Desmoid-type fibromatosis is a rare disease that can result in hydronephrosis. Hydronephrosis associated with desmoid-type fibromatosis often requires surgery or ureteral stent insertion. Although radiation therapy is recommended for inoperable cases of desmoid-type fibromatosis, there has been no report of treatment for hydronephrosis associated with desmoid-type fibromatosis by radiation therapy alone. We herein report a case of successful treatment for inoperable recurrence of desmoid-type fibromatosis with unilateral hydronephrosis by radiation therapy alone.A 43-year-old Japanese female underwent resection of desmoid-type fibromatosis in the right inguinal region and combined resection of the right external iliac vein 5 years before. Other treatment was not performed because of her pregnancy. Four years after surgery, desmoid-type fibromatosis recurred in the right pelvic wall. Cyclooxygenase-2 selective inhibitor treatment was given for 1 year, but her desmoid-type fibromatosis enlarged to more than 10 cm, and she had swelling of her right leg and hydronephrosis of her right kidney. The patient received 50.4 Gy in 28 fractions of prophylactic irradiation using 10 MV X-ray and 9 Gy in five fractions of a sequential boost for the recurrent desmoid-type fibromatosis. Although there was temporary tumor progression at 1 month after radiation therapy, slow regression of the tumor was seen. At 5 years after radiation therapy, there was no disease progression or severe complications.We experienced successful treatment for an inoperable case of desmoid-type fibromatosis with hydronephrosis. Moderate-dose radiation therapy alone is an effective and feasible approach for the management of hydronephrosis associated with desmoid-type fibromatosis. Desmoid-type fibromatosis (DTF) is a rare disease that occurs in 2–4 people per million people annually , 2. AbdoWide resection is required for treating DTF in some cases, but recurrence is frequent . RadiatiA 38-year-old Japanese female in the 15th week of pregnancy presented with a dull pain in her right inguinal region. She had no medical history and no family history of familial adenomatous polyposis. Magnetic resonance imaging (MRI) revealed a tumor around the external iliac artery to the femoral artery just below the right abdominal wall Fig. . RT afteAt 3 years after surgery, marginal recurrence of DTF in the right pelvic wall was diagnosed by MRI. Cyclooxygenase-2 selective inhibitor treatment was given for 1 year. However, MRI showed enlargement of the recurrent tumor measuring 6.8 × 10.0 cm in size Fig. . Her labDefinitive RT was performed using 10 MV X-ray. The patient received 50.4 Gy in 28 fractions of prophylactic irradiation for the right inguinal region of the postoperative lesion with a margin of 5 cm added to macroscopic tumors. After initial irradiation of 50.4 Gy, the patient received 9 Gy in five fractions of a sequential boost for the right pelvic wall tumor to a total dose of 59.4 Gy (Fig. At 1 month after RT, there was temporary tumor progression and the tumor was 7.9 × 10.5 cm in size Fig. . Howeveret al. reported that 5-year local recurrence-free survival rate after surgery for abdominal wall DTF was 91% [et al. also reported that pelvic radiation therapy did not induce any deterioration in renal function or degree of hydronephrosis [Abdominal wall DTF has been shown to have a relatively good outcome. Crago  was 91% . Accordi was 91% . Treatme was 91% . In our  was 91% , 11. Theephrosis .We were able to treat DTF with hydronephrosis by radiation therapy without surgical treatment or ureteral stent insertion, which the patient rejected. Our results indicated that radiation therapy without stenting for hydronephrosis in a patient with normal renal function is one of the options for treatment of DTF. A previous study showed that medication therapy alone for DTF with hydronephrosis was successful . As far In our case of abdominal wall DTF, one of the causes of recurrence was considered to be that RT could not be performed after the initial surgical treatment despite the presence of a positive margin because the patient selected to continue her pregnancy. The clinical behavior of DTF in females who are pregnant is not clear. Marco Fiore reported that DTF progression during or after pregnancy can usually be safely managed by watchful waiting alone . AnotherAdditionally, conservative management has recently been recommended. Retrospective studies have shown that patients can respond to drug therapy and that spontaneous regression can occur after recurrence , 17, butThere have been few reports with detailed description of the method of RT for progressive recurrent DTF. Several studies have indicated that 50 Gy is sufficient for postoperative patients , 20.TherWe experienced successful treatment for a case of progressive recurrent DTF with unilateral hydronephrosis that could not be sufficiently treated initially because the patient was pregnant. Despite the difficulty of continuing conservative treatment after surgery due to tumor progression and onset of hydronephrosis, prevention of recurrence and successful local control without severe complications were achieved in our case for 5 years after RT alone with a moderate dose.


Composite hemangioendothelioma is an extraordinarily rare form of vascular neoplasm which develops predominantly in the skins and soft tissues of the adults. Neuroendocrine marker expression in composite hemangioendothelioma is considered as specifically relevant to the more aggressive behavior.The patient was a 71-year-old man complaining continuous back pain. Computed tomography (CT) showed that 10 cm of contrast-enhanced soft tissue mass was occurring on the right posterior chest wall and developing adjacent to the spinal canal. Via the laminectomy, the tumor end was identified and separated from the dura mater. Then, via the posterolateral thoracotomy, the en bloc resection was achieved by separating the tumor from the diaphragm and vertebras. Histologic examination showed a complex combination of epithelioid and retiform hemangioendothelioma areas which were positive for anti-synaptophysin staining. At 12-month follow-up, there were no signs of tumor recurrence on CT, and the patient had no symptom.We achieved the complete resection of a huge thoracic neuroendocrine composite hemangioendothelioma developing adjacent to the spinal canal. The combination of the posterior lumbar laminectomy and the following posterior thoracotomy is a viable approach to radically resect a thoracic neuroendocrine composite hemangioendothelioma involving chest wall.The online version contains supplementary material available at 10.1186/s40792-021-01331-3. Composite hemangioendothelioma is an extraordinarily rare type of endothelial tumor developing chiefly in the skin and the superficial soft tissue of the adults, showing the distinctive combination of retiform and epithelioid features . Only foThe patient was a 71-year-old man complaining continuous pain on the right back. Imaging studies showed that 10 cm of contrast-enhanced soft tissue mass on the right posterior chest wall was involving the 11th and 12th ribs, and developing to the spinal canal via the Th11–Th12 intervertebral foramen (Fig. ® sheet and the muscle flap coverings which the latissimus dorsi muscle and the longissimus dorsi muscle had been partially spared for at the beginning of the surgery. Microscopically, the tumor was characterized as an admixture of epithelioid and retiform areas (Fig. The patient was anesthetized on prone position. Skin incision was determined to perform the posterior spinal fusion and prepare the skin and muscle flap for covering the chest wall defect after the tumor resection, i.e., longitudinal midline skin incision over the spine to expose the right side of the thoracic spine and the second incision along the rib and over the tumor. After splitting the spinalis thoracis muscle, the Th11–Th12 transverse processes and the Th11–L1 spinous processes were cut off at the levels of the base and the lamina was partially removed to approach the Th11 spinal canal. The Th11 thoracic nerve was dissected at the level of the root so that the proximal end of the tumor was clearly identified as developing to the exact proximity of the dura mater (Fig. In the 5th edition of the World Health Organization classification of Tumors of Soft Tissue and Bone, composite hemangioendothelioma is defined as a locally aggressive, rarely metastasizing vascular neoplasm, containing an admixture of histologically distinct components . To our In terms of surgical strategy, in the current case, we were able to successfully separate the tumor from the dura matter via the laminectomy, and determine the resection line on the anterolateral part of the vertebra, diaphragm, and chest wall via the following posterolateral thoracotomy. The current approach can be also a viable option for achieving the radical resection of not only thoracic composite hemangioendothelioma, but any other types of soft tissue neoplasms which occur deep in the posterior chest wall and develop adjacent to the vertebra and the spinal canal. An adjuvant radiotherapy is also to be considered against the minimal or positive margins, even though the significance in reducing locoregional recurrence of such rare vascular tumors is unclear.Via the posterior lumbar laminectomy and the following posterior thoracotomy, we achieved the complete resection of a huge thoracic neuroendocrine composite hemangioendothelioma developing adjacent to the spinal canal.Additional file 1: Video S1. Microscopic procedures.Additional file 2: Video S2. Macroscopic procedures.


