The ovePreoperative ureteral radiographic imaging by intravenous urography or computed tomography (CT) has been highlighted in cases of large pelvic masses, pelvic inflammatory disease, or endometriosis. Furthermore, ureteric stenting could facilitate the identification of anatomy in the surgical field in complex cases or in cases suspected of congenital variations/anomalies, minimizing iatrogenic ureteric injury. However, it is not recommended on a routine basis. .The diagnosis and follow-up assessments of ureteric duplication can be performed usually with ultrasonography, intravenous pyelography (IVP), voiding cystourethrography (VCUG), CT, and magnetic resonance urography (MRU). In general, ultrasonography is considered to be the first-line imaging modality as it can demonstrate findings suggestive of duplication, such as asymmetrical renal lengths, separated renal pelvises, or disproportionate, pelvicalyceal dilation of the upper and lower poles, presence of ureterocele, especially in females, where it has a stronger association with duplicated collecting system. Also, VCUG is the standard imaging method performed in the assessment for vesicoureteral reflux, which is common in duplicated collecting system cases. IVP has been used to assess the morphology and function of the urinary tract. Following intravenous administration of contrast agent, the appearance of the kidneys, ureters, and bladder are examined for obstruction, scarring, or other abnormalities by radiographs and fluoroscopy. Furthermore, comparing MRU with CT, the spatial resolution of MRU is less than that of CT; however, the contrast resolution is superior and hence the urine in the collecting system acts as a contrast agent in MRU. The main findings of duplicated renal collecting systems are the same as on CT as they were described above in ultrasonography. On the other hand, CT reconstruction software can generate very good images of the collecting systems . OverallFinally, with regards to the treatment, it should be guided by the symptoms secondary to diagnosis as well as by the renal function. In the study of Siomou et al., out of the 75 duplicated collecting systems, 24 were managed surgically (19 with complete duplication and five with incomplete). Of the 40 duplex systems which were complicated with vesicoureteral reflux, 26 were managed conservatively with the prophylactic use of antibiotics . During,The importance of a systematic approach in the identification of the relevant anatomy and landmarks, taking into consideration the variation of the duplicated collecting system, is highlighted in this case report providing an insight into this congenital anomaly not only to the surgeons who are operating in the field of the lesser pelvis but also to radiologists whose role is crucial in the multi-disciplinary team input to each case.


Dogs are valuable models for spontaneous intracranial neoplasia in humans. The most common intracranial neoplasm in dogs is meningioma, followed by glioma and choroid plexus tumors. Pilocytic astrocytoma (PA) is a form of glioma and is a common childhood and adolescent tumor in humans. There have been two previous reports of PA in dogs. These cases were both adult dogs and the clinical and imaging features were not described. This report describes a 23-week-old female intact American Bulldog with a 2-week progressive history of neurologic dysfunction. Magnetic resonance imaging (MRI) revealed a large hemorrhagic mass that was confirmed on post-mortem examination to be a high-grade astrocytoma with pilocytic morphology. This case is the first to report a high-grade astrocytoma with pilocytic morphology in a juvenile dog.A 23-week-old female intact American Bulldog was presented for a two-week history of progressive circling to the right, twitching, and altered mentation. Magnetic resonance imaging (MRI) revealed a non-contrast enhancing hemorrhagic mass centered in the right thalamus with concurrent subdural and intraventricular hemorrhage. Post-mortem histologic examination of the brain confirmed a mass centered on the thalamus with histomorphologic features consistent with a high-grade astrocytoma with pilocytic morphology. To the authors’ knowledge, the present case is the first to report clinical and imaging characteristics of a high-grade astrocytoma with pilocytic morphology in a young dog. The incidence of intracranial neoplasia in dogs is reported to be 14.5 in 100,000 . GliomasPilocytic Astrocytoma (PA) is a common pediatric tumor in humans, accounting for 15.4% of childhood