Giant cystadenocarcinomas of the ovary are rarely described conditions.The authors describe a 57-year-old Brazilian woman who presented with an increase in abdominal girth in February 2003. Imaging studies showed a giant abdominal pelvic mass with probable origin in the right ovary. Cancer antigen-125 was elevated, while carcinoembrionic antigen and alpha-fetoprotein were normal. Total abdominal hysterectomy, bilateral salpingoophorectomy and omentectomy were done. The mass weighed 40Kg, and the histopathology study revealed a mucinous cystadenocarcinoma. She underwent chemotherapy with paclitaxel and cisplatin with no side effects. Under follow-up for more than 10 years, she is asymptomatic and with normal imaging and laboratory parameters, including the cancer antigen-125 marker.This huge tumor evolved for a long time unsuspected and without metastases in a patient from a developing region. The diagnostic and management challenges posed by this unexpected and unusual presentation of an ovarian cystadenocarcinoma are discussed. Mucinous cystadenomas have origins from inclusions and invaginations of the ovarian celomic epithelium and persistence of Müllerian cells, or from Wolffian epithelium and teratomas . They ofExtra-large benign and malignant cysts of the ovary are uncommon and involve diagnostic and management challenges -7, and dThis report concerns an unsuspected giant mucinous ovarian cystadenocarcinoma in a 57-year-old woman with a huge abdominal enlargement. The main objective of this report is to call attention to ovarian epithelial cysts in the outpatient clinics and primary care services, contributing to a decrease in any under-diagnosis, misdiagnosis and underreporting that might occur.A 57-year-old Brazilian woman was referred to our hospital because of an increase in abdominal girth in February 2003. She said she was overweight, and had noted a rapid increase in abdominal girth during the last year. Her menarche occurred at the age of 15, she always had regular periods, and had used oral contraceptives for two years. She had eight pregnancies, producing eight children, and underwent tubal ligation at the age of 29. Her menopause occurred at the age of 46, and she never used hormonal therapy. She underwent an appendectomy and cholecystectomy, respectively, at the ages of 16 and 29, and had arterial hypertension and dyslipidemia. She denied the use of tobacco, alcohol or illicit drugs. Physical examination revealed pale skin, a temperature of 36.1°C (97°F), a respiratory rate of 25 breaths/minute, a pulse rate of 100 beats per minute, blood pressure at 180/140mmHg; normal cardiac and respiratory sounds; her abdomen was distended and tense, and her abdominal circumference measured 134cm  mucinous cystadenoma of the right ovary [et al. removed by laparotomy a giant (21kg) mucinous cystadenoma of the right ovary detected in a 52-year-old patient who presented with morbid obesity and acute abdominal pain [et al. reported two 15-year-old girls with giant benign ovarian tumors (teratoma and cystadenoma) presenting with different clinical features and elevation of tumor markers [et al. utilizing open surgery in a 55-year-old patient who presented with intense abdominal pain soon after a paracentesis to rule out the hypothesis of liver cirrhosis. Determinations of tumor markers were normal [Women with abdominal-pelvic masses constitute a challenging condition in general practice, because the clinical features and findings from physical examination are usually nonspecific. Moreover, concomitance with overweight and obesity can be additional diagnostic pitfalls. Imaging studies of the abdomen can contribute in ruling out the main alternative hypotheses. Although tumor markers can be a useful tool for differential diagnosis of malignant cysts, some authors have described elevated levels of these markers in patients with benign tumors. Cevik and Guldur reported a 13-year-old patient with abdominal enlargement and pain due to a giant 40×30×20cm) mucinous cystadenoma of the left ovary. The levels of CEA, CA 19–9 and CA-125 were high, but α-fetoprotein and human chorionic antigen were normal [0cm mucinet al. described two cases of benign mural nodules suggesting sarcomas in right ovarian mucinous cystadenomas; one of them was a giant (22×18cm) mass. In both cases, the women described a progressive infraumbilical swelling and abdominal pain [Major diagnostic difficulties are often posed if inner nodules are disclosed in these cystic cavities, because this finding must be considered as indicative of a malignant tumor ,15. In onal pain . Malignanal pain .et al. described a 13-year-old girl with an asymptomatic giant (25×25cm) CAdB on the left ovary. The tumor marker evaluation was negative, and a Pfannenstiel laparotomy was performed [Based on cell origin , ovarianerformed . The interformed . Noteworerformed , but somOur patient presented with an asymptomatic increase in abdominal girth, which was associated with a giant mucinous cystadenocarcinoma found in her right ovary. She did not search for medical help for one year of the abdominal change, which allegedly was self-interpreted as an increase in her usual overweight.Despite diagnostic delay, the cystadenocarcinoma was removed before any dissemination. Our present case study gives evidence of unsuspected development of a malignant giant abdominal tumor in a woman from a developing region. Case reports contribute to an increase in the suspicion index about uncommon conditions in primary care settings and in specialized services, and may decrease possible under-diagnosis, misdiagnosis and underreporting.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 US: Abdominal ultrasonography; CA-125: Cancer Antigen-125; CAdB: Borderline cystadenoma; CEA: Carcinoembrionic antigen; CT: Computed tomography.The authors declare that they have no competing interests.SHML and VMS were the major contributors in writing the manuscript, and interpreted the clinical, complementary and surgical data. ACD performed the macroscopic evaluation of the tumor and interpreted the histological examinations. SHML and VPC participated in the ovarian tumor operation and treated the patient. FRDM was a contributor in writing the manuscript, and did the literature search. All authors read and approved the final manuscript.