Spontaneous ureteric rupture is uncommon and has a wide range of presentations. Accurate diagnosis and timely treatment is necessary to avoid potential serious complications.We present the case of a 55-year-old female who presented with severe right lower abdominal pain with rebound tenderness, vomiting, and a single episode of hematuria. A computed tomography with intravenous contrast of the abdomen and pelvis showed a 0.3-centimeter right upper ureteric calculus, with hydronephrosis and ureteric rupture. In view of the scan findings, a diagnosis of spontaneous ureteric rupture secondary to urolithiasis was made. The patient underwent a percutaneous nephrostomy and ureteric stenting.Spontaneous rupture of the ureter is an uncommon diagnosis for which clinical and laboratory signs may not always be reliably present. A high index of suspicion is required for diagnosis, which is usually confirmed on advanced imaging. It may occur in serious complications of urinoma and abscess formation. As such, accurate diagnosis and timely treatment is crucial. Spontaneous ureteric rupture, or extravasation of the urine from the ureter in the absence of trauma or iatrogenic ureteric manipulation, is a rare condition. The literature, especially in emergency medicine, is fairly sparse. It may mimic many other causes of acute abdomen and itself requires prompt treatment. Thus, it is a diagnosis for which emergency physicians (EP) should have a high index of suspicion. We report a case of spontaneous ureteric rupture in the presence of a small, obstructing ureteric calculus. Informed consent was obtained from the patient for publication of this case report.A 55-year-old female presented to the emergency department (ED) with acute onset right lower abdominal pain a few hours prior. The pain was non-radiating, constant, and gradually increasing in intensity. She reported one episode of gross hematuria at home with the onset of the pain but the hematuria resolved subsequently. The patient had vomiting with the pain but did not have any other gastrointestinal symptoms. She did not have any fever or chills.The patient had no past medical problems but a previous surgical history of a total hysterectomy and bilateral salpingo-oopherectomy performed four years prior and a laparoscopic procedure converted to open deroofing of a hepatic cyst three years prior. During a computed tomography (CT) of the abdomen and pelvis ordered by her hepatobiliary surgeon three years prior, a small, right kidney mid-pole stone was noted. However, as it was not causing any symptoms and was relatively small in size, it was conservatively managed.On physical examination in the ED, she was afebrile and hemodynamically stable. Examination of the abdomen revealed tenderness over the right flank and iliac fossa with rebound tenderness but no guarding.3 per microliter (μL) (reference range 4.5 – 11.0×103/μL) with mild neutrophilia. Serum electrolyte and creatinine levels were within normal range. Urinalysis showed microscopic hematuria with no casts. Point-of-care ultrasound did not reveal any intraperitoneal free fluid or the presence of an abdominal aortic aneurysm, and no findings suggestive of cholecystitis.Initial blood investigations showed a total white cell count of 11×10The differential diagnosis in this patient was broad and included appendicitis, renal colic, diverticulitis, etc. However, the presence of rebound tenderness and persistent pain prompted the decision for the patient to undergo a CT of the abdomen and pelvis in the ED. Meanwhile, intravenous ceftriaxone was administered in view of the presence of peritonitis. The contrast-enhanced CT showed no evidence of acute appendicitis but found a 0.3-centimeter right upper ureteric calculus, with upstream hydronephrosis and ureteric rupture –3.What do we already know about this clinical entity?Spontaneous ureteric rupture is a rare urological emergency that has only been described in case reports and series, mainly in surgical and urological literature.What makes this presentation of disease reportable?Neither symptoms nor simple radiological or lab tests are reliable for diagnosis. Only computed tomography (CT) done in view of peritonitis revealed the diagnosis.What is the major learning point?Ureteric rupture requires prompt treatment. Providers need to be aware of this condition and when a CT should be done to minimize diagnostic delay.How might this improve emergency medicine practice?By increasing awareness about this rare disease, emergency physicians may have a higher index of suspicion and be better able to diagnose and manage this emergency.Given the imaging findings, the patient was admitted to the urology service. She underwent urgent right percutaneous nephrostomy on the day of admission. Three days later, anterograde ureteric stenting was performed with a confirmatory nephrostogram the following day. She was subsequently discharged with a follow-up CT of the kidneys, ureters, and bladder in three weeks and urology clinic follow-up after the scan.Spontaneous ureteric rupture is a rare urological emergency, which to our knowledge, has only been described in case reports and case series. The most common cause is lithiasis; other possible etiologies include metastatic invasion of the ureter, urinary retention from neurogenic bladder, connective tissue diseases, retroperitoneal fibrosis,4The clinical presentation of this condition is more relevant to the ED environment, but the existing literature provides little information on this topic. Sudden onset of abdominal or flank pain seems to be common.Point-of-care ultrasound detection of urolithiasis and hydronephrosis has limitations,Complications that may arise from this condition, which EPs should be cognizant of, include formation of urinomas, or perinephric or retroperitoneal abscesses. Urosepsis and its attendant risks is also possible following ureteric or perinephric rupture. Thus, prompt treatment and admission of this diagnosis, once made, is necessary.Spontaneous ureteric rupture is an uncommon urological emergency. Not only are signs and symptoms varied and at times non-specific, laboratory and plain radiographic studies also provide little definitive evidence for diagnosis. Diagnosis is usually only made if emergency physicians order advanced imaging. To avoid potential complications, EPs need to be aware and have a high index of suspicion of this condition to make an accurate and timely diagnosis of ureteric rupture.


Angiomyolipoma is the most frequent neoplasm of the kidney. It may have a wide range of clinical manifestations, but it is usually detected incidentally on cross-sectional images. Rupture and hemorrhage of angiomyolipoma is an important concern for large lesions. We present the case of a 42-year-old female, with no history of urinary stones, who presented with a sudden-onset left flank pain for eight hours. Upon examination, she was tachycardic and hypotensive. Abdominal examination revealed a left-sided flank mass. Abdominal computed tomography (CT) scan with intravenous contrast demonstrated a heterogeneous mass lesion in the left kidney with mixed attenuation and had a macroscopic fat density that was surrounded by a large hematoma. Subsequently, transcatheter renal artery embolization was performed with no complications. The procedure was able to control the active bleeding. Then, a total nephrectomy was performed, and the hematoma was evacuated during laparotomy. Spontaneous nontraumatic renal hemorrhage is a very rare condition. Clinicians should keep a high index of suspicion for this condition when they encounter a patient with the clinical triad of flank pain, flank mass, and hypotension. The case demonstrated the role of endovascular embolization of angiomyolipoma to control life-threatening hemorrhage. Angiomyolipoma is the most common benign solid tumor of the kidney. It has female predilection and is often identified in adulthood . This tuWe present the case of a 42-year-old female who was brought to the emergency department by ambulance because she developed a sudden-onset left flank pain for eight hours before presentation. The pain was non-radiating, and she described it as stabbing in nature. It was associated with nausea and dizziness. She scored the pain as 8 out of 10 on the severity scale. She reported that the pain was not related to urination, posture, or meals. The patient used over-the-counter analgesics for the pain with no clinical improvement. The medical history of the patient was remarkable for beta-thalassemia minor and allergic rhinitis. She underwent a tonsillectomy at the age of 14 years. She was not on any medications. The patient reported having an allergy to penicillin. She was a nonsmoker and never consumed alcohol. The family history was significant for glucose-6-phosphate dehydrogenase deficiency.Upon examination, the patient was in severe pain and appeared pale and sweaty. She did not have any signs of respiratory distress. Her vital signs included tachycardia (120 bpm), hypotension (88/50 mmHg), normal respiratory rate (14 bpm), and normal temperature (36.8°C). Abdominal examination revealed a left-sided mass with generalized tenderness. Considering the hypovolemic status of the patient, aggressive intravenous hydration was initiated.The initial laboratory investigations revealed a decreased hemoglobin level 10.9 g/dL) with normal leukocytes  and platelets . She also had a mild elevation in the urea and creatinine levels. Urinalysis findings were normal. The liver function test and inflammatory markers were within the reference range  scan with intravenous contrast. The scan demonstrated a heterogeneous mass lesion in the left kidney with mixed attenuation and had macroscopic fat density. The kidney was surrounded by a hematoma that exerted mass effect upon the kidney. Such findings conferred the diagnosis of ruptured renal angiomyolipoma with active hemorrhage Figure .The case was discussed with the interventional radiology department, and the plan for embolization was made. Transcatheter renal artery angiography demonstrated abnormal vasculature. Particulate embolic agents were administered selectively to occlude the abnormal vessels Figure . The embSubsequently, a laparotomy operation was performed to resect the tumor. A total nephrectomy was performed, and the hematoma was evacuated. Histopathological examination of the specimen confirmed the diagnosis of angiomyolipoma. The patient was discharged on the fifth postoperative day. After three months of follow-up, the patient remained asymptomatic with normal renal function tests.We presented the case of a middle-aged woman with angiomyolipoma that presented with spontaneous nontraumatic rupture for which she underwent endovascular embolization and surgical resection. The spontaneous nontraumatic renal hemorrhage, also known as Wunderlich syndrome, is a rare condition with bleeding in the subcapsular and perinephric spaces . WunderlWunderlich syndrome presents with the classic triad of acute flank pain, flank mass, and signs of hypovolemic shock. As in the present case, the most common etiology of Wunderlich syndrome is a neoplastic lesion, with angiomyolipoma being the commonest . In contImaging studies are crucial to making the diagnosis of Wunderlich syndrome. A computed tomography scan demonstrates the presence of hemorrhage surrounding the kidney along with the primary lesion if present. Notably, the computed tomography scan can make the diagnosis of angiomyolipoma, the most common neoplastic cause of Wunderlich syndrome, with high accuracy and confidence . The manSpontaneous nontraumatic renal hemorrhage is a very rare condition. Clinicians should keep a high index of suspicion for this condition when they encounter a patient with the clinical triad of flank pain, flank mass, and hypotension. A computed tomography scan can make the diagnosis promptly. The case highlights the value of endovascular embolization of angiomyolipoma to control life-threatening bleeding.


