We report a rare case of 50-year-old Moroccan woman with local recurrence of a subcutaneous hydatid cyst in proximity to the medial surface of the tibia and another cyst at the tibialis posterior muscle in the absence of liver, lung und spleen involvement. The first surgery was done in another hospital three years ago; no adjuvant treatment was performed after surgery. Recurrence was diagnosed according to the MRI appearance, serological and pathological findings. The patient underwent complete excision of the subcutaneous cyst with two centimeters of the medial gastrocnemius muscle; the tibialis posterior muscle cyst was intraoperatively drained and irrigated with scolicidal agent as it was next to the posterior tibial pedicle. A periopertive anthelmintic chemotherapy was administered. Two years after the patient showed no recurrence. This case report and literature review describe an approach to the diagnosis and management of this pathological entity. Echinococcosis has its highest prevalence in countries, where the common intermediate hosts, sheep and cattle, are raised  , 2. The A 50 year old Moroccan female patient of rural origin presented in Avicenna hospital Rabat-Morocco with history of pain and swelling of the medial aspect of the right leg for two years. The patient had been operated, in another hospital, three years earlier for echinococcosis of the subcutaneous tissue of the right leg with no adjuvant treatment. No history of trauma, fever and weight loss was reported. A painful mass  next theRecurrent disease is defined as the appearance of new active cysts after therapy. It occurs after surgical or radiologic intervention and manifests as reappearance of live cysts at the site of a previously treated cyst or the appearance of new disease resulting from procedure-related spillage . The reaDetermining the ideal therapeutic approach for a recurrent subcutaneous and muscular hydatid cyst can be quite challenging for the surgeon. Moreover, the rarity of the disease renders the decision making on the favorable treatment quite difficult. Nevertheless, once the diagnosis is established, the surgeon should consider performing a radical procedure with periopertive administration of anthelmathic chemotherapy aiming in minimizing the possibility of a recurrence. A long term follow-up is required in all cases.


Retinoblastoma is a curable intraocular malignancy in children. However, in clinical practice, retinoblastoma can sometimes be misdiagnosed and mismanaged, leading to extraocular extension and even death. In this report, a series of 3 cases are related that emphasize the conditions and consequences resulting from misdiagnosis and mismanagement of retinoblastoma. The clinical features, imaging findings, histopatholigical examination, and management in 3 case reports of children with misdiagnosed retinoblastoma are presented. Two of the cases received pars plana vitrectomy after being misdiagnosed with Coats disease or ocular blunt trauma, whereas the third case received evisceration after being misdiagnosed with suppurative endophthalmitis. When the diagnosis of retinoblastoma had been confirmed after a second surgery was performed in our hospital, only 2 of the cases received adjuvant orbital radiotherapy. All 3 cases died of systemic tumor metastases. Intraocular surgical procedures should be avoided in any equivocal case until the possibility of latent retinoblastoma is eliminated.We strongly recommend that early enucleation be executed as soon as possible followed by postoperative adjuvant therapy under conditions wherein an intraocular surgery was inadvertently performed in an eye with retinoblastoma. Here we present 3 cases of Chinese children referred to us for management of advanced retinoblastoma. Two of these cases were subjected to pars plana vitrectomy after being misdiagnosed with Coats disease or ocular blunt trauma. The third case was subjected to evisceration after being misdiagnosed with suppurative endophthalmitis. All 3 cases developed massive orbital extensions after intraocular surgeries and eventually died as a result of tumor metastases. Written informed consents were obtained from the participants or their guardians before the surgeries. This study was approved by the Institutional Review Board of the Zhongshan Ophthalmic Center, Guangzhou, China.Retinoblastoma is the most common primary intraocular malignancy in children.A 5-year-old girl presented with pain in the left eye (LE) accompanied by headache, nausea, and vomiting that were present for 11 months. The patient had been misdiagnosed with Coats disease and had received a pars plana vitrectomy combined with endophotocoagulation 1 year before admission to our hospital.+2. Ectropion, mixed conjunctive congestion, band-shaped degeneration of the cornea, neovascularization of the iris, occlusion of the pupil, and opacification of the lens were also detected within the LE. The LE developed a pseudohypopyon resembling a masquerade syndrome . There was secondary glaucoma present in the LE, with an intraocular pressure of Te Figure A. Examine Figure B.Enucleation of the LE was performed during her hospitalization. Histopathological examination revealed a poorly differentiated retinoblastoma in the extraocular stage, showing combined endophytic and exophytic growth patterns, involving the choroid, sclera, optic disc, and optic nerve, and extended to the margins of resection Figure C. PostopA 3-year-old girl was admitted presenting with LE pain for 2 months and proptosis for 1 week. The patient injured her LE in a fall 3 months earlier. She was diagnosed with ocular blunt trauma of the LE consisting of cataract, uveitis, retinal detachment, optic nerve contusion, and secondary glaucoma. The B-scan ultrasonography of the LE showed retinal detachment and vitreous opacity , whereas that of the RE was normal. On ocular slit lamp examination of the LE, eyelid edema, severe subconjunctival hemorrhage, and opacification of cornea were detected and a gray-white neoplasm with a size of 2.0 × 2.5 cm was observed beside the LE , toxocariasis in 2 patients (18%), toxoplasmosis in 1 patient (9%), and endophthalmitis in 1 patient (9%). Enucleation was necessary in all 11 patients, combined with adjuvant treatment of radiotherapy and chemotherapy. Only 1 patient died of metastatic retinoblastoma 24 months later.Coats disease characteristically causes a chronic, unilateral, progressive retinal detachment associated with massive subretinal exudation from permeable telangiectatic retinal blood vessels.9 Accordingly, it is not usually applied in the treatment of retinoblastoma. However, when the intraocular tumor has been controlled or successfully managed, vitrectomy can provide a means for managing complications of retinoblastoma, including retinal detachment and vitreous hemorrhage. Vitrectomy is also applicable for localized vitreous seeds of retinoblastoma, although it should be used judiciously because of the risk of tumor cell dispersion. Moreover, use of vitrectomy requires that the retinal tumor has been stable for a sufficient period of time before vitrectomy and a limited number of vitreous seeds are present.10 The potential for orbital recurrence of retinoblastoma is unlikely, unless intraocular surgeries  were performed prior to enucleation without a diagnosis of retinoblastoma.12Vitrectomy or pars plana vitrectomy represent aggressive modalities for mechanical removal of intraocular retinoblastoma because of the risk of tumor cell dispersion from the sclerotomies and recurrence of the intraocular tumor.7 If an intraocular surgical procedure has been performed in an equivocal case, histopathological analysis of the intraocular tissue is strongly suggested, and enucleation should be performed as soon as possible after a diagnosis of retinoblastoma has been established.12Endophthalmitis, which can produce a “masquerade syndrome,” is sometimes confused with retinoblastoma because of leukocoria, hypopyon, or hyphema, as in our case 3. Infectious causes of endophthalmitis in patients <3 years of age include bacteria, congenital toxoplasmosis, toxocariasis, syphilis, and herpes simplex virus. Typically, in children with endophthalmitis, a previous ocular injection, pain, hypopyon, and elevated intraocular pressure are the main clinical findings. Such symptoms are all quite different from those of retinoblastoma. In our case 3, the ocular contents were eviscerated, rather than enucleation, which exacerbated tumor extraocular extension and resulted in massive orbital infiltration. Moreover, the lack of any histopathological examination of the ocular contents contributed to a 2-year delay in the diagnosis of retinoblastoma. Therefore, ocular content evisceration is prohibited in the treatment of retinoblastoma because of the high risk of tumor extension. In any case of unilateral leukocoria with signs of intraocular inflammation, the diagnosis of retinoblastoma must be considered.In this report, 3 cases of orbital metastasis of retinoblastoma were presented to our department. In all cases, an intraocular surgery had been performed prior to establishing a diagnosis of retinoblastoma and no specific treatment for reducing the subsequent recurrence likelihood of retinoblastoma was initiated. When the diagnosis of retinoblastoma had been confirmed after the second surgery in our hospital, the patient of case 1 refused any adjuvant treatments. Although cases 2 and 3 did have adjuvant orbital radiotherapy at their local hospitals, these postoperative radiotherapies were delayed for 2 and 4 months, respectively, and only case 2 received systemic chemotherapy to prevent tumor dissemination. Sadly, all 3 children died of systemic tumor metastases.8 If an intraocular surgical procedure is performed in an eye with suspected retinoblastoma, enucleation combined with adjuvant chemotherapy or orbital radiotherapy should be performed in the early postoperative period.11 We strongly recommend postoperative adjuvant therapy, even when there is no histopathological evidence of local extraocular tumor spread.11In summary, retinoblastoma may present with atypical features such as vitreous hemorrhage or signs of vitreous inflammation. Vitrectomy should be avoided in these cases until the possibility of latent retinoblastoma has been eliminated.


The Rapunzel syndrome refers to an uncommon and rare form of trichobezoar that extends past the stomach into the small intestines. The Rapunzel syndrome is usually found in young female patients with a history of psychiatric disorders, mainly trichotillomania and trichophagia. We describe a case of Rapunzel syndrome in a 15-year-old girl who presented with abdominal pain, vomiting, and weight loss. We performed a surgical laparotomy and successfully removed a huge trichobezoar extending into the small intestine. A bezoar is a mass of foreign and intrinsic material found in the gastrointestinal tract, most commonly in the stomach. The term bezoar derives from the Arabic word “Badzehr” or the Persian word “padzhar” which means antidote . BezoarsA 15-year-old girl was admitted to our hospital with a 2-month history of appetite loss and moderate weight loss. Episodic abdominal pain and intermittent vomiting worsened after meals. According to her parents, the girl pulled out her own hair and swallowed it, since the age of 5. On examination, the patient was anemic with normal vital signs. Physical examination of her abdomen revealed a large and firm palpable mass from epigastrium to the periumbilical region and hyperactive bowel sounds.μm3, and a mean corpuscular hemoglobin (MCH) of 17.7 pgm, while the rest of the laboratory test findings were all within normal limits. On further evaluation, an ultrasound of the abdomen confirmed the presence of a large intragastric mass and an abdominal computed tomography (CT) scan revealed a huge hypodense and heterogeneous gastric mass, appearing as a mesh, with multiple air bubbles seen within and surrounding the mass  of 56.4 the mass . The masthe mass .Due to the bulky size of the trichobezoar, we decided that the most efficient and safe way of removing it was an elective laparotomy. Upper midline incision was performed and a longitudinal gastrotomy was made in the anterior wall of the distended stomach. The trichobezoar was identified and extracted . The masThe RS, as a very rare and unusual form of trichobezoar, was originally described by Vaughan Jr. et al. in 1968 , the actThe signs and symptoms which characterize the RS depend on the size of the trichobezoar and the presence of complications. RS is typically characterised by signs and symptoms of gastric outlet obstruction or malabsorption-related complications. According to Naik et al. , the mosUpper gastrointestinal endoscopy is considered to be the gold standard for the diagnosis of the trichobezoar; however, it does not prove the existence of the RS. The abdominal CT scan is the most accurate imaging test concerning the presence of trichobezoars, since it demonstrates heterogeneous, mottled intraluminal mass with low attenuation and air trapping . FurtherRecurrence of RS is extremely rare, with the total number of recurrences reported in the literature, being 3 . The mosIn conclusion, despite the fact that the RS is an uncommon disorder, it should be included in the differential diagnosis of young female patients with a history of trichophagia and trichotillomania, chronic abdominal pain, nausea, and vomiting. As far as the diagnosis is concerned, it can be easily made with the use of endoscopy and abdominal CT scan, with the latter being the most accurate imaging test concerning the presence of trichobezoars. Management options for the treatment of the RS include surgical removal by laparotomy or laparoscopically, while laparotomy is widely considered as the treatment of choice for complicated trichobezoars. Recurrence of RS is extremely rare; however long-term psychiatric/psychological support and follow-up should be performed in order to prevent any recurrences.