Pyloric duplication is an extremely rare gastrointestinal tract malformation in neonates. This is the first case report of pyloric duplication in our country (Lithuania).We report the case of a 2-day-old Lithuanian girl who suffered from pyloric duplication mimicking an alternative common bile duct cyst or other intra-abdominal organs cysts. A laparotomy was performed and the cystic formation of the pyloric area was successfully resected. The postoperative course was uneventful.There are only a few reports describing abdominal masses caused by pyloric duplication mimicking common bile duct cyst or other intra-abdominal organs cysts. Therefore thorough clinical and instrumental examination is needed to determine the most accurate diagnosis that allows one to choose the right treatment. The term “intestinal duplication” was first used by Fitz, but was not widely used until popularized by Ladd in 1937 . True pyWe present the case of a 2-day-old Lithuanian girl of third pregnancy and third birth who was born after a gestation period of 39 weeks. She was delivered by Cesarean section. Her weight at birth was 3450g and her Apgar score was evaluated as 10 out of 10 points. An ultrasound was done in the 23rd week of gestation and a cyst in the fetal abdominal cavity was detected. The newborn was admitted to our neonatal intensive care unit two days after birth because of vomiting. A palpable tumor was found in the upper right of her abdomen through inspection and palpation. An X-ray of her abdomen revealed dislocation of her intestines to the left side of the abdomen (Figure The cyst began to grow and consequently symptoms of gastrointestinal obstruction began to present. Three weeks after her birth a laparotomy was performed. The cystic formation in the pyloric area was found and resected Figure . HistopaA duplication cyst of the pylorus is an extremely rare congenital anomaly of the gastrointestinal tract -9. Dupli1. Duplication cyst of the pylorus is an extremely rare congenital anomaly of the gastrointestinal tract.2. Thorough clinical and instrumental examination helps to determine the most accurate diagnosis and allows one to choose the right treatment.A written informed consent was obtained from the parents 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-in-Chief of this journal.The authors declare that they have no competing interests.KT and PG performed the surgery, provided all operative details and photographic material. KT was a major contributor in editing, revising and final approval of the version of the manuscript to be published. PG contributed in patient care, acquisition of data and has been involved in drafting the manuscript. JP was a major contributor in writing and drafting of the manuscript, edited the final version of the manuscript, did the data analysis and reviewed the literature. KT, PG, and JP were involved in the conception and design, analysis of the data, preparation of the manuscript. All authors have given final approval of the version to be published.


Leiomyomas are the benign tumors of the smooth muscle that usually arise in the gastrointestinal system and in the uterus. Oral leiomyomas are uncommon due to the paucity of the smooth muscles in the mouth (except in blood vessels) and those of the mandible are extremely rare. Leiomyomas have been classified as solid, angiomyoma (vascular leiomyoma), and epithelioid variants. Here, we report a rare case of leiomyoma of the mandible in a 9-year-old child, together with conventional histopathologic and immunohistochemical findings. Leiomyoma was first reported by Blanc in 1884. It is fo61A 9-year-old boy reported to the Department of Oral and Maxillofacial Pathology, SIBAR Institute of Dental sciences, with an asymptomatic extraoral swelling on the right body of the mandible, which had been evident for 2 months . AccordiOn radiographic investigations, the panoramic radiograph of the right mandibular premolar-molar region showed faint periosteal reaction in the vicinity of permanent first molar. The developing tooth buds of right canine and premolars appeared to be uninfluenced by the pathosis in the region as compared to the left side. The periosteal bone reaction was noted . As the The lesion was removed under general anesthesia via an extraoral approach. A well-circumscribed lesion was observed and the lesion was easily removed from the surrounding tissues. Macroscopically, the lesion was roughly a spherical mass, brownish white in color, measuring 1 × 1 × 1 cm and was firm in consistency. Microscopically, the tissue section exhibited cellular areas intermingled with collagenous and hyalinized areas. The cells were predominantly spindle shaped and showed elongated nuclei with fusiform or blunt ends . The tisAs all the spindle cell neoplasms show similar histopathologic features, immunohistochemistry (IHC) was suggested. IHC was performed against vimentin, smooth muscle actin and S-100 protein. The tumor was positive for α-smooth muscle actin  and vimeThe occurrence of leiomyoma is rare in the oral cavity. It accounts for 0.42% of all soft tissue lesions in the oral cavity. Intraoss169et al, reported that they can be seen at any age ranging from infancy to 75 years and are most commonly seen in the middle-aged group.[Huseyin Koca ed group.7 It exhied group. The mosted group. Intraossed group. The raried group.3 In our Radiographically, angioleiomyomas manifest as unilocular or multilocular radiolucent lesions with either an ill-defined or a well-defined sclerotic border. Cortical expansion of the alveolar plates may be evident. Root resorption has also been shown. In our cAccording to World Health Organization, leiomyomas are classified into three histological groups: (a) vascular (angioleiomyoma), accounts for 74% of the cases; (b) solid, accounts for 25% of the cases; (c) epithelioid (leiomyoblastomas), accounts for less than 1% of the cases.5 Histolog1The histopathologic diagnosis of leiomyoma is relatively difficult because of the resemblance to many other tumors with spindle-shaped cells. The differential diagnosis would include myofibroma, hemangiopericytoma, neurofibroma, neurilemmoma, nodular fasciitis, fibrous histiocytoma and schwannoma.4Immunohistochemically, leiomyomas are reactive with vimentin, desmin, α-smooth muscle actin and muscle-specific actin. In our case, immunohistochemical findings revealed positivity for vimentin, smooth muscle actin and negativity for S-100 protein. These features helped us to exclude fibroblastic and neural tumors while indicating myogenic differentiation of the tumor.The malignant transformation rate has not been reported in the literature. In gener12In summary, leiomyoma in the oral cavity is rare and is still a rarer entity in mandible due to paucity of the smooth muscles. But it should be considered in the clinical differential diagnosis in the pediatric patients due to its wide age range of occurrence. Even though in the present case, clinicoradiographic and histologic findings favored a benign process, a careful periodic evaluation should be planned as benign lesions have metastasized after latency period.