Anal canal duplication (ACD) is a very rare condition, diagnosed and treated mostly in childhood. Less than 90 cases have been reported in the literature so far. We are presenting a case of a young woman who underwent surgical excision of the duplication when she was 27 years old. The patient was unaware of her condition and was referred from a gynaecological office to the surgical department with a history of perianal discomfort and mucus discharge. Local examination showed an external orifice posterior to the anal opening, on the median line, which had the macroscopic appearance of a secondary anal orifice. The opening was about 0.5 cm in diameter. Exploration of the tract revealed a length of about 4 cm. MRI described the aforementioned tract, parallel to the anal canal, with no other anomalies mentioned. Under spinal anesthesia, with the patient in jackknife position, the accessory anal canal was surgically excised. The pathology report showed the presence of smooth muscle fibers and typical anal glands in the specimen. After a five-year follow-up, the patient showed no recurrence or any other related local symptoms. Absence of perianal abscess from the patient history, along with the macroscopic aspect of the opening similar to a secondary anal orifice on the midline, should raise the suspicion of ACD. Due to the lack of bothersome symptomatology, the patient did not seek any special investigations for her condition until she was in her late twenties. ACD is a very rare condition in adults that might pass unnoticed, but a midline opening posterior to the anus should always raise the suspicion of a secondary anal canal. Surgery is the only cure for this condition with good results after a proper pre-operative workout to reveal others simultaneous malformations. Anal canal duplications (ACD) are the rarest malformations of the digestive tract, seen and treated mostly in childhood. They are described as a consequence of duplication of the dorsal cloaca in an early developmental stage or a consequence of recanalization of a cloacal membrane excess in late embryonic life ,2. Hoda A 27-year-old woman was seen in the outpatient clinic of the surgical department, with a second anal opening situated along the midline, posterior to the normal anal canal opening . The patDuring the examination, an additional opening was revealed posterior to the anal canal opening, situated on the midline. There was no pain on palpation of the perianal region or any other findings on digital rectal examination of the normal anal canal. The ACD orifice had a smaller diameter than the evaluator’s finger; therefore, only instrumental exploration was performed in order to determine the length of the tract—around 4 cm, and the presence of any intraluminal secretions—none were discovered. Anoscopy showed a normal anal canal with first degree hemorrhoids, and no communication between the two tracts was found when hydrogen peroxide and methylene blue were injected in the ACD.Patient consent was obtained in order to take pictures of the lesion—the strange, perineal second orifice, which looked like a mini-anus, with a structure mimicking the dentate line—and to have it evaluated by our clinic’s panel of experts. After our department meeting, the suspicion of an ACD was raised and an MRI was performed for further evaluation. MRI described a tract with a length of 4 cm situated posterior to the anal canal, with no communication between the two structures.Possible differential diagnoses were perianal fistula—usually developing on the sides and being accompanied by abscess, or other malformations of the anorectal region, such as dermoid cyst, presacral teratoma, lumbosacral meningocele, spina bifida, and others. MRI ruled out these differential diagnoses. The patient was admitted for surgical intervention under regional anesthesia. She was placed in a jackknife position with the legs abducted at 60 degrees. The ACD was removed using electrocautery. Special care was taken to prevent any anal sphincter damage. At the proximal end of the ACD, some small cysts were discovered. The whole tract was excised, and the skin was close after the procedure with absorbable sutures. Postoperative recovery was uneventful, and the patient was discharged on the third post-operative day. The histologic examination of the specimen revealed the presence of stratified squamous epithelium  and tranThe outcome was favorable, and the patient was completely healed with no recurrence after a five-year follow-up.ACD is a very rare malformation, in adults it is even rarer because most of the lesions are operated in childhood and infancy. Being so rare, clinicians have difficulties in recognizing ACD, usually mistakenly diagnosing it as a perianal fistula or perianal abscess. Ochiai et al. defined ACD, based upon three features: the presence of squamous epithelium in the caudal end, transitional epithelium in the cranial end, and smooth-muscle cells in the wall of the lesion . The parThese complementary features should be evaluated in the context of the anatomical presence of an additional anus-like opening, posterior to the normal anus, usually situated on the midline. The only case in the literature where an ACD opening was described outside of midline at five o’clock was presented by Rezvan et al. . Some auUsually, the parents or the doctors discover the presence of an ACD during early stages of life, but sometimes because of the lack of symptoms, the lesion is noticed only in adulthood, when complications occur. Apart from the presence of an additional opening, ACD often becomes symptomatic, with local infection and discharge as the most common signs . ImagingFistulography can also be used to exclude the presence of fistulous tracts and/or communication with the anal or rectal lumens. Anoscopy can be used in the fashion described in the work-up of our case, with injection of hydrogen peroxide and methylene blue in order to rule out a communication with the anal canal. Echoendoscopy can be a useful tool, allowing the identification of the anal sphincter, the ACD tract, and its dimensions . Surgery is the only curative option. Discussions about the therapeutic indications for asymptomatic ACD have been ongoing and are based on a report from Duke and Galvin,  releasedACD is a very rare condition in adults that might pass unnoticed, but a midline opening posterior to the anus should always raise the suspicion of a secondary anal canal. Surgery is the only cure for this condition with good results after a proper preoperative workout to reveal other simultaneous malformations. Definitive diagnostic has to be supported by the pathology report, confirming the presence of squamous and transition zone-type epithelium, smooth muscle fibers, and sometimes of anal glands. This could explain the mucus and fluid discharge. The lack of perianal abscess history in a young patient should prompt this differential diagnostic.


Lumbosacral Tarlov cysts (TCs) have rarely been seen to rupture. Here, we report an unusual presentation of a ruptured TC with intraspinal hemorrhage mimicking carcinomatosis. Pathological diagnosis was obtained using percutaneous biopsy. A conservative approach was utilized and an excellent outcome was achieved. Thus, in cases such as this, a ruptured hemorrhagic TC should be on the differential diagnosis to drive appropriate clinical management decisions. Tarlov cysts (TCs), also known as perineural cysts, are spinal intradural cerebrospinal fluid (CSF)-filled sacs that form within the nerve root sleeves. These lesions are often found in the sacral region of the spinal column. Though predominantly asymptomatic, TCs may compress spinal nerve roots and clinically present with low back or leg pain and radiculopathy ,2. To ouA 71-year-old woman with no significant past medical history presented with acute-onset headache, severe low back pain, and urinary retention. Physical examination was notable for sacrococcygeal tenderness to palpation and pain-limited antigravity motor strength in the legs. Head imaging was unremarkable. Lumbosacral MRI demonstrated a 3 cm heterogeneously enhancing mass-like lesion at the S3-S4 level. Additionally, diffuse cauda equina nerve root and leptomeningeal enhancement were noted Figure .TCs, or perineural cysts, are intradural CSF-filled sacs found within the lumbosacral spine . They arTCs are most commonly asymptomatic and are generally found incidentally during radiographic studies performed for other reasons. Symptomatic TCs have a reported incidence of 1% in the adult population ,12,19. EAcute symptomatic presentation with cyst rupture and hemorrhage as seen in this case is quite rare. Godel et al. reported a case of a 38-year-old woman who developed acute radiculopathy in such a case . Yates eTC rupture resulting in symptomatic intraspinal hemorrhage is rare. The possibility of symptomatic TCs merits a high degree of suspicion, especially if radiographic findings are suggestive of a neoplastic etiology. Conservative management is indicated in such a setting where TC is suspected, including cautious monitoring for clinical deterioration.


Salivary gland tumors are relatively uncommon with most being benign. When diagnosed the most common benign and malignant tumors are pleomorphic adenoma and mucoepidermoid carcinoma (MEC), respectively. However, not uncommonly, it is difficult to differentiate between the histopathological entities, leading to a diagnostic dilemma that can impact a patient’s treatment and prognosis. A 24-year-old woman presented with a three-year history of asymptomatic left-sided facial swelling. She denied any prior history of head and neck radiation. There was no history of alcohol consumption or smoking exposure and there was no personal or family history of head and neck cancers. Additionally, she did not have any known occupational or environmental exposures. Due to the chronicity and painless nature of this facial mass, our patient did not pursue evaluation initially. Subsequently, she experienced an increase in size and pain for a few months exacerbated by swallowing. She had no other symptoms. On physical examination, a 3 x 3 cm left parotid gland mass was noted. There was no associated head or neck lymphadenopathy and compression of the left facial mass did not elicit secretions from the opening of Stensen’s duct. Due to the rapid increase in size, she was sent for CT neck/soft tissue with contrast which confirmed a 3.56 x 2.67 cm solid nodule within the superficial portion of the left parotid gland. This was followed by an MRI orbit/face/neck with and without contrast, for further delineation, which demonstrated a 4 x 3.7 x 3 cm complex heterogeneous mass within the superficial left parotid gland. Thereafter the patient underwent an uncomplicated ultrasound-guided biopsy of the parotid mass. The histopathological appraisal concluded that this was a cellular pleomorphic adenoma, with mucinous and squamous metaplasia with reactive lymph nodes. Due to the new rapid increase in size and intense painful nature of this tumor, nerve-sparing left parotidectomy, fat grafting and reconstruction were completed. Cellular pleomorphic adenomas are benign low-grade neoplasms, typified as biphasic with both epithelial and myoepithelial components. However, they have increased cellularity and focally increased mitotic activity, not advanced enough to qualify as malignant. The presence of mucinous and squamous metaplasia is of diagnostic interest as it makes diagnosis on fine-needle aspiration (FNA) morphologically challenging. These findings are typical of MEC and on FNA can be misleading in the setting of a pleomorphic adenoma. However, on histopathological evaluation of the gross specimen along with immunohistochemical staining the diagnosis is made much easier. A diagnosis of MEC would have potentially required neck dissection and adjuvant therapy with a potential increased risk of morbidity and mortality. This case emphasises the importance of an adequate tissue biopsy in regards to parotid gland tumors to optimise a patient's care plan. Salivary gland tumors are rare, making up less than 5% of all head and neck neoplasms . The majA 24-year-old woman who immigrated from Liberia to the USA approximately one year ago presented our outpatient clinic with a complaint of a left-sided facial swelling for the past three years. She initially noticed this left lower jaw swelling approximately three years ago but left it untreated due to the chronic nature of the swelling and her relatively asymptomatic course. However, subsequently, she noticed an interval increase in the size of the swelling associated with pain. This was aggravated by palpation of the swelling, lying on her left side and swallowing. There was no associated fever, drainage from the swelling, increased salivary production, anorexia, weight loss, dyspnea, dysphagia, hoarseness, ophthalmoplegia, hearing loss, gait disturbance, or known tuberculosis exposure. She did not endorse smoking, alcohol consumption or use illicit drugs. There was no personal or family history of cancers. Additionally, she did not have any known occupational or environmental exposures. This was on the background of having no known medical history of any chronic illness.The patient was well developed with vital signs within normal limits. On physical examination, there was a 3 x 3 cm, mildly tender, non-mobile, round, firm, non-compressible and non-reducible swelling at the angle of mandible abutting the lobule of the left ear Figure . No cervDue to the rapid increase in size and a concern for possible malignancy, the patient was sent for a CT scan of soft tissue of neck with contrast that confirmed a solid nodule in the superficial portion left parotid gland measuring 3.56 x 2.67 cm that appeared to be homogeneous and had a well-defined margin Figure , fat gra  Cellular pleomorphic adenomas are benign low-grade neoplasms, typified as biphasic with both epithelial and myoepithelial components . In thisOn gross evaluation, the majority of these tumors measure between 2 and 4 cm . Facial Imaging modalities such as CT scan and MRI assist in staging the pleomorphic adenoma. MRI is superior to CT Scan as it informs about the depth of involvement, surrounding structures including bone and the neurovascular bundle that can assist in guiding surgery. With respect to other diagnostic modalities, an incisional biopsy is contraindicated as it leads to an increased chance of recurrence . We acknHistopathologically, MEC is classified into low, intermediate and high grade, with low grade (48%) being more common than high grade (38.7%) and intermediate grade 13.3%) being the least common. The degree of cytological atypia, cyst formation and relative numbers of mucous, epidermoid and intermediate cells are the determinants of tumor grade 3.3% bein. Some plSubsequent to the inconclusive FNAC report revealing squamous and mucinous metaplasia and staging imaging not showing regional or distant disease, the patient was scheduled to undergo surgery. Thereafter she underwent a superficial parotidectomy with excision of the left parotid mass and two reactive lymph nodes. The histopathological evaluation of the gross specimen with squamous and mucinous metaplasia was not advanced enough to be classified as malignant. There was the absence of atypia, metastasis, tumor necrosis and the presence of minimal cellular proliferative activity in the context of a slow-growing nature of tumor. It was important to make the most accurate diagnosis as labelling the tumor as MEC would have potentially required neck dissection and adjuvant therapy with potential increased risk of morbidity and mortality. Nerve-sparing enucleoresection and superficial parotidectomy formed the mainstay of surgical treatment. Generally, treatment depends upon tumor size, location and extent of local structure involvement . ExtremeSalivary gland tumors are uncommon. When they do occur, they may present as a diagnostic challenge necessitating a multidisciplinary approach for tissue sampling and eventual appraisal. It is imperative to make the correct diagnosis as this determines management course. Combined clinicopathological assessment is the recommended approach as either in isolation may yield equivocal results. This case highlights the importance of obtaining an adequate tissue biopsy for appropriate histopathological appraisal in order to guide subsequent management of the tumor.