Leiomyomas are the most common pelvic tumors in women. About 20–30% of women older than 35 are affected. Rare conditions of leiomyomas have extrauterine locations. Myxoid degeneration is a rare type of leiomyoma degeneration. We report a case of solid-cystic myxoid leiomyoma in a 53-year-old woman complained of constipation, urinary hesitation, and malodorous vaginal discharge with palpable 17 × 12 cm mass between vagina and rectum. Regarding the inferior location of the mass, a perineal approach was used to enucleate it. This rare location has not been mentioned before. The woman was finally diagnosed by pathologists which was myxoid leiomyoma. Leiomyomas are the most common benign tumor in women . In geneA 53-year-old woman with no previous medical history was admitted to our center complaining of malodorous vaginal discharge and sever constipation. Her symptoms began a year ago; the patient suffered from moderate constipation and urinary hesitancy. Her symptoms developed to severe constipation, urinary hesitancy, and malodorous vaginal discharge with inability to defecate unless in standing position.Physical examination and digital rectal exam combined with bimanual transvaginal exam revealed a solid rubbery mass about (12 × 10 cm) between the posterior wall of vagina and anterior wall of the rectum with no obvious limits. The examination under general anesthesia demonstrated the same findings.3). Vaginal secretions were sent to analysis and revealed fibrin, erythrocytes, and very rare benign endometrial elements.Laboratory findings were within normal limits, except for a mild leukocytosis  showed a (17 × 12 × 10 cm) solid-cystic mass extending from sacrum posteriorly to pubic symphysis anteriorly and to perineal skin inferiorly, with mass effect . SigmoidUpon laparotomy, a lithotomy position was used, and an abdominal approach was established. The uterine was found enlarged with no other abnormalities. The peritoneum of rectouterine pouch was incised and the big mass was found with no connection to the vagina or the rectum. A perineal incision was made and the mass was enucleated en bloc  without Macroscopically, the mass measuring 15 × 15 × 6.5 cm, having well demarcated borders, cut surface revealed a myxoid appearance with occasional nodules of white fasciculated tissue.Microscopically, the nodules are composed of fascicles and bundles of spindle cells having elongated bland nuclei; the remaining tissue showed thick-walled blood vessels within edematous myxoid stroma. Neither necrosis nor irregular mitotic activity could be seen .Immunohistochemistry revealed nuclear positive response on estrogen receptor  and a neMalignancy can be excluded and the final diagnosis is consistent with myxoid leiomyoma.Leiomyomas are the most common pelvic tumors in women , 7. TheyLeiomyomas are usually asymptomatic and discovered through routine ultrasound. Some patients present with mass effect symptoms such as hydroureteronephrosis in retroperitoneal masses, postcoital bleeding in cervical masses, constipation, and urinary hesitancy. In our case, a malodorous vaginal discharge was reported due to leiomyoma's position between vagina and rectum. Since malignancy is more common in retroperitoneal smooth muscle, radiologic study (CT or magnetic resonance imaging (MRI)) is mandatory to evaluate the mass and its relationship to the adjacent structures and blood vessels . AlthougAll previous reports mentioned that the laparotomy and the laparoscopic surgery through the abdomen are the best ways to resect these tumors. In this case, the abdominal approach was not able to help reach the mass and enable its resection. Transperineal incision allowed the enucleation of the mass.The histopathologic application could not definitely confirm the origin of the tumor, whether it arose from the genital tract or from the tissue in the retroperitoneum. The rectum, vaginal canal, and Denonvillier fascia (since it contains smooth muscle cells and blood vessels ) can allLeiomyomas are benign tumors of smooth muscle. Extrauterine tumors are rare manifestation and can be found anywhere in the body. The resection of the pelvic low tumors may occur through transperineal incision. Malignancies should always be ruled out in retroperitoneal leiomyomas.


A case of immature gastric teratoma in a neonate is being reported here. The neonate was presented with abdominal mass and distension and managed with excision of mass; the patient is doing fine postoperatively.  A 15-day-oldmale child was brought to the hospital with complaint of abdominal distention and a palpable mass in left upper abdomen since birth. There were no other generalised or systemic complaints. The child was born through a full term vaginal delivery and cried immediately after birth. Child was on breast feed and there was no history of exposure to any drugs or radiation to the mother in the ante-natal period. Abdominal examination revealed a firm, irregular mass in left upper abdomen. Complete blood count revealed Hb of 18.0 g/dL and total leukocyte count of 11,500/cm3. Other biochemical investigations were within normal limits. Ultrasonography revealed a solid-cystic mass with mixed echogenicity in left upper abdomen and continuous with gastric outline. Computed tomography (CT) demonstrated a large, lobulated, heterogeneous, exogastric mass attached to larger curvature of stomach with solid and cystic components with calcification and fat attenuation, suggestive of teratoma . An apparent invasion to surrounding structures was not seen. Exploratory laparotomy was performed which revealed a large solid-cystic mass (9.5x5x3cm) arising from postero-inferior wall of the stomach along its greater curvature. The mass was lying outside the stomach without any extension into the lumen of the stomach. The mass was excised en-block and the defect was repaired. The postoperative period was uneventful and child was discharged on 7th postoperative day. The child is still on follow up with serum alpha-feto protein level and is doing well. Histopathological examination showed presence of stratified squamous epithelium, dermal appendages, gut mucosa, respiratory lining, and cartilage along with multifocal aggregates of immature neuro-ectodermal tissue, suggestive of immature gastric teratoma. Teratoma is the most frequent tumor among germ cell neoplasms in children.[1-4]In infancy and childhood the commonest site of teratoma are sacrococcygeal region (60-65%), gonadal (10-20%), mediastinal (5-10%), presacral (5%) and rarely intracranial, retroperitoneal and cervical.[5]There is a striking male predominance of gastric teratoma with only seven cases (7%) occurring in females.[4]In most of the cases, the chief complaints are abdominal distention and lump but sometimes respiratory difficulty can be caused by upward displacement of the diaphragm by the tumor. Some infants can present with vomiting, hematemesis or malena because of ulceration of overlying mucosa in cases of endogastric component.[2] Although gastric teratoma can arise from any part of the stomach, common sites are the lesser curvature of stomach, antrum and fundus of stomach along the posterior wall. Majority of gastric teratoma are exogastric representing approximately 60% of the cases while endogastric growths are present in about 30% of the cases. Mixed exogastric and endogastric growths are rare.[2]In our case the mass was exogastric without any endogastric component attached to greater curvature. Differential diagnosis in paediatric age group should include neuroblastoma, Wilms tumor, hepatoblastoma, rhabdomyosarcoma, liposarcoma, and retroperitoneal teratoma. Histopathologically gastric teratoma can be mature or immature based on the presence and differentiation of neuroglial tissue. Mature gastric teratoma contains mature glial tissue along with other derivatives of all germinal layers and vice versa. Prognosis is excellent as complete excision with primary closure of gastric wall defect give recurrence free survival without adjuvant chemotherapy and radiotherapy. But few cases of immature teratoma with malignant transformation have been described in the literature so regular follow up with abdominal USG and serum AFP is advised in each case.[1]Source of Support: NilConflict of Interest: Nil


Angiomyolipoma (AML) arising in the liver is rare and usually benign, but it occasionally has malignant potential. A 58-year-old man with a liver tumor identified by a previous doctor with features suggestive of hepatocellular carcinoma on computed tomography (CT) underwent anterior segmentectomy of the liver in 2006. Microscopically, the tumor was composed of exclusively epithelioid cells that were scatteredly positive for human melanoma black 45 on immunohistochemistry. Accordingly, primary hepatic epithelioid AML (eAML) was diagnosed. The patient was subsequently referred to our hospital for follow-up after hepatectomy. He had event-free survival for nearly 7 years. In 2013, two well-defined round nodules were detected in the right lung field by chest CT, and partial pneumonectomy was performed for diagnosis and treatment. Histological examination of the resected lung tissue showed that it was morphologically and immunohistochemically identical to his primary hepatic eAML, leading to the diagnosis of pulmonary metastasis. This paper demonstrates a rare case of malignant hepatic eAML with late recurrence in the lung after hepatectomy. Angiomyolipoma (AML) is a relatively rare mesenchymal tumor. Histologically, it is composed of blood vessels, adipose tissue, and smooth muscle. AML is most commonly observed in the kidney, followed by the liver. The tumor is thought to originate from pluripotent perivascular epithelioid cells (PECs), although its normal tissue counterpart remains unclear , 2. PEC A 58-year-old man was incidentally found to have a liver tumor measuring 20 mm by abdominal ultrasonography during a routine company health examination in 2004. The tumor grew in size over the next 2 years, and closer inspections were conducted by a previous physician in 2006. Enhanced computed tomography (CT) of the abdomen showed a 70-mm mass with hemorrhagic and necrotic changes in the right lobe of the liver (segment 5), with early arterial phase staining and late phase washout, which seemed to be consistent with hepatocellular carcinoma (HCC) Fig. . AnterioTSC1 and TSC2, was not detected. The patient has remained recurrence-free on a closer follow-up schedule for 2 years after pneumonectomy.After hepatectomy, the patient was referred to our hospital and followed every 6 months on an outpatient basis. No hepatic recurrence was observed over nearly 7 years. In 2013, a chest X-ray showed a well-defined nodule in the right lung field Fig. . Chest CSince it was first described by Ishak  in 1976,Immunohistochemically, there was a difference in the degree of staining for HMB-45 between primary and metastatic lesion. Considering intratumoral heterogeneity has been observed in various malignancies , this diThere is a paucity of detailed data on the metastasis or recurrence pattern of malignant hepatic AML. As summarized in Table Four patients died due to disease recurrence, indicating an unfavorable prognosis after recurrence , 12–14. In conclusion, we reported a case of malignant hepatic AML with late recurrence in the lung and examined the characteristics of recurrence in patients with malignant hepatic AML. The prognosis of patients with malignant hepatic AML is poor. In this respect, we regard hepatic AML as a borderline malignant tumor, and aggressive therapeutic intervention is recommended since surgical resection is indisputably the most reliable curative treatment.Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.