Approximately 200,000 new cases of melanoma are diagnosed worldwide each year. Skin metastases are a frequent event, occurring in 18.2% of cases. This can be distressing for the patient, as the number and size of cutaneous lesions increases, often worsened by ulceration, bleeding and pain. Electrochemotherapy (ECT) is a local modality for the treatment of cutaneous or subcutaneous tumors that allows delivery of low- and non-permeant drugs into cells. ECT has been used in palliative management of metastatic melanoma to improve patients’ quality of life. This is, to our knowledge, the first application of ECT as neoadjuvant treatment of metastatic subcutaneous melanoma.TM as neoadjuvant treatment permitting conservative surgery three months later.A 44-year-old Caucasian woman underwent extensive surgical resection of a melanoma, with a Breslow thickness of 1.5 mm, located on the right side of her scalp. No further treatment was given and the woman remained well until she came to our attention with a large nodule in her right cheek. Whole-body fluorodeoxyglucose positron emission tomography/computed tomography (FDG PET/CT) was performed for staging and treatment monitoring. Baseline FDG PET/CT showed the lesion in the cheek to have a maximal standardized uptake value (SUVmax) of 19.5 with no evidence of further disease spread. Fine needle aspiration cytology confirmed the presence of metastatic melanoma. The patient underwent two sessions of ECT with intravenous injections of bleomycin using a CliniporatorFollow-up PET/CT three months after the first ECT treatment showed a marked decrease in SUVmax to 5. Further monitoring was performed through monthly PET/CT studies. Multiple cytology examinations showed necrotic tissue. Conservative surgery was carried out three months after the second ECT. Reconstruction was easily achieved through a rotation flap. Pathological examination of the specimen showed necrotic tissue without residual melanoma. One year after the last ECT treatment, the patient was disease-free as determined by contrast-enhanced CT and PET/-CT scans with a good functional and aesthetic result.ECT represents a safe and effective therapeutic approach that is associated with clear benefits in terms of quality of life  and may, in the neoadjuvant setting as reported here, offer the option of more conservative surgery and an improved cosmetic effect with complete local tumor control. Approximately 200,000 new cases of melanoma are diagnosed worldwide each year ,2. Skin A 44-year-old Caucasian woman underwent extensive surgical resection of a melanoma, with a Breslow thickness of 1.5 mm, located on the right side of her scalp. No further treatment was given and the woman remained well until she presented to our attention when a large nodule appeared in her right cheek. Physical examination revealed a 3.5 cm maximum diameter lesion partly adhered to the adjacent overlying skin but with no apparent infiltration of the inner oral mucosa, as confirmed by computed tomography (CT) scan. The whole-body CT scan revealed a 3.5 cm maximum diameter sandglass-shaped large mass, engaging the right cheek, with the upper extremity close to the floor of the orbit. No other metastatic localization was detected. Whole-body fluorodeoxyglucose positron emission tomography/CT (FDG PET/CT) was performed for staging and treatment monitoring under standardized conditions that included fasting for at least 6 h, administration of 3.7 MBq/kg of FDG and imaging one hour after injection. The baseline FDG PET/CT study showed the lesion in the cheek to have a maximal standardized uptake value (SUVmax) of 19.5 with no evidence of further disease spread  as elective treatment. The patient signed a detailed informed consent for the different therapeutic options. ECT treatment was performed after the approval of an appropriate ethics committee  in compliance with Helsinki Declaration, following internationally recognized guidelines. The first neoadjuvant ECT treatment was performed with the patient receiving intravenous (IV) bleomycin 15000 IU/m [Standard radical surgery would have required a large resection of the whole cheek, with wide safety margins of the mass, including the skin and oral mucosa surface, branches of the mandibolar facial nerve and part of the orbit floor. Instead, the patient was spared this and underwent two sessions of ECT using a CliniporatorConservative surgery was carried out three months after the second ECT, and the mass excised, including a narrow rim of healthy tissue margin and a small triangle of adherent skin, sparing the inner layer, the oral mucosa and the floor of the orbit. Reconstruction was easily achieved through a rotation flap. Pathological examination of the specimen, which included a solid lesion with a maximum diameter of 1.3 cm, showed necrotic tissue without residual melanoma. Six months after surgery, the SUVmax in the region of the lesion had further decreased to 1.3 Figure . One yeaApplication of short and high-intensity electric pulses (reversible electroporation) facilitates the intracellular delivery of administered drugs. For example, the cytotoxic effect of bleomycin is increased by more than 8000-fold .Electroporation of the cell membrane causes significant vascular changes in the tumor region, inducing arteriolar vasoconstriction with the reduction of tumor tissue blood perfusion (vascular lock). This effect becomes irreversible when electric pulses are associated with chemotherapy (vascular disrupting effect), leading to an additional cascade. Tumor cell death, due to the effect of bleomycin, is a result of long-term lack of oxygen and nutrients and the accumulation of catalytic products. These effects also induce short-term cessation of bleeding and the palliation of hemorrhaging and ulcerated cutaneous lesions ,12.Currently, ECT indications include the local control of single and in-transit metastatic melanoma skin nodules not amenable to surgery or isolated limb perfusion or infusion, non-melanoma skin cancer, local recurrences and skin metastases from breast cancer, skin metastases from head and neck cancer and local recurrences in the oral cavity -19. SincIn the local control of cutaneous and subcutaneous metastatic lesions, ECT overcomes the low efficacy of classical chemotherapy and may mean surgery can be avoided. However, to our knowledge, its use has never previously been described in a neoadjuvant fashion to reduce the surgical extension in patients with metastatic melanoma.Interestingly, ECT has been employed as a tissue-sparing treatment to reduce tumor burden for further treatments and surgery in other tumor types. One report described the use of preoperative ECT of anal melanoma to enable surgical resection with organ and function-sparing effect while a second case described ECT of a digital chondrosarcoma to avoid finger amputation ,25. In bECT represents a safe and effective therapeutic approach that is associated with clear benefits in terms of quality of life  and may, in the neoadjuvant setting as reported here, offer the option of more conservative surgery and an improved cosmetic effect with complete local tumor control.Written informed consent was obtained from patient for publication of her clinical details and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.The authors declare that they have no competing interests.NM enrolled the patient and drafted the manuscript. CC1 collected data and followed the patient’s course. SM performed surgical procedure. GDM partecipated surgical procedure. GB performed pathology studies. PAA performed electrochemotherapy. CC4 performed imaging studies. LA performed imaging studies. All authors read and approved the final manuscript.