Bladder endometriosis is an infrequent cause of a focal bladder mass and may masquerade as a neoplasm on ultrasound imaging. This diagnostic dilemma can be resolved with a multiparametric MRI, which shows characteristic hemorrhagic signals of this entity. We present an unusual case of bladder endometriosis where the patient complained of lower abdominal pain without urinary symptoms and was found to have a bladder mass on ultrasonography. This mass was further investigated by an MRI of the pelvis, which revealed characteristic features of endometriosis; the diagnosis was confirmed on subsequent laparoscopy. Endometriosis is a commonly encountered chronic benign gynecological condition characterized by the presence of functional endometrial glands and stroma-like lesions outside the uterus. In rare cases, the involvement of the urinary system due to endometriosis has been observed. However, the most common site of the urinary system involvement due to this entity is the posterior wall of the urinary bladder, which is in close proximity with the uterus, and hence vulnerable to direct spread from the uterus . In thisA 31-year-old unmarried female presented with complaints of lower abdominal pain and severe dysmenorrhea. Her pain had gradually increased in intensity over time. Clinically, the patient was vitally stable, and her abdomen was soft and non-tender. A review of her records showed that she had undergone two previous ultrasounds; one had been performed nine years back in 2012, which had shown large multiloculated cystic lesions in both adnexa and cul-de-sac with low-level internal echoes suggestive of bilateral ovarian endometriomas. At least two intramural fibroids had also been identified in the uterine body. Subsequently, she had undergone a laparoscopic cystectomy, confirming endometrioma. She had later undergone laparotomy for myomectomy, adhesiolysis, and bilateral ovarian cystectomy. Her follow-up/second ultrasound had been done in 2016, which had shown new development of multiple uterine fibroids without any ovarian pathology.Based on her current symptoms, she again underwent an ultrasound pelvis after an interval period of five years, which demonstrated a bulky anteverted and anteflexed enlarged uterus with multiple intramural and subserosal fibroids of variable sizes. The urinary bladder base revealed a slightly lobulated well-defined hypoechoic immobile mass without any significant vascularity, measuring 22 x 20 x 36 mm Figure . Both ovThe MRI pelvis showed an enlarged uterus with multiple uterine fibroids and an irregular mixed signal intensity mass along the posterior wall of the urinary bladder. This lesion was showing intravesical extension and was posteriorly in contact with the uterus with indistinct fat planes Figure . On careEndometriosis is a benign condition characterized by the presence of heterotopic endometrial tissue that structurally and functionally behaves as endometrial glands or stroma . It has The patients commonly present with infertility or pain-related symptoms, including cyclic pelvic pain, dysmenorrhea, dyspareunia, dysuria, and dyschezia . BladderVarious theories have been proposed in the literature regarding the cause of endometriosis, and the most preferred one is retrograde menstruation. It has been found that during menstruation, there is spillage of endometrial cells in the anterior cul-de-sac, which is the most dependent portion, just like the posterior cul-de-sac, hence representing a favorable site for deep endometriosis. It is proposed to perform imaging before or during menstruation as the lesions become more perceptible .Laparoscopic evaluation and histological confirmation are the gold standard methods for the diagnosis of endometriosis . ValuablOn ultrasound, endometriotic nodules appear as well-defined, solid, hypoechoic, irregular masses. They may contain low-level echoes or small cystic spaces and often show no blood flow on color Doppler. Anteverted-retroflexed uterus ("question mark sign") is often seen with severe posterior compartment deep infiltrating endometriosis. The "kissing ovaries" sign signifies ovaries that are adherent to one another posterior to the uterus and are frequently seen with bilateral endometriomas . Deep inMRI is a robust imaging technique for the assessment of bladder endometriosis. Whenever there is a high suspicion of cancer, MRI is a reliable modality with its superior tissue characterization, better multiplanar capability, higher contrast resolution, and better delineation of bladder wall layers, in comparison with ultrasonography. The typical appearance of bladder endometriosis on MRI is usually of low signal intensity on T2 weighting with intermediate signal intensity on T1 weighting, and foci of high signal intensity on T1 and T2 weighting . AccordiFew "pearls of wisdom"/recommendations regarding the use of MRI for the diagnosis and characterization of pelvic endometriosis are presented here. (a) T1-weighted fat-suppressed sequences should be included in all-female pelvis MR examinations because this sequence is very helpful in detecting small endometriomas and differentiating them from mature cystic teratomas. Peritoneal endometriotic deposits usually appear bright on T1-weighted images, representing blood products, and hence the best sequence to pick this abnormality is the T1-weighted fat-saturated sequence. (b) It must be taken into account that endometriomas and endometriotic foci can demonstrate restricted diffusion and mimic pelvic malignancies. (c) The ancillary finding of hematosalpinx and endometriomas with "T2 shading" sign  are suggestive of pelvic endometriosis. These ancillary findings were not present in our case to support the diagnosis. (d) Solid deep infiltrating endometriosis, as seen in our case, can involve the uterus, cul-de-sac, pelvic ligaments, anterior rectosigmoid colon, bladder, as well as surgical scars. The best sequence to detect deep infiltrating endometriosis and associated fibrosis is the T2-weighted MR sequence without fat suppression. These lesions demonstrate ill-defined margins and T2 hypointense signals as a result of fibrosis along with foci of T2 hyperintensity representing ectopic endometrial glands. These typical MR signal characteristics can help in establishing the diagnosis ,14.This case report highlights the importance of a collaborative effort involving clinicians and radiologists in patient management. In a nutshell, our report illustrated typical MRI features of bladder endometriosis in a patient with atypical clinical presentation of the disease. Familiarity with imaging techniques and background knowledge of this rare entity are of paramount importance to reach an early diagnosis and avoid unnecessary interventions, thereby reducing morbidity and mortality among patients.


Nevoid hyperkeratosis of nipple and areola (NHNA) has been classified into 3 types: type 1 is associated with an epidermal nevus; type 2 with various dermatoses such as the Darier disease (DD), chronic eczema, and cutaneous T-cell lymphoma; type 3 is of unknown etiology. Despite the multifactorial association, a consistent morphological feature of NHNA is the presence of brown verrucous plaques involving the nipple and areola area, making the clinical distinction difficult [A 25-year-old male with skin type V presented with multiple variably pigmented lesions all over the body since the age of 5. He reported a history of swelling affecting the bilateral nipple-areola complex (NAC) for 1 year. Lesions were foul-smelling and exacerbating during summer. He did not report a similar family history. On cutaneous examination, the bilateral NAC showed erythematous to gray verrucous plaques that hindered the visibility of the nipples . DermoscDermoscopic features of NHNA are sparsely reported. Mazzella et al. reported multiple blue-gray globules and leaf-like areas in the case of type 3 NHNA . In the Here we reported a characteristic dermoscopic pattern found in a case of DD associated NHNA, which can be valuable for future diagnosis and for differentiating it from other NHNA causes.