Castleman disease is a lymphoproliferative disorder of lymph tissue. Albeit benign there is a tendency to transform into lymphoma in some cases.We present a rare case of Castleman tumour presenting as a solitary pulmonary tumor with extension into the oblique fissure on the left lung necessitating a pneumonectomy.A 20 year fit and well lady presenting with constant inter-scapular back pain of 4 months, left hilar shadow on chest roentgenogram and CT scan revealing a centrally based left hilar mass originating from left upper lobe. There was no evidence of mediastinal lymphadenopathy, secondaries or enlargement of lymph nodes in the rest of the body.Patient was taken up for surgery. Thoracotomy revealed a firm well encapsulated 4*4 cm, highly vascular, mass in close relation to hilar structures, crossing the oblique fissure into the lower lobe and with no invasion of hilar structures, or mediastinal lymphadenopathy. The mass was removed en bloc with a pneumonectomy. Histopathology confirmed a hyaline vascular type of Castleman tumor of lung.Isolated pulmonary parenchymal Castleman disease is extremely rare and reported in less than 10 case reports worldwide. It commonly presents as a central mass and requires en bloc resection along with lobectomy/pneumonectomy. The potential for malignant transformation is justification for surgery albeit most cases are only diagnosed post resection. This is an important differential diagnosis for a well circumscribed, benign, centrally placed tumor of the lung and is a justifiable rare indication for pneumonectomy.Written informed consent was obtained from the patient for publication of this abstract and any accompanying images. A copy of the written consent is available for review by the Editor of this journal.


Purpose. Appendicular endometriosis mimicking appendicitis is a rare finding. Inguinal tumor in the course of appendicular endometriosis located within an inguinal hernia sac and infiltrating the periosteum of the pubic bone has not yet been described. Case Report. This paper describes a case of a rapidly enlarging, solid, unmovable, very painful upon palpation inguinal tumor, in a 36-year-old nulliparous woman. During surgery, a hard (approximately 4 cm in diameter) tumor infiltrating the periosteum of the right pubic bone and continuous with the inguinal hernia sac was dissected. The distal segment of the vermiform appendix was an element of the dissected tumor. Histological examination revealed endometriosis of the distal vermiform appendix. After 6 months of hormone treatment, she was referred for reoperation due to tumor recurrence. Once again histological examination of the resected tissue revealed endometriosis. There was no further recurrence of the disease with goserelin therapy. In addition to the case report, we present a review of the literature about endometriosis involving the vermiform appendix and the inguinal canal (Amyand's hernia). Conclusion. This case expands the list of differential diagnoses of nodules found in the inguinal region of women. Appendicitis is the most common surgical disease. The intraoperative finding of an inflamed appendix in the inguinal hernia sac is a rare pathology. It was first described in 1736 by Amyand, who removed a ruptured appendix located within the inguinal hernia of an 11-year-old boy suffering from a spontaneous appendico-cutaneous fistula [Appendicular endometriosis mimicking appendicitis is another rare finding –9. ArévaA 36-year-old woman with regular menstruation cycles, presented with pain in the right lower abdomen under observation at our surgical outpatient clinic 6 months previously, returned to the clinic complaining of a painful mass localized on the right pubic bone. She had noticed the mass 3 days prior. An open right inguinal canal had previously been diagnosed by passing a fingertip during a painless palpation, but sac formation was not detected during the cough test.3/μL).The patient was urgently referred to the inpatient surgery department. The inguinal mass was a solid, unmovable, very painful upon palpation inguinal tumor, which had a diameter of approximately 4 cm, and was located over the right pubic bone. The skin above the tumor was warm. The patient reported regular menses and was menstruating on admission. The laboratory workup revealed only a borderline elevated white blood cell count  infiltrating the periosteum of the right pubic bone in continuity with the inguinal hernia sac was dissected. The distal segment of the vermiform appendix 10 cm long) was an element of the dissected tumor. The vermiform appendix was removed, and the stump of the cecum was sutured after disinfection with iodine and returned to the peritoneal cavity. Histological examination revealed endometriosis of the distal vermiform appendix , which did not influence her menstrual cycle, she was referred for reoperation due to tumor recurrence.On admission, another 4 cm unmovable tumor, which was painless on palpation, was discovered. No other symptoms were reported. The right inguinal canal was closed and the tumor was excised completely (with approximately 1 cm margins) under spinal anesthesia. Histological examination revealed endometriosis. The postoperative course was complicated by local infection, which subsided during an empiric antibiotic therapy.After surgery, the patient again underwent treatment with goserelin  for 9 months, this time followed by a monophasic contraceptive (drospirenone + ethinyl estradiol) under the supervision of the Gynecologic Outpatient Clinic. No recurrence of the disease has been reported.A case of a tumor of the pubic bone with appendicular endometriosis is unique in surgical and gynecological literature. The tumor infiltrating the superior pubic rami probably developed slowly and was asymptomatic for considerable time. The sudden appearance of symptoms was not connected to trauma or gynecological, bone, or joint disease. Interestingly, the patient did not report menstrual pain, which is typical for endometriosis. In our opinion, the development of pain was related to the infiltration of periosteum in the late stage of the disease. It remains a matter of speculation whether the endometriosis initially involved the peritoneum of the inguinal hernial sac and then involved the vermiform appendix, or vice versa.The presence of the vermiform appendix in the inguinal hernia sac accounts for 1% of all cases of inguinal hernia, whereas acute appendicitis at this location accounts for approximately 0.1%–0.13% of cases –13.Appendicular endometriosis is a rare pathology, usually diagnosed during operations for acute appendicitis , 15. ThiThe diagnosis of appendicular endometriosis infiltrating pubic bone is difficult. Only surgical exploration and histological examination allow the proper diagnosis in such cases.This case expands the list of differential diagnoses of nodules found in the inguinal region of women.


Odontomas are odontogenic benign tumors composed of dental tissue. Majority of these lesions are asymptomatic and are often detected on routine radiographs. They can be thought of as “tooth hamartomas" with the lesion consisting of various tooth components. They are divided histologically into complex odontomas and compound odontomas. This paper describes the case of a compound odontoma in a 13-year-old girl diagnosed due to the retention of the primary right mandibular second molar. A surgical excision was performed without disturbing the unerupted premolar. The results achieved indicate that early diagnosis of odontomas enables adoption of less complex treatment, a better prognosis, and displacement or devitalisation of adjacent tooth. Odontomas are the most common odontogenic tumors of the jaws, characterized by their slow growth and nonaggressive behavior . The terOdontomas are considered to be developmental anomalies resulting from the growth of completely differentiated epithelial and mesenchymal cells that give rise to ameloblasts and odontoblasts . They arThe etiology of odontoma is still unclear . Local tThe World Health Organization (WHO) 2005 has classified odontomas according to the histopathological findings: complex odontomas, in which the dental tissues are well formed but exhibit an amorphous and more or less disorderly arrangement, and the compound odontomas, in which the dental tissues are normal, arranged in an orderly pattern, but their size and conformation are altered, giving rise to multiple small teeth like elements called odontoids or denticles .The majority of odontomas which are located in the anterior region of the maxilla are compound, while the great majority of odontomas located in the posterior areas, especially in the mandible, are of the complex types .Management consists of excision. Prognosis after treatment is very favorable, with scant relapse , 10, 11.A 13-year-old female patient reported to the Department of Pedodontics with chief complaint of pain in mandibular right posterior region. Patient's medical and family history were inconclusive. Intraoral examination revealed the presence of occlusal caries on mandibular primary right second molar. The radiographic examination revealed the presence of occlusal caries involving enamel, dentin and approaching pulp Figures An OPG revealed the same findings as those of the intraoral periapical radiograph After achieving adequate local anaesthesia, a mucoperiosteal flap was reflected from the distal surface of the mandibular right primary first molar to the mesial surface of mandibular right permanent first molar on the labial surface. A layer of bone overlying the lesion was removed using a round surgical bur under constant irrigation with saline solution . The calThe surgical site was curetted and irrigated with povidone iodine-saline solution. After hemostasis was achieved, the flap was approximated and closed primarily with 3.0 silk sutures and postoperative radiograph was taken . SuturesSix months postoperatively, OPG was advised to check for the eruption of second permanent premolar  and reveOdontomas are nonaggressive, hamartomatous developmental malformations or lesions of odontogenic origin appearing as small, solitary, or multiple radiopaque lesions found on routine radiographic examinations . They arThe etiology of odontomes remains unknown . It has In 1914, Gabell et al. grouped odontomes according to their developmental origin into: epithelial, composite  and connective tissue , 17. AccWHO defined a compound odontoma as a malformation in which all the dental tissues are represented in a more orderly pattern so that the lesion consists of many tooth like structures. Most of the structures do not morphologically resemble the teeth of the normal dentition but, in each one enamel, dentin, cementum, and pulp, are arranged as in the normal tooth . ComplexThe incidence of compound odontome ranges between 9% and 37%. The majority of odontomas in the anterior segment of the jaws are compound composite in type 61%), whereas the majority of odontomas in the posterior segment are complex composite in type (34%) %, wherea. InteresRadiographic examination seems to be the most effective clinical method of differentiating between the two types , 26. ComThe case described in this report was initially diagnosed as compound odontoma based on the radiographic findings. This diagnosis was later confirmed by histopathologic examination of the lesion .Odontomas, both compound and complex, must be examined microscopically to establish a definitive diagnosis , 14.Kaban states that odontomas are easily enucleated, and displaced adjacent teeth by the lesion are seldom traumatized during surgical excision because they are usually separated by a septum of bone . An odonThus, a thorough visual, manual, and radiographic examination should be performed for all the pediatric patients who present with clinical evidence of delayed eruption, missing tooth, or temporary tooth displacement, with or without history of trauma.adopt a less complex and less expensive treatment,ensure better prognosis,avoid relapse of the lesion,avoid displacement or devitalisation of adjacent tooth.Early diagnosis of odontomas helps us to adopt a l