Subsequent biopsy revealed a paraganglioma that was treated with chemotherapy. This case represents an unusual presentation of a paraganglioma associated with gastrointestinal (GI) bleeding and highlights the utility of CTA and 123MIBG in evaluation and treatment.A 10-year-old boy presented with a 3-day history of worsening abdominal pain, fever, emesis and melena. Abdominal ultrasound revealed a right upper quadrant mass that was confirmed by computed tomography angiogram (CTA), which showed an 8 cm well-defined retroperitoneal vascular mass.  Among neuroendocrine tumors, paragangliomas may initially grow insidiously, lacking biochemical secretion , and theA previously healthy 10-year-old African American boy presented with a 3-day history of fever and periumbilical abdominal pain and a 24-hour history of nonbloody, nonbilious emesis, tenesmus, and melena. Past medical and family histories were noncontributory. In the emergency room, he was dehydrated, afebrile, tachycardic, and normotensive. Abdominal exam was soft, with mild epigastric tenderness, without appreciable hepatosplenomegaly or masses. Stool was hemoccult positive. Initial laboratory studies were significant for hemoglobin 4 g/dL (13–16), MCV 80.1 fL (78–98), BUN 12 mg/dL (7–18), creatinine 0.8 mg/dL (0.3–0.8), and albumin 2.7 g/dL (3.7–5.6). Coagulation factors were normal but inflammatory markers were slightly elevated. Stool bacterial, viral, and parasitic pathogens were negative. Initial management included intravenous fluids rehydration followed by packed red blood cells. He was admitted to the GI service for further workup. Within 8 hours of presentation, abdominal examination suggested a new right upper quadrant fullness that quickly progressed from a doughy consistency to a discrete, dense 10 cm round mass in the middle of the abdomen. A broad differential diagnosis for GI bleeding and abdominal pain was entertained, including infectious etiologies, inflammatory bowel disease, Meckel's diverticulum, and malignancies. An extensive workup to investigate these possibilities followed.Abdominal radiograph showed midline displacement of bowel loops. Abdominal ultrasound revealed a right upper quadrant mass displacing the pancreatic head and compressing the inferior vena cava (IVC). CTA indicated an 8 cm well-defined retroperitoneal mass. This highly vascular mass closely abutted the third portion of the duodenum with poor definition of the fat planes between mass and bowel, significantly compressing the IVC. The lesion was primarily fed by the gastroduodenal and superior mesenteric arteries. The mass also abutted the right kidney, resulting in pelvicaliectasis Figures 123MIBG scan indicated uptake only in the abdominal mass. Urinary catecholamines and tumor markers such as alpha-fetoprotein were negative. A CT-guided biopsy with histology and electron microscopy were consistent with a neuroendocrine tumor, specifically, a paraganglioma.Given the concern for a GI tumor, staging evaluation including chest CT, brain magnetic resonance imaging (MRI), bone scan, and bone marrow biopsy were performed, all negative. 123MIBG uptake. Surgical resection 5 months later revealed a tumor adherent to the inferior mesenteric vein, aorta, mesenteric vessels, vena cava, and ureter, which were dissected away. At the duodenal adhesion, a malignant ulcer was eroding into the third part of the duodenum. Near total gross resection with positive microscopic margins and negative lymph node sampling was achieved. Genetic testing revealed a germline point mutation in the mitochondrial complex II enzyme succinate dehydrogenase (SDH) gene, subunit B. Over the course of 24 hours, he underwent exploratory laparotomy, which revealed a mass densely adherent to the third portion of the duodenum. Definitive upfront resection was deferred. Two cycles of neoadjuvant chemotherapy  resulted in no change in the mass size or degree of After the ultrasound and CT imaging, the differential was modified to include invasive GI tumors, including stromal tumors (GIST), neuroendocrine tumors, embryonal tumors, germ cell tumors, or tumors of nerve sheath origin. Rarely, tumors are associated with GI bleeding when eroding through intestinal mucosa .Paragangliomas are exceedingly rare chromaffin tissue complexes of the neuroendocrine system, distributed along paravertebral and paraaortic axes. Paragangliomas commonly occur in the region between the inferior mesenteric artery and aortic bifurcation known as the organ of Zuckerkandl but can occur in all locations where paragangliomas are found , 4. ParaThe specific gene defects associated with paraganglioma development involve the B, C, and D subunits of the SDH gene. While both B and D mutations increase the risk of malignant progression as compared to sporadic cases, SDHB-positive tumors are more aggressive –12, with123MIBG is a highly specific test that detects the catecholamine-secreting nature of a tumor. As 123MIBG testing is not 100% sensitive, other nuclear imaging modalities should be considered. [18F]FDG PET (positron emission tomography) is the preferred functional imaging modality for staging and treatment of SDHB-positive paraganglioma [Radiographically, CT or MRI is the initial test of choice. Paragangliomas are vascular tumors that commonly contain necrotic, cystic, or hemorrhagic areas that can be identified by MRI. anglioma .131MIBG (131iodine metaiodobenzylguanidine) are currently being evaluated for patients with metastases [Treatment for localized paraganglioma is primarily surgical. For unresectable or metastatic paragangliomas, no clear standard of care exists. When indicated, sarcoma-like therapies are often employed. Combinations of cyclophosphamide, vincristine, and dacarbazine have been shown to induce tumor regression; however, the survival benefits of such therapy is unclear and has the potential for significant late effects in adolescent patients . Experimtastases , and tartastases .123MIBG exams every six months as well as annual head, neck, and chest imaging for metachronous tumors along the neuroendocrine axis. The patient was treated with 3 courses of 131MIBG therapy and is currently in remission on maintenance therapy with oral sunitinib. Our patient had an SDHB-related paraganglioma. Parental testing revealed an identical mutation in the patient's mother, who has not experienced any tumors to date. Three of the patient's siblings have been tested and 2 carry germline SDHB mutations and are currently undergoing tumor surveillance. He has had follow-up imaging including MRI and In summary, gastrointestinal bleeding from paraganglioma invading the duodenum is extremely rare in children. Although rare, neuroendocrine tumors should be entertained in the differential diagnosis of pain or GI bleeding, specifically if associated with an abdominal mass. Combinations of imaging techniques are key in the correct identification, characterization, evaluation, and management of these tumors.