Background: Cerebellar liponeurocytoma is an extremely rare benign tumor which generally occurs in cerebellum and is almost always solitary. Multifocal cerebellar liponeurocytoma is exceedingly rare, only 8 cases has been reported so far. Herein we present the 9th case of multifocal cerebellar liponeurocytoma in a 70-year-old woman with the complete clinical course and comprehensive imaging findings.Case Presentation: A 70-year-old woman presented with a history of intermittent headache for 5 years. Computed tomography (CT) and magnetic resonance imaging (MRI) of the brain have been performed and suggested a diagnosis of teratoma based on the imaging findings. After the surgical resection of the lesion, histopathological and immunohistochemical analyses revealed neuronal, glial, and lipomatous components and confirmed the diagnosis of multifocal cerebellar liponeurocytoma after surgical resection. During the 2-year follow-up period, the patient showed no signs of recurrence or metastasis.Conclusion: We described the radiological characteristics and clinical course of an exceedingly rare case of multifocal cerebellar liponeurocytoma in the cerebellar vermis and temporal lobe. The clear multifocality makes this case unusual. Cerebellar liponeurocytoma (CLN), which was first described in 1978 by Bechtel et al.  due to rA 70-year-old woman presented with unsteady walk without any obvious inducement in June 2018. She also described a history of occasionally falls, memory loss, hearing and vision loss, nausea, and vomiting for 3 months. In her previous medical history, she had intermittent headache and dizziness for 5 years. Neurological examinations at admission were unremarkable. An elevated level of neuron-specific enolase  was found. Blood pressure, pulse, temperature, routine laboratory tests  and other serum tumor markers were within normal ranges, including carbohydrate antigen 242 , carbohydrate antigen 125 , carbohydrate antigen 153 , carbohydrate antigen 199 , alpha-fetoprotein serum (0.0 ng/ml), carcinoembryonic antigen (0.5 ng/ml), and β-human choriogonadotropin (2.6 U/L).* 3.4 cm) in the temporal lobe with curvilinear calcification (* 2.8 * 3 cm) in the cerebellar vermis with predominantly fatty density and ill-defined margin caused compression of ambient cisterns. The bone window showed the mild compression of perilesional cranial bones (* 4.3 * 4.6 cm) (Computed tomography (CT) and contrast-enhanced magnetic resonance imaging (MRI) were performed in June 2018. CT revealed a large heterogeneous mass   without  4.6 cm) . JudgingDue to the unbearable headache caused by obstructive hydrocephalus, midline suboccipital craniotomy, and near-total excision of the cerebellar mass was performed, and the left temporal lobe lesions was left untreated since it is asymptomatic at time. On intraoperative examination, we observed a grayish cerebellar tumor with firm consistency, and abundant blood supply, that was not well-demarcated from the surrounding tissue. Microscopic examination showed a glioneuronal tumor with dense and diverse tumor cells, accompanied by extensive vascular hyperplasia, and papillary structures along with scattered adipose tissue and lymphatic structures in excised tumor mass . GeneralThe patient was discharged 12 days after the surgery. Postoperative MRI indicated the cerebellar mass was removed near-total . The temThe first case of CLN was reported by Bechtel et al. in 1978 . AlthougCLN is composed of cells with neuronal differentiation and focal lipomatosis . The hisOur patient presented with multifocal lesions involving both temporal lobe and cerebellar vermis. Only eight cases with multifocal lesions have been previously reported. In 1997, the first case of multifocal CLNs in a 44-year-old man was reported by Horoupian et al.  SubsequeIn our case, the relative small lesion in the cerebellum increased slowly in size and showed an aggressive feature during the 5-years follow-up period. Jenkinson et al.  reportedCerebellar hemisphere is the most common site, while fourth ventricle is the second common site for CLN. The fat composition was observed within the tumor in all cases. Calcification and hemorrhage were observed in one case, while cystic changes were found in five cases. CLN was typically isointense or hyperintense on T1WI. On T2WI and fluid-attenuated inversion recovery (FLAIR) images, the tumor was hyperintense. Heterogeneous contrast enhancement was found in nearly all cases. Edema in surrounding tissue was rare. On CT, the tumor was variably iso-to hypodense (compared to brain parenchyma) with focal areas of marked hypoattenuation corresponding to fat component . The keyDifferential diagnosis of CLN includes medulloblastoma and intracranial teratoma. Medulloblastoma usually presents at younger age (<20 years old) with CT showing a slightly hyperintense lesion in the fourth ventricle. Cystic changes and calcification are commonly seen in tumors. The lesion is usually isointense or hypointense on T1WI, while hyperintense on T2WI on MRI. Intracranial teratoma are benign tumors occurring mainly in the pineal and sellar region, mostly in young men.In conclusion, CLN is a rare intracranial tumor with unknown etiology, which can occur in the cerebellum or supratentorial parenchyma. The distinctive imaging feature of CLN is the fatty component within the lesion. An accurate diagnosis based solely on its imaging findings preoperatively is usually is difficult due to its rarity. Surgery is the primary treatment of choice for large masses causing symptoms. Long-term follow-up is needed for unresected small lesions.The original contributions presented in the study are included in the article/The studies involving human participants were reviewed and approved by the ethics committee of the Second Affiliated Hospital, Zhejiang University School of Medicine. The patients/participants provided their written informed consent to participate in this study.CW performed the data acquisition. CW and SW performed the radiological images analysis. CW, SW, and XX performed the manuscript preparation. All authors contributed to the article and approved the submitted version.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.


Hepatic metastases from carcinoid tumours are typically solid, hypervascular lesions on imaging. The cystic form, mimicking an abscess, is extremely rare. We report a case of a 48-year-old female presenting with a large hepatic mass that was diagnosed as a hepatic abscess, but the ultrasound-guided biopsy showed well-differentiated grade 1 neuroendocrine tumour. CT scan of chest, abdomen and pelvis was performed, looking for the primary tumour, it revealed an endobronchial mass of the right inferior lobe. Lung biopsy by rigid bronchoscopy was taken confirming the diagnosis of a typical carcinoid tumour. Figure 1). Contrast-enhanced abdominal CT scans at the arterial and portal phase revealed a well-defined, round-cystic lesion (Density of 18 HU) occupying the entire right lobe of the liver, measuring 18 × 13 × 19 cm, having a thick enhancing wall suggestive of a liver abscess  and a C-reactive protein value of 70 mg/L. Abdominal ultrasonography showed a large, well-defined, round cystic lesion occupying the entire right lobe of the liver, with hypoechoic center and an irregular wall , especially carcinoid tumours, which originate commonly in the gastrointestinal tract or in other locations such as the lung, kidneys or the ovaries.2 These tumours can be classified as typical or atypical based on their histological features.3 Atypical carcinoid has a more aggressive biological behaviour than typical one with a high risk of recurrence and distant metastases.4 Metastases occur in 15% of typical carcinoid tumours and 30–50% of atypical carcinoid tumors of the lung, and usually involve the liver, bone, adrenal glands and brain.Bronchial carcinoids, as in our case, account for 2% of primary lung tumours and approximately 25% of all carcinoid tumours.In this article, we report a case of liver metastases from typical carcinoid tumours of the lung, mimicking a liver abscess.5Liver metastases do not have pathognomonic symptoms, they are often silent. The common presentation of liver abscess includes fever, chills and right upper quadrant pain. These symptoms may be either present in liver metastases, because of secondary infection or superinfection of spontaneous necrosis.5,6In the absence of pathognomonic symptoms, imaging, especially CT and MRI help the diagnosis. The typical CT imaging features of hepatic abscess are the presence of air bubbles or air-fluid level, double-target sign enhancement. Some characteristic CT findings may help differentiate between hepatic abscess and mass. Abscess shows inner air density, multilayered enhancement, double target or cluster sign, sharp margin and air biliary gram. It can be associated with pleural effusion, atelectasis of the lower lungs. However, a liver mass such as cholangiocarcinoma and hepatic metastasis would have a solid component with peripheral rim-like enhancement. It usually manifests with capsular retraction, focal bile duct dilatation and associate lymph node enlargement.7 The arterial hyperenhancement is due to a rich hepatic arterial supply. They may develop central necrosis or cysts, in this case, imaging features are atypical and can mimic liver abscesses in the pyogenic phase.8,9Liver metastases of NETs, as in our case typically appear as solid, hypervascular masses with marked contrast-enhancement in the arterial and portal phases.9,10The appearance of liver metastases on MRI can also mimic hepatic abscesses, they both appear hypointense on T1W images and hyperintense on T1W images with wall enhancement. Therefore, the role of apparent diffusion coefficient (ADC) calculated from diffusion-weighted imaging (DWI) allows the characterization of benign and malignant lesions.10The necrotic portion of a tumour has a low viscosity and, therefore, a high ADCs. In contrast, the necrotic portion of an abscess cavity is filled with pus, which contains inflammatory cells, bacteria, necrotic tissue and proteinaceous exuded plasma with a very high viscosity and cellularity, thereby resulting in markedly decreased ADCs.8,9Fine-needle aspiration (FNA) is considered the gold standard with a yield that is highly sensitive. However, the process of fine-needle aspiration and its interpretation is fraught with risks. One study reported superiority of needle biopsy over FNA cytology allowing the demonstration of better architectural, cellular as well as immunohistochemical evaluation of liver lesions in biopsy specimens.10Because of non-specific radiological features, percutaneous biopsy with histopathology studies may be required for a definitive diagnosis.In the absence of typical clinical symptoms and because of non-specific radiological features, the diagnosis of liver metastasis and its differentiation from hepatic abscesses may still be challenging, especially in patients with unknown malignancy. Percutaneous biopsy is essential for a definitive diagnosis.The appearance of hepatic metastasis in CT scans can mimic hepatic abscess.It is important to make and consider the differential diagnosis to guide the therapeutic strategy.MRI and especially diffusion-weighted sequence have an important role to differentiate hepatic metastasis from abscesses.In the absence of a definitive diagnosis, percutaneous biopsy with histopathological studies may be required.


Rhabdomyosarcoma (RMS) is a very rare, highly malignant neoplasm thought to originate from the pluripotent mesenchymal tissue. Predominantly diagnosed among children and teenagers, however they can also be encountered in adults. There are a few risk factors associated with RMS like family history of malignancy and genetic syndromes like neurofibromatosis type 1, Li-Fraumeni syndrome, Noonan syndrome and Beckwith-Wiedemann syndrome; however, most cases of RMS are sporadic. Other factors like radiotherapy for other malignancy and pre-natal radiation exposure also are associated with increased risk of developing RMS. The most common reported sites for RMS are head, neck, trunk, pelvis and lower limbs. Omental involvement of primary RMS has been rarely reported in the literature. Principally, the survival of treated RMS cases has improved, primarily due to multidisciplinary management approaches. In this paper, we report a case of primary pleomorphic RMS in a 50-year-old female who presented with abdominal pain. The tumour nomenclature is derived from the Greek words rhabdo, which means rod shape, and myo, which means muscle. Rhabdomyosarcoma (RMS) is recognised as the most frequent childhood soft-tissue sarcomas, and it is encountered in more than 50% of all soft-tissue sarcomas in the paediatric and adolescent age groups . It is lA 50-year-old female Libyan patient presented with a recent history of abdominal pain associated with vomiting and constipation. Two years prior to the presentation, she was diagnosed with ovarian papillary serous cyst adenocarcinoma FIGO stage II C  treated with total abdominal hysterectomy, bilateral salpingo-oophorectomy and inguinal lymph node dissection in addition to six cycles of chemotherapy . Clinically, there was a tender mass felt at the right lower abdominal quadrant close to umbilicus area. Laboratory findings showed that haemoglobin level was 7.7 g/dL, and other results were not remarkable. A computed tomography (CT) scan revealed a 6.5 × 6.3 × 5.5 cm ill-defined enhanced soft-tissue lesion posterior to the right anterior abdominal wall muscles, corresponding to L2-L4 level. The right-side abdominal wall muscle was thickened and surrounded by omental fatty stranding. There was no clear cleavage between the mass and the anterior abdominal wall as shown in Figure These findings were confirmed by a magnetic resonance imaging (MRI) study, which showed a well-capsulated non-enhancing heterogeneous intraabdominal extraperitoneal lesion seen adherent to the posterior aspect of the right transversus abdominis muscle as shown in Figure Initially, it was assumed that the mass was a malignant tumour due to ovarian cancer recurrence to the omentum or retroperitoneum. Imaging-guided core biopsy was performed; however, the sample was inadequate. The patient declined a repeat attempt and her choice was to go straight for surgery, and the staging CT chest and pelvis did not reveal any additional disease foci. The patient underwent an exploratory laparotomy, which revealed a large right-sided abdominal mass about 10 × 10 cm in size involving the omentum; therefore, it was completely resected with part of the anterior abdominal wall muscle and ascending colon that was adherent to the tumour. Gross examination showed that the resected firm tissue mass measured 11 × 9 × 7 cm with central necrosis and haemorrhage as shown in Figure Microscopically, it showed fragments of high-grade malignant mesenchymal tumour composed of densely cellular fascicles of spindle cells alternating with hypocellular myxoid areas creating marble-like appearance and admixed with sheet of round polygonal cells with densely eosinophilic cytoplasm associated with wide areas of tumour necrosis. The malignant cells display moderate to marked nuclear pleomorphism and high mitotic figures. Many large bizarre tumour cells are seen. The tumour cells are seen invading the adipose tissue and the serosal surface of the large bowel wall as shown in Figure Postoperatively, the patient recovered uneventfully and resumed her daily activities few days after surgery and the patient was referred to the oncologist for further management. Follow-up MRI abdomen was performed 7 and 10 months after the surgery and revealed no evidence of disease recurrence.The omentum may harbour a metastatic tumour from extra/intra-abdominal primary tumours but, on the other hand, primary tumours originating from the omentum may be encountered; however, they are very rare. RMS is a very rare, highly malignant neoplasm thought to arise in immature striated skeletal muscle cells at any age and any site of the body. Generally, RMS is the most often encountered subtype of soft-tissue sarcomas, and it only accounts for 3% of childhood cancers and 2% of adolescent cancers . It is eThe current treatment of RMS is multifaceted and comprises surgical excision, chemotherapy and radiotherapy. PRMS is treated like soft-tissue sarcoma using a multidrug chemotherapy protocol that includes ifosfamide, doxorubicin and mesna. Regarding the radiotherapy, the proton beam therapy is the standard type of radiotherapy for most of the RMS cases. The targeted RMS therapy is increasingly being incorporated into clinical practice, as IGF1R (insulin-like growth factor 1 receptor)-directed targeted therapy has revolutionised RMS tumour treatment; however, further development is still required in this field. Some authors claimed that patients with localised RMS can be cured or in remission for a long period, and the outcomes in those with metastatic or recurrent RMS remain poor. Generally speaking, and due to the inefficiency of the adjuvant therapy, the outcome remains poor in the majority of the reported cases . Also, tIn this case we have described what we believe to be the third case of primary omental RMS in an adult to be reported in the literature, and the first case ever reported of PRMS of the omentum in adults or children. RMS should be considered in the differential diagnosis of a malignant omental mass in an adult, especially in the absence of another primary site of the tumour, and this is to ensure early diagnosis and appropriate management. PRMS rarity results in the fact that the available information regarding the disease's clinical and biological characteristics are very limited; further multi-institutional trials and researches that are more challenging, however, are still required.


A 44-year-old male presented to the ER with severe abdominal pain and a reported syncope episode. Focused abdominal sonography in trauma and abdominal exam demonstrated free fluid and peritonitis. CT scan demonstrated a metallic object in the stomach with free air and fluid throughout the abdomen. The following report is a rare case presentation of a perforated stomach secondary to a migrated esophageal stent used for the treatment of a benign esophageal stricture refractory to other treatment modalities. Esophageal stents are utilized for many different purposes, including treatment of benign or malignant esophageal disease such as small, contained esophageal perforations, strictures, and unresectable malignancies. This also includes progressive chronic dysphagia that has failed medical management and other conservative measures. In regard to chronic dysphagia, the decision for stent placement is decided after serial dilations and failed medical management. Refractory benign esophageal strictures (RBES) can be defined as stenosis <14 mm after serial esophageal dilations within two weeks or failure to maintain a diameter >14 mm after four weeks of esophageal dilations . Stents A 44-year-old chronically unhealthy male with a history of alcohol abuse, gastroesophageal reflux disease (GERD), hypertension, esophageal stricture, and dysphagia was previously seen by thoracic surgery for evaluation of weight loss and continued difficulty swallowing. He had previously undergone several esophagogastroduodenoscopies (EGDs) with serial dilations and only mildly improved dysphagia. No reports of peptic ulcer disease (PUD) were identified during previous EGDs. Images and biopsies were negative for esophageal malignancy or mass but did demonstrate severe stenosis. Due to recurrent symptoms and progressive dysphagia resulting in weight loss and the inability to swallow liquids and medications, he was treated with repeat esophageal dilation and placement of a 19 mm x 120 mm fully covered EndoMAXX esophageal stent by Merit Medical Systems. During outpatient follow-up, four weeks later, he was progressing well and able to swallow oral liquids and solids. The patient reported a 20 lb weight gain since stent placement and was pleased with his post-operative progression. Five months later, the patient presented to the ED following a syncope episode and acute onset abdominal pain. In the ER, due to the uncertain presentation, he was treated as a trauma patient. He underwent workup with a Focused Abdominal Sonography in Trauma (FAST) exam, which demonstrated intra-abdominal free fluid. Physical examination demonstrated severe tenderness consistent with peritonitis, as well as transient hypotension that responded to IV resuscitation. A CT scan of the abdomen and pelvis was obtained and demonstrated free fluid, air, and an intra-abdominal metallic object (Figure Esophageal strictures are caused by many different pathologies, including GERD, radiation, cancer, esophageal resection, immunologic disorders, and more. The disease can be very aggravating and debilitating for affected individuals. Typically, about two in three patients will be successfully treated with serial dilations. However, one-third may require further management. Complex strictures are defined as being longer than 2 cm, tortuous, and severely narrowed diameter . In thisEsophageal stents are an essential tool that has been shown to treat dysphagia successfully, secondary to refractory esophageal structures. Migration is a complication that is more likely to occur early following placement but can occur at a later date, although rare. Intestinal obstruction is more commonly diagnosed following evaluation of a migrated esophageal stent. Intestinal perforation is possible but rare. Perforation of the stomach is extremely rare, and no identified case reports have been identified during the literature review to formulate this report. As with any gastrointestinal perforation, if not treated quickly, it can result in severe sepsis and patient demise.


We present a case of nondiagnosed comminuted stress fracture of the base of the proximal phalanx associated with flexor digitalis longus rupture in a teenage athlete. To our knowledge, there is no description in the literature of such a combination of injuries due to delayed diagnosis and incorrect treatment, including injections in the metatarsophalangeal joint of the second toe. The present article focuses on the importance of clinical suspicion of this rare lesion to achieve early diagnosis and avoid surgical treatment. Finally, the surgical technique used to treat this uncommon injury in the chronic phase, which yielded an excellent outcome in this teenage athlete, is described. Stress fractures mainly occur in the foot, especially in runners and/or long-distance athletes. The most common location is the central metatarsals . HoweverDiagnosis must be based on an adequate physical examination and plain orthogonal X-rays. In case of doubt, MRI could also be performed, which has special utility in the acute phase of injury, which can go unnoticed in X-rays. Early diagnosis is very important because it can influence the choice of treatment strategy and subsequent prognosis. In cases reported by Pitsis et al.  and YamaStress fractures can occur in all of the tarsal bones, although the main location is the metatarsals . RegardiWe describe a case involving a combination of lesions in the metatarsophalangeal joint of the second toe in a teenage triathlete, its diagnosis, treatment, and two-year follow-up. To our knowledge, this is the first such case to be reported.We present a case involving a 15-year-old female triathlete with a 3-month history of pain in the forefoot. Despite pain and swelling in the metatarsophalangeal joint, the patient continued to compete and was treated with corticosteroid injections in that joint and discharged with orthosis. In the clinical examination, the only remarkable aspect was slight subtalar pronation and a a shortening of the gastrocnemius muscle. She had not sustained any previous trauma and did not have any systemic diseases. Immediately after intra-articular injections, she described spontaneous weakness in flexion of the second toe and a flexible claw toe .Plain weight-bearing X-rays were performed, in which the bone lesion remained undiagnosed ; howeverNo similar cases with such a combination of injuries were found in the scientific literature. As such, after failure of conservative treatment , foot and ankle sports surgeons were consulted and decided to perform surgical treatment.Based on CT findings , a treatDuring postoperative care, the patient started walking with a postsurgical shoe the day after surgery and started immediately. She started passive movements of the toe 3 weeks after surgery. Six weeks postoperatively, active movement of the metatarsophalangeal joint was started, and normal shoes without orthoses were allowed; she did recover well for the claw toe deformity in this period. Sport practice was resumed 8 weeks after surgery, and running was allowed in postoperative week 12, after verification of full fracture consolidation. The patient returned to normal sporting activity 16 weeks postsurgery, exhibiting excellent evolution in the 2-year follow-up according to the American Orthopaedic Foot and Ankle Society forefoot scale . She did not have any arthrosis or osteoarthritic changes during this time.This is the first case described in literature that combines a stress fracture of the proximal phalanx of second toe and flexor digitorum longus rupture. It is important to make a correct differential diagnosis; this is essential—if not mandatory—before applying intra-articular injections, which can mask underlying injuries and also lead to others as what happened in our case.Despite the lack of reports similar to ours, we consider examination of the plantar plate using dynamic ultrasound of capital importance because bone avulsion and tendon rupture can go unnoticed on MRI because it is performed in the chronic phase. We also think that most comminuted fractures go underdiagnosed, due to patients not consulting in the acute moment and also because of the lack of knowledge in its diagnosis.We think that the rupture of the tendon in our patient was a result of the steroid injection, mainly because the patient did not have any predisposing situation for this to occur and also because it happened close in time to the injection.Our patient was treated surgically due to chronic evolution of the injury and the failure of conservative treatment. We performed an osteo-suture and a second and third metatarsal Weil osteotomy to correct the probable biomechanical etiology, dismissing isolated repair of the fracture, as Yamaguchi et al.  reportedAs Maas et al.  describe


Cervical radiculopathy is a common clinical condition with an annual incidence of 85/10,000. Refractory cases with positive disco-vertebral imaging findings are routinely referred to the Neurosurgeon for evaluation and treatment. In the absence of a clearcut compressive etiology, other rarer but surgically curable causes must be considered before recommending conservative management. We discuss the case of an otherwise active, healthy patient with an invalidating, refractory, relapsing nuchal pain and cervical radiculopathy. Only careful and state-of-the-art neuroimaging led to the correct diagnosis: an osteoid osteoma of the right C6 lamina was diagnosed and microsurgically resected allowing complete recovery and cure. The clinical features of these rare tumors in this unusual location are reviewed. The case is relevant for multifold reasons: it draws attention to rare conditions which can mimic radicular compression; emphasizes the need for a careful evaluation and appreciation of specific clinical symptoms and signs associated with non-compressive radiculopathies; prompts planning of a state of the art imaging workup, in order to rule out such an elusive tumor. All these measures minimize the risk of overlooking the present and other rare pathologies, sparing patients a long path of time-consuming, frustrating and cost-ineffective studies and treatment modalities. Cervical radiculopathy is a relatively common  . CompresA 62 years old otherwise healthy woman complained of a four-month history of progressive nuchal pain radiating to the right upper limb. She also reported tingling and numbness in the C6-C7 right dermatomes. The pain became excruciating at night, often waking up the patient and causing reactive depressive changes in her mood. Although significantly relieved by NSAIDs, prolonged intake was limited by reflux esophagitis causing hiccups which further interfered with night rest. Besides tenderness in the palpation of the right paraspinal nuchal muscles, pain evoked by flexion/extension and lateral neck bent, no neurological signs were observed. MRI highlighted vast signal alteration (hypo in T1 and hyper in T2 and STIR images) involving the paraspinal tissues mainly on the right side at C5-C7, extending epidurally and along the corresponding foramina. In the same location, soft tissues strongly enhanced, consistent with increased vascular permeability. No reduction of spinal canal or foramina by disk, osteophytes or masses was detected. An infectious spinal process was suggested but ruled out based on the absence of fever and laboratory signs. Following six weeks of observation and symptomatic medical therapy, the patient underwent further MRI study including T1 pre- and post-contrast fat saturation sequences : previous findings were better highlighted and as well as changes in bone signal in the C6 and C7 spinous processes (low in T1 and high in STIR) and C6 neural arch Figure , B.The latter appeared swollen at the right hemilamina which harboured a focal heterogeneous tumor: at high-resolution multiplanar CT, the nidus showed the hallmark “target” appearance of an osteoid osteoma  is one of the more frequent conditions related to dysfunction of cervical spinal nerve root. The annual incidence varies from 1,79 to 3,5/1,000 according to the most recent epidemiological studies ,3. Age gOsteoid osteomas (OOs) are small (invariably less than 1.5 cm), benign bone tumors that rarely arise in the spine with a predilection for the neural arch 75%) ,7. A sli5% ,7. A While axial pain is the rule, radicular involvement with clearcut radiculopathy in OOs are much rarer; in most such cases compression by the tumor is advocated, while to our knowledge only two non-compressive cases have been described to date: the Authors documented intense periradicular infiltration by lymphocytes and plasma cell at histology, in a T 11 OO [The diagnosis of spine OOs is challenging and often delayed due to a specific symptoms and the small size of the lesion which can be easily missed not only at plain X-rays in classic projections but also at CT if appropriate slice thickness, orientation or multiplanar reconstructions are not carried out. While radionuclide scanning has been indicated as a valuable localizing tool ,7,11, MROur case however demonstrates that good depiction of reactive changes in the vicinity of the tumor by use of adequate MRI sequences is the key to correct diagnosis. In our experience, fat saturation techniques have been proven especially valuable: among them, Dixon technique allows a more uniform and less affected by artifacts suppression of fat signal than many other techniques . The advThe treatment of choice for OOs is surgical resection. Depending mainly on the location and accessibility of the lesion, open surgery and CT-guided radiofrequency ablation (RFA) have been advocated. Pipola et al.  comparedCervical radiculopathy in the absence of disco-vertebral compression should be carefully evaluated for other potentially surgical causes, OOs among them. High-quality MRI studies are crucial in the imaging workup, since adequate sequences, namely fat suppression techniques  can best define the inflammatory changes in the surrounding tissues, triggered by the secretion of PGE from the tumor nidus. The nidus though can be easily missed due to the small size and variable signal features. High-resolution CT scan with multiplanar reconstructions targeted on MRI indications is therefore a compulsory adjunct in the OO presurgical workup. The PGE biochemical storm involving muscles, ligaments, bone and neural structures within the spinal canal underlies the characteristic clinical syndrome  and justifies excellent, though transient response to NSAIDs. Radiculopathy is a less common feature in OOs and has been seldom reported. Open surgery is the gold standard of treatment: resection in healthy bone margins provides negligible recurrence rates and is preferable to RFA, in the vast majority of patients. Additional instrumented surgery is seldom needed.


Observing a metastatic malignant melanoma and its primary lesion at the same time is rare. The histopathological detection of any unusual pigmented lesion is critical. Observing a metastatic malignant melanoma and its primary lesion at the same time is rare. The histopathological detection of any unusual pigmented lesion is critical. Any unusual pigmented lesion after surgical removal should be microscopically investigated. In the case of metastatic melanoma of parotid with no specific primary origin, patient’s history is helpful. Malignant melanoma is a rare type of cancer in salivary glands, most of which are likely to be metastatic. Malignant melanoma of parotid glands occurs almost invariably as metastases from a primary tumor that is located in the skin or the mucous membranes of the head and neck regions.2A 19‐year‐old woman with pain and swelling in her right parotid gland was referred to Shohada Hospital (Tehran‐Iran) with no underlying diseases. She has complained of a mobile mass on the parotid region for a duration of 3 months. In her clinical examination, the oral mucosa and overlying skin were observed as intact with normal color. The patient's magnetic resonance imaging (MRI) indicated a high signal of a lobular‐contoured, as a 4 × 2.5 cm solid mass lesion in the superior lobe of her right parotid gland Figure . MultinuTo discover the primary origin of the recent melanoma lesion, a comprehensive past medical history was taken from the study case. As a result, we found that she had a history of congenital scalp pigmented nevus with no pathological consultation or any report of metastasis after 2 years Figure . Conside3Melanoma is the most serious skin cancer with a high mortality rate that reminds the necessity of performing the histopathological analysis of any pigmented lesion after biopsy, as well as the detection of the history of pigmented lesions.Generally, malignant melanomas of the parotid gland are metastatic and have maxillofacial skin origin.According to a research by Greene and Bernier,Addressing the study by Prayson et al., 12 cases were reported who were all diagnosed with malignant melanomas of parotid with cutaneous origins of head and neck areas, except one that had an unknown origin. It is interesting that neither none of them were bilateral nor MRI was used for localizing their tumors.4Melanomas of the head and neck areas can be metastatic to local lymph nodes and parotid glands. Considering the high mortality rate of this malignancy, all pigmented lesions with unusual features, large size, irregular pigmentation, unknown duration, or recent enlargement should be submitted for microscopic examinations. Furthermore, recording the exact past medical history of the affected patients is necessary. In the case of any malignancy, neglecting the marginal safety in the surgical operations of pigmented lesions can increase the probability of the metastasis.The authors declare that they have no conflict of interest.Author 1: Acquisition of data and diagnosis of the lesion. Author 2: Study supervision and diagnosis of the lesion. Author 3: Initiate the preparation of this case report for scientific publication and surgery of case. Author 4: Study search and design, analysis, and interpretation of data.Shohada_e_Tajrish Educational Hospital, Shahid Beheshti University of Medical Sciences, Oral and Maxillofacial Pathology Department approved this report.The authors of this article have obtained all required consent forms from the patient. Her consent has been given for the images and other clinical information to be reported in this article excluding her name.