We present the case of a 52-year-old male patient, hospitalized on an emergency basis in the University Emergency Hospital in Bucharest, after being diagnosed with pneumoperitoneum acute abdomen, for which emergency surgery was mandatory. A 3,5-4 cm malignant gastric perforation, ascitis and peritoneal carcinomatosis were found. The histopathological exam revealed infiltrative mucinous gastric carcinoma with epiploic metastasis. Due to the lack of available gastric material, an atypical surgical solution was performed: gastric packing with epiploic material by means of transgastric traction.The solution proved to be successful for short-term recovery. The underlying condition was not focused on, the patient being directed to the Oncology Department.Acute gastric perforation is a rare complication of gastric cancer, and the association with gastric linitis is uncommon. This specific histopathological condition made the classical surgical repair techniques unsuitable for the presented case and an atypical solution had to be performed. Even if gastric cancer has been diagnosed pre- or perioperatively, it may still be difficult to assess the true extent of the carcinoma and to determine the local operability, due to the inflammatory changes associated with peritonitis, that can affect both the gastric walls and the abdominal lymph-nodes [3],5]. Perforated gastric cancer is a rare condition with a reported incidence of less than 1%, and, generally, present with histories and symptoms that do not obviously differ from those of benign gastric perforation. In a review of literature, the ideal operation for treating the malignancy is still in discussion, as it is dependent on various factors such as the hemodynamic stability of the patient, the surgical expertise, the type and stage of the malignancy [3]-5]. Based on these facts, a two-stage operation is considered by some authors the correct attitude in perforated gastric cancer. In most cases, the initial operation should be directed towards the treatment of perforation and peritonitis. After the recovery of the patient and the histological confirmation of the malignancy, an adequate staging can be completed and radical oncological operation for gastric cancer may be planned, if appropriate .The mucinous gastric carcinoma (MGC) is defined as an adenocarcinoma in which a substantial amount of extracellular mucin (more than 50% of the tumor) is retained within the tumor and it represents only 2% to 6% of all stomach cancers. In a review of literature, MGC is characterized by deeper invasion, more frequent lymph node metastasis, a more advanced pathologic stage at the moment of diagnosis, more frequent lymphatic invasion, and a poorer survival than in NMGC. Though, the poorer survival prognosis is not related with the histopathological features “per se”, but with the late diagnosis [8]. Papadopoulos et al. proposed that mucin interferes with the inflammatory response and the immunologic recognition of tumor cells [9].There are several theories that explain the aggressive invasion of the gastric wall. One of them states that the extracellular mucin acts as an infiltrating medium into the surrounding stroma and assists the penetration of deeper layers by tumor cells .Due to the local anatomopathological aspects, none of the classical curative or palliative techniques could be successfully applied in the presented case. Local intraoperatory findings were surprised by the discrepancy between the anamnesis and the discrete clinical symptoms and, on the other hand, the severity and extension of the gastric lesions: mucinous gastric carcinoma with extensive infiltration of the walls, giant perforation of 3,5/ 4 cm, with anfractuous borders, suggestive of a long term gastric sufferance. The stomach was rigid and difficult to mobilize at the moment and intraoperative risk became prohibitive. Due to rare histopathological encountering (perforation on gastric linitis plastica), an atypical solution had to be chosen, using the greater omentum as material for the surgical repair of the defect [


Cavernous hemangioma in the thymus is a rare presentation in mediastinal hemangiomas. The diagnosis is difficult to make promptly because both invasive and noninvasive examinations usually fail to distinguish it from other tumors of the mediastinum. Their clinical presentations depends on their size and their involvement with adjacent mediastinal structures.We treated a 52-year-old man with thymic cavernous hemangioma that was incidentally detected by chest radiography during a routine health check, and had been misdiagnosed as thymoma before the operation. The tumor was completely resected by thymectomy via video-assisted thoracic surgery. The pathological tissue was diagnosed as a cavernous hemangioma, and no phlebolith was observed in the center.We reported this case of thymic cavernous hemangioma for its extremely rare occurrence in the thymus. The preoperative diagnosis remains a challenge both clinically and radiologically. It is still difficult to distinguish this tumor from other tumors in the thymus. Furthermore, biopsies might not result in a definitive diagnosis. Finally, surgical resection provides material for histopathologic diagnosis. To facilitate the preoperative diagnosis of such a rare tumor, more cases will need to be reported. Cavernous hemangioma is a rare tumor of the mediastinum, accounting for 0.5% or less of all mediastinal tumors . The diaA 52-year-old man was referred to our hospital for assessment of a suspicious mass that was detected on a chest radiography during a routine health check. He denied symptoms, including chest pain, hoarseness, hemoptysis, cough, dry cough, and dyspnea. He was a nonsmoker and had no exposure to any environmental fumes or dust.Physical examination revealed normal breathing sounds in both lung fields. Laboratory findings were within normal limits. In these examinations, hematology test results and biochemistry test results were within regular levels. Plain chest computed tomography (CT) displayed a circumscribed soft tissue mass that was located in the anterior mediastinum, measuring 2.1 × 1.4 cm  rather than a median sternotomy. The thymectomy was performed in the lateral decubitus position, utilizing a three-port, right-sided VATS approach under general anesthesia administered through a double-lumen endotracheal tube. Three entry ports were made at the third and sixth intercostal spaces of the right posterior axillary, midaxillary, and midclavicular lines. The camera was introduced via trocar through the lower port at the sixth intercostal space of the midaxillary line. The operative procedure involved removal of the entire thymus gland including the anterior mediastinal fat, with careful attention to preservation of the phrenic nerve and control of thymic venous tributaries draining into the left brachiocephalic vein. There was no invasion into adjacent structures. After careful attention to hemostasis, a chest tube was placed to drain the pleural cavity, and the right lung was reinflated under direct vision. Macroscopically, examination of the resected specimen showed an encapsulated tumor measuring 2.2 × 1.2 × 0.8 cm in the thymus that could be palpated as a soft nodule. The cut surface seemed to have some fresh blood and the soft tissue was taupe, and no phlebolith was discerned in the center. Histopathologically, it was diagnosed as a cavernous hemangioma consisting essentially of a large number of dilated vessels with a single layer of endothelial cells with no signs of atypia or mitosis. Most of the channels contained red blood cells in the cavity , the axilla (20%), and other organs (5%). Histologically, any dilated lymphatic spaces are lined with attenuated endothelial cells resembling the cells that line normal lymphatics. The lymphatic spaces are usually filled with proteinaceous eosinophilic fluid. The supporting stroma is composed of collagen and may contain lymphocytes and lymphoid aggregates. Angiolipoma is a rare benign tumor consisting of mature adipose tissue and blood vessels. Thymomas are classified using the Masaoka staging system, including stages I, II, III, and IV, and the new World Health Organization schema .As the mass was in the thymus, we considered thymectomy necessary to extirpate the lesion completely and that extirpation by VATS might be a better option to treat this condition than median sternotomy. Surgical resection is associated with excellent long-term prognosis Table .Table 1We reported this case of thymic cavernous hemangioma for its extremely rare occurrence in the thymus. Preoperative diagnosis remains a challenge both clinically and radiologically. It is still difficult to distinguish the disease from other tumors in the thymus. Furthermore, biopsies may not result in a definitive diagnosis. Finally, surgical resection provides material for histopathologic diagnosis. To facilitate the preoperative diagnosis of such a rare tumor, more cases will need to be reported.Written informed consent was obtained from the patient for the publication of this case report and accompanying images. A copy of the written consent is available for review by the editor-in-chief of this journal.


Clear cell sarcoma of soft tissues is a very rare, malignant soft tissue tumor that usually arises in the extremities, with a predilection for the lower limbs. This report presents a 45-year-old male with a painless mass in the right lumbar region for one year. Magnetic resonance imaging showed a 3.6×3.2×1.5-cm soft tissue mass of the right lumbar region. The tumoral cells had pleomorphic nuclei and large amounts of clear cytoplasm, and a proportion of the cells contained melanin. Immunohistochemical analysis was performed, which identified that the cells were positive for S-100, MITF and HMB-45 tumor markers. The patient underwent a postoperative chemotherapy protocol and had no local recurrences at one year post-surgery. Clear cell sarcoma of soft tissues (CCSST) was first described by Enzinger in 1965 . PatientA 45-year-old male presented to our department with a painless mass in the right lumbar region of one year. The patient related that the mass had remained stable for 11 months, with no pain or increase in size. One month prior to presenting at the hospital, the patient reported that the mass had started to enlarge, elicit pain and affect the general wellbeing of the patient. The patient did not have personal or familiar history of cutaneous malignancy. Magnetic resonance imaging identified a 3.6×3.2×1.5-cm soft tissue mass of the right lumbar region.Physical examination revealed a subcutaneous irregular firm mass in the right lumbar region region measuring 3.5×3.0 cm. There were no signs of superficial skin inflammation and clinical examination did not reveal other relevant cutaneous lesions.An open biopsy was performed and the gross specimen measured 3.5×3×1.2 cm. The gross specimen was described as a gray-white, homogenous, rubbery tissue with no attachment to the skin. Tumoral cells were arranged in sheets or small nests, and were separated by connective tissue septa . The celThe patient was started on a postoperative chemotherapy protocol. Radiotherapy was not administered, as requested by the patient. The patient had no local recurrences at the one year post-surgery follow-up and there were no complications from the chemotherapy treatment.CCSST is currently a distinct entity classified, by the World Health Organization, as soft tissue and bone tumors . CCSST iPain above the tumor site may be experienced in 33–50% of all the patients that suffer from CCSST. Primary CCSST usually arises in deeper soft tissues, bound to an adjacent tendon or aponeurosis . FrequenHistologically, CCSST is composed of round and/or fusiform cells that are arranged in nests and separated by fibrocollagenous tissue. The cells have pleomorphic nuclei and large amounts of clear cytoplasm, while the spindle-shaped cells exhibit palely stained eosinophilic cytoplasm .A small number of giant cells, with >12–15 nuclei, may be observed , but theThere are no specific immunoreactive markers used to delineate clear cell sarcoma. S-100 and HMB-45 are often used to differentiate clear cell sarcoma from epithelial tumors and synovial sarcoma, and faint keratin immunoreactivity has been observed in clear cell sarcoma ,13. ThisAn important differential diagnosis is metastatic MM. A possible source of a primary lesion must first be excluded before making a diagnosis of CCSST . In contSurgical resection, adjuvant radio- or chemotherapy or a combination of these three treatments has no reported significant advantage of one therapy over another , althouget al (Patients with CCSST have a variable unpredictable course of the disease. Lymph node metastasis has been reported in a high number of cases . The othet al , patient


Resectable isolated multiple metastases to the pancreas from renal cell carcinoma are rare. In this report, we describe a patient with multiple metastases of renal cell carcinoma to the pancreas who was treated with pylorus-preserving total pancreatectomy.18F-2-fluoro-2-deoxyglucose positron emission tomography/computed tomography showed no accumulation in her body other than the pancreas. Because of concerns about her quality of life after total pancreatectomy, she underwent pylorus-preserving total pancreatectomy. After the resection, her control of blood sugar and quality of life were generally satisfactory. She died as a result of gastrointestinal bleeding 35 months after undergoing pancreatectomy.The patient was a 58-year-old Asian woman who had undergone right nephrectomy for renal cell carcinoma 20 years earlier. In 2008, she was diagnosed with multiple metastases of renal cell carcinoma to the pancreas by abdominal computed tomography during routine follow-up for renal cell carcinoma. Pancreatic metastasectomy should be considered, even for multiple metastases, when the primary tumor is renal cell carcinoma and the metastatic lesions are isolated. Metastatic tumors of the pancreas are rare. Most patients with metastases to the pancreas are not candidates for resection, because the lesions are often widespread. It has been reported that only 1.8 % of patients who undergo pancreatectomy do so for metastatic cancer , 3, withSurgical resection has been reported to improve the prognosis of patients with RCC . Only 1118F-2-fluoro-2-deoxyglucose positron emission tomography/computed tomography (FDG-PET/CT) showed FDG accumulation in the tail of the pancreas  , partial left nephrectomy (2006), and partial chest wall resection (2007) for metastases from RCC, and she was started on interferon therapy in 2007. In 2008, during routine follow-up, abdominal computed tomography (CT) revealed multiple space-occupying legions in the pancreas, but she had no subjective symptoms. Her carcinoembryonic antigen and carbohydrate antigen 19-9 levels were within normal limits. Contrast-enhanced abdominal CT revealed multiple stained nodules in the pancreas  Fig. . PreoperAs metastases occurred while the patient was being treated with interferon, surgery was indicated. Intraoperative ultrasonography showed more than four nodules in the pancreas from the head to the tail, but there was no evidence of lymph node swelling or peritoneal dissemination. She underwent PPTP with splenectomy. Because all blood supply to the stomach comes from the left gastric artery via intramural vessels and all blood drains from the stomach through the left gastric vein, close attention was paid to preservation of these vessels. Her pancreas contained ten macroscopic and more than eleven microscopic metastatic lesions Figs.  and 5. TTwo weeks after PPTP, the patient fell during a hypoglycemic episode and broke her right femur. Subsequently, however, her control of blood sugar was generally satisfactory. Adjuvant therapy consisted of interleukin (IL)-2  for 2 years, after which IL-2 therapy was discontinued because the patient was experiencing severe nausea and vomiting. There was no evidence of RCC recurrence 28 months after PPTP, but she changed hospitals thereafter. We were informed by a local hospital that she died as a result of gastrointestinal bleeding 35 months after pancreatic resection.Resectable isolated multiple metastases to the pancreas from RCC are rare. Most of these patients are not candidates for surgical treatment. In this report, we describe a rare case of isolated multiple metastases to the pancreas from RCC treated by PPTP.Diagnosis of metastases to the pancreas from RCC is often difficult, and thus knowledge of a patient’s medical history is important when the pancreatic mass is initially detected. In our patient, however, the diagnosis was relatively easy because she had been followed for a long period of time after nephrectomy. In general, contrast-enhanced CT, MRI, and FDG-PET/CT are used for the differential diagnosis of nodules in the pancreas. Metastases to the pancreas from RCC are detected as hypervascular tumors on contrast-enhanced CT and dynamic MRI and as accumulations of FDG. Small-sized metastatic RCCs, less than 15mm to 20mm in diameter, have been reported to be homogeneously enhanced . In our The kidney is the most common primary tumor site 70.5 %) of metastases to the pancreas , with ma % of metThe surgical procedure to choose depends on the location of the metastases. Distal pancreatectomy is the treatment of choice for patients with solitary lesions in the body or tail of the pancreas, whereas pancreatoduodenectomy is usually performed in patients with solitary lesions in the head of the pancreas. In general, TP is performed in patients with widespread or multiple tumors because it is as safe as the pylorus-preserving Whipple procedure for the treatment of benign and malignant neoplasms of the pancreas . TP withAnother organ-preserving TP procedure for pancreatic neoplasms is duodenum-preserving total pancreatectomy (DPTP), which was performed on a patient with multiple metastases of the pancreas from RCC . PreservIn this case report, we describe the benefits of surgical resection in a patient with multiple isolated pancreatic metastases from RCC. Organ-preserving TP is the treatment of choice for these patients, as shown by their better quality of life after resection.Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.


Abdominal wall defects are associated with other intra-abdominal anomalies. We report two neonates with omphalocele associated with intra-abdominal anomalies. One neonate had multicystic kidney. Other neonate had duplication cyst of ileum which was missed during initial closure in neonatal life. The birth prevalence of omphalocele is 1 in 5000 births. More than 50% of cases have other serious defects involving the alimentary tract and cardiovascular, genitourinary, musculo-skeletal, and central nervous systems. Chromosomal anomalies are seen in 30-40% cases of omphalocele [1-3]. We report two neonates with omphalocele associated with intra-abdominal pathology. Case 1: A newborn baby presented with giant omphalocele (7x8 cm),  with no other gross associated anomalies. The baby passed meconium within the first 24 hours of life. Ultrasound examination revealed abnormal right kidney consisted of multiple cysts of 10x12 cm in total. Echocardiogram was normal. At operation, facial closure of the defect was possible after nephrectomy for multicystic right kidney . The histopathological examination confirmed the diagnosis of multicystic dysplastic kidney.Case 2:A 2-month-old male baby, previously operated on 2nd day of life for omphalocele minor, presented with abdominal distention associated with regurgitation of feed and irritability. On examination, there was a palpable mass which was soft, smooth, and slightly mobile. The erect abdominal radiograph showed a soft tissue shadow in the right iliac fossa pushing the bowel to the other side. CT scan of the abdomen showed huge cystic mass in the abdomen . On exploration there was a cystic duplication of the terminal ileum which was excised along with resection of the bowel related to the cyst with end to end anastomosis. Patient is doing fine on follow-up.Both of our cases add to the cases of omphalocele associated with intra-abdominal congenital anomalies. Only few cases of omphalocele have been associated with multicystic dysplastic kidney. Kawakita et al [2] reported a case of omphalocele associated with multiple anomalies including bilateral multicystic kidneys, atrial septal defects, and anomalies of the face and hands. The patient died of respiratory distress due to potter’s syndrome. Our case had unilateral renal anomaly and thus had good prognosis. Removal of dysplastic kidney further eased us in facial closure of the abdominal wall defect. Three cases of antenatally diagnosed enteric duplications have been reported in literature . In our 2nd case, the omphalocele was small and duplication cyst was missed owing to small defect. Later on, it presented with abdominal distension due to accumulation of bowel contents in the communicating duplication cyst of the terminal ileum. Other intra-abdominal anomalies are reported with omphalocele as malrotation, atresia, Meckels diverticulum, polycystic kidney and hydronephrosis.Source of Support: NilConflict of Interest: None


Primary central nervous system lymphoma (PCNSL) may rarely be preceded by “sentinel demyelination,” a pathologic entity characterized by histologically confirmed demyelinating inflammatory brain lesions that mimic multiple sclerosis (MS) or acute disseminated encephalomyelitis (ADEM). Interpreting the overlapping radiologic and clinical characteristics associated with each of these conditions—contrast-enhancing demyelination of white matter and relapsing and remitting steroid-responsive symptoms respectively—can be a significant diagnostic challenge.We describe a 57-year-old woman with an unusual clinical course who presented with multi-focal enhancing white matter lesions demonstrated to be inflammatory demyelination by brain biopsy. Despite a good initial response to steroids and rituximab for treatment of presumed tumefactive multiple sclerosis, the patient’s condition rapidly deteriorated, and a repeat brain biopsy six months later was consistent with a diagnosis of diffuse large B-cell lymphoma.Early clinical suspicion for PCNSL and awareness that biopsied lesions may initially show sentinel demyelination suggestive of alternate diagnoses may be essential for early initiation of appropriate therapies and mitigation of disease progression. Clinical, pathophysiological, and diagnostic aspects of sentinel demyelination and PCNSL are discussed. Primary central nervous system lymphomas (PCNSL) are rare neoplasms that account for less than 1 % of all brain tumors . Though PCNSL rarely presents with steroid-responsive, multifocal demyelinating “sentinel” lesions characterized by a predominance of T-cell infiltrates and few B-cells –4. TheseHere we describe a case of a steroid-responsive, histologically-confirmed inflammatory demyelinating lesion which proved later to be a rapidly progressing PCNSL diagnosed on repeat biopsy. We offer guidelines to the approach to a brain biopsy in older adults, to facilitate increased diagnostic accuracy.In October 2009, a previously healthy 57-year-old woman presented with a six week history of progressive fatigue, confusion and headache associated with a right inferior homonymous quadrantanopsia. The patient reported frequently bumping into objects as well as several recent falls. She denied alcohol or drug use and was not taking any prescribed medications. Physical exam revealed a mild right hemiparesis and a wide-based gait, in addition to the visual field defect. There was no relevant past medical, family, or psychosocial history. The patient’s clinical timeline is summarized in Table A brain MRI revealed multiple enhancing lesions, including right frontal and left parieto-occipital mass lesions, that are nonspecific but concerning for demyelination, an infectious or inflammatory process, or neoplasm Fig. .Fig. 1BrFollowing pre-operative corticosteroid treatment (dexamethasone 6 mg IV q6h for 3 days), the left parieto-occipital lesion was biopsied Fig. , revealiThe patient was diagnosed with acute multi-focal demyelination, presumed to be acute disseminated encephalomyelitis or tumefactive multiple sclerosis, and treated with intravenous corticosteroids. She improved rapidly but shortly after discharge developed worsening right-sided weakness and dysarthria and returned several weeks later. At that time, she was alert but mildly disoriented, with intact sensation to temperature, pinprick and vibration, brisk deep tendon reflexes throughout, and bilateral ankle clonus. Gait again was wide-based and unsteady.Repeat MRI showed decreased edema and enhancement of the right-sided lesions, but an increase in the size of the left-sided lesions with vasogenic edema and subfalcine herniation Fig. . The patHow did two biopsies of the same lesion, separated only by months, yield such different histopathological results?As this case demonstrates, PCNSL may be preceded by demyelinating lesions –4, 7 sugCould the co-existence of “sentinel demyelination” and PCNSL be simply coincidental and unrelated pathologies? Pure coincidence has been hypothesized when MS and PCNSL have been found in two different locations in the same patient separated in time by many years . In our surrounding lymphoma has never been described to our knowledge.Is it possible that the first biopsy missed the "true" lesion harboring malignant B-cell clones? If so, how do we explain the presence of inflammatory demyelination? A paraneoplastic phenomenon is possible, as anti-MOG antibodies were found in the sera of a patient with sentinel demyelinating lesions preceding PCNSL . These mCould tumefactive demyelination have transformed into B-cell lymphoma ? In our Could the T-cell infiltrates in the initial biopsy have represented a cell-mediated immune response against the lymphoma, thereby masking a diagnosis of PCNSL , 11? ThiThe observation that the presence of non-malignant infiltrates consisting predominantly of T-cells correlates with improved survival in follicular lymphoma and reports of spontaneous regression of lymphoma in immunocompetent individuals support the existence of a suppressive cell-mediated anti-tumor response –18.up to 50 % of cases [Did treatment with corticosteroids mask the presence of malignant B cells? Many B-cell lymphomas, including PCNSL, are steroid-responsive , 19, 20,of cases .Summarizing 15 cases from the literature, combined with our own case presentation, Table While each patient presentation is unique, there are some clinical “red flags” that should increase suspicion for PCNSL Table . ExampleBarkhof and modified McDonald criteria can be used to predict the risk of progression from a clinically isolated syndrome (CIS) or ADEM to MS , 24. IdeThorough approaches to both radiographic and histopathologic differential diagnoses in such cases have been detailed elsewhere , 20, 25.If a brain biopsy is being considered in this context of initial diagnostic workup, steroids should be withheld, unless rapid neurological deterioration is present, as most patients with PCNSL tolerate deferral of steroid therapy as long as biopsy is performed in a timely manner . HoweverThis case serves as a reminder that PCNSL can be preceded by sentinel lesions indistinguishable from the demyelination of MS or ADEM, and that steroid treatment before biopsy obscures a diagnosis of PCNSL. Clinicians must be vigilant to realize that not all histologic and radiologic evidence of demyelination correspond to a primary demyelinating disease. It is important to maintain a high level of suspicion for PCNSL to facilitate early diagnosis and treatment, particularly with specific clinical and diagnostic features Table . The comth WMA General Assembly, Seoul, Republic of Korea, October 2008).Not applicable for a case report on this patient that was treated without a research intention. This case report does not meet the definition of human research by the United States Department of Health and Human Services or the Food and Drug Administration guidelines. This is also in accordance with the ethical manual and guidelines of the World Medical Association (see 59Written informed consent was obtained from the next of kin of the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the editor of this journal.All data supporting our findings are contained within the manuscript.


Appendiceal mucocele is a rare cause of acute abdomen. Mucinous appendiceal neoplasms represent 0.2–0.7% of all appendix specimens. The aim of this study is to report a case of a mucinous appendiceal neoplasm presented as acute appendicitis, discussing the clinical and surgical approach in the emergency setting. A 72-year-old female patient was admitted to the emergency department with a clinical examination indicative of acute abdomen. The patient underwent abdominal computed tomography scan which revealed a cystic lesion in the right iliac fossa measuring 8.3 × 5.2 × 4.1 cm, with calcified walls, and a mean density indicative of high protein content. The patient was taken to the operating room and a right hemicolectomy was performed. The postoperative course was unremarkable. The histopathological examination revealed a low-grade mucinous appendiceal neoplasm with negative regional lymph nodes. Ultrasound and CT are useful in diagnosing appendiceal mucocele and synchronous cancers in the emergency setting. The initial operation should include appendectomy and resection of the appendicular mesenteric fat along with any fluid collection for cytologic examination. During urgent appendectomy it is important to consider every mucocele as malignant in order to avoid iatrogenic perforation causing pseudomyxoma peritonei. Although laparotomy is recommended, the laparoscopic approach is not contraindicated. Appendiceal mucoceles (AM) or mucinous neoplasms are rare lesions characterized by a distended and mucus-filled appendix. They represent 0.2–0.7% of all appendix specimens –5. HistoThe aim of this study is to report a case of giant appendiceal mucocele presented as acute appendicitis, discussing the clinical and surgical approach in the emergency setting.A 72-year-old female patient was admitted to the emergency department complaining of severe pain on her right lower quadrate (RLQ) of the abdomen with duration of 12 hours. Fever of 38°C was also present. Her clinical examination was indicative of acute abdomen with a palpable mass in the right iliac fossa. Her past medical history included dyslipidemia, hypertension, chronic constipation, lower extremity varices, and a total left hip replacement. Standard laboratory examination showed mild leukocytosis with increase of neutrophil count.The patient underwent a nonenhanced 16X multidetector abdominal CT scan, after oral administration of contrast solution . There was a cystic lesion in the right iliac fossa measuring 8.3 × 5.2 × 4.1 cm, contiguous to the cecum, consistent with a dilated appendix . The lesThe patient was taken to the operating room after being given intravenous antibiotics (cefuroxime 1.5 gr and metronidazole 500 mg) and low-molecular-weight heparin. Taking into consideration the size of the lesion, the possibility of a malignant neoplasm and the prospective of an emergency right hemicolectomy in an elder patient, the open approach was decided. A midline incision was made and the peritoneal cavity was entered. An 8 × 5 × 4 cm mucocele of the appendix was found . There wWe presented the diagnostic and therapeutic approach in the emergency setting of a case of a giant appendiceal mucinous neoplasm presented as acute appendicitis in an elderly patient. Appendiceal mucinous neoplasms can be presented as acute appendicitis in only 8% to 14% of the cases , 10. FurThe term appendiceal mucocele refers to a dilated appendix with increased intraluminal accumulation of mucus. Chronic obstruction of the appendix either by mucus or as a result of mucosal hyperplasia and benign or malignant neoplasms cause the appendiceal mucocele , 12. BenA consensus for classification and pathologic reporting of mucinous appendiceal neoplasia was recently published . It was Recent reports showed a male predominance (5 : 2) . HoweverRuiz-Tovar et al. reported 14% of their patients had an intraoperative diagnosis of appendicitis with AM . Omari eFrom those patients with perforation or extravasation, up to 83% may have a malignant mucocele . A maligUltrasound and CT imaging studies are valuable for the detection of AM and can be easily performed in the emergency setting , 18, 23.The optimal surgical approach for treating an appendiceal mucocele remains controversial. Traditionally neoplasms of the appendix more than 2 cm in diameter are managed by right colon resection. The rationale for this approach is the resection of occult lymph nodal metastases within the ileocolic lymphatic system . At the The laparoscopic approach has been described for the management of the appendiceal mucocele and is still recommended by some authors in selected patients , 33. SinThe initial operation should include appendectomy with en-block resection of the appendicular mesenteric fat and any fluid or mucus must be recovered for cytologic examination , 36. InsAfter an initial urgent operation if the histological diagnosis reveals positive lymph nodes, adenocarcinoma of the intestine, mucinous adenocarcinoma, carcinoid or adenocarcinoid tumors larger than 2.0 cm, or high mitotic rate, a right hemicolectomy should be performed , 40. PatIn case of an appendiceal specimen with perforation and adenomucinosis, follow-up with CT scans every six months for five years and also CEA and CA19-9 tumor marker surveillance is recommended , 46. If In conclusion appendiceal mucocele is a rare cause of acute abdomen. Ultrasound and CT are useful in diagnosing appendiceal mucocele and synchronous cancers in the emergency setting. However, the diagnosis is intraoperative and on histopathological examination. The initial operation should include appendectomy and resection of the appendicular mesenteric fat along with any fluid or mucus collection for cytologic examination. During urgent appendectomy it is important to consider every mucocele as malignant in order to avoid iatrogenic perforation causing pseudomyxoma peritonei. Although laparotomy is recommended, the laparoscopic approach is not contraindicated.


Granulomatosis with polyangiitis (GPA) sometimes involves the eye orbit; however, choroidal involvements in GPA had been rarely reported. We report a rare case presenting with a choroidal mass in an 83-year-old Japanese woman who presented with left eye pain. Diagnostic biopsy revealed necrotizing vasculitis with infiltrates of inflammatory cells. Diagnosis was localized granulomatosis with polyangiitis. Combined treatments with corticosteroid plus azathioprine resolved the choroidal mass region. Although treatment with corticosteroid and immunosuppressive agents improves the prognosis of the disease, ocular morbidity is still well recognized. Clinicians should consider a differential diagnosis of GPA in patients with inflammatory choroidal tumors. Granulomatosis with polyangiitis (GPA) is classically characterized as the triad of necrotizing granulomatosis lesions of the upper and lower respiratory tract, focal segmental glomerulonephritis, and necrotizing vasculitis . The vasAn 83-year-old Japanese woman was referred to us with an 8-month history of left periorbital and left eye pain associated with headache migrating temporal region. The patient subsequently developed conjunctivitis of the left eye. The medical history included unstable angina 3 months previously that was treated with percutaneous coronary intervention. Upon admission, the patient had a temperature of 37.3°C, pulse 91/min, and blood pressure 90/63 mmHg. Her lungs were clear, and her abdomen was soft without hepatosplenomegaly. Neurological examinations revealed no nervous system involvements including cranial nerve palsy or peripheral neuropathy. The patient's pupils were both 3.0 mm, and motility was normal. Confrontational visual-field examinations were normal in both eyes. Intraocular pressures were 16 mmHg on the right and 17 mmHg on the left. Funduscopic examination revealed no abnormalities of the optic nerve or retinal vessels of both eyes.μL with normal renal function and urinalysis.Laboratory results are shown in Enzyme-linked immunosorbent assays (ELISAs) showed positive results for MPO-ANCA  and negative results on an anti-proteinase-3- (PR3-) ANCA . Computed tomography (CT) of the chest and abdomen revealed no abnormal findings (data not shown). In magnetic resonance imaging (MRI) scans of the orbits, diffuse-weighted images (DWI) showed a mass lesion in the interior of left eye. T2-weighted image (T2WI) showed a choroidal tumor with low intensity in left eye . MRI alsWe performed diagnostic biopsy of the choroidal mass. Histopathological evaluation of the specimen revealed granulomatous choroiditis with necrotizing vasculitis with infiltrates of inflammatory cells . Also, sWe treated the patient with oral prednisolone (30 mg/day). The symptoms rapidly resolved. MRI findings indicated that the choroidal tumor was also rapidly resolving . Two monWe report the case of a choroidal tumor in a patient with GPA. Choroidal tumors are not uncommon, but the development of a choroidal tumor caused by GPA is rare. To our knowledge, no choroidal tumor as a direct consequence of GPA has been described, although retrobulbar granuloma is one of the serious complications of GPA . It is oIn our case, histological examination showed a chronic necrotizing granulomatous inflammation localized in the choroidal tissues, thus creating the picture of a solid choroidal tumor. These clinical and histological findings seem to indicate that choroidal granuloma may be another ocular manifestation of GPA. Although GPA can affect any systemic organ, ophthalmological manifestations occur in 28~77% of GPA patients . The mosThe gold standard treatment for GPA combines glucocorticoids (GCs) and cyclophosphamide (CYP) . More seIn summary, we report a case of choroidal tumor in a patient with granulomatous choroiditis secondary to GPA. GPA is a rare entity that can affect almost any organ system. To our knowledge, no choroidal tumor has been previously described as a direct consequence of GPA. Although ophthalmic involvement is relatively common during the course of the disease, choroidal involvement is rare. It is important, however, to consider GPA in the differential diagnosis of a choroidal tumor. Early diagnosis and an appropriate interdisciplinary approach to management are required to decrease morbidity in patients with GPA-mediated inflammatory ocular disease.


Dear Sir,A congenital epulis is a rare benign jaw tumor of neonate and is also known as “congenital gingival granular cell tumor”. The Greek word ‘‘epulis’’ means ‘‘swelling of the gingiva’’. It was first described in 1871 by Neumann; hence the alternative name is Neumann’s Tumor [1]. It usually presents at birth with an obvious mass arising from the gingival mucosa of the maxilla or mandible. It commonly presents in neonates, although prenatal diagnosis with ultrasound has been reported as early as 26 weeks gestation [2]. The lesion usually arises from the gingival mucosa of the maxilla or mandible (maxillary/mandibular ratio 3:1). However, it has also been described on the tongue [3].There is a marked female preponderance of 10:1. This lesion is often pedunculated, flesh-pink colored, firm with a smooth or lobulated surface and in general solitary. However, multiple lesions may also occur in up to 10% [1]. The size of the mass varies from a few millimeters to few cm in diameter. Large lesions may interfere with respiration, feeding or adequate closure of the mouth. The lesions commonly interfere with feeding. Its etiology, histopathogenesis and natural history is still unclear [4]. The diagnosis is usually made on clinical grounds alone, although difficulties may arise when the size of the lesion is small, or the index of suspicion is low. Although few cases of spontaneous regression have been reported, the recommended treatment is surgical excision under local or general anaesthesia [5]. There are no reports of recurrence, or malignant change.A 2.5-kg neonate born by normal term vaginal delivery at home was brought for something protruding from the mouth causing feeding problem. On examination, a round, 3x2 cm pedunculated, smooth, pink-colored soft tissue mass covered by mucous membrane was protruding from the mouth, attached to the maxillary incisor region . Mass was firm in consistency and was not tender on palpation. Adjacent tissue appears normal on examination. The lesion prevented the patient from feeding but did not interfere with respiration. A differential diagnosis of congenital epulis, hemangioma, and teratoma was made. She was booked for excision of this mass under general anesthesia. Mass was removed with an elliptical incision to the peduncles and hemostasis was achieved with diathermy. Postoperative recovery was uneventful. The child was breastfeeding the day after surgery, and discharged home the following day. Excised tissue was sent for histopathological examination. Its microscopic examination showed a benign tumor composed of sheets of closely packed, large, polygonal cells, with abundant eosinophilic granular cytoplasm with small central nuclei consistent with histopathology of congenital epulis.Histologically, congenital epulis shows remarkable similarity with the more common Granular Cell Tumors (GCTs). The clinical presentation of this congenital tumor can be distressing due to its size and aggressive appearance, it is important for the attending pediatricians, pediatric surgeon to be aware of this rare congenital tumor.Source of Support: NilConflict of Interest: None declared


Primary hepatic neuroendocrine tumors (PHNET) are extremely rare and difficult to distinguish from primary and metastatic liver cancers since PHNETs blood supply comes from the liver artery. This study aims to investigate CT and MR imaging findings of primary hepatic neuroendocrine tumor (PHNET) and correlation with the 2010 WHO pathological classification.We examined CT and MRI scans from 29 patients who were diagnosed with PHNET and correlated the data with the 2010 WHO classification of neuroendocrine tumors.−3 mm2/s versus grade 2: 1.26 ± 0.23× 10−3 mm2/s versus grade 3: 1.14 ± 0.17× 10−3 mm2/s).According to the 2010 WHO classification system, PHNETs are divided into three grades based on histological criteria. Grade 1 tumors are singular, solid nodules with enhancement at the arterial phase on CT and MRI scans. In grade 1 tumors, the dynamic-contrast enhancement curve shows rapid wash-in in the arterial phase. Grade 2 tumors can have a singular or multiple distribution pattern, necrosis, and nodule or marginal ring-like enhancements. Grade 3 tumors have multiple lesions, internal necrosis, and evidence of hemorrhage. Portal venous tumor thrombus was seen in one case. As tumor grades increase, the capsule begins to lose integrity and tumor apparent diffusion coefficient (ADC) values decrease(grade 1: 1.39 ± 0.20× 10CT and MRI can reflect tumor grade and pathological features of PHNETs, which are helpful in accurately diagnosing PHNETs. Neuroendocrine carcinoma (NEC) is rare and originates in the gastrointestinal tract, neuroendocrine cells, and pancreas. Although it resembles adenocarcinoma, well-differentiated NEC behaves biologically in a more benign fashion . HoweverHowever, there is some controversy surrounding whether CT and MRI scans can predict tumor grade and malignancy. One research group reported that the CT features of hepatic neuroendocrine tumors vary and do not correlate with their pathologic diagnoses . AnotherIn this study, we retrospectively investigated 29 patients diagnosed with PHNET by surgery or puncture and summarized the characteristics of CT and MRI scans as a way to improve the accurate of diagnosis of PHNET. Additionally, we correlated our findings with the 2010 WHO classification of gastrointestinal neuroendocrine tumors.Between May 2004 and December 2013, 29 patients  with PHNETs were admitted to Beijing Chaoyang Hospital of Capital University of Medical Sciences. Of these 29 patients, 5 cases had carcinoid syndrome, 7 patients experienced pain in the right upper abdomen, 9 patients complained of abdominal discomfort, PHNETs were identified in 6 patients by routine ultrasound, and 2 patients had liver tumors detected by a chest CT exam. All patients underwent a hepatectomy or an ultrasound- or CT-guided liver puncture. PHNETs were confirmed by pathology and immunohistochemistry. Serum levels of alpha-feto protein(AFP) were normal (less than 40 ng/ml), viral hepatitis B antigen was negative, and evidence of liver cirrhosis was not observed.All patients underwent CT and MRI scanning and provided written informed consent. The Hospital institutional review board approved this study.Abdominal helical CT examinations were performed with multi-detector row scanners . Food and water were deprived 4–6 h before enhanced CT examinations. To show contrast between the liver and digestive tract, an oral positive contrast agent  was used. The patients were in the supine position, scanning range was from the right diaphragm to the kidney, slice thickness was 5 mm with helical scan and the slice thickness of the reconstruction image was 1.25 mm with a pitch of 0.984:1. All patients received nonionic intravenous contrast material , which was administered at a rate of 4 mL/s  using a mechanical power injector  set at 230 mAs and 120 kVp. Thereafter, hepatic arterial, portal venous phase, and delayed phase images were acquired with delays of 25 s, 50–60 s, and 2–3 min, respectively. The slice thickness and reconstruction slice thickness were the same with unenhanced CT. Multi-plane reconstruction (MPR) images were obtained at ADW 4.2 workstation .Eighty-three percent of patients (24 out of 29) underwent abdominal MRI examinations in a 3.0-T system with a phased-array torso coil for signal reception . A respiratory-triggered T2-weighted fast spin-echo pulse sequence with fat-suppressed technique was performed. The acquisition parameters were as follows: TR/TE (msec), 4000–5000/81; flip angle, 140°; field of view, 38 cm; matrix, 256 × 256; number of sections, 30; section thickness, 6 mm; interval; 1 mm; and one signal acquired. A breath-hold T1-weighted fast low angle shot (FLASH) GRE sequence was performed in all patients with a chemical shift technique. The parameters were set as TR/TE (msec), 210/2.45, 210/3.83; flip angle, 65°; field of view, 38 cm; matrix, 256 × 256; number of sections, 30; section thickness, 6 mm; interval, 1 mm; and one signal acquired. Three-Dimensional Volume Interpolated Breath-hold Examination (3D-VIBE) was performed during hepatic arterial, portal venous and delayed phases at 25 s, 60–70 s and 2 min after administration of a gadolinium-based contrast medium (0.1 mmol/kg) at a rate of 3–5 mL/s, followed by a saline flush (20 mL). The parameters were as follows: TR/TE (msec), 3.68/1.22; Flip angle, 10°; field of view, 35 cm; matrix, 256 × 256; number of sections, 80; and acquisition time, 21 s.2 along the three orthogonal directions. The sequence was obtained within two breath-holding periods for the whole liver. The parameters included: TR = 4000 ms, TE = 66 ms, matrix = 138 × 128, FOV = 32 to 38 cm adapted to individual body size, twenty-four slices for each b factor for entire liver, thickness = 6 mm, interslice gap = 0.6 mm, average = 2, bandwidth = 1735 Hz/pixel, and acquisition time = 24 s for each breath-holding period. The parallel imaging algorithms (GRAPPA) with an acceleration factor of 2 were added. Spectral fat saturation was employed systematically to suppress the chemical-shift artifacts. ADC maps regarding isotropic images were automatically acquired and all mean ADCs of the lesions were measured on those maps. For patients who did not hold their breath within 24 s, we induced slices for each b factor and added the thickness of the slice and the gap. The acquisition time in scanning the whole liver for hold-breath one time could be reduced to 16 s.Diffusion-weighted MR imaging was performed before dynamic imaging, using a single-shot spin-echo echo planar imaging (EPI) sequence with b factors of 0 and 800 s/mmTwo radiologists blindly analyzed dynamic-contrast enhancement curves  and liver lesions on unenhanced and post-enhanced CT and MRI scans. Measurements of attenuation avoided cystic, necrotic and hemorrhagic areas. If tumors were cystic or predominantly cystic, regions of interest (ROI) on unenhanced and post-enhanced CT and MRI scans were placed in the solid area or margins. Regions of interest (ROI) on diffusion-weighted images were carefully identified in the margins or solid area and ADC values were recorded. Experienced pathologists evaluated hematoxylin-eosin (HE) slides and PHNET grades were defined according to the 2010 WHO classification of gastrointestinal neuroendocrine tumors.Grade 1(G1): the mitotic rate was < 2 per 10 high power field and the Ki-67 index was ≤ 2 %. Grade 2(G2): the mitotic rate was 2–20 per 10 high power field, and the Ki-67 index was 3–20 %. Grade 3 (G3): the mitotic rate was >20 per 10 high power field and the Ki-67 index > 20 %Using pathology and the 2010 WHO classification of PHNET, eight cases were defined as G1, 10 cases were defined G2, and 11 cases were defined G3.All 19 singular lesions  underwent surgical resection. The maximum diameter of the mass was 8 cm with a clear border. Twelve lesions were predominantly solid, one case showed calcification, and the remaining 6 cases were cystic-solid or predominantly cystic.HE staining of G1 lesions revealed well-differentiated round or ovoid cells arranged in nests and glandular duct or chrysanthemum formation. The mitosis rate ranged from 0 to 2 per 10 high power fields. In G2 lesions, the cell arrangements were irregular, with mild differentiation and minimal nuclei atypia. The mean mitosis rate was 10 (range: 4 to 16) per 10 high power field. G3 lesions were poorly differentiated with large nuclei and the mean mitosis rate was 27 (range: 22 to 40) per 10 high power field.Histology showed positive staining for the neuroendocrine marker, synaptophysin (Syn) and CT imaging revealed singular lesions (8 cm) with clear borders and irregular margins. We found multiple liver lesions of different sizes. One PHNET case progressed to tumor thrombus in the portal vein with no evidence of liver cirrhosis in the surrounding normal tissue.−3 mm2/s versus (2.0 ± 0.38 × 10−3 mm2/s). All lesions showed enhanced capsules in the delayed phase of post-enhancement MRI scans. These data further confirm that G1 lesions were benign or low-grade malignant tumors and distinguishable from hepato-cellular carcinoma.Based on CT imaging Table  and MRI −3 mm2/s versus 2.02 ± 0.26 × 10−3 mm2/s, respectively). Nodular or marginal-ring enhancements were presented on post-enhanced MR images. Dynamic enhanced curves (9/10) with CT and MRI appeared as type III in the tumor margin or solid area.For the ten patients with G2 PHNETs, CT scans revealed both single and multiple lesions with necrosis Table . Nodular−3 mm2/s versus 1.97 ± 0.30 × 10−3 mm2/s, respectively). In one case, tumor thrombus appeared as a filling-defect in the portal vein.Four out of eleven cases with G3 PHNETs were singular and the remaining seven cases appeared as diffuse, multi-nodular or marginal-ring enhancement on CT. Three multi-nodular cases were misdiagnosed as metastases and two cases were misdiagnosed as hepatocellular carcinoma with diffuse intra-liver metastases. This misdiagnosis further suggests that a combination of a detailed clinical history and lab tests are essential in the accurate diagnosis of PHNET. The dynamic-contrast enhancement curves illustrated type III. Hemorrhage was seen in one tumor  and increase the likelihood of a correct diagnosis. A tumor that originates from neuroendocrine cells is a neuroendocrine tumor (NET). There are two types of NETs: neural neuroendocrine tumors and epithelial neuroendocrine tumors. Primary hepatic neuroendocrine tumors (PHNETs) develop in the intrahepatic bile duct epithelium and have an extremely low incidence of 0.17 % .G1 and G2 NETs are low grade neoplasms and have a good prognosis. Neuroendocrine carcinomas (NECs) are G3 neoplasms that are structurally similar to NETs. The neuroendocrine markers synaptophysin (Syn) and chromogranin A (CgA) are diffusely expressed and accompanied by nuclear atypia, focal necrosis, and a high mitosis rate (>20/10 HPF).Hepatic NECs have a better prognosis than hepatocellular carcinoma , but chaZhu Z et al.  reportedADC values showed that the PHNET had restricted diffusion compared with the surrounding normal liver, which confirmed that the PHNET was malignant. As PHNET lesions grew in size, the number of intrahepatic lesions increased from single to multiple, focal hemorrhage, necrosis, and portal venous thrombosis became prevalent . Su M etA rich blood supply from the hepatic artery is one characteristic of PHNETs, which is reflected in the type of dynamic enhancement curves. In our study all lesions were markedly enhanced in the arterial phase and the reconstruction of the arterial phase confirmed a rich blood-supply. However, it is difficult to distinguish PHNETs from other diseases such as primary hepatocellular carcinoma (HCC), hepatic adenoma and focal nodular hyperplasia (FNH), hepatic epithelial angiomyolipoma and liver metastases, which also receive their blood supply the liver artery. Primary hepatocellular carcinoma (HCC) has the typical manifestations of marked arterial enhancement and shows a washout pattern that makes it easy to misdiagnose PHNET with tumor thrombus as HCC. The majority of HCC patients has a history of hepatitis, liver cirrhosis, and increased serum levels of alpha-feto protein (AFP). In contrast, PHNETs show a washout in portal venous phase and higher enhancements in delayed phases compared to the surrounding liver parenchyma. Hepatitis, liver cirrhosis, portal vein thrombosis, and increased serum AFP levels are uncommon in PHNET patients. Hepatic adenoma and focal nodular hyperplasia (FNH) has a portal venous phase enhancement that is equal to or slightly higher than normal liver tissue. Hemorrhage and necrosis are rarely seen in FNH. Individuals who have a history of taking oral contraceptives have a higher incidence of hepatic adenomas . AccordiOne limitation of our study is the small sample size, which results from the low incidence of this rare tumor. Therefore, future studies must include more cases, which will allow for the inclusion of statistical data.In conclusion, the appearances of CT and MRI reflect the biologically benign fashion of G1 and G2 PHNETs and the malignant growth of G3 PHNETs. This data could be used to diagnose PHNETS and distinguish grade 1 and 2 tumors from grade 3 tumors, which typically show multiplicity and central necrosis.


Solitary extramedullary plasmacytoma (EMP) of the spleen is a rare condition. The present study describes the case of a 23-year-old female with an extremely rare solitary EMP of the spleen. Upon examination, the tumor demonstrated unusual and notable multiple-phase spiral computed tomography (CT) findings. The lesion was a solitary, well-defined mass, with areas of variable splenic necrosis and cystic degeneration. Contrast-enhanced CT revealed progressive enhancement of the lesion in the cystic wall, internal septa and solid portion, a finding that has not previously been described. The patient underwent a splenectomy and recovered without complications. No evidence of tumor recurrence has occurred during the past two years of follow-up. To the best of our knowledge, this is the first study to examine the CT findings of a solitary EMP of the spleen. The study aimed to investigate the imaging features of solitary EMP, in particular the multiple-phase spiral CT findings, and raise awareness of the disease to reduce misdiagnoses. Solitary extramedullary plasmacytoma (EMP) is a rare plasma cell neoplasm that occurs most frequently in head and neck soft-tissue regions, with the nasopharynx, nasal cavity and paranasal sinuses most commonly affected . The occ9/l and a white blood cell (WBC) count of 8.0×109/l . The differential WBC count identified a total of 1.3% eosinophils, 25.3% lymphocytes and 6.2% monocytes monocytes . In addition, the tumor marker levels of carcinoembryonic antigen, α-fetoprotein, carbohydrate antigen (CA)19-9 and CA-125 were normal. An abdominal ultrasound revealed a non-uniform, low echo, 8.4×7.5-cm, round lesion in the spleen. A number of radiological and laboratory tests were performed in order to eliminate systemic plasmacytoma. The full blood count and levels of creatinine, serum calcium and uric acid were within the normal ranges. The chest CT scan was normal, with the absence of any pulmonary lesions or mediastinal lymph nodes. In addition, the emission computed tomography skeletal survey was normal.A 23-year-old female was admitted to the Second Affiliated Hospital, Zhejiang University School of Medicine  following the discovery of a splenic mass during a routine abdominal ultrasound. The patient had a history of bleeding gums, but not of nausea, dyspnea, fever, abdominal pain or intubation. Furthermore, no positive findings were identified during a physical examination. The results of laboratory tests revealed a hemoglobin level of 129 g/l, a platelet count of 259×10The pre-contrast CT revealed that the spleen was enlarged and contained a solitary, well-defined mass, with areas of variable cystic degeneration and necrosis. The mean CT attenuation value of the solid portion was 39 HU, while the values observed for the areas of necrosis and cystic degeneration were 21–23 HU . FollowiThe patient was transferred to the Department of Surgery at the Second Affiliated Hospital, Zhejiang University School of Medicine, where a laparotomy and splenectomy were performed. The resected spleen was enlarged, with lymphoma-like tumors infiltrating the parenchyma. Histopathology revealed a dense monoclonal infiltrate of plasma cells with eccentrically situated nuclei and a mild degree of nuclear polymorphism. In addition to a variety of binuclear cellular forms with scattered mitoses, perinuclear halos were observed in a number of the cells . The immPost-operatively, the patient recovered without complications, and no evidence of tumor recurrence has occurred during the past two years of follow-up.In total, <10% of patients with plasma cell neoplasms present with solitary plasmacytoma (SP). SP is classified according to location as either solitary plasmacytoma of the bone (SPB) or EMP . The majet al (The median age of patients with either SPB or EMP is 55 years old . In the et al  advocateet al –4, one pet al . TreatmeOwing to the rarity of the condition, the CT findings of splenic EMP, in particular the radiological manifestations of multiple-phase spiral CT, have not been well-studied. A number of studies investigating the CT findings of EMPs in other organs were identified during the literature search for the present study. One study, which examined a case of pancreatic EMP, demonstrated a large homogeneously-enhancing mass in the region of the pancreatic head . A seconA number of differential diagnoses exist that should be considered when a large, solitary, splenic mass with variable areas of necrosis is detected . MalignaThe present case study described the case of a 23-year-old female with a large SP of the spleen. To the best of our knowledge, this patient was the youngest of all reported cases. Although EMP of the spleen is extremely rare, and a diagnosis can only be confirmed by pathological examination, the disease should be considered during the differential diagnosis of large, splenic, malignant tumors. In order to reduce the misdiagnosis of EMP, future studies should focus on extending existing clinicopathological knowledge of the disease.