and adolescent brain tumors ,8. In thThere are two previous reports of PA in dogs . These tA 23-week-old female intact American Bulldog was presented to the Emergency and Critical Care Department at the Schwarzman Animal Medical Center for evaluation of two weeks of progressive circling, twitching, hyporexia, hypodipsia, and diarrhea. Approximately four weeks prior to presentation, the patient had been relinquished to new owners. The progressive neurologic deficits were noted after the change in ownership. Prior medical history was unknown.On presentation, the patient weighed 6.5 kg with a body condition score of 4 out of 9. Physical examination revealed hypothermia (temperature 95.5 °F), mild bradycardia (72 beats per minute), and moderate dehydration. The peripheral blood pressure was within normal limits. Comprehensive bloodwork was performed and revealed a severe hypernatremia  and severe hyperchloremia . Other blood work changes including the mild electrolyte, protein, and liver enzyme abnormalities were consistent with dehydration and the dog’s juvenile age. On neurologic examination, the dog was obtunded but responsive. She had a mild generalized ataxia and circled to the right. She displayed a right head turn, left head tilt, low head carriage, and fine head tremors. Cranial nerve examination identified a positional vertical to rotary nystagmus, absent menace in the left eye, and decreased nasal sensation on the left. Spinal reflexes and postural reactions were unremarkable. Neuroanatomic localization was multifocal with suspicion for right forebrain and left brainstem involvement, specifically the central vestibular system. Differentials for the patient’s neurologic signs included primary structural intracranial disease  or less likely metabolic/reactive etiologies. Due to the unknown duration and severity of the hypernatremia, it was elected to gradually correct the sodium elevation over three days prior to pursuing an MRI under general anesthesia.The patient was hospitalized and started on a fluid treatment plan to correct the free water deficit over 72 h with 5% dextrose in water (D5W) (15 mL/hour) and Plasmalyte (maintenance rate of 60 mL/kg/day). The patient was also treated with Keppra (25 mg/kg IV every 8 h), Cerenia (1 mg/kg IV every 24 h), Metronidazole (15 mg/kg IV every 12 h), and Visbiome (1 capsule PO every 12 h). By the fourth day of hospitalization, the patient’s hypernatremia and hyperchloremia had significantly improved, but remained mildly elevated . Additionally, the patient was persistently hypothermic with only mild improvement in her diarrhea. Her neurologic examination remained unchanged despite supportive care and correction of her electrolyte abnormalities.After three days of hospitalization, the patient was determined to be metabolically stable for anesthesia and an MRI was recommended. The dog was premedicated with IV administration of Butorphanol (0.2 mg/kg) and Maropitant (1 mg/kg). General anesthesia was induced with Midazolam (0.2 mg/kg IV) and Propofol (0.83 mg/kg IV) and maintained with the inhalant anesthetic isoflurane (0.75–1%). A constant rate infusion of dopamine (5–10 μg/kg/min) was utilized for blood pressure support due to persistent hypotension while under general anesthesia.Magnetic resonance images of the brain were acquired using a 1.5 Tesla magnet and human knee coil  with appropriate repetition time (TR) and echo time (TE) parameters. The study included T2-weighted images in sagittal and transverse ; pre- and post-contrast T1-weighted images in sagittal, transverse, and dorsal ; a T2W-FLAIR sequence in transverse ; and a gradient echo (T2*) sequence in transverse . The post contrast T1-weighted images were obtained after manual intravenous injection of the gadolinium-based contrast medium gadodiamide .The MRI revealed an irregularly marginated, heterogenous, well circumscribed, non-contrast enhancing mass in the right thalamus and caudal right frontal lobe mostly displaying signal void on the T2 and T2* weighted images and T1W hypointensity . The dorA postmortem examination was performed. Preliminary external brain evaluation on autopsy confirmed a large subdural hematoma. When viewed from the ventral surface, there was mild asymmetry of the diencephalon due to marked expansion of the right side and lesser expansion of the left side. Spanning from the region of the optic chiasm to the transverse fibers of the pons and bordered by the piriform lobes, the ventral aspect of the rostral perforated substance, optic chiasm, tuber cinereum, mamillary bodies, and crus cerebri contained dozens of coalescing areas of hemorrhage. The brain was fixed in 10% neutral buffered formalin for 7 days. Subsequently, the brain was serial sectioned after formalin fixation. Centered on the thalamus and spanning from the caudal frontal lobe to the level of the caudal piriform lobe was an approximately 2.6 cm × 1.8 cm (at its widest dimension), ovoid, firm, mottled tan, brown and red mass enveloped in hemorrhage . The sur2). The peritumoral neuroparenchyma was variably compressed and secondary tissue reactions included multifocal areas of parenchymal loss, hemorrhage, and edema; swollen axons (spheroids); Gitter cell infiltration; moderate gemistocytic astrocytes; moderate to severe neovascularization; and mononuclear perivascular cuffs. Further, histology confirmed subdural hematoma, intraventricular obstructive hydrocephalus, and histiocytic, lymphoplasmacytic, neutrophilic meningitis. Glial fibrillary acidic protein (GFAP) immunohistochemistry displayed diffuse and marked immunoreactivity within the mass. Based on the histomorphologic features, this tumor was classified as a high-grade astrocytoma with pilocytic morphology.Histologically, the mass consisted of elongated bipolar cells with delicate streaming cytoplasmic processes on an eosinophilic fibrillary stroma with infrequent loosely arranged regions . AdditioAstrocytoma is the most commonly reported primary intra-axial tumor in dogs, and some brachycephalic breeds have a genetic predisposition ,4,5. PriThe histopathology of the two previously reported cases of PA in dogs was very similar to the current case; the tumors contained fibrillated elongated cells; Rosenthal fibers; microcyst formation, and positive GFAP immunoreactivity . These hThe MRI findings in this case were unusual. Similar to the typical canine astrocytoma, the lesion was T1-weighted hypointense . In contIn humans, the MRI features of PA have been well described. PA preferentially affects the cerebellum with other common locations including the optic pathway, hypothalamus, third ventricle, and brainstem. Less commonly, PA can be found in the cerebrum and spinal cord ,28. PA gThe neuroanatomic localization in this case was multifocal, which is uncommon in cases with a solitary tumor, and was likely due to the hemorrhage, edema, and herniation secondary to the primary mass. The abnormal mentation, circling to the right, and right head turn were likely due to the right sided thalamic lesion. The hypothermia and hypodipsia were likely due to the secondary effects on the hypothalamus from hemorrhage and edema. The left head tilt, nystagmus, and ataxia were attributed to the increased intracranial pressure and secondary transtentorial herniation compressing the vestibular system. Tremors, which ultimately improved with correction of electrolyte derangements, were felt to be secondary to the hypernatremia, though a pain response cannot be entirely ruled out.The severity of the hemorrhage in this case was striking and was likely due to the atypical tumor-associated blood vessels and encroachment on surrounding cerebral, meningeal, and potentially choroid plexus blood vessels. The intratumoral perivascular hemosiderin laden macrophages supported that the hemorrhage was ongoing within the tumor itself. There were additional regions of hemorrhage at the cranial-most aspect of the tumor  and the caudal-most aspect of the tumor . The hemorrhage in the 3rd ventricle with potential involvement of the choroid plexus may have led to more significant bleeding overall, however, invasion of the choroid plexus was not confirmed on histology. Additionally, there were regions where the tumor bordered or obscured choroidal and meningeal surfaces, suggesting potential encroachment of associated blood vessels, which may have led to the large subdural hematoma. The microvascular proliferation seen in this case is one criterion for increased grade. Other features of increased grade were not seen. Although rare, there have been cases reported in the human literature of PA lesions with significant internal hemorrhage .Based on the clinical history, MRI findings, gross and histopathologic examination of the brain, this patient was diagnosed with a high-grade astrocytoma with pilocytic morphology. This is the first report of a pilocytic phenotype in a young dog, and the first report to describe the clinical and MRI characteristics. Neoplasia should be considered as a differential diagnosis of dogs with an intra-axial lesion of any age.


Paragangliomas are extremely rare neuroendocrine tumors with an incidence of 0.3 cases per million in children. They arise from several sites in the body including the orbit, ear, nose, larynx, carotid area, mediastinum, duodenum, and the genitourinary tract. Germline mutations have been identified in paragangliomas involving the proto-oncogene RET, tumor suppressor genes VHL, and NF1 The commonest clinical presentation is hypertension but incidentally detected forms have been reported during imaging and on a few occasions the diagnosis was missed. The authors report on a case of a 12-year-old male with a history of fever, left flank pain, vomiting, and headaches, finally diagnosed by pathological examination of a resected retroperitoneal mass invading the proximal left ureter. Immunohistochemistry was positive for neuron-specific enolase, NSE (chief cells), and S-100 protein (sustentacular cells) all consistent with paragangliomas. The challenges faced by the team in the preoperative evaluation and surgical treatment are reported. Paragangliomas are exceedingly rare neuroendocrine tumors, with a reported incidence of 0.3 cases per million in the pediatric population -6. In 19Patient information: we herein present a 12-year-old Nigerian primary school pupil referred to us with a history of fever, left flank pain, vomiting, and headaches. He is the first of two children, a product of normal term delivery, and fully immunized. There was no similar problem in the family. His parents were separated and he lived with his grandmother. He was referred to the pediatricians for control of hypertension after which he had excision of an incidentally detected retroperitoneal paraganglioma involving the proximal left ureter with ureteroureterostomy.Clinical findings: on clinical evaluation, his Blood Pressure was markedly elevated (200/120 mmHg) but no mass was detected on abdominal examination. Serum electrolytes, urea and creatinine, full blood count, and urinalysis were all within normal limits. He commenced higher doses of Lisinopril 10 mg and Aldomet 250 mg with improvement in his blood pressure readings. Abdominal ultrasound revealed left hydrocalicosis and intravenous urography showed prompt excretion of contrast on the right but delayed nephrogram on the left with clubbing and hydronephrosis  close to the abdominal aorta, encasing the proximal left ureter was discovered and converted to a midline transperitoneal approach which made complete excision of the mass easier  measuring 8x6.5x4cm , urine VMA/Creatinine 2.28 micromol/24hrs (0 - 4.7) urine HVA/24hrs 16 micromol/24hrs (0 - 39) and urine HVA/Creatinine 3 micromol/24hrs (0-7.9). At 4 months follow-up, an intravenous urogram showed prompt bilateral excretion, mild left hydronephrosis, and normal ureters bilaterally. He was followed up for 2 years, initially with no remarkable events.Patient perspective: the informant and caregiver of the patient reported satisfactory care and outcome of the treatment her grandson received during the period of admission and follow-up visits to the urology clinic. He was followed up for two years leading up to this case report.Informed consent: ethical approval was not applicable in this case report. Informed consent was obtained from the caregiver for this publication and any accompanying images.Paraganglioma in children is a rare catecholamine-secreting tumor that requires a high index of suspicion and supporting diagnostic investigations in making the correct diagnosis -3,5,6,8.et al. in their study noted that hypertension should be sought in patients with dilated or obstructed upper urinary tract and considered this as an indication for surgery and relief of obstruction cures hypertension in the greater majority of patients [This case presents a possible lack of perception and challenges posed to clinicians in the management of paragangliomas in children, especially in low-income settings. The index patient had hypertension secondary to an incidentally detected retroperitoneal paraganglioma at surgery causing left ureteropelvic junction obstruction. de Waard D patients ,21. In ipatients . Pain frpatients ,7,23-25.patients ,17,26,27et al. observed that flank pain is a common symptom in these cases [The average age of onset of paragangliomas in children is 11 years with a male to female ratio of 2: 1 and this is comparable to the index case . Similarse cases ,21. Theyet al. and Abdelkhalek et al. as being partly responsible for delay in diagnosis and oftentimes misdiagnosis in resource-poor settings [Among the indications for surgical intervention in children with hydronephrosis are hypertension, flank pain, renal impairment, infection, and stones, the initial two features being apparent in our patient ,20,21,23settings ,17. In asettings . Two weesettings ,18. To fThe absence of cytokeratin expression by paragangliomas at immunohistochemistry has been used by pathologists to separate them from other neuroendocrine tumors . ImmunohThe occurrence of hypertension in the pediatric age group should arouse a need to screen for pheochromocytoma-paraganglioma syndrome. Early treatment is crucial to avoid life-threatening cardiovascular complications. This case highlights the significance of a high index of suspicion and detailed clinical evaluation in identifying these patients and offering them timely treatment. The importance of entertaining a broad differential diagnosis and carrying out thorough laboratory and imaging investigations hold the key to correct diagnosis and successful management. The mainstay of treatment remains surgery, and the outcome is good following gross complete resection. To the best of our knowledge, this is one of the few reports of surgical management of paragangliomas in children and the first detailed report of the immunohistochemistry characteristics of the tumor in our environment.