Hyperplastic diffuse nephroblastomatosis (HDNBM), also called universal nephroblastomatosis  is a rarHDNBM is a well-defined disease, and should not be confounded with the persistence of nephrogenic rests (NR) , with or without association to WT. This is a serious problem in the literature, as many authors do not separate those entities. It is, indeed, a very common mistake to call both HDNBM and persistence of NR “nephroblastomatosis” and to include in the same paper cases of HDNBM and persistence of NR  with no distinction.HDNBM typically attains children in their first year of life and demands a differential diagnosis with other causes of bilateral nephromegaly, most commonly benign diseases  and renal lymphoma (extremely rare in the first year of life). The rarity of HDNBM and the fact that the condition is mostly asymptomatic, presenting as incidental abdominal masses, give rise to late diagnosis and delay of chemotherapy, which may allow the development/late treatment of WT. Unfortunately, HCNBM has been associated with a higher frequency of anaplastic WT, perhaps due to a selection of non-responsive cell lineages by chemotherapy .HDNBM is clinically diagnosed. Histopathology usually cannot differentiate between secondary WT and NBM foci in needle biopsy specimens . This maIn this paper, we review the scarce literature dedicated to HDNBM and summarize two cases treated in on a referral service of Pediatric Urology/Oncology.We made an analytic descriptive non-systematic literature review about HDNBM. The key words “HYPERPLASTIC DIFFUSE nephroblastomatosis” or “UNIVERSAL NEPHROBLASTOMATOSIS” were used to find papers through PUBMED, with no language or time limitations. The abstracts were then reviewed. Papers dealing primarily with the persistence of NR in WT cases  were eliminated, as our aim was to review primary HDNBM as an individual clinical entity. The selected papers were then read in toto. Any other papers of interest retrievable from the references were also reviewed. Gray literature was not included. This literature search resulted in 20 papers reviewed in toto .Two clinical cases of HDNBM presenting in the last 10 years to a referral hospital for Pediatric Oncology were also reported, for the sake of illustration of clinical characteristics and image exams of HDNBM.Ethical Committee consent was waived, as this research project involved only literature review and retrospective review of anonymized data from case reports. Both parents consented to a review and publication of anonymized clinical data, including photographs and image exams in medical journals or conferences.Two patients treated in our institution  in the last 10 years motivated this review.Two cases /10 years, despite being a limited number, seems to conform to the disease incidence suggested by Perlman et al in the United States (<2 patients/year)  on the medium third of the right kidney. The patient was then sent to Pediatric Oncology with a presumptive diagnosis of stage 1 Wilms tumor associated with HDNBN and submitted to chemotherapy (actinomycin+vincristine). Fourteen weeks later almost all nodules had regressed, except for the nodule on the medium third of the right kidney (23x21x33mm). Chemotherapy was continued, with the same protocol. 15 and 23 weeks later the nodule persisted (23x22mm), associated with a smaller nodule on the same kidney (6x6mm) and an 11x7mm one in the medium third of the contralateral kidney. 80 weeks after the beginning of chemotherapy a CT demonstrated another exophytic nodule on the medium third of the right kidney (26x24x22mm) and 2 nodules on the medium third of the left kidney (12x10x9mm). The patient was submitted to a limited resection, including two nodules presenting in the right kidney. Histopathology diagnosed WT without anaplasia, involved surgical limits, and no nodal metastases. Chemotherapy was resumed. 118 weeks after beginning chemotherapy a CT showed right kidney hypotrophy. The left kidney was bigger than expected for the age of the patient (120x46mm), and no kidney nodules were detected. Unfortunately, 2.5 years later the patient presented lumbar and leg pain, which evolved into paraplegia, caused by vertebral invasion and spinal compression (T9-T12) by a tumor demonstrated on MRI. The mass was resected and diagnosed as recurrent WT. Three months later a pulmonary nodule and an abdominal nodule next to the left adrenal gland/superior pole of the left kidney were detected and second-line chemotherapy was initiated . After three cycles the pulmonary nodule disappeared on CT, but the abdominal nodule persisted and now measured 32mm (diameter). After 8 chemotherapy cycles a biopsy of the right kidney was described as recurrence of WT and the patient was sent to palliative treatment. Surprisingly, she is alive with no evidence of tumor on image exams, 2.5 years later. Supposedly, the later biopsy diagnosed persistent NR as WT. A preliminary analysis of this clinical case shows the clinical and histological difficulties to differentiate NR nodules and WT and the ill-defined treatment protocols addressing this condition.Our other patient was a 2 months-old baby girl, diagnosed with bilateral nephromegaly at 2 days of age . The child had a prenatal diagnosis of nephromegaly and the kidneys were described on perinatal ultrasound as enlarged and hypoechoic with loss of cortico-medullary differentiation. The patient was referred to Pediatric Nephrology, which decided to follow-up on the baby expectantly with a provisional diagnosis of autosomal dominant polycystic disease. At 7 months-old a new ultrasound showed worsening bilateral nephromegaly with multiple heterogenous hypoechoic nodules in both kidneys. The diagnosis of HDNBM was then considered. CT showed multiple bilateral hypodense renal nodules that did not enhance after endovenous contrast injection, static kidney scintigraphy (DMSA) showed asymmetrical enlarged heterogeneous kidneys presenting irregular contour and multiple hypocaptating areas throughout the parenchyma. The child was admitted at 9 months-old, presenting ventilatory restriction. Right and left kidneys measured 132x67mm and 133x65mm, respectively . Coagulation problems that needed treatment were associated. A percutaneous kidney biopsy showed immature nephrogenic tissue. The child was then submitted to chemotherapy designed to treat WT stage 1 according to SIOP protocol, with a good response. After 6 cycles the kidneys nodules regressed. The patient remains asymptomatic for 1 year, with no evidence of malignant degeneration to WT. This other case illustrates again the difficulties with diagnosis and rare clinical symptoms of HDNBM (ventilatory and coagulation problems).Literature data about HDNBM are scarce and low quality, mostly due to the rarity of the disease. There are probably undiagnosed/unreported cases registered as bilateral WT. Conjoint review of NBM and HDNBM adds to the confusion and makes it difficult to differentiate the natural history of NBM  nests presenting in syndromic patients and stage 5 Wilms tumors) and HDNBM -5, whichMost retrieved papers consisted either of multicentric retrospective reviews reporting on long periods of time , 6 or caNR may be unrelated to disease and simply mature in due time or relate to defects of renal embryological maturation and pre-malignant status, especially in syndromes related to high WT risk . NR per, 3:HDNBM ;Bilateral sporadic stage 5 WT ;Bilateral WT related to high risk syndromes ;“Common” unilateral non-syndromic WT  .Autopsies in neonates detect NR in <1% of cases, mostly microscopic , 16. ThiNBM/persistence of NR, HDNBM and WT are different diseases, while interrelated.NR nests may be perilobar  or intralobar . They may also be classified according to their stage of development . Only hyperplastic NR are active and are the physio-pathologic basis of HDNBM, including malignant degeneration. For histologic details concerning NR and their relationship to WT, please see the excellent review by Hennigar et al. .HDNBM is a pre-malignant condition showing diffuse and bilateral nephromegaly. The cortical surface of both kidneys is composed mainly of hyperplastic blastematous tissue nodules, caused by the massive proliferation of diffuse foci of metanephric persistent tissue throughout the parenchyma .The disease is typical of the first two years of life: no patient older than 3 years of age has been reported to this moment. Children typically present with bilateral diffuse nephromegaly, with an irregular surface . The medRadiologically the kidneys are massively enlarged, with a shell-like nodular expansion of the cortex. The main characteristics of the disease by imaging are:Ultrasound: homogenous multiple hypoechogenic solid peripheral nodules, that may be confounded with the multiple cysts typical of hereditary polycystic dominant kidney disease.Static scintigraphy (DMSA): nephromegaly with multiple “cold” areas.CT: multiple isodense hypocaptating peripheral uniform nodules. The unaffected compressed central parenchyma shows a characteristic striated appearance, with dentate spiculations (“stag antler” appearance), that may also be shown in excretory urography. This “stag antler” appearance may delay the correct diagnosis by misdiagnosing dominant polycystic renal disease, especially as ultrasound may wrongly assume the multiple hypoechogenic nodules as cysts. True cysts may rarely be present and add to the diagnostic difficulties . SecondaMRI (gold standard): nodules hypointense to the cortex and isointense to the medulla in T1 and hyperintense in T2 weighted images, hypocaptating as compared to normal kidney tissue  Figure-. MRI canMacroscopically DHNBM shows as a rind-like expansion of the renal cortex with tan-white discrete nodules, corresponding roughly to CT and MRI descriptions. WT and NBM nodules usually cannot be differentiated in a needle biopsy, and open biopsies are debatable, as they may transform WT cases into ≥stage 3. This represents a serious hindrance, as the ONLY histological distinction between metanephric persistent tissue and WT is the fibrous pseudo-capsule encircling malignant nodules. In other words, WT and NBM nodules share the same histopathological description  and logical , but active treatment may be able to select the aggressive cell lineages, enabling future development of anaplastic tumors (6 times more common than among cases of sporadic WT) .HDNBM normally responds to chemotherapy with quick regression of the proliferative nodules, but the length of the treatment is controversial and the problem may recur , 7. RegrGrowth, recurrence or non-responsiveness of any nodule during chemotherapy suggests WT, and the tumor should be resected, preferably using nephron-sparing techniques , 13, 14.HDNBM is a rare condition affecting children on their first two years of life, commonly presenting either as bilateral nephromegaly and/or malignant degeneration (WT). The diagnosis depends on the presence of bilateral nodular nephromegaly. MRI offers the best accuracy among image exams. A restricted biopsy is unable to distinguish between HDNBM and WT. HDNBM is treated by chemotherapy, generally with the same protocol used to treat stage 1 WT, but the duration of the treatment remains to be determined. Secondary malignancy attains most patients, especially after a late diagnosis. The incidence of anaplastic WT is disproportionately high.


Congenital Bochdalek hernia (BH) in an adult is rare and has an unusual presentation. They are confined to the pediatric age group with an incidence of 1:3,000 live births. It rarely persists asymptomatic until adulthood. Surgical repair by thoracic, abdominal, or thoraco-abdominal approach is the treatment of choice with diaphragmatic reconstruction in associated diaphragmatic agenesis. With only 10 cases of BH with partial diaphragmatic agenesis reported to date, we discuss the rarity, unusual presentation, and management of BH in a young adult with sickle cell disease that has not been reported in the literature. Adult congenital Bochdalek hernia (BH) is uncommon and usually confined to the pediatric population with an incidence of 1:3,000 live births, 0.17% on abdominal computed tomography,A 24-year-old female with a sickle cell disease presented with mild chest pain, an episode of vomiting, and no history of trauma or chronic illness; she was hemodynamically stable, afebrile with regular abdomen examination. Chest examination revealed decreased left air entry, chest X-ray showed left consolidation, and ultrasound thorax confirmed the presence of bowel loops and left kidney within the thoracic cavity. Contrast-enhanced computed tomography (CECT) of the thorax and abdomen showed colon, small bowel, left kidney in the thoracic cavity with scoliosis (Cobb angle of 24 degrees), fused 12th thoracic and 1st lumbar vertebral bodies, and displaced left diaphragmatic crus and its defect ,1B.The patient received preoperative antibiotics, nebulization, and bowel preparation with normal blood investigation. Following left thoracotomy, the thoracic cavity was accessed through the fifth intercostal space, which contained a hypoplastic lung, the colon, small intestine up to the duodenal jejunal flexure, and left kidney with no hernia sac. The normal colonic mesentery was adherent to the aorta and pericardium . FollowCzech anatomist Vincent Alexander Bochdalek first described congenital adult diaphragmatic hernia in 1848.Displaced diaphragmatic crus is explained by the mal-development of at least three of the four parts of the hemidiaphragm anlage, the unpaired ventral portion from the septum transversum, the unpaired dorsolateral portions from the pleuroperitoneal membranes, or the medial portion from persisting parts of the primary mesentery.Surgical repair is the treatment of choice, either by thoracoscopic/laparoscopic approach or by an open repair. We performed an open thoraco-abdominal approach as repositioning of intrathoracic content into the abdominal cavity was not possible through the defect as mesentery of the colon was adherent to the descending thoracic aorta and pericardium as well as dense adhesions within the defect and adhesions confined to the abdominal wall restricted manual reduction. Acute splenic sequestration crises are a complication of sickle cell disease. However, as there is a lack of evidence from the randomized control trials showing that splenectomy improves survival and decreases morbidity in patients with sickle cell disease, hence splenectomy was not considered.Our case is unique in presentation. First, its association with intrathoracic ectopic kidney has been reported in less than 0.25% of the cases and further association of intrathoracic ectopic kidney with vertebral fusion has been reported only once in the literature.Adult congenital BH is rare, can have unusual presentation, and perioperative management in sickle cell disease is challenging, requiring multimodal management.