Paratesticular fibrous pseudotumors represent benign new growths confined to intrascrotal structures. Both pathogenesis and clinical management are little understood due to the rarity of the lesion, with less than 200 cases reported to date. Recently, paratesticular fibrous pseudotumors have been postulated to be immunoglobulin G4-related, pathogenetically. Here we report two cases of patients with paratesticular fibrous pseudotumor to highlight the clinical features of this rare disease and we report the immunohistochemical examinations to support the theory of paratesticular fibrous pseudotumor being an immunoglobulin G4-related disease.A 28-year-old white man presented with a painless intrascrotal mass. After a clinical examination, a malignant growth was suspected. His ultrasound results revealed a well-demarcated hypoechoic lesion of 1.5cm in diameter at the spermatic cord. Our patient underwent local excision. His follow-up has been uneventful for 12 years. The second case was an 18-year-old white man who presented with a painless scrotal mass suspicious of testicular tumor. A magnetic resonance imaging scan revealed a 3cm mass at the spermatic cord with very low signal density on T2-weighted imaging and a low and inhomogeneous uptake of gadolinium contrast agent on T1-weighted, fat-suppressed imaging. Following local excision, our patient has been well for 18 months.On histological examination, both of the lesions consisted of collagen-rich hyalinized fibrotic tissue with storiform features. There were lymphofollicular infiltrates and, sporadically, also venulitis. The immunoglobulin G4 staining (in case 2) showed an infiltrate of 10 to 15 positive cells per high-power field on average, corresponding to a proportion of 40% in evaluable hot spots. The two patients with paratesticular fibrous pseudotumor presented within a time span of 15 years. During that time, 400 patients with testicular germ cell tumors had been treated in our institution.The specific histological features documented in our case lend support to the theory of paratesticular fibrous pseudotumor being an immunoglobulin G4-related sclerosing disorder. Paratesticular fibrous pseudotumors usually occur in young adulthood. Clinically, paratesticular fibrous pseudotumor can mimic testicular malignancy. Ultrasonographic findings are largely unspecific, however, scrotal magnetic resonance imaging may aid in discriminating the lesion from malignant tumors. Local excision, whenever technically feasible, is the preferred treatment of paratesticular fibrous pseudotumor. Fibrous pseudotumors represent a clinicopathological challenge because of their unknown origin, and their widely variable topographical as well as morphological features. A fibrous pseudotumor confined to paratesticular structures was first described by Balloch in 1904 . Only 6%Based on the contention that these growths were of reactive rather than neoplastic origin, Mostofi and Price explicitly supported the term ‘fibrous pseudotumors’ , which hClinical management of PFPs is largely unresolved. Misapprehension does occur in the majority of cases mainly because these intrascrotal masses may mimic testicular neoplasms . Due to . Surgical exposure by trans-scrotal incision revealed a solid, well-delineated nodule at the spermatic cord distinct from the testis and the epididymis. Local excision was easily accomplished. His postoperative recovery was uneventful. After 12 years of follow-up neither recurrence of intrascrotal disorders nor any other serious diseases have been noted.The first case was a 28-year-old white patient who presented with a right-sided painless scrotal mass that had been slowly growing for four years. His medical history did not involve any predisposing conditions. On clinical examination, a hazelnut-like, hard and mobile nodule was detected by palpation in the right-sided paratesticular region. An ultrasound scan revealed a 1.5 × 1.5cm clearly demarcated hypoechoic lesion at the spermatic cord cranial to the testicleThe second case was a 19-year-old white man who presented with a right-sided painless scrotal mass that had appeared six months previously. His medical history involved a tonsillectomy but no specific events predisposing to scrotal diseases. Our patient was in excellent general condition. A prune-sized solid mass was palpable in the paratesticular region  reported a mean age at presentation of 42 years [In a recent review, Miyamoto et al. 20 reported42 years . It thusAccording to previous reports, PFPs would more commonly arise on the left side . NoteworWith respect to localization, PFPs are observed on the tunica vaginalis, the epididymis, and the tunica albuginea in 76%, 10%, and 14% of cases, respectively [Clinically, both of our cases presented as painless intrascrotal nodules. Their history was remarkably long, comprising of four years in the first of our cases, however, similar findings have been reported previously .Suspicion of testicular malignancy had prompted admission to hospital in both of our cases. Fortunately, preoperative scrotal imaging afforded excluding of this diagnosis and pursuing a testis-sparing surgical approach in both cases. The typical ultrasonographic features of PFP involve sharp margins of the lesion, and localization of the lesion outside of the testicular parenchyma. Usually, the sonographic pattern is homogeneously hypoechoic . MRI of Histologically, PFPs present with the typical histomorphological picture of paucicellular hyalinized tissue with bundles of collagen fibers interspersed by spindle cells and plasma cells as well as lymphocytes . Immunohet al.[Notwithstanding, a probably more specific observation is the significantly elevated IgG4 cell count that was first reported by Bösmüller et al. and now et al.. If threet al.,13,14. TWith respect to the treatment of PFP, conservative surgery, that is local excision of the mass, is sufficient . Our twoParatesticular fibrous pseudotumors represent a truly rare entity with a peak incidence in young adulthood. There is growing evidence that these nonmalignant growths belong to the group of IgG4-related sclerosing diseases. Clinically, PFPs may be confused with malignant testicular tumors. Ultrasonography and particularly scrotal MRI provide appropriate preoperative assessment of the lesion. Testis-sparing excision of the nodule should always be attempted.Written informed consent was obtained from the two patients for publication of this case report and any accompanying images. Copies of the written consent forms are available for review by the Editor-in-Chief of this journal.The authors declare that they have no competing interests.KPD conceived the study, analyzed and interpreted the clinical parameters of the patients in light of the available literature, and wrote most parts of the manuscript. WJS performed the surgical procedures, assisted in the clinical management of the patients, took responsibility for obtaining informed consent for publication, made substantial contributions to the acquisition of clinical data, and was an important participant in writing and editing the manuscript. UF performed and analyzed the imaging procedures performed on the patients and was a major participant in writing the manuscript. MNW performed the histological examination of the surgical specimens, and wrote the pathohistological sections of the manuscript. All authors read and approved the final manuscript.KPD is a consultant urologist and has been working in clinical urology for 30 years. He has a particular interest and expertise in scrotal diseases. WJS is a trainee physician in urology. UF is a senior radiologist with a particular interest and expertise in MR imaging. MNW is a staff pathologist dedicated to surgical pathology.