Systemic mastocytosis is a rare disease resulting from infiltration of atypical mast cells in multiple organ systems and present with variety of symptoms. Primary appendiceal and cecal mass with isolated abdominal pain as a presenting feature in systemic mastocytosis have not been reported in literature up to now.We described a 69- years-old female with systemic mastocytosis who presented with chronic abdominal pain and recent progression. On imaging of the abdomen and pelvis showed a mass in cecum. The patient underwent surgery and histopathologic evaluation of cecal and appendiceal masses revealed uniform small round cell tumor with eosinophilic cytoplasm admixed with many eosinophils distorting normal colonic mucosal architecture. The neoplastic cells showed positive expression of CD117 and Mast cell tryptase. According to all these considerations systemic mastocytosis was confirmed as the diagnosis.Isolated abdominal pain and primary large intestinal mass are uncommon features of systemic mastocytosis. This case report informed physicians and pathologists to consider it as one of differential diagnosis. •Mastocytosis is an uncommon neoplastic infiltration of mast cells in one or multiple organs.•Isolated abdominal pain and primary large intestinal mass are uncommon features of systemic mastocytosis.•Mastocytosis reveal common surprising clinical signs that to conclude lead to inadequate treatment. mastocytosis. Majority of patients with Mastocytosis have evidence of cutaneous involvement and less of them present with other organs involvement include the spleen, lymph nodes, liver and gastrointestinal (GI) tract . Mastocy2A 63- years-old woman with chief complaint of chronic abdominal pain and recent worsening referred to surgery department of Sina hospital affiliated to Tehran University of Medical Sciences in November 2019. She didn't have complained any other symptoms such as nausea, vomiting, diarrhea, loss of appetite, weight loss, skin rashes or flushing. In physical examination, vital signs were normal. Abdomen was soft in palpation without any tenderness and appearance abnormalities. Cardiopulmunary, skin and other organ systems examination were unremarkable. She had no familial history, past medical, and past surgical history. Laboratory analysis showed mild leukocytosis, other hematologic, biochemical and urine tests were all unremarkable. On imaging, one mass in cecum was seen. Patient was scheduled for right hemicolectomy and para-aortic lymph node dissection by expert colorectal surgeon. The specimen was received at Sina hospital pathology lab for histopathologic examination consists of a segment of colon, cecum, terminal ileum and appendix. Colon and terminal ileum were 26cm in length and 4.5cm in maximum diameter. On opening a whitish solid submucosal mass was seen in cecum measuring 2x1.5 × 1cm. Also, a 0.7cm whitish lesion was noted in the base of appendix. Microscopic examination for both cecal and appendiceal lesion revealed infiltration of monotonous neoplastic cells with round to ovoid nuclei, eosinophilic cytoplasm, prominent nucleoli and distinct cell boundaries distorting the normal colonic mucosal architecture, also many eosinophils were present admixed with neoplastic cells . Immunoh3Mastocytosis is a rare disease resulting infiltration and accumulation of a clonal, neoplastic proliferation of morphologically and immunophenotypically abnormal mast cells in one or multiple organ systems  cause di4Isolated Mastocytosis in the GI tract is rare but they must be careful in the differential diagnosis of various GI masses. Difficulties in the differential diagnosis might happen in connection with an unexpected setting. They reveal common surprising clinical signs that to conclude lead to inadequate treatment. It is our hope, that with such a report, we are able to reduce misdiagnosis and improve the outcome of these patients.Ethical approval is not needed due to the fact that the treatment of the patient was based on approved options and was not controversial.No source to be stated.AEJ: major contribution of the idea, study design and revise the paper, EN: pathologist and writing the paper, NM: data collection and follow up.Name of the registry:Unique Identifying number or registration IDHyperlink to your specific registration (must be publicly accessible and will be checked)Elham Nazar is Guarantor of this submission.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.Not commissioned, externally peer-reviewed.There is no conflict to be declared.


Hereditary multiple intestinal atresia (HMIA) is a rare form of intestinal atresia that affects the gastrointestinal tract from the pylorus to the rectum. A few cases have been reported in the literature. Here, we report the case of a three-day-old girl who was referred to our hospital as a case of upper intestinal obstruction. After initial resuscitation, the radiological examination revealed pyloric obstruction, multiple intra-abdominal calcifications, and rectal atresia making the diagnosis of HMIA most likely. Exploratory laparotomy revealed multiple intestinal atresia, the first started at the pylorus, the second was at the level of the duodenojejunal junction, and there were also multiple small bowel atresias. The colon was a cord-like structure, and there was rectal atresia. Multiple resections of the atretic intestinal segment followed by multiple anastomoses, terminal ileostomy, and the use of a trans-anastomotic tube were performed. In this study, the clinical picture, radiological findings, and management are described and compared to the findings reported in the literature. One-third of all cases of neonatal intestinal obstruction are caused by intestinal atresia. Multiple intestinal atresia is a rare form of intestinal atresia characterized by the presence of several atretic segments in the small or large bowel, resulting in signs of intestinal obstruction in infants, such as vomiting, abdominal bloating, and failing to pass meconium. Moreover, multiple atresia is associated with an increased risk of mortality .tetratricopeptide repeat domain-containing protein 7A (TTC7A) gene [TTC7A is often associated with severe intestinal defects and severe combined immunodeficiency (SCID) and inflammatory bowel disease [Hereditary multiple intestinal atresia (HMIA), a severe congenital disorder, was first defined by Guttman and colleagues in 1973 . The gen disease . Multipl disease -6.To date, most studies have been reported in Europe and the United States, with limited cases reported in Saudi Arabia. Thus, the rarity and treatment challenges of the disease led us to present a case and review the literature.A preterm three-day-old girl was born by cesarean section to healthy non-consanguineous parents at 36 weeks of gestation; her birth weight was 1.7 kg. She was referred to our hospital as a case of intestinal obstruction from a peripheral hospital where an oral contrast study was done, and it was diagnosed as duodenal obstruction. However, the prenatal follow-up did not raise the diagnosis of intestinal obstruction.Upon arrival, the baby presented with vomiting and failure to pass meconium. On examination, the patient had stable respiratory and hemodynamic status with no evident dysmorphic features. The abdomen was soft, lax, and not distended. A rectal tube was inserted but failed to pass beyond 3 cm. Laboratory studies were unremarkable. A plain abdominal X-ray showed a hugely dilated stomach Figure  with remAfter a period of stabilization, she underwent an exploratory laparotomy, which revealed multiple intestinal atresia, the first atresia was at the pylorus, and the second atresia was at the duodenojejunal junction. There was also multiple atresia of the small bowel, with multiple completely atretic cord-like segments of the intestine. The remaining part of the small intestinal was segmented, with every segment measuring about 2-3 cm containing whitish calcified material. The colon was also found to be completely atretic, with a closed lumen like a cord (Figure Postoperatively, the baby was shifted to the neonatal intestinal care unit and was kept on total parenteral nutrition with intravenous antibiotics. On follow-up, there was a minute whitish secretion through the trans-anastomotic tube. Multiple trials of feeding the baby through the nasogastric tube failed. Subsequently, the condition of the baby deteriorated due to gram-negative sepsis in the blood, and, unfortunately, she died after three weeks of the surgery. In our case, the short life of the baby did not allow further investigations to confirm the presence of SCID.The rarest form of multiple atresia is HMIA which affects the gastrointestinal tract from the stomach to the rectum ,7. The ePrenatal diagnosis is still an issue. Ultrasound can raise suspicion of HMIA, especially in the presence of polyhydramnios, gastric dilatation, and intraluminal calcification; however, it cannot distinguish HMIA from other types of intestinal atresia despite advancements in the detection of intestinal anomalies ,10,11. HThe aim of the surgical intervention should be to restore the continuity of the gastrointestinal tract and maintain the maximum length of the viable bowel ,7,8,12. The comparison in Table Medical supportive management and early surgical intervention to restore the continuity of the gastrointestinal tract through multiple resections and anastomoses is the usual course of treatment based on the literature and our own experience. Moreover, a small bowel transplant or bone marrow transplant or even gene therapy may be a viable therapy option; however, further research is required to confirm this line of treatment. Although this is currently a rare disease, each case study can provide us the opportunity to learn more about the true pathophysiology.HMIA should be considered in cases of neonatal intestinal obstruction with radiological signs combined with pyloric obstruction, rectal obstruction, and multiple intraluminal intestinal calcifications. The family should be aware of the prognosis. Further immunological and genetic workup in the future might clarify the disease and improve the prognosis.


Background: Liposarcoma (LPS) is one of the most common soft-tissue sarcomas. However, intrathoracic LPS is rare, as only 1% of all LPS cases are found in the thorax. Methods: A systematic literature review through PubMed and Embase databases was performed. Only eligible case reports and case series reporting intrathoracic LPS in adult patients were included. Kaplan–Meier curves were calculated to evaluate the survival rate of included patients based on the histological subtype of LPS. Results: 123 studies reporting 197 patients were included. We added a case of a 69-year-old female patient with recurrent giant intrathoracic LPS. The primary tumor measured 15.1cm × 22.9 cm × 21.9 cm and weighed 3100 g. Six months later, the patient was admitted to the hospital with another intrathoracic tumor measuring 9.5 cm × 9 cm× 1.4 cm. The immunohistochemical studies showed expression of murine double minute 2 (MDM2) antigen in both primary and recurrent tumor cells. Conclusions: Dyspnea, chest pain, and cough were the most common symptoms reported in included studies. Overall, the 5-year survival rate was 62%. The highest survival was observed in well-differentiated LPS patients (80%) and the lowest in myxoid LPS (31%). Liposarcoma (LPS) is one of the most frequent soft-tissue malignancies, accounting for 20% of all cases. LPS originates from abnormal differentiation of adipocytes or lipoblasts and usually develops in patients between the 5th and 7th decade of life . On the We report on a 69-year-old female patient admitted to the Department of Cardiology due to gradually increasing dyspnea and weakness for the past two years. Throughout this period, the patient remained under regular cardiological control. Her medical history included gastrectomy due to cancer 30 years prior, thyroidectomy due to a goiter 20 years earlier, hysterectomy due to fibroids, cholecystectomy, and lipoma resection of the neck region. The patient quit smoking 30 years prior to admission. Before that, the patient had smoked for 20 years. In addition, she suffered from coronary artery disease, atrial fibrillation, and arterial hypertension. The patient was in the process of qualifying for cardiac resynchronization therapy (CRT). However, a chest X-ray showed an abnormal mass in the right thoracic cavity. During hospitalization, computed tomography (CT) confirmed a massive lesion in the right thoracic cavity, 15.1 cm × 22.9 cm × 21.9 cm in size, with a 5 cm strand passing to the left side between the heart and descending aorta. The tumor had a heterogeneous adipose tissue density and contained soft tissue lesions and calcifications . The lesIn August 2022, a control chest CT revealed a recurrent tumor measuring 9.5 cm × 9 cm × 1.4 cm in the posterior mediastinum . TherefoThe article follows CARE guidelines for case reports and the Preferred Reporting Items for Systematic Reviews and Meta-Analysis (PRISMA) ,4. InforA total of 123 case reports and case series with 198 patients were included in this systematic review. The clinicopathological characteristics of included patients are presented in Liposarcoma is a mesenchymal tumor first reported by Virchow in 1860 . In 2013The clinical course of LPS patients mostly depends on tumor location and size. In this review, 16% of patients were asymptomatic, with tumors found incidentally. In time, the tumor starts to compress adjacent structures and patients become symptomatic. Dyspnea was the most common complaint (50%), followed by chest pain (29%). Our patient also reported dyspnea on admission and CT revealed compression of the right middle and lower lobes. A similar clinical presentation was observed by Baheti et al., who reviewed patients with intrathoracic synovial sarcoma. A total of 21% of patients were asymptomatic, while 49% and 19% of complaints represented chest pain and dyspnea, respectively . AccordiThe most frequent diagnostic procedures in this systematic review included CT, MRI, PET, and biopsy. A CT scan of LPS showed heterogeneous mass with nodules, fibrotic, and adipose tissues . On the As demonstrated in this review, surgical resection is the most common treatment strategy in intrathoracic LPS (188 patients (96%)). Complete resection is one of the most significant factors in LPS treatment. Leaving even a small fragment of the tumor or its capsule worsens survival and increases the risk of recurrence. Proper surgical access determines treatment success. Minor lesions can be successfully removed using the VATS technique. Open surgeries are usually performed in larger lesions. Typically, a posterolateral or anterolateral thoracotomy is used, depending on the location of the lesion and the operator’s preference. The anterior mediastinal lesions are more demanding and may involve large vessels. However, in certain clinical situations, a more extensive incision, which provides a better exposure, is required. The literature describes the removal of such lesions by bilateral anterior thoracotomy, median sternotomy, or clamshell access. Yet another example is tumors infiltrating the posterior mediastinum. Taki et al. described a demanding case that required simultaneous esophageal resection . Large tp = 0.087) [Furthermore, additional RTH was reported in 35 patients. A similar strategy is performed in retroperitoneal LPS . There i= 0.087) . Finding= 0.087) . MLPS wa= 0.087) . Chemoth= 0.087) . Current= 0.087) . MLPS an= 0.087) . Eribuli= 0.087) . A phase= 0.087) . The use= 0.087) . In a re= 0.087) . In a mu= 0.087) . Additio= 0.087) .Due to its uncommon location and many potential histological presentations, there is a broad differential diagnosis, including thymolipoma mesothelioma, teratoma, leiomyosarcoma, or fibrosarcoma. Additionally, there are various tumors composed of adipose tissue with different prognosis and management . Therefore, immunohistochemical and molecular methods have been studied extensively in LPS. WDLPS and DDLPS develop due to amplification of the 12q13-15 chromosome segment. As a result, in both of those tumors, amplification of MDM2 (suppression of p53) and CDK4 (cell cycle regulator) oncogenes can be found by immunohistochemistry or in situ hybridization (ISH) . Since tThis systematic review cannot be considered without certain limitations. Firstly, articles in languages other than English and without full-text access were excluded. Therefore, we could have missed some case reports of high relevance. Secondly, selection bias should be acknowledged. Furthermore, only a certain number of studies reported detailed information regarding radiological or pathological findings and follow-up. Intrathoracic LPS is a rare disease mostly reported in case reports and case series. More prospective studies with large sample sizes are necessary to understand clinicopathological features fully.To conclude, intrathoracic LPS is a rare neoplasm. Symptoms, if present, are usually due to compression of the surrounding organs. Due to the uncommon location, heterogeneous features in imaging, and multiple possible histological presentations, differential diagnosis is broad. Recent advances in pathology facilitate a precise preoperative diagnosis, which is essential for proper patient management. As demonstrated in this review, only a complete surgical resection offers the best possible outcomes. There are no guidelines regarding additional use of radio- and chemotherapy and this issue should be further examined. Unresectable LPS might be treated with eribulin, while further treatment agents are under constant examination with promising results. LPS patients require a long-term follow-up period, as recurrence may develop years postoperatively.