Diffuse hepatic hemangiomatosis (DHH) is an uncommon vascular lesion, though hemangiomas are the commonest benign tumors of the liver. The etiology is largely unknown to date; however, its association with giant cavernous hemangiomas (GCH) has been reported in the literature. We present herein, the case of a 37-year-old hypothyroid woman with abdominal fullness for 2 months. The contrast-enhanced computed tomography revealed multiple well-encapsulated lesions involving the liver lobes and was diagnosed as giant cavernous hemangiomas. Most of them, except the deep-seated ones, were enucleated. Histopathological examination highlighted the presence of GCH with irregular margin, replacement of hepatic parenchyma, and presence of multiple micro-hemangiomas suggesting the possibility of DHH further substantiated by retrospective radiological assessment. No extrahepatic vascular lesion was noted, and the post-operative recovery and follow-up were uneventful. Adult DHH is an uncommon entity. The diagnosis of DHH and its distinction from GCH are important from the management and prognostic point of view as recurrence, extrahepatic manifestations, features of consumption coagulopathy, and death from the complications are not uncommon. The prevalence of hemangioma, a benign vascular tumor, varies from 5 to 20% in different studies.1,3,4,6-Diffuse hepatic hemangiomatosis (DHH) is characterized by innumerable hemangiomas invading and replacing the liver parenchyma. DHH can be limited to a hepatic lobe or involve the hepatic parenchyma diffusely.1,20,2,The etiopathogenesis of DHH is uncertain, although it has been diagnosed in association with hereditary disorders, such as skeletal hemangiomatosis and Osler-Weber-Rendu disease, and drugs like oral contraceptives (OC) and metoclopramide.9A 37-year-old woman presented with right upper quadrant fullness and shortness of breath for 2 months. Her medical history included the diagnosis of hypothyroidism on L- thyroxin (25 mcg/day), antidepressants usage over the last 8 years, and was submitted to an uneventful open appendicectomy (gangrenous appendicitis with peritonitis) one month before the most recent complaints. She denied alcoholism, diabetes, or hypertension. Her liver function tests were normal apart from mild elevation of serum alanine aminotransferase (ALT) . The levels of carcinoembryonic antigen (CEA), CA-19-9, α-fetoprotein (AFP) were normal. The serological workup for all hepatotropic viruses was negative.The contrast-enhanced computed tomography (CECT) scan showed an enlarged liver (liver span of 20 cm) with multiple heterogeneous hypodense lesions involving both the lobes. These lesions showed peripheral nodular enhancement. The largest lesion measuring 11×8×11.3 cm was located in the left lobe with medial extension in the gastrohepatic region abutting the gastric lesser curvature laterally, the antropyloric region inferiorly, and the body and the tail of the pancreas posteriorly A. Anothein situ due to its proximity to these vessels.During surgical enucleation of the vascular masses, dense adhesions of the liver with both the anterior abdominal wall and transverse colon were noted C. The laThe enucleation specimens from the right lobe, left lobe, and Segment VIII measured 8×6×4.5 cm, 9×7×5 cm, and 3.5×2×2 cm, respectively. The cut surfaces of all these specimens revealed well-encapsulated, dark red to tan-brown colored masses. These masses showed a firm to a spongy cut surface with a few perilesional cherry-colored honeycomb foci D.Microscopically, all lesions were relatively well-circumscribed A and parThe adjacent liver parenchyma maintained lobular architecture; however, the large majority of the portal tracts and the central veins showed expansion due to the formation of micro-angiomas along with dilatation of the native vessels. These portal tracts and central veins showed irregular shapes due to micro-angioma formation A. These Postoperatively, the patient was hemodynamically stable and was discharged on day 5 with L-thyroxine  following endocrinologist consultation. After 6 months of follow-up, she is doing well, and no fresh complaints have been registered. No extrahepatic lesion was noted in further follow-up. The abdominal ultrasonography revealed the presence of 57×47 mm lesion that could not be resected due to its location (close to the middle hepatic vein) along with another small lesion close to the resection limit (segment II) measuring 16×15 mm.-2,13,Diffuse hepatic hemagiomatosis (DHH) is an unusual benign vascular lesion of the liver in adults, with less than 20 reported cases in the literature . It is c1,20,DHH usually presents with abdominal pain and distension similar to the index case. The presence of jaundice can result from the parenchymal replacement by the vascular lesions, mass effect, and hepatic dysfunction due to ischemia. The mass effect with an obstructive component appears significant due to the presence of jaundice along with clay-colored stools in the absence of any ductopenia. A normal to mild elevation of the liver function tests has been documented in the literature.2,4,7,On CT scan, DHH is observed as a nodular or infiltrative hypoattenuating lesion with indistinct borders in contrast to GCH. This feature was missed on CECT, although appreciated retrospectively in the index case. Delayed centripetal enhancement is the characteristic finding in contrasted studies, however the infiltrative borders can be overlooked in the case of isolated DHH without multisystemic involvement, similar to the index case.10,3,8,5,Histological features of DHH include thin-walled, cystically dilated, non-anastomosing, vascular channels with infiltration of the native hepatic parenchyma and without any encapsulation/ circumscription, the latter two features separating it from a CH. These vascular channels are lined by a single layer of flat endothelial cells with no significant nuclear atypia. Secondary changes like degeneration of the vessel wall, calcification, intracavitary erythrocyte deposition, and thrombosis are usually seen similar to CH.1,22,17,18,9,7,8,11,12,The occurrence of adult DHH is an uncommon event although its localized form, namely CH is the commonest benign hepatocytic lesion. 10The index case illustrates an isolated adult DHH in association with GCH. The diagnosis can be suspected using radiological modalities like USG, CT, or MRI; however, a definitive diagnosis requires histopathological evaluation. The characteristic peripheral or delayed enhancement on CT coupled with the morphology on which a diffuse replacement and lack of capsular restriction of these vascular lesions are usually helpful. Surgical resection alone might not be satisfactory if the involvement of bilateral lobes and a deep-seated location is encountered wherein radiation can be tried. Recurrences are common, and liver transplantation is the last resort to treatment.


A 65-year-old lady was referred for cross-sectional imaging of the pancreas in view of a history of uncontrolled blood glucose. She was an ex-smoker and suffered from non-insulin-dependent diabetes mellitus and hypertension. She denied any abdominal symptoms, and the examination of her abdomen was unremarkable. Blood investigations were within normal limits.Magnetic resonance (MR) imaging incidentally revealed a broad-based ampullary lesion measuring 2 × 1.6 cm which demonstrated restricted diffusion and heterogeneous enhancement on dynamic contrast-enhanced sequences . ComputeA duodenoscopy established the presence of a prominent ampulla; multiple biopsies were taken through the bite-on-bite technique. Histological analysis revealed the proliferation of bland spindle-shaped mesenchymal cells with occasional ganglion cells. Both components were S-100 positive , whereas. Follow-up MR imaging will be organized yearly until any alteration in radiological features.A panel of investigations to assess for endocrinological activity, including catecholamines and androgenic hormones, was normal. Following discussion at a multidisciplinary team meeting, a decision was taken to manage this lesion conservativelyThe radiological and histological features of this incidental lesion are consistent with those of a ganglioneuroma, a rare neuronal tumor, in the ampulla. 1 These lesions are composed of ganglion cells, Schwann cells, and fibrous tissue,2 in the absence of immature elements, thus distinguishing them from neuroblastomas and ganglioneuroblastomas.Ganglioneuromas are rare neuronal tumors derived from primordial neural crest cells, which are undifferentiated cells of the sympathetic nervous system.1 They most commonly arise in the paravertebral sympathetic chains of the posterior mediastinum or paraspinal retroperitoneum, as well as in the adrenal medulla. They very rarely occur in the gastrointestinal tract, where they have a predilection for the colon and the duodenum. These benign hamartomatous tumors can potentially arise anywhere along with the peripheral autonomic ganglion sites.1 Lesions are usually endocrinologically inactive and are often discovered incidentally. Alternatively, they may secrete catecholamines, vasoactive intestinal polypeptide, or androgenic hormones, resulting in hypertension, diarrhea, and/or virilization.Patients may be asymptomatic or may present with non-specific symptoms such as abdominal pain.3 Less than a quarter will demonstrate fine and speckled calcifications. Computed tomography is the preferred imaging technique. It will characteristically reveal the presence of well-defined, solid, encapsulated masses that are iso- to hypoattenuating to muscle.3 It provides information regarding tumor size, organ of origin, tissue invasion, vascular encasement, adenopathy, and calcifications.3On imaging, lesions are well-circumscribed and can be large.3 Lesions typically have relatively intermediate signal intensity on all sequences.3Magnetic resonance imaging can allow for better tissue discrimination, hence enabling evaluation of the organ of origin and regional invasion.3 Moreover, calcifications tend to be discrete and punctate rather than coarse or amorphous.3 The finding of distant metastases also sets ganglioneuromas apart from their malignant counterparts.The radiological appearance of these benign lesions is similar to that of malignant neuroblastomas and ganglioneuroblastomas. Differentiation is occasionally possible based on the lack of irregular contours, tissue invasion, and vessel encasing which are features of the more aggressive tumors.1 On gross pathologic analysis, ganglioneuromas are typically white, firm, well-circumscribed, and nodular tumors.2 Microscopically, the tumors are composed of an intimate admixture of spindle cells and ganglion cells. Immunohistochemistry of the spindle cells will reveal S-100-positivity, which confirms the tumor’s neural lineage.2 The presence of ganglion cells can be confirmed by staining for neuron-specific enolase and neurofilament; this will allow differentiation from other neurogenic spindle cell lesions such as neurofibromas.2Thorough tissue sampling is required in order to confirm the diagnosis.4 Prognosis after resection is excellent and recurrence is rare.1 Malignant transformation has been very rarely reported,5 thus continued follow-up is required in patients who are managed conservatively.Complete surgical resection to confirm the diagnosis can be offered, however, this depends on the location. Endoscopic papillectomy is also a safe and effective option for ampullary lesions.


Mesenteric vein thrombosis (MVT) in a pregnant patient is a rare condition that seems to be associated with the pregnancy pró-thrombotic state. This can lead to severe circumstances such as intestinal hemorrhagic ischemia, sepsis, abortion and death. Abdominal assessment is challenging due to the anatomical and physiological changes during pregnancy. MVT clinical and complementary evaluation are nonspecific, making essential an image exam. We report a case of a 33-years-old woman at 11 weeks of gestation. She sought medical evaluation due to abdominal pain and had an appendicitis diagnosis, which was treated by laparoscopic surgery. One week later, she came back complaining of nonspecific abdominal pain. So an extensive evaluation was made, and the diagnosis of MVT and intestinal ischemia was concluded. She underwent laparotomy exploration and anticoagulation, having a good evolution and so was discharged on the sixth post-operative day. While mild abdominal pain is common, severe abdominal pain during pregnancy is usually an ominous sign that needs prompt management to avoid harm to maternal and fetal health , 2. The Nonetheless, mesenteric vein thrombosis (MVT) is a rare and not yet well understood potentially life-threatening condition in pregnant patients . As a coWe present a case of a first trimester pregnant woman diagnosed with MVT and bowel loop ischemia 1 week after laparoscopic appendectomy.A 33-year-old woman, in the 11th week of pregnancy, presented with 5-day history of abdominal pain that has worsened in the last hours. She also referred anorexia and diarrhea. There were no obstetric or gynecological complaints. At evaluation, she was stable and had pain on right iliac fossa with negative Blumberg sign. She denied previous morbidities or surgeries. Her first child was born a year ago by normal delivery of an uneventful pregnancy.Abdominal ultrasonography examination confirmed the diagnosis appendicitis. She underwent laparoscopic appendicectomy, and catarrhal appendicitis was identified. The surgery was uneventful, and the patient was discharged on the first post-operative day (POD).Eight days later, she returned complaining of diffuse abdominal pain within 3 days of evolution. She had stable signs, pain on mesogastrium palpation, without peritoneal irritation. Laboratory exam with mild leukocytosis and increased C-reactive protein (CRP) (28 mg/dl) and abdominal ultrasonography showed small amounts of free fluid in the pelvis and parietal thickening in a small bowel loop.Magnetic resonance image (MRI) was performed. MVT and venous ischemia of a small bowel loop were identified  and 2. SDoppler ultrasonography performed on the first and fifth PODs identified superior and inferior MVT . AnticoaThe recovery was uneventful. The intraabdominal Penrose drain was withdrawn and the patient received hospital discharge on the sixth POD. She was reevaluated in the 14th POD and had no complaints. The thrombophilia investigation is scheduled to be performed after puerperium in order to avoid false-positives results as consequence of physiological changes of pregnancy.It is well known that physiological changes occur during pregnancy which contribute to a state of hypercoagulability . As a coVenous mesenteric ischemia (VMI) occurs less frequently than arterial ischemia, representing 5–15% of all acute mesenteric ischemia cases . It is aet al. [Guan et al.  evaluateClinical signs and symptoms include abdominal pain and distention, nausea and vomiting, while common laboratory findings are leukocytosis and elevated CRP , 9. ThusThe decision regarding surgical versus endovascular management relies on the patient’s clinical status. In patients without peritonitis, endovascular therapy can be considered as the first-line approach . EmergenIn the absence of surgical indication, MVT can be managed with supportive measures, anticoagulation and broad-spectrum antibiotics. A 6-month therapy is enough for patients with reversible causes of thrombosis, as opposed to lifelong therapy for those with hypercoagulable disorders or for whom an etiology was not established (idiopathic MVT) , 9, 10.In conclusion, we report a rare case of appendicitis followed by VMT with VMI in a 33-year-old pregnant woman who underwent exploratory laparotomy. A high index of clinical suspicion is necessary for an accurate diagnosis and prompt treatment of this life-threatening condition. Although rare, physicians should be aware of the hypercoagulable state during pregnancy and its natural risk.The authors declare that they have no conflict of interest and that the ethical principles were followed.This study did not receive any specific grant from funding agencies in the public, commercial or non-profit sectors.This study complies with institutional/national ethical standards. There is no need for evaluation by National Research Ethics Commission to short report.


Dear Editors,Neuromyelitis optica spectrum disorder (NMOSD), preferentially affecting the optic nerves and spinal cord, is a representative inflammatory demyelinating disease of the central nervous system (CNS). The presence of antibodies targeting the water channel aquaporin 4 (AQP4) can be demonstrated in the serum of most patients.A 46‐year‐old woman complained of acute progressive bilateral lower extremity numbness and weakness, along with dysuria and constipation. Neurological examination revealed paraplegia  and remarkable sensory dysfunction below the nipple plane. A positive Babinski sign was observed on the left side.Extensive laboratory workup was unrevealing, including tumor markers, pathogenic, inflammatory, and immunological assessments. Spinal magnetic resonance imaging (MRI) showed longitudinally extensive T2 hyperintense lesions involving the cervical and thoracic cord without enhancement Figure . Chest cThe diagnosis of NMOSD was made. The patient was treated with intravenous methylprednisolone  and plasma exchange . Long‐term immunosuppressive therapy was suggested, but the patient refused.The patient was then referred for surgery. The hepatic mass was removed, and postoperative pathological examination indicated a poorly differentiated intrahepatic cholangiocarcinoma. Immunohistochemical staining of the neoplastic tissue samples was performed, demonstrating the absence of AQP4 expression on the surface of tumor cells Figure .Unfortunately, no substantial improvement was observed after immunotherapy and oncotherapy (MRC grade 0 in both legs). Eighteen months later, at the latest visit, partial recovery of neurological disability was achieved (MRC grade 2 in both legs), and no neurological relapse and tumor recurrence were reported. The patient declined to repeat the AQP4 antibody test and spinal MRI during the follow‐up.The LETM with seropositive AQP4 antibodies in this patient made the definite diagnosis of NMOSD based on the 2015 diagnostic criteria.The correlation between NMOSD and concurrent tumors remains uncertain. AQP4 is highly expressed in some tumors and may have a role in its migration. AQP4 antibodies are produced by the immune system to prevent tumors from spreading.According to the PNS diagnostic criteria defined in 2004, cancer present within 2 years of diagnosis established the possible paraneoplastic NMOSD in our patient.Treatment of NMOSD associated with tumors mainly comprises oncotherapy and immunotherapy. However, it remains controversial whether long‐term immunosuppression is required for these patients. Immunosuppressive agents have been prescribed to prevent future recurrence, including azathioprine, mycophenolate mofetil, rituximab, or tocilizumab.In conclusion, our patient with cholangiocarcinoma broadens the phenotypic spectrum of NMOSD associated with tumors. Although rare, a systemic screening for neoplasms is required in AQP4 antibody‐positive NMOSD patients with the first attack. If found, AQP4 staining of tumor cells is recommended to evaluate the potential role of the tumor in the development of NMOSD and determine the optimal therapeutic schedule for these patients.The authors declare that they have no conflict of interest.Informed consent was obtained from the patient for publication of this paper.


Teaching Point: In patients with familial multiple cavernous malformation syndrome with acute focal neurological deficit, a symptomatic spinal cavernous malformation must be included in the differential diagnosis. A 29-year-old male patient with a known history of familial multiple cavernous malformation syndrome (FCMS) presented with new-onset pyramidal signs during routing follow-up. Initial brain computed tomography (CT) showed multiple hyperdense cerebral lesions corresponding to known cerebral cavernous malformations (CCM), without apparent acute hemorrhage. Because of persisting symptoms, magnetic resonance imaging (MRI) of the thoracic spine was performed 10 days later. T2-weighted images (T2-WI) showed a intramedullary round hyperintense focus with a hypo-intense rim at the level of the 4th thoracic vertebral body (Th4) . Typical (type 2) lesions have a mixed T2-signal centrally with a hypo-intense hemosiderin rim. Bi-directional T2- and T1-hypo-intense extension in the spinal cord representing intramedullary hemorrhage is often seen in SCM. Unlike in CCM, popcorn-like appearance is uncommon. Appearance on T1-WI is variable [Most patients are treated conservatively but surgical resection may be necessary in cases with severe or recurrent symptoms. Patients with FCMS may benefit from follow-up using brain and spinal MRI although no widely used guidelines exist.


Intussusception is a surgical emergency in which a part of the intestine slides into the distal adjacent part. Adult colocolic intussusception secondary to a tumoral process is a rare but serious clinical condition requiring immediate surgical intervention.We report a case of a 65-year-old male patient presenting with abdominal pain and distention, subsequently diagnosed with colocolic intussusception in the descending colon with closed-loop bowel obstruction with impending caecal perforation. An exophytic mass on the descending colon was discovered intra-operatively, prompting a subtotal colectomy with ileosigmoidal anastomosis and loop ileostomy with the suspicion of malignancy. The histopathological examination of the surgical specimen concluded a moderately-differentiated colonic adenocarcinoma with 40% mucinous component.Adult intestinal intussusception is a rare but serious condition differing greatly in etiology from its pediatric counterpart. Its preoperative diagnosis is challenging in adults, which appears to be due to its imprecise presenting signs and symptoms; thus, the condition can be mistaken for other causes of intestinal obstruction. Adenocarcinomas remain the most common cause of malignant tumors in the colon, which also makes them one of the causes for colocolic intussusception.Intussusception can appear as a surgical emergency even in the elderly, necessitating prompt surgical intervention to avoid intestinal ischemia and gangrene. Its diagnosis can be aided to a great degree by CT imaging. •Colocolic intussusception is a rare surgical emergency in adults and is often associated with neoplastic lead points.•Clinical diagnosis of the disease is challenging because of its non-specific presenting features. However, its diagnosis can be aided to a great degree by CT imaging.•The disease necessitates prompt surgical intervention to avoid intestinal ischemia and gangrene. Intestinal intussusception is infrequent in adults unlike pediatric intussusception, accounting for only around 5% of all intussusceptions cases In the majority of cases, the precipitating lesion is a bowel malignancy, thus making surgical resection the preferred choice of treatment for intussusception in adults In adults, intussusception presents with variable clinical manifestations. Since it rarely presents with the classic triad of vomiting, abdominal pain, and passage of blood per rectum, its preoperative clinical diagnosis is challenging. Abdominal Computed Tomography (CT) is the imaging modality of choice for the diagnosis of intussusception in adults. Pathognomonic signs of the condition include “target” or “sausage-shaped” masses in the outer intussuscipiens and inner intussusceptum In this study, we report a rare case of colocolic intussusception in an elderly patient. The patient was correctly diagnosed using an abdominal CT scan, which highlights the essential role of imaging in the diagnosis of intussusception in adults. The imaging also dictates the type of surgical intervention required for the patient. This case has been reported in line with the SCARE criteria 2A 65-year-old male presented to the emergency department with the chief complaint of insidious onset, colicky, moderately severe, and non-radiating abdominal pain for 1 week followed by 5 days of obstipation and a lump in the right side of the abdomen. He also provided a history of a non-bloody diarrheal episode about 2 weeks back. His appetite was reduced with an unintentional weight loss of 10 lbs. but he denied any history of fever, nausea, vomiting, hematochezia /melena. His family history was non-contributory and he had no significant past medical history. Social history of occasional alcohol consumption and tobacco use were positive. The patient denied any illicit drug use.On examination, the patient was alert and his vital signs were normal. He was afebrile with a blood pressure of 110/80 mmHg, pulse rate of 80 beats per minute, respiratory rate of 18 breaths per minute, and SpO2 of 97% in room air.On abdominal examination, mild tenderness was elicited on right lower quadrant without rebound tenderness and guarding. An ill-defined soft, smooth mass could be palpated on the right side of the abdomen with an extension from the right lumbar region to the right iliac fossa. The abdomen was distended with increased bowel sound without any sign of peritonitis. All the hernial sites were intact. On digital rectal examination, no mass could be felt but the glove was stained with blood. Other systemic examinations were found to be normal.Laboratory investigations were within normal limits with hemoglobin of 12.3 g/dL An abdominal X-ray revealed multiple dilated large bowel loops with few air-fluid levels suggesting large bowel obstruction . UltrasoThe clinical and radiological findings in the patient established that this was a case of colocolic intussusception causing closed-loop intestinal obstruction due to a competent ileocecal valve. The patient was then promptly administered intravenous fluids and anitbiotics and underwent nasogastric decompression. Emergency laparotomy was done and on exploring the abdomen, a large bowel loop invaginating into the descending colon with a large colonic mass as the lead point was revealed. The proximal bowel loops were dilated with the caecum being hugely dilated with impending perforation. The colonic mass was suspected to be malignant in origin. Thus, a standard subtotal colectomy  with ileOn opening the specimen, an exophytic, friable mass of size 8.5 cm × 7 cm × 7 cm was seen arising from the posteromedial wall of the descending colon .Fig. 4CuHistopathological examination of the cut sections from mass showed neoplastic tubular to cystic glands infiltrating the mucosal lamina propria, submucosa, muscularis propria, and perimuscular fibrous connective tissue. The glands were lined by pseudostratified columnar epithelium with loss of polarity and nuclear hyperchromasia. Mitosis constituted 5/10 HPFs and intraluminal necrosis was present. This led to the diagnosis of moderately differentiated adenocarcinoma with 40% of mucinous components (T3 N0 Mx) .Fig. 5SeThe post-operative period was uneventful and the patient recovered well. On 6-month follow-up patient was doing well and disease free.3Intussusception is an uncommon cause of intestinal obstruction in adults representing 1% of bowel obstructions and 0.003% to 0.02% of all hospital admissions An intussusception with a lead point can clinically present in a variety of ways. Most commonly patients experience intermittent or episodic pain, blood in stool, nausea, and vomiting. The complications such as bowel obstruction, infarction, hemorrhage, perforation, or peritonitis may change the clinical presentation. Neoplastic-related symptomatology may include weight loss, melena, constipation, generalized fatigue/malaise, and/or a palpable mass on physical examination Radiological investigations are extremely helpful for the diagnosis of intussusception. Abdominal X-ray shows air-fluid levels and dilated bowel loops related to the occlusive syndrome. Ultrasonography can be useful to visualize the classical target sign on a transverse view. Abdominal CT is the most sensitive study to diagnose intussusceptions with a diagnostic accuracy of 83% and is preferred to other imaging studies like colonoscopy, ultrasonography, X-ray, and small bowel series Early surgical intervention is required soon after the diagnosis due to the high occurrence of bowel ischemia and bowel gangrene In this case, dilated proximal bowel loops and a hugely dilated caecum with impending perforation was discovered on laparotomy. The excessive dilatation of the caecum may be due to a competent ileocecal valve in the presence of bowel obstruction. The excessive dilatation of the caecum may cause the appearance of fragile regions due to precarious vascular anastomosis as the caecum becomes dependent on parietal infusions. This increases the risk of perforation and hence resulting in fecal peritonitis, increasing morbidity and mortality In this case, an exophytic friable mass was found intraoperatively in the descending colon. Histopathological examination of the mass revealed moderately differentiated adenocarcinoma with 40% mucinous component. In practice, nearly 70% of colorectal adenocarcinomas are diagnosed as moderately differentiated carcinoma 4Intussusception of the colon in the elderly occurs rarely and in most cases is secondary to carcinoma. This article elaborates the case of an elderly man with colocolic intussusception due to adenocarcinoma of the descending colon. Thus, intussusception should be considered in the differential diagnosis of patients who present with a longstanding history of vague abdominal pain and weight loss. The workup must include additional radiographic studies like ultrasound or CT scan of the abdomen at the time of presentation. Finally, mandatory operative intervention is required after the diagnosis of intussusception in the elderly due to the high risk of malignancy.None.Not required.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.Diptee Poudel (DP) = Study concept, Data collection, Writing - original draft preparation.Narendra Maharjan (NM), Shankar Raj Lamichhane (SRL), Ajay KC (AK) = surgical therapy for the patient.Diptee Poudel (DP), Narendra Maharjan (NM), Shankar Raj Lamichhane (SRL), Ajay KC (AK) = Editing and writing.All the authors read and approved the final manuscript.Not applicable.Dr. Diptee Poudel.Not commissioned, externally peer-reviewed.The authors declare no competing interests.


Subependymal giant cell astrocytoma (SEGA) is a unique brain tumor that constitutes one of the major diagnostic criteria of tuberous sclerosis complex (TSC). It rarely occurs de novo after adolescence. SEGA tends to originate from the ventricular walls, provoking obstructive hydrocephalus, raised intracranial pressure, and papilledema with plausible visual problems. We present a case of large SEGA in a 33-year-old-TSC patient with a higher-than-expected age for the first presentation. His chief complaint was visual acuity deterioration combined with headaches. Microsurgical removal of the tumor was carried out. Obstructive hydrocephalus and papilledema resolved after treatment. Although SEGA-TSC is considered a tumor of children and adolescents, it can present for the first time in adulthood. In TSC patients, periodic imaging follow-up is recommended and any visual symptoms should prompt exclusion of intracranial tumors. Tuberous sclerosis complex (TSC) is an autosomal dominant hereditary phacomatosis that can affect any organ. The most common presentation of the disease is benign multiple hamartomas that may appear in the brain, heart, kidneys, eyes, and skin ,2.The International Tuberous Sclerosis Complex Consensus Group updated the clinical diagnostic criteria for tuberous sclerosis complex in 2012. Subependymal giant cell astrocytoma (SEGA) was classified as a major criterion among others, such as subependymal nodules, cortical tubers, and retinal astrocytoma . IsolateSEGA grows on the walls of the lateral ventricles and foramen of Monro, as well as the third ventricle to a less extent . ObstrucWe present the case of a 33-year-old male with known TSC who presented with visual acuity impairment and headaches because of a large SEGA in the left lateral ventricle with resulting hydrocephalus. The presentation of SEGA for the first time at this age is extremely unusual and has only been reported in a few cases. The case has been reported in line with the SCARE Criteria .A 33-year-old male patient presented to the ophthalmologic clinic complaining of gradually deteriorating visual acuity in the left eye over the last several months. He also reported headache episodes of increasing frequency. One year ago, the patient was diagnosed with tuberous sclerosis complex (TSC) based on six major clinical and radiological criteria Figure : ungual The initial evaluation revealed an intact visual acuity of 20/20 in the right eye. In the left eye, the visual acuity was low at 20/100 and could be corrected to 20/40. The pupils were round and regular with normal direct and indirect light responses. The fundoscopy revealed papilledema stage I and stage II according to the Frisen staging system in the right and left eyes, respectively. The rest of the ophthalmological assessment, as well as visual fields, was within the normal limits.Magnetic resonance imaging (MRI) with gadolinium enhancement showed cortical tubers, subependymal nodules, and a large, enhancing lesion on the wall of the dilated left lateral ventricle Figure . The lesThe patient was referred to the neurosurgical department, where surgical removal of the tumor was performed using a left frontal, ultrasound-assisted, transcortical transventricular approach using a microscopic technique. An external ventricular drain was placed before closure. The histological examination confirmed the diagnosis of astrocytoma.The symptoms resolved at one month postoperatively and there was a significant improvement in the visual acuity as well as papilledema in the left eye. The postoperative MRI after three months showed complete resolution of hydrocephalous. The patient was well after six months.Tuberous sclerosis complex, also known as Bourneville Pringle disease, was initially described by von Recklinghausen in 1862 . It is aThe morbidity and mortality associated with this disease are attributed mainly to neurological lesions . AlthougThe incidence of SEGA in TSC varies from 5% to 14% . SEGA teTSC-SEGA originates mainly in the lateral periventricular regions adjacent to the foramen of Monro , and, toWhile focal neurological symptoms and headaches are frequent complaints , the decIn our patient, surgical intervention was indicated due to the symptomatic obstructive hydrocephalus. Surgery is a safe and effective treatment of SEGA and can be performed using multiple surgical or neuroendoscopic approaches  as well Subependymal giant cell astrocytoma is a rare, benign tumor of the central nervous system that constitutes a major criterium of the tuberous sclerosis complex. Although it is considered a tumor of children and adolescents, the occurrence and manifestation in later adulthood may still happen. Periodic imaging follow-up is recommended. New complaints in those patients, such as visual symptoms, should prompt thorough investigation with TSC kept in mind.


Ewing's sarcoma rarely presents in bones of the feet. Surgical management usually includes amputation. Limb sparing surgery is anecdotal.We report the case of a 13‐year‐old boy with an Ewing sarcoma in his calcaneus who had a calcaneal reconstruction with total calcaneus allograft after induction chemotherapy.At 42 months of follow‐up our patient remains disease free and functionally intact. A review of the exceptional limb salvage procedure options for malignant calcaneus tumor was performed. Physical examination showed a swollen ankle with mild tenderness and slightly decreased range of motion. No fever or weight lost was found. Radiography and computed tomography (CT) revealed an expansive lytic lesion in the calcaneum with cortical break and no associated periosteal reaction. Magnetic resonance imaging (MRI) of the ankle showed a well‐defined bony lesion, isointense to T1‐weighted and heterogeneously hyperintense to T2‐weighted images, associated with a soft tissue mass. The tumor showed intense enhancement after contrast administration due to its high vascularity and also presented internal foci of necrosis Figure . CT and EWSR1‐FLY1 fusion gene by RT‐PCR. A limb salvage surgery with a total calcanectomy and reconstruction with allograft calcaneus was planned since the calcaneus was almost completely involved, and there was no evidence of metastasis. The patient was admitted at the oncology ward and started on chemotherapy following the Practice Guidelines based on the Spanish sarcoma group 21 protocol (GEIS‐21).−2, doxorubicin 75 mg m−2 and vincristine 2 mg m−2; and cycles 3 and 5 with ifosfamide 9 g m−2 and etoposide 500 mg m−2.Ultrasound‐guided biopsy was performed. Histologically biopsy showed a malignant tumor composed of uniform small round cells, diffusely distributed. The cells showed strong membranous immunohistochemical expression of CD99. Molecular study revealed a 2.1The surgery was performed by specialist's pediatric oncologic orthopedic surgeons. Under spinal/epidural anesthesia, the patient was set in supine position with affected thigh elevated and pressurized by tourniquet. A lateral calcaneus approach extended with a posterior‐medial ankle approach was used to expose the subtalar joint and performed the total calcanectomy, avoiding the sural nerve and fibular tendons Figure . After tThe average size of the calcaneus was taken from the images obtained by foot MRI and CT scan. The calcaneal allograft was selected for being similar to the original in shape and size, being the best option available in the bone bank of the Barcelona Tissue Bank.2.2Pathological examination of the surgical specimen confirmed the calcaneus Ewing sarcoma with clear surgical margins, the closest ones were covered by periosteum, without infiltration, and soft tissue margins were of 5 mm Figure . There wFollow‐up radiography at 12 months showed normal position of the allograft and screws, with progressing healing of the arthrodeses  Figure . Follow‐3With a conservative surgical management of a malignancy, it is difficult to achieve both adequate surgical margins and a functional reconstruction.In our patient, a reconstruction with a frozen calcaneus after the total calcanectomy for the Ewing's sarcoma was performed. Reconstructive techniques for the hindfoot after total calcanectomy include osteocutaneous distally pedicled, free flap pedicled, prosthesis and allograftReconstruction with frozen calcaneus allograft after a total calcanectomy for the malignant bone tumor has been reported previously. Ottolenghi and PetracchiIn our patient, the calcaneus allograft was fixed in place with compression screws after the joint surfaces were resect, as needed, to obtain a proper fit. Because the rare of the use of total calcaneus allograft, no biomechanical studies of the mode of fixation have been made. Based on the current literature we preferred a double arthrodesis with screws. Degeorge et al. made the calcaneocuboid arthrodesis with a Blount's staple, however, they reported that their patient developed a varus of the hindfoot.Ewing sarcoma generally arises from diaphysis of long bones and axial skeleton; however, rarely, the small bones in the feet including calcaneus can be affected. We think that in skeletally immature children, reconstruction with calcaneus bone allograft is a good alternative among several options that are available such as custom‐made prosthesis, fibular or iliac crest flaps. We describe the use of this technique with a 42‐months follow‐up. At last follow‐up, our patient was disease free and had an excellent functional outcome. Further studies are needed to compare the available surgical options, including their functional outcome, complications and cost.4Total calcaneal allograft reconstruction of an Ewing's calcaneus sarcoma in a child is a good alternative limb salvage surgical treatment among several options that are available such as custom‐made prosthesis, fibular flaps, or iliac crest flaps.Ferran Torner: Conceptualization (lead); investigation ; methodology ; supervision ; validation ; writing – review and editing . Jorge H. Nuñez: Conceptualization ; investigation ; methodology ; writing – original draft . Emilio José Inarejos Clemente: Investigation ; supervision ; writing – review and editing . Moira Garraus: Investigation ; methodology ; supervision ; validation ; writing – review and editing . Mariona Suñol: Conceptualization ; investigation ; methodology ; supervision ; validation ; writing – review and editing . Aníbal D. Martínez: Conceptualization ; investigation ; methodology ; resources ; supervision ; writing – review and editing . David Moreno: Conceptualization ; investigation ; methodology ; supervision ; validation ; writing – review and editing .The authors have stated explicitly that there are no conflicts of interest in connection with this article.This material is the authors' own original work, which has not been previously published elsewhere. The study was performed in accordance with the ethical standards aslaid down in the 1964 Declaration of Helsinki.


Small bowel obstructions are a common general surgery occurrence. In a patient with prior abdominal surgeries, the usual diagnosis is secondary to adhesions. The management is typically conservative, which usually avoids operative intervention. Computed tomography (CT) scans help diagnosticians take a snapshot inside the abdomen; however, CT scans are not perfect and intra-abdominal pathologies can be missed requiring surgery. We present a case of an atypical small bowel obstruction. The initial CT scan showed a transition point in the right lower quadrant, which was managed non-operatively. One week later, the patient then re-presented with hematochezia following an outpatient pill cam procedure. Imaging showed the presence of a new small bowel mass, which was not seen on imaging done 1 week ago or from 10 months prior. He was then taken for a diagnostic laparoscopy, in which a small bowel mass was found, pathology positive for recurrent renal cell carcinoma. It is estimated that small bowel obstructions account for ~12–16% of surgical admissions, resulting in 300 000 annual operations in the USA , 5. TheySmall bowel obstructions are typically managed with bowel rest, nasogastric tube decompression and fluids. Obstructions secondary to adhesions are successfully managed non-operatively about 70–80% of the time , 7. The The patient is a 69-year-old male with a past medical history of hypertension, chronic kidney disease, chronic obstructive pulmonary disease, right renal cancer status post-laparoscopic nephrectomy in 2016  who presented to the emergency room (ER) with abdominal pain. The patient recently underwent an esophagogastroduodenoscopy and a colonoscopy the week prior as a workup for anemia, which were unremarkable. Since then, he started having intermittent abdominal pain and bloating. The CT abdomen and pelvis without contrast in the ER showed multiple dilated small bowel loops with a transition point in the right lower quadrant  and 2. DThe next week, unbeknownst to his Gastroenterologist about his recent small bowel obstruction admission, he then proceeded with a capsule endoscopy study to assess for other causes of his anemia. He tolerated the pre-operative bowel preparation without issues. After swallowing the capsule, he subsequently had bloody bowel movements. He then went to the ER and found to have a hemoglobin of 7.1, which was decreased from his prior week’s hospitalization, 8.4. A CT angiography (CTA) abdomen and pelvis was then performed that showed a 4 × 6 × 4.1 cm mass in the right upper quadrant with the capsule endoscopy at the level of the mass  and 2. TSurgery revealed a small bowel tumor in the jejunum  and 4, wAccording to the Surveillance, Epidemiology, and End Results database, the 5-year survival of stage IV renal cell carcinoma is 12% . Renal cSurveillance protocol varies by the American Urologic Association and the National Comprehensive Cancer Network. Based on this patient’s staging, both guidelines recommend follow-up CT chest imaging, as well as CT, magnetic resonance imaging or ultrasound of the abdomen every 3–6 months until 5 years , 13. AddThis is a rare case of a metastatic renal cell carcinoma leading to anemia and a small bowel obstruction that was identified after a capsule endoscopy procedure. Prior non-contrast CT a week prior and another CT with IV contrast 10 months prior were negative for a small bowel mass. Therefore, this 6 cm mass remained undetected by prior imaging and was only found after he became symptomatic from the capsule itself. Even the capsule images were ineffective. CT scans have a high rate of sensitivity and specificity, but are not flawless. When adhesive disease is not the cause of a bowel obstruction, it is important to look for other possible etiologies. As medicine continues to advance, new strategies for monitoring kidney cancer recurrence are on the horizon.


Supernumerary kidney (SNK) is a rare congenital anatomical variation usually detected incidentally via imaging. Although a random finding, it may present with hydronephrosis, calculi or malignancy. Both its vascularization and its drainage vary significantly, depending on its location and shape. Riedel’s lobe is a normal, though rare, variant of liver anatomy presenting either as a downward projection of the inferior border of the right liver lobe or as a triangular pyramidoid projection to the right of the gallbladder.We present a case of a 71-year-old man who was initially admitted to the hospital for backache. Computed tomography (CT) imaging revealed the simultaneous occurrence of a left supernumerary kidney and Riedel’s lobe. The SNK lay caudally to the normal kidney, it was supplied by a branch of the superior mesenteric artery and its drainage was supported by a bifid ureter. The Riedel’s lobe represented the “tongue-like” variant without causing any symptoms to the patient.Both entities should be monitored carefully, as their presence may require surgical management should they raise a sequence of symptoms or, as in this case, modify the surgical plans in the context of other coexisting medical events. A supernumerary kidney (SΝK) is an encapsulated additional parenchymal organ, supported by its individual vasculature and collecting system. It is considered a very rare congenital anatomical variation with only a few cases reported worldwide ,2. The dThe type of SNK drainage by an individual ureter or a bifid ureter can distinguish two separate entities of this condition. The location of the supernumerary kidney in relation to the ipsilateral kidney usually follows the type of its drainage: when it is located cranially, an individual ureter is present, and when caudally, which is more common, it is drained by a bifid ureter. Blood supply also varies depending mostly on the position of the additional kidney compared to the major one ,4. SNK iA Riedel’s lobe is an anatomical variation of the liver, appearing as a downward tongue-like projection of the inferior border of the right lobe or as a triangular pyramidoid projection to the right of the gallbladder . It is uThis report presents a unique case of a 71-years-old male with a supernumerary kidney on the left side, as well as a Riedel’s hepatic lobe.A 71-year-old male was admitted to the University Hospital of Alexandroupolis complaining about a severe backache. Anti-inflammatory drugs were administered to him and he was discharged. A month later, the recurrence of the symptoms led to his admission for further clinical investigation. Laboratory tests revealed anemia and acute renal failure which was treated conservatively. Anemia was thoroughly investigated for its origin and a sigmoid colon mass was detected via imaging.Pre-surgery testing revealed an aneurysm of a jejunal branch of the superior mesenteric artery. A Riedel’s lobe of the “tongue-like” variant, as well as a supernumerary left kidney, were also detected, as random imaging findings. The SNK was fully separated from the normal kidney and located caudally to the latter. Its drainage was supported by a bifid ureter and its vascularization was supplied by a separate artery derived from the superior mesenteric artery Figures -3.The SNK’s dimensions were 6.53 x 3.26 cm, significantly smaller than the normal kidney that usually ranges between 10 and 14 cm long in males , which required an ileostomy procedure. The postoperative course of the patient remained eventful as renal failure caused severe electrolytic disorders. Two months post-surgery, the patient died from septic shock due to a failed intestinal anastomosis and intraabdominal contents leakage.We report a unique case of a 71-year-old male with a supernumerary kidney on the left side concurrent with a Riedel’s liver lobe.SNK is an uncommon anomaly with less than 100 cases reported to date. It can be associated with several congenital abnormalities such as ectopic ureteric opening, ureteral and vaginal atresia, among others. When this is the case, this cluster of rare diseases further complicates the standardization of a diagnosis protocol. Still, SNK is considered mostly a random imaging finding .According to the available data, either side presents equal frequency in relation to the supernumerary kidney’s location. In our case, the SNK was located on the left side without the “horseshoe” malformation, following the typical pattern. The drainage system of the SNK varies significantly from an independent to a bifid ureter with the latter being the most anticipated type of drainage and appearing in our case. The SNK typically depends for its arterial supply on its own separate artery, albeit anatomical vascular variations may occur. Once again, the SNK under study abides by the rule of a separate artery.Although SNK is mostly asymptomatic, it can be accompanied by a number of symptoms, such as hypertension, abdominal discomfort, hydronephrosis, or present as a palpable mass. Even though none of the above-mentioned clinical signs, except for a complaint of backache, were present in our case, the patient was diagnosed with acute renal failure soon after his second admission to the hospital. Nevertheless, the clinical findings of our patient’s several comorbidities suggest that neither back pain nor acute renal failure can be directly associated with the SNK.Riedel’s lobe is an uncommonly encountered morphological variation of the hepatic lobulation, where the inferior border of liver segments 5 and 6 projects inferiorly to the right of the gallbladder . It presTo our knowledge, this duet of anatomical variants depicts a sui generis case that has not been previously reported. The left location of the SNK, contralateral to Riedel’s lobe, invalidates any attempt to form hypotheses about an embryological connection of their coexistence. Furthermore, taking into consideration that Riedel’s lobe is more frequently encountered in females, with a male to female ratio of 1:3, the case we present exhibits even greater peculiarity ,11,13.Both anatomical entities require careful management to ensure an optimal outcome for the patient. SNK management depends on renal function, patient’s symptoms, and related complications. Although asymptomatic or minimally symptomatic cases are favored for a scheduled follow-up to detect early complications, surgical intervention (nephrectomy) is considered the treatment of choice for SNKs with associated comorbidities. It is important to highlight that scientific data suggest that variations in renal vascular anatomy such as multiple renal arteries that inevitably accompany an SNK, or renal parenchymal congenital anomalies, may affect aortic endograft procedures . Prior cRegarding Riedel’s lobe, it is a mostly asymptomatic accessory liver lobulation that usually requires no special treatment than a regular follow-up and monitoring . SurgeryRandom cases enrich physicians’ perspectives. A third kidney with an abnormal Riedel’s lobe is a rare medical event that may raise diagnostic issues and create surgical complications during abdominal interventions. Modern imaging techniques (CT) with advanced protocols play a vital role in assisting physicians' choice of treatment in a more personalized approach towards the patient. Although both entities are rarely linked with major complications, it is important to be attentive, especially in cases where the surgical approach is highly considered. Another interesting point that needs to be brought forward is the embryological descent of these cases, an approach mostly neglected as physicians focus on treating symptoms.In general, anatomic aberrations, even those that don’t normally disrupt the body’s function, could accompany, induce, or perplex clinical signs and should always be kept in mind. Case reports contribute to this pathway enhancing our knowledge of anatomic variations.


Esophageal gastrointestinal stromal tumors (GISTs) are rare tumors of the gastrointestinal interstitium, and comprise less than 0.7% of all GISTs. The presentation of esophageal GIST is relatively benign, commonly characterized by symptoms of dysphagia and gastrointestinal bleed. On the contrary, it is highly unusual for these tumors to present as surgical emergencies.Here, we describe a case of hemothorax secondary to the rupture of a massive (19 cm) esophageal GIST in a 79-year-old male. The patient presented with mild back pain, vomiting, and hypotension. A CT scan revealed significant mediastinal enlargement and left hemothorax. We conducted an emergency thoracotomy which revealed a 19 × 15 × 7 cm ruptured esophageal tumor that was bleeding profusely into the left thoracic cavity. Piecemeal resection without esophagectomy was performed to achieve hemostasis. Pathological evaluation of resected tissue confirmed the diagnosis of GIST. The patient was provided adjuvant imatinib therapy and remains progression-free at the 10-month follow-up.To the best of our knowledge, this is the first reported case of life-threatening hemothorax caused by a ruptured esophageal GIST. Findings from this case may aid in the diagnosis and management of these rare tumors. Gastrointestinal stromal tumors (GISTs) are rare tumors of the gastrointestinal interstitium and esophageal GISTs are particularly unusual, accounting for only 0.7% of all GISTs . ResearcA 79-year-old male arrived by ambulance to our emergency department with chief complaints of nausea and vomiting after eating lunch. The patient was hypotensive and tachycardic on arrival and a physical exam was notable only for mild back pain and crackles in the left lower lung field. Past medical history included hypertension, type 2 diabetes mellitus, chronic kidney disease, and gastro-esophageal reflux disease which were all managed by medical therapy. As insufficient kidney function contraindicated contrast use, a non-contrast computed tomography (CT) scan was obtained Fig. , which rThe patient was placed in the right lateral decubitus position and the thoracotomy incision was made along the left sixth intercostal space. Independent ventilation of the right lung was established prior to thoracotomy. A massive hemothorax was noted upon entering the left thoracic cavity. After draining approximately 2 l of blood, a large tumor roughly 20 cm in size, situated caudal and posterior to the heart in the lower posterior mediastinum became visible. The tumor compressed the surrounding structures, but was encapsulated and well demarcated. Careful examination revealed that a portion of the capsule had ruptured and was hemorrhaging into the thoracic cavity (Fig. Histologically, the tumor was composed of disarrayed fascicles of spindle cells with palely eosinophilic fibrillary cytoplasm Fig. b. The miThe patient’s recovery from surgery was unremarkable. He was discharged on postoperative day 34. Adjuvant therapy with 400 mg imatinib mesylate daily was started as an outpatient, but discontinued after 1 month of therapy due to side-effects. The patient was offered second-line treatment with sunitinib, but declined because of his stable clinical status. Gastroscopy conducted at 2 months post-operation showed no sign of mucosal invasion or esophageal stricture (Fig. We describe the successful surgical management of a rare case of esophageal GIST rupture. We believe this case is notable for several points. Firstly, the case is educational because the patient’s presenting symptoms closely mimicked that of esophageal perforation, a condition characterized by chest or back pain accompanied by mediastinal enlargement and pleural effusion on imaging studies. Notable deviations from the usual presentation of perforation were the lack of free air findings on imaging and the relatively mild pain experienced by the patient. In such cases, esophageal tumor rupture should be considered as a differential diagnosis.Furthermore, an esophageal GIST rupturing into the thoracic cavity instead of the esophageal lumen is a very rare phenomenon. GISTs rupturing into the gastrointestinal lumen are not uncommon. For example, gastrointestinal bleeding due to tumor invasion is a relatively common presentation of esophageal GIST, with a reported occurrence rate of one in eight cases . In starAnother noteworthy aspect of this case is that we conducted an emergent operation. Esophageal GISTs do not typically present as surgical emergencies, and hence, there are very few published examples of management in such situations –8. One sFinally, the case begs the discussion of whether incomplete resection has survival benefit in esophageal GIST. Because of the rarity of esophageal GIST, the studies to guide us on this topic do not exist. One paper that may inform us is a retrospective study of 239 patients with metastatic GIST who were on imatinib therapy and who were also provided with incomplete resection or debulking surgery of the tumor . SurgeryWe describe urgent surgical management of a ruptured giant esophageal GIST by emergency thoracotomy and subsequent “piecemeal” resection without esophagectomy. Although rare, ruptured esophageal GIST represents a potential differential in patients presenting with hemothorax and mediastinal enlargement.


Bronchogenic cyst is a congenital aberration of bronchopulmonary malformation with bronchial-type, pseudostratified cylindrical epithelium. They are usually discovered in the mediastinum and intrapulmonary but are rarely encountered in retroperitoneum. We report a case of the retroperitoneal bronchogenic cyst and perform a literature review to summarize the safety of laparoscopic resection for this rare disease.We report a 57-year-old woman who was admitted to our hospital with no clinical symptoms and was found by chance to have masses in the adrenal gland area during a routine physical examination. An abdominal CT examination revealed a cystic lesion was found in the left suprarenal region. Afterward, the patient underwent a laparoscopic exploration. Histopathological findings confirmed the diagnosis of a retroperitoneal bronchogenic cyst. The patient recovered uneventfully without signs of recurrence during a 1-year follow-up period.Bronchogenic cyst is rare in the retroperitoneal region. It should be considered as one of the differential diagnoses of a retroperitoneal neoplasm, especially in the left retroperitoneal region. Laparoscopic surgery is technically feasible and safe for the treatment of patients with a retroperitoneal bronchogenic cyst. Bronchogenic cysts (BCs) arise from abnormal budding of the foregut during embryogenesis, which is a benign congenital aberration of bronchopulmonary foregut malformation . It is tOn January 4, 2021, a 57-year-old woman was referred to the West China Hospital for evaluation of a left adrenal neoplasm suspected of being an adrenal tumor. The patient had no symptoms and the mass was incidentally discovered on medical examination. There were no obvious abnormalities in her medical history or physical examination. Additionally, she did not have any underlying diseases or take any medications. Routine laboratory investigations such as complete blood counts and liver and kidney function tests were within normal ranges. Specifically, negative results were obtained for all adrenal gland hormones and serum tumor markers , and a bipolar clamp coagulation system . Dissociation and exposure of the mass were achieved through the ultrasound knife, along with clamping the blood vessels leading to and from the tumor by a titanium clip. Intraoperatively, a 4×3cm cyst mass was observed behind the head of the pancreas and on the left side of the abdominal aorta, which had an unclear boundary with the surrounding tissues and was closely adhered to the diaphragm. The entire mass was completely removed. Afterward, we repaired the damaged diaphragm and performed complete hemostasis of the wound. The resection specimen was collected in a plastic bag and removed via small incisions around the umbilical cord. An orthopedic drainage tube was inserted into the left retroperitoneal cavity, and slightly bloody fluid was collected. The operation lasted 200 min, and the estimated blood loss was 20 ml with no transfusion.During the surgery, the patient was positioned supine. Using Veress needles, a 1.5-cm incision near the upper navel edge was made to establish pneumoperitoneum with a constant pressure of 13 mm Hg. Four trocars were used: a 5 mm trocar was inserted on the left side of the umbilicus, and two 12 mm trocars were placed below the right costal margin in the midclavicular line and mid-axillary line, respectively. The rest 12 mm trocar was installed under the xiphoid process. The procedure was performed using an ultrasonic surgical aspirator , an ultrasonic system  for the publication of any potentially identifiable images or data included in this article.JL and QF contributed to the study concept and design. HL, JX, and QF contributed to the investigation and writing the original draft. HL, JX, and QF contributed to data collection. QF,JL and ZC revised the paper. All authors contributed to the article and approved the submitted version.This work was supported by the 2021 Sichuan Science and Technology Plan Project “International cooperation in science and technology innovation/technological innovation cooperation in Hong Kong, Macao and Taiwan“ (2021YFH0095) and Sichuan University from 0 to 1 project (No. 2022SCUH0017).The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.All claims expressed in this article are solely those of the authors and do not necessarily represent those of their affiliated organizations, or those of the publisher, the editors and the reviewers. Any product that may be evaluated in this article, or claim that may be made by its manufacturer, is not guaranteed or endorsed by the publisher.


A 14-year-old girl with bilateral chronic posterior scleritis was referred to us for poor control of ocular inflammation. There was an incidental finding of choroidal osteoma bilaterally whereby the choroidal mass in her right eye demonstrated a significant tumor growth in a short duration of two months. The right eye choroidal tumor was orangy in color with a well-defined margin, two-disc diameter in size, and located at the macula encroaching the fovea. Multimodal imaging assessments, including serial color fundus photo, enhanced depth imaging optical coherence tomography (EDI-OCT), and B scan ultrasonography monitoring, confirmed a continuous rapid growth of choroidal osteoma with episodes of scleritis flare-ups. Furthermore, intolerance toward second-line immunosuppressants and loss of follow-ups during the coronavirus disease 2019 (COVID-19) pandemic lockdown led to frequent relapses of her posterior scleritis. Therefore, a new treatment plan was designed, and close monitoring of choroidal osteoma growth and control of posterior scleritis were initiated. Subsequently, bilateral posterior scleritis remained quiescent, and her vision remained stable with stagnant growth of choroidal osteoma. Choroidal osteoma is a rare, benign osseous tumor within the choroid. It is usually unilateral and occurs in the juxtapupillary region of the eyes in healthy women in the second to third decades of life. Choroidal osteomas tend to be orange-red in color in the early stages, and later develop a yellowish tint secondary to retinal pigment epithelium (RPE) depigmentation . It was A 14-year-old girl with a prior history of bilateral posterior scleritis treated at a secondary hospital was referred to a facility in the central region of Malaysia for resistant steroid therapy for scleritis control. She underwent a thorough autoimmune and infective workup, but the results were unremarkable. Initially, she was started on oral methotrexate (MTX) 15 mg per week combined with gradual tapering of oral prednisolone. However, she developed flare-ups when oral prednisolone was tapered to 10 mg OD or below. Subsequently, she was co-managed with the Pediatric Rheumatology team for the optimization of second-line treatment. Oral MTX was switched to the subcutaneous route, given poor tolerance to the oral form. After that, scleritis control was achieved and maintained for about a year. However, after a year of being quiescent, she developed frequent relapses in her right eye. Hence, subcutaneous MTX was further increased to 17.5 mg per week, and oral prednisolone was escalated to an immunosuppressive dose. These improved the flare-ups, but upon tapering of prednisolone below 20 mg, she began to experience mild aches over her right eye. She was subsequently referred to the Uveitis Clinic by the Pediatric Rheumatology team.Upon our first review, her visual acuity for both eyes was 20/30. Anterior segments were unremarkable, and intraocular pressures were normal. There were no signs of intraocular inflammation or steroid toxicity. However, we noted bilateral optic disc hyperemia. There was an incidental finding of a two-disc diameter orangy choroidal mass at the macular region in her right eye Figure  and a onEnhanced depth imaging optical coherence tomography (EDI-OCT) demonstrated a mass with a spongy form appearance in the choroidal layer Figures -4 3]. T. T3]. ThUveitic workup was repeated. The Mantoux test, TB Quantiferon, anti-nuclear antibodies (ANA), rheumatoid factor (RF), antineutrophil cytoplasmic antibodies (ANCA), and venereal disease research laboratories (VDRL) were all negative. Therefore, she was treated as a case of bilateral choroidal osteoma likely induced by poorly controlled posterior scleritis. She was continued on oral prednisolone 15 mg OD and subcutaneous MTX 17.5 mg per week. After two months, the right eye choroidal mass significantly increased in size, encroaching the fovea Figure , while tAlthough the characteristic clinical appearance of choroidal osteoma has been extensively described in the literature -6, due tDespite the availability of well-documented case reports and reviews ,4,8, theOn histopathology, choroidal osteoma consists of bony trabeculae between an altered choriocapillaris and the outer choroidal layers . In addiSubretinal mass in posterior scleritis is not uncommon -18. NoduConcurrent choroidal osteoma and posterior scleritis are rare. Based on our literature review, this is likely the third case report to describe it. The previous two case reports were published by Trimble et al.  and NairWe are also still considering the possibility of IgG4-related inflammatory disease due to the recurrent "behavior" of posterior scleritis and choroidal osteoma association in our patient, based on the study by Nair et al. . HoweverChoroidal osteoma is a rare benign ossifying tumor of the choroid. This case report highlights the enlargement of the choroidal osteoma with recurrent posterior scleritis, and clinicians should be vigilant about its presence. Decalcification of the tumor, choroidal neovascularization, and RPE atrophy can result in potential blindness. Hence, early detection, good control of ocular inflammation, and continuous monitoring of the tumor growth with multimodal imaging are essential to prevent permanent blindness in these patients.


Oncocytic adrenal cortical neoplasms are rare cases and are divided into oncocytoma, oncocytic neoplasms of uncertain malignant potential and oncocytic adrenal cortical carcinomas, based on the Lin–Weiss–Bisceglia (LWB) histological system adopted in the current World Health Organization (WHO). We reported a 42-year-old female diagnosed with an oncocytic neoplasm of uncertain malignant potential initially, which turned out to be a carcinoma owing to distant metastasis to the scalp and lung. To our knowledge, this is the first published case of oncocytic adrenal cortical carcinoma with scalp metastasis. This case also highlights the limitation of the current diagnostic algorithm and emphasizes the importance of two parameters (PHH3 and Ki-67) for determining the malignant potential of oncocytic adrenal cortical neoplasms. Adrenal cortical carcinoma (ACC) is the most common primary cancer in the adrenal gland and is subclassified into four histologic variants: conventional, oncocytic, myxoid, and sarcomatoid. The majority is the conventional variant, which accounts for 90%, followed by the oncocytic variant.“Oncocyte” was first introduced in 1931 by Hamperl, referring to a cell with abundant granular eosinophilic cytoplasms, which are confirmed by an abundance of mitochondria ultrastructurally or immunohistochemically reactive to antimitochondrial antibody (AMA) mES-13 .Adrenal oncocytic neoplasms (AONs) are an extremely rare variant of adrenocortical tumors that include adrenal cortical oncocytoma, oncocytic adrenal cortical neoplasm with uncertain malignant potential, and oncocytic adrenal cortical carcinoma. Since the first case was confirmed by electron microscopy in 1991, only 56 cases were diagnosed as oncocytic adrenal cortical carcinoma until 2021 .Based on current limited cases, the prognosis of oncocytic ACC appears to be better than for the conventional type. Only seven cases reported developed distant metastases, and the liver, lung, bone, and ovary were the most common sites for them  3]..3].A 42-year-old female with a history of asthma without medication control came to our emergency department owing to diffuse abdominal pain in June 2020. The physical examination revealed epigastric and left upper quadrant tenderness as well as left costovertebral angle tenderness. The laboratory examination results showed as follows: Cortisol: 9.32 ug/dL (5 to 25 ug/dL), Testosterone: 26 ng/dL (4.94–32.01 nmol/L), Aldosterone: 56.5 pg/mL (20–90 pg/mL), P.R.A.: <0.14 ng/mL/h (1.02 ± 0.12 ng/mL/h), V.M.A.: 2.2 mg/24 h (2–7 mg/24 h). Abdominal CT revealed a heterogeneous low-density mass (size: 3.9 × 2.9 cm) over the left supra-renal space, accompanied with a low attenuation area in the center, suggestive of necrosis a. A lapaGrossly, this yellow tumor measured 3.9 × 2.9 × 2.5 cm in size with a brownish central necrosis, smooth outer surface and intact adrenal capsule b. MicrosIn August 2021, the patient came back to our hospital due to a small nodule found over her right scalp. The image examination revealed a 1.17 cm soft tissue mass at the right frontal scalp, and the following PET scan revealed several sub-centimeter nodules throughout the lung field. Therefore, a surgical resection of this scalp tumor and wedge resection of pulmonary nodules were performed. The pathology of both the scalp and lung tumors showed metastatic oncocytic adrenal cortical carcinoma, confirmed by a panel of immunohistochemistry.It can be difficult to diagnose the oncocytic variant of ACC, even when helped by ancillary tests. Krishnamurthy et al. shared a similar opinion, suggesting that the only unquestionable criterion of malignancy in an AON is the presence of metastasis or invasion (capsular and/or vascular) .The Weiss criteria for malignancy in ACC cannot be directly applied to AONs because of the consistent presence of eosinophilic cytoplasm, a diffuse growth pattern, and a high nuclear grade . AONs arThis case was initially reported as an oncocytic adrenal cortical neoplasm with an uncertain malignant potential based on the Lin–Weiss–Bisceglia criteria, owing to the presence of sinusoidal invasion and necrosis but the lack of any major criteria.Unfortunately, distant scalp and pulmonary metastases were found about one year later. Owing to the rapid progression of the disease, we wondered whether this AON could be diagnosed as a malignancy the first time.Multifactorial diagnostic schemes have been proposed to aid in the diagnosis of ACCs . The HelPhospho-histone H3 (PHH3) immunostaining is a valid, accurate, and reproducible surrogate marker of the standard mitotic count in adrenal cortical tumors, especially in ACCs with a low mitotic index . PHH3 isIn the current WHO classification, the Lin–Weiss–Bisceglia (LWB) criteria proposed in 2004 are adopted to differentiate between AONs that are benign, borderline malignant potential, or malignant neoplasms. However, several articles have emphasized the importance of the Ki-67 proliferative index as a parameter for predicting diagnosis as well as prognosis. We provided a case which was initially diagnosed as an oncocytic adrenal cortical neoplasm with an uncertain malignant potential following LWB criteria. However, it turned out to be a malignancy owing to distant scalp and lung metastases about one year later. After reevaluating the mitotic count and proliferative index in hotspots by PHH3 and Ki-67 immunostaining, this case could be initially diagnosed as oncocytic ACC by either the Lin–Weiss–Bisceglia histological system or the Helsinki score. Hence, we would like to share this extremely rare case and emphasize the importance of PHH3 (identifying mitotic figures) and Ki-67 (proliferative index) in hotspots as ancillary tests for determining the malignant potential of AONs.


Ascaris migration from the intestine into the peritoneal cavity is rarely seen and the usual presentation is the acute abdomen. Our case report is of a young male who got admitted after a roadside accident with polytrauma including blunt trauma abdomen. When the patient was taken up for exploratory laparotomy, a freely lying tubular structure was noticed in the pelvis and small intestinal perforation. On inspection, it turned out to be an Ascaris worm. This is a case report of a rare presentation of Ascaris lumbricoides with jejunal perforation following blunt trauma. This blunt trauma could have been the cause of an intestinal perforation resulting from a concealed presence of an impending Ascaris perforation.  Ascaris lumbricoides is one of the most common helminthic infections throughout the world with the highest prevalence in developing countries like India, Bangladesh, and China. Although rare, perforation peritonitis is a known complication of ascariasis [Ascaris produces a lytic secretion, and this together with the nibbling effect of the head of the Ascaris can lead to perforation of the normal bowel wall [cariasis . It is pwel wall .Ascaris lumbricoides-associated intestinal perforation with blunt abdominal trauma. Though the clinical presentation is usually delayed if there is mild trauma, this mild trauma could have led to intestinal perforation, accelerating the already present impending ascariasis perforation [This is a report of a rare case of foration .Ascaris was found in pelvic collection roughly 20cm in length  after a thorough peritoneal wash with saline. Two drains were placed in the pelvis and Morison's pouch. Postoperatively the patient was given a triple antibiotic cover and recovered well. The patient was allowed oral intake of food on postoperative day three. On review of the patient’s history, it was noticed that he frequently used to eat from street vendors. The history ruled out worms in stool, abdominal pain, or any history of intestinal perforation. He was treated with a course of albendazole.A 28-year-old male presented to the casualty after a roadside accident with polytrauma. On examination, the patient had multiple fractures of the femur, tibia, radius, and facial injuries. A history of blunt trauma to the abdomen was present. No complaint of pain in the abdomen on presentation. The abdomen was soft and non-tender. Ultrasonography and x-ray of the abdomen were grossly normal. On the third day of admission, the patient complained of bleeding per rectum along with pain abdomen. On examination, the abdomen was distended but no tenderness or mass was palpable. Contrast-enhanced CT (CECT) abdomen showed pneumoperitoneum, small bowel distended with multiple air-fluid levels, the largest being 5.1 cm, smooth narrowing of distal ileum stricture with moderate ascites. Urgent explorative laparotomy was planned, which was done under general anaesthesia with endotracheal intubation. During laparotomy, a single 1x1 cm perforation was found near the distal end of the jejunum on the anti-mesenteric border  are avoided in patients with intestinal obstruction because paralyzing the worms may lead to difficulty during surgery. In such cases, albendazole and mebendazole are preferred .Ascaris as seen in this patient.In this case, blunt trauma along with an impending perforation led to jejunal perforation and symptoms associated with it. Perforation was repaired primarily. Therefore, this rare case has highlighted the chances of blunt trauma leading to perforation of the small intestine of an impending perforation by


Multicystic biliary hamartoma (MCBH) is a very rare hepatic benign neoplasm that manifests as a localized cystic-solid mass. Only 17 cases have been described in the literature to date. MCBH diagnosis is currently dependent on imaging and pathology following surgical resection and no precise standards are in place.This case study involves a middle-aged male patient with a history of drinking but no other liver diseases. A routine ultrasound examination showed a 6.0 × 5.5 cm inhomogeneous echo mass in the right lobe of the liver. The patient experienced no discomfort or other symptoms, and blood tests were normal. Imaging revealed a localized cystic-solid neoplasm in segment 6 of the liver that did not have the features of a malignant tumor. Surgical resection was performed. Based on imaging, macroscopic examination, and histological results, a final diagnosis of MCBH was made.The imaging and pathological features of MCBH were summarized based on the published case reports to date. As a non-invasive examination, the imaging features will aid in the diagnosis of MCBH. Furthermore, these features, along with tumor size and patient symptoms, will facilitate clinicians in selecting surgical resection or follow-up for individual patients. Patients with hepatic neoplasm are frequently encountered in our clinic. Common causes include hepatocellular carcinoma, cholangiocarcinoma, secondary malignant liver tumors, hemangioma, and abscesses. Diagnosis is dependent on whether there is chronic liver disease, a history of malignant tumors, positive tumor markers, particular imaging features, and pathological manifestations. Some rare hepatic cystic lesions, including mesenchymal hamartoma (HMH), Von Meyenburg's complex (VMC), Caroli's disease, Biliary cystadenoma, ciliated hepatic foregut cyst (CHFC), intraductal papillary neoplasm of the bile duct (IPNB), are also encountered.Multicystic biliary hamartoma (MCBH) is a very rare hepatic localized cystic–solid neoplasm. MCBH diagnosis is still based on the imaging and pathological characteristics of surgically resected specimens due to a lack of characteristic diagnostic criteria in imaging, effective serological markers, or genetic detection. This case study in a 44-year-old male patient describes the eighteenth reported case of MCBH. Since this was considered a focal benign neoplasm, surgical resection was performed. A diagnosis of MCBH was made using a combination of imaging, macroscopic examination, and histological results.In 2010, Ryu Y et al. —were within the normal range. Serological tests for hepatitis B and hepatitis C were negative. Autoantibodies related to autoimmune liver disease, thyroid function, and ceruloplasmin were within the normal range. Physical examination showed no positive disease indicators.The patient then underwent an imaging examination. A second ultrasound revealed multiple small irregularly shaped hypoechoic masses with slightly hyperechoic septae in segment 6 of the liver (S6), and a total size of approximately 6.0 × 5.5 cm. Contrast-enhanced ultrasound (CEUS) showed a cystic-solid lesion with honeycomb-like enhancement in the arterial phase, in which multiple disordered unreinforced tubular columnar areas were seen. No obvious papillary structure was found. The enhanced region was slowly cleared in the portal and delayed phases. Abdominal contrast-enhanced computed tomography (CT) scans  showed aSince MCBH is a localized cystic-solid lesion, it can be difficult to diagnose by needle biopsy due to limited sampling of the lesion and heterogeneous distribution of the tumor components. After communicating with the patient and his relatives, surgical resection was performed. This was an open operation. The lesion could not be observed in the liver surface. The intraoperative ultrasonic testing was performed and the lesion was located in the right posterior segment VI of liver. Anatomical resection of segment VI was performed and the resection margin was more than 1 cm to the lesion. No enlarged lymph nodes were found during the operation. The residual liver had no tumors and showed healthy texture by intraoperative ultrasound. The operation was successful, lasting about 2 h, and the intraoperative bleeding was 100 ml.The surgical specimen revealed an approximately 6.0 × 5.5 cm nodular mass. A cystic-solid lesion with a honeycomb appearance and gray-white, medium texture, was seen in a section of the resected specimen. The lesion was composed of diffuse, cystically dilated ductal structures that were approximately 0.1–1.5 cm in diameter and surrounded by fibrous tissue. The lesion was filled with clear, colorless liquid surrounded by normal liver tissue.Low-power microscopy displayed a relative clearance boundary in the lesion area that consisted of ductal structures, periductal glands, fibrous connective tissues, and blood vessels. Ductal structures were cystically dilated and irregularly angulated. Bile-stained materials were observed in some ducts and the peripheral bile ducts were not dilated. High-power microscopy showed that the ductal epithelium was composed of a monolayered columnar and cuboidal epithelium that was morphologically identical to biliary epithelium. Fibrous connective tissue around the ducts contained only mild lymphocytic infiltration. Normal hepatocytes were observed between the cystic ducts. There were no smooth muscle elements or ovarian-like stroma, and there were no atypical cells or papillary growth of the epithelial cells. Synchronous biliary hamartomas, nodules, steatosis, or significant fibrosis were not observed in the non-lesion liver tissue. Immunohistochemistry showed CK7 and CK19 positivity in the dilated duct epithelium and CD34 positivity in the vessels. Ki-67 antigen staining revealed the proliferative activity of individual cells .Based on the clinical manifestations, imaging and histological results, the final diagnosis was confirmed as MCBH. The patient recovered well after the operation and was discharged from the hospital. At 6 mo postoperatively, the patient was still alive.MCBH is a very rare hepatic benign neoplasm. It was first reported in 2005  proposedHMH is a large, well-circumscribed, multiloculated cystic mass  that canVMC is characterized by discretely distributed, well-defined, cystically dilated bile ducts. Contrary to the relatively large size of nodules in MCBH, the nodules in VMC are small (<1.5 cm), usually between 0.2 and 0.5 cm in diameter. No enhancement is seen using enhanced CT/MRI . The curThe typical imaging manifestation of Caroli's disease includes enlarged intrahepatic bile ducts that communicate with the bile duct system , and accBiliary cystadenoma is rarely encountered in males. The condition manifests as multiple septa in the large cyst which are divided into multiple small cysts of different sizes, usually with “ovarian-like” stroma. Imaging of the patient in the current case study revealed multiple tubular shadows twisted into a honeycomb-like lesion with solid components in the middle. There were clear differences in the imaging manifestations of these two diseases. In addition to the lack of “ovarian-like” stroma, the current case did not support biliary cystadenoma. CHFC has a unilocular cystic appearance and the presence of a four-layered cyst wall . A ciliaIPNB is characterized by marked dilation or cystic lesions of the bile ducts with papillary structures  which arAll published literature on MCBH were collected. Only 17 cases have been recorded in the literature to date, with the one described here being the eighteenth case . MRI of MCBH is a very rare hepatic benign neoplasm that is associated with a localized cystic-solid mass. The incidence and natural history of this disease remain unknown. In the absence of characteristic diagnostic imaging criteria, effective serological markers, or genetic detection, diagnosis is dependent on imaging combined with histology after surgical resection. In this case study, we summarize the imaging and histological features of this disease. Importantly, we focus on those imaging characteristics that aid the diagnosis of MCBH using non-invasive methods. Imaging results combined with neoplasm size and patient symptoms will facilitate clinicians in selecting surgery or follow-up for individual patients, thereby preventing the need to rely on simple surgical resection and consequently reducing pain and economic burden for patients.


Atrophoderma of Pasini and Pierini (APP) is a rare cutaneous condition of unknown etiology, predominantly affecting young women. It is a dermatologic disorder characterized by hyperpigmented depressed patches of dermal atrophy. Patients usually present with single or multiple asymptomatic, violaceous, and sharply demarcated areas of depressed plaques; of note, unlike morphea, there is no surrounding erythema or induration. In this report, we discuss a case of a 43-year-old South Asian male patient who presented with an eight-month history of multiple asymptomatic, and depressed bluish-brown plaques that had appeared over his body. The clinicopathological correlation was consistent with a diagnosis of idiopathic atrophoderma of Pasini and Pierini (IAPP). We also engage in a review of the literature on IAPP. Atrophoderma of Pasini and Pierini (APP) is an extremely uncommon dermatologic condition, with only around 200 cases reported in the medical literature so far. Idiopathic atrophoderma was first reported by Pasini in 1923, where he described the findings related to this condition as progressive atrophic dermal lesions over the trunk. Later, in 1936, Pierini and Vivoli conducted further studies on this cutaneous condition, defining and describing the lesions in more detail, as well as shedding further light on the potential link between this condition and morphea [This dermal manifestation is usually benign in nature, presenting as sharply demarcated atrophic hyperpigmented lesions, violaceous brown in color, multiple, and varying in size with a bilateral symmetrical distribution. These lesions may also present as hypopigmented lesions or skin-colored lesions in people of color . These aA 43-year-old South Asian male, with no known past medical history, presented with an eight-month history of asymptomatic, hyperpigmented patches, characterized by discrete central atrophy. The lesions were violaceous in color, and ovoid in shape, appearing gradually over the dorsal aspect of hands bilaterally, and over the medial aspect of both thighs. Small multiple unilateral lesions were also noted over the lateral aspect of the right lower limb. There was no history of any recent or distant trauma, tick bites, new medication use, or any recent fungal infections reported by the patient. The patient reported that these lesions had shown the same form, texture, and color since their initial appearance, with slow and nominal growth in size noted over the course of the past eight months. Both his prior medical and familial history was deemed unremarkable. With the exception of these dermal lesions, the patient’s general health and overall physical condition were exceptional.On dermatological examination, there were two large hyperpigmented violaceous patches with atrophic changes noted on the bilateral dorsum of hands, measuring 3 x 4 cm and 4 x 6 cm in size Figures , 2. SmalIn order to differentiate between the various differential diagnoses, two punch biopsies were obtained, with a clean border of adjacent normal skin for further microscopic examinations and correlation Figure . One bioAfter an extensive study of the clinicopathological evidence for this case, the findings were deemed consistent with the clinical impression of APP. The final diagnosis was explained to the patient and he was reassured regarding the benign course of this condition. It was also explained to him that currently there are no definitive treatments for this disorder and that the areas of depressed skin tend to be persistent, but the hyperpigmentation could be alleviated with pigment-reducing lasers, and he was advised to do so in a laser clinic.IAPP is an extremely rare skin disorder. Fortunately, it is a cutaneous-limited disease with no known systemic implications. This dermal disorder has an insidious onset, and it is most commonly seen in the second and third decades of life, with a higher prevalence in women than men by a ratio of 6:1 in adults . NeverthThe exact etiology of this cutaneous atrophy is still unknown. The hypothesis of whether atrophoderma is a solitary entity or an atrophic variant of morphea is still being debated. APP is thought to share a pathogenetic pathway with classic morphea . Indeed,In some cases, genetic influences have also been observed to play a role in triggering APP. Studies have shown that families with a predisposition to C2 deficiency (C2D) have a higher likelihood of developing IAPP; moreover, there have been other studies that showed clustering among some families . Having Borrelia burgdorferi (B. burgdorferi) spirochete is a trigger for this condition in some cases [In another study , it has me cases ,15.B. burgdorferi, and patients did not develop any new lesions during the follow-up periods ranging from three months to eight years [As for treatment options, there are no definitive treatments for IAPP yet. Some therapies that have been studied in the literature include hydroxychloroquine 400 mg daily for a year. Some studies suggest that it may be an effective therapeutic option for chronic refractory IAPP . Hydroxyht years ,15. It hht years . All theGiven the lack of reliably effective treatment for IAPP, a conservative approach to management is preferred. In recent studies, the use of cosmetic camouflage makeup and Q-switched Alexandrite lasers have been studied in small lightly hyperpigmented lesions of IAPP to improve its cosmetic appearance and the quality of life for patients ,19. In aIAPP is an uncommon cutaneous condition characterized by hyperpigmented atrophic patches, which have no definitive pathogenesis or etiology. They are brownish-violaceous lesions that appear and increase in size and number gradually and slowly over the course of many years. The relationship between morphea and IAPP is controversial and is still undergoing further study. There are no definitive treatments for this condition, but further studies are underway and appear to be promising. Emphasizing the benign nature of the disease to the patient is the most important and often the only step in the management of this condition.