What abnormalities are seen on brain CT scan?What is the most probable diagnosis?What additional work-up do you request for this patient?What is the possible prognosis of this patient?A 19 years old woman came to our clinic with chief complaints of progressive headache and blurred vision since 3 weeks ago. Headache was initially intermittent but gradually progressed and became constant and aggravated in the recumbent position. Furthermore, she had complaints of blurred vision and binocular diplopia especially in far distance. On neurologic examination, she had bilateral papilledema and bilateral sixth nerve palsies. The rest of examination was unremarkable. The brain CT scan is presented in The brain CT scan shows well circumscribed cystic lesion with a mural nodule in the right cerebellar hemisphere that has mass effect on the fourth ventricle and subsequently non communicating hydrocephalous.Cystic solid lesion of the cerebellar hemisphere that has solid component located near the pial surface and posterior aspect of the cyst, making cerebellar hemangioblastoma (HB) the most likely diagnosis.The diagnostic work-up of suspected hemangioblastoma consists of complete neural axis magnetic resonance imaging (MRI), abdominal CT scan and ultrasonography, and detailed ophthalmologic evaluation to reveal the presence of associated lesions that may be a part of von-Hippel-Lindau disease complex.Hemangioblastomas are benign tumors and generally are not invasive. These tumors have good prognosis and favorable survival when treated appropriately.1Hemangioblastomas are rare tumors, which account for 1-2.5% of all intracranial neoplasms and 10% of posterior cranial fossa tumors with male to female ratio of 2:1. Hemangioblastomas may develop at any age but more likely happen in the third to fifth decades of life.1The clinical manifestation of hemangioblastomas depends on the anatomical location and growth patterns. Cerebellar hemangioblastoma may manifest itself with headache, symptoms of increased intracranial pressure  or signs of cerebellar dysfunction and altered mental status, in decreasing order of frequency.2 Therefore diagnostic work-up of suspected hemangioblastoma includes detailed ophthalmologic examination, complete neural axis imaging for detection of multiple hemangioblastomas and abdominal CT scan, and ultrasonography for surveillance of renal, adrenal and pancreatic tumors.3Most of hemangioblastomas are sporadic; however, they are associated with von-Hipple-Lindau as an autosomal dominant inherited disease in about 34% of cases.4 On brain CT scan, mural nodule is isodense with fluid density surrounding cyst. Furthermore, mural nodule has avid enhancement with contrast, unlike the cyst wall that usually does not enhance.4 On brain MRI hemangioblastoma has hypo to isointense mural nodule with fluid filled cyst on T1W, hyperintense mural nodule and flow voids predominantly at the periphery of the cyst on T2W and avid enhancement of mural nodule without cyst wall with contrast.4Hemangioblastomas are well defined homogenous lesions composed of cyst with a solid enhancing mural nodule in 60% of cases. In the remaining cases, the tumor is solid with no cystic cavity.1Cerebellum is the main location of both JPA and hemangioblastoma that mostly present as a well defined solid-cystic lesion; but hemangioblastoma occur dominantly in adult and mural nodule located at the periphery and posterior with pial layer attach and has abnormal flow voids.5 Ganglioglioma usually develops in children and young adults and rarely arises from cerebellum.6Furthermore, hemangioblastoma can be differentiated radiographically from ganglioglioma, according to the presence of flow voids and peripheral location of mural nodule in hemangioblastoma and variable pattern of mural nodule enhancement in ganglioglioma.6Medulloblastomas have characteristic features of hyperattenuated vermian mass with surrounding vasogenic edema in brain CT scan, decreased apparent diffusion coefficient (ADC) values and mainly affect children with less than 10 years of age.8Ependymomas occur frequently in children and most of lesions arisefrom fourth ventricle with heterogenous appearances due to calcification, hemorrhage and necrosis.7GBM happen rarely in the posterior fossa and present as a heterogenous mass with surrounding edema and rim enhancement of the thick irregular wall on MRI.9Hemangioblastoma is the most common primary posterior fossa tumor in adult that occur in 80% of cases in the cerebellum. Although the imaging features are characteristic, other cystic-solid cerebellar lesions such as juvenile pilocytic astrocytoma (JPA), ganglioglioma, medulloblastoma, ependymoma, metastases, and glioblastoma multiformis (GBM) should be considered in the differential diagnosis.2 Considering surgery for hemangioblastomas are made according to the neurologic signs and symptoms related to the anatomic location of the tumor. Symptomatic patients and asymptomatic ones with enlargement of solid lesions and development of cyst on follow-up imaging have benefited from surgery.2 The prognosis is usually very good and most cases do not develop any long-term neurologic sequela; however, partial removal of the tumor, multiple hemangioblastomas, and young age at the time of diagnosis are associated with higher rate of recurrence.2Surgical resection is a standard treatment for hemangioblastoma, and for large lesions preoperative embolization may be helpful. In patients with incomplete resection of the tumor, adjunctive radiotherapy can be used.In conclusion, it is essential to look for characteristic features of hemangioblastoma in every patient presented with solid-cystic lesion in the cerebellar hemisphere, to seek associated abnormalities, and to treat the lesion appropriately as soon as possible to prevent permanent neurologic sequela.


Synovial sarcomas commonly occur in the extremities of young adults. A primary occurrence in the mediastinum is very rare with only a few reported cases in the world literature. This paper is about a 42-year-old male who presented with chest pain and dyspnoea on exertion. Imaging showed an anterior mediastinal mass with adhesions to the lung. Pathological examination of the resected mass showed a biphasic neoplasm with a spindle cell component admixed with gland-like elements. The tumour showed positive staining with cytokeratin, epithelial membrane antigen, and Bcl-2 confirming the diagnosis of a biphasic synovial sarcoma. A wide range of neoplasms, both primary and metastatic, occur in the mediastinum, which pose considerable diagnostic difficulties. A synovial sarcoma should always be considered in the differential diagnosis, and immunohistochemistry is an important adjuvant tool in this situation. This paper highlights the importance of recognizing an unusual presentation of this aggressive neoplasm to aid appropriate clinical management. Synovial sarcoma is a distinctive malignant soft tissue neoplasm that commonly occurs in the extremities of young adults. Although frequently associated with joints, tendons and bursal structures, it is now believed that this tumour does not originate from synovial cells as originally postulated. In fact, these tumours show epithelial differentiation and are thought to be derived from pluripotent mesenchymal cells capable of epithelial differentiation . Uncommonly, synovial sarcomas have been reported from rare sites such as head and neck region, abdomen, thoracic cavity, and mediastinum. We have described here a case of biphasic synovial sarcoma occurring primarily in the mediastinum. The mediastinum is a rare site for occurrence of a primary synovial sarcoma with only a few case reports and series published in the literature till date. A wide range of tumours occur in the mediastinum, and the differential diagnosis of these tumours can be quite challenging. A 42-year-old man presented with symptoms of right-sided chest pain, not related to physical activity or exertion. He also complained of shortness of breath on exertion. There was no history of cough, fever, or weight loss. Physical examination did not reveal anything significant. His haematological parameters were normal. The chest radiograph (PA view) showed a well-defined radio opaque shadow with no calcification or cavitations with its medial border merging with the right border of the heart, which was suggestive of an anterior mediastinal mass . Lateral The gross specimen consisted of about 250 gm of grey white soft tissue with extensive areas of necrosis. Also received was a lobe of lung measuring 9 × 9 × 2 cm. On microscopic examination, a biphasic neoplasm was noted with well formed glandular epithelial structures admixed with a spindle cell component . The spi On immunohistochemistry, the glandular component showed cytoplasmic positivity for the epithelial markers cytokeratin and epithelial membrane antigen (EMA). The spindle cell component stained strongly with vimentin and bcl-2 . The tumSynovial sarcoma is a rare and aggressive malignant soft tissue tumour occurring most commonly in the extremities of young adults. Other sites from which this tumour has been reported include the lung, pleura, chest wall and very rarely, the mediastinum –6. Only Witkin et al. for the first time in 1989 reported 4 cases of biphasic synovial sarcomas of the mediastinum . A serieThe differential diagnosis of a primary synovial sarcoma in the mediastinum is complex as a wide array of primary and metastatic tumours occur in this site. The biphasic synovial sarcoma must be distinguished from metastatic sarcomatoid carcinoma and biphasic malignant mesothelioma. In a sarcomatoid carcinoma, the spindle cells as well as the epithelial elements show atypical features. The glandular structures in a synovial sarcoma do not exhibit much pleomorphism. Biphasic mesothelioma can show morphological features very similar to a biphasic synovial sarcoma. However grossly, a mesothelioma presents as diffuse plaque like tumour masses on the pleural or pericardial surfaces, whereas a synovial sarcoma presents as a discrete mass in the mediastinum. Our case presented as a discrete mass in the mediastinum and showed adhesions to the pleura. Other spindle cell tumours that commonly occur in the mediastinum like solitary fibrous tumour and malignant peripheral nerve sheath tumour (MPNST) were excluded in this case, as the tumour cells were negative for CD34 and S100 respectively. The gross findings in combination with the morphological and immunohistochemical profile confirmed the diagnosis of a synovial sarcoma in our case. Bcl-2 is the product of a mitochondrial oncogene which inhibits apoptosis. This oncoprotein is found to be expressed in a variety of benign and malignant spindle cell tumours of the skin and soft tissue . Its green bloc resection of the mass.Synovial sarcomas are highly aggressive tumours, and complete surgical excision remains the mainstay of treatment. Radiotherapy is often required to obtain local control of the disease with adjuvant chemotherapy. Adequate pre operative work up such as a fine needle aspirate (FNA) or a needle biopsy might have offered a clue to the diagnosis in this case perhaps leading to better preoperative planning and  In conclusion, synovial sarcoma must be entertained in the differential diagnosis of spindle cell as well as biphasic tumours arising in the mediastinum. This case report emphasizes the importance of prompt clinical suspicion, accurate histopathological diagnosis, and use of appropriate immunohistochemical markers in the diagnosis of this unusual tumour in an unusual site.


Winged scapula is defined as the prominence of the medial border of the scapula. The classic etiopathology of scapular winging are injuries to the spinal accessory or long thoracic nerves resulting respectively in trapezius and serratus anterior palsy. To the best of our knowledge, there are only few reports of scapular lesions being mistaken for winging of the scapula. We report a rare case of a large scapular osteochondroma arising from the medial border and causing a pseudowinging of the scapula.A 17-year-old Caucasian boy came to us complaining about a winged left scapula. The patient had a complete painless range of motion, but a large hard bony swelling was palpable along the medial border of his left scapula. A grating sensation was felt when his arm was passively abducted and/or elevated causing discomfort. A lesion revealed on X-rays was diagnosed as an osteochondroma of the medial border of his scapula. After preoperative examinations, he underwent open surgery in order to remove the lesion. A histological examination confirmed the clinical diagnosis of osteochondroma. A clinical examination 3 months later showed a full and painless range of motion, the absence of the grating sensation during passive abduction and elevation and the complete disappearance of his left shoulder deformity. After 2 years of follow-up, there were no clinical or radiological signs of recurrence.Despite its rarity osteochondroma should be considered in the differential diagnosis for any adolescent presenting with a winging of the scapula. Winged scapula is defined as the prominence of the medial border of the scapula . It is oA 17-year-old Caucasian boy presented with a deformity of his left scapula which developed gradually over a period of 5 months. He did not complain about pain or any other symptom. He did not undergo any course of pharmacological or physical therapy. There was no history of trauma, and family history was not contributory. A clinical examination of his shoulder showed an important asymmetry of his scapulae with his left side elevated from his thoracic cage Figure . The preOsteochondroma is the most common benign bone tumor, representing 15% of all bone tumors  and 45% This case report describes a rare presentation of an osteochondroma leading to a pseudowinging of the scapula and it highlights the importance of including this kind of pathology in the differential diagnosis for any adolescent presenting with a winged scapula.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.The authors declare that they have no competing interests.CC conceived, conceptualized and reviewed the manuscript. VF and GI wrote the manuscript and reviewed the existing literature on the topic. RP and RD contributed to the surgery and elaborated the clinical figures. VP and CDR performed the histological examination of the osteochondroma and elaborated the histological figures. All authors read and approved the final manuscript.


Iatrogenic right diaphragmatic hernia is very rare. We report the first case of a patient who had a diaphragmatic hernia after laparoscopic fenestration of liver cyst. A herniorrhaphy of the diaphragmatic defect was carried out after reducing the herniated organ. The postoperative course was uneventful. Diaphragmatic hernias are not as common as the traumatic type. Surgeons can easily miss diaphragmatic injuries during the operation especially after laparoscopy. Late diagnosis of iatrogenic diaphragmatic hernias is frequent. Ct scan is helpful for diagnosis. Surgery is the treatment of diaphragmatic hernia at the time of diagnosis, even with asymptomatic patients. The incidence of iatrogenic diaphragmatic hernia after surgery may be reduced if the surgeon checks for the integrity of the diaphragm before the end of the operation. A review of the literature is also performed regarding this rare complication. Liver cysts are benign congenital malformations resulting from isolated aberrant biliary ducts . LaparosA 61-year-old female with a past medical history of laparoscopic fenestration, one year ago, of a huge right liver benign cyst Figure  and 3. LSurgery is the mainstay of therapy in benign liver cyst. Lin and al  first deThe diaphragmatic hernia is a herniation of abdominal structures within the thoracic cavity. It can be either congenital or acquired. Diaphragmatic acquired defects are most commonly traumatic in origin, followed by iatrogenic lesions and spontaneous defects . These aA late presentation of a iatrogenic hernia diaphragm was reported in 5%–62% of cases in different series, with the longest reported delay of 35 years .Grasping instrument and electrocautery and dissection near of the diaphragm may cause diaphragmatic injuries after surgery. The energy generated by these instruments especially the ultrasonic activated scissor can provoke a weak point in the diaphragm .That’s why surgeons must be careful handling the instruments, thermofusion and ultrasonic dissector during laparoscopy ,19. A smPatients with large diaphragmatic defects can have critical problems shortly after surgery due to cardiorespiratory disturbances. Unexplained pain in post operative is not specific but should suspect this complication. Other patients may be asymptomatic or have vague symptoms, which may delay the diagnosis. Our patient presented pain one year after the first surgery. The diagnosis of a cyst recurrence was suspected firstly but not the diagnosis of a diaphragmatic hernia.The clinical features are usually chronic symptoms such as upper abdominal and lower chest pain, nausea, dyspnea, and reflux after meals, which may develop into an acute presentation with severe epigastric pain, vomiting, and intestinal obstruction ,19.The radiological diagnosis is often complex and includes several imaging modalities . Chest rSome authors think that the thoracotomy is the elective surgical approach that can correct anatomical restoration of the chest and abdominal cavity especially when it is the approach during the initial surgical procedure -24. ThouTreatment by laparoscopy is feasible with a shorter length of stay. This approach is especially used in left diaphragmatic hernia repair ,26.Because of liver bulk, right side hernia is not amenable to laparoscopic repair, with a high level of conversion. However some authors described this approach with success . In our Herniated contents are reduced, the muscular defect is treated and an endothoracic drain is placed . In someThe majority of these defects can be repaired safely with non-absorbable sutures without the need for a prosthetic mesh ,28. WithIatrogenic herniation of abdominal contents after laparoscopic fenestration of liver cyst is a rare complication. Iatrogenic diaphragmatic injury can be missed during surgery. Surgeon must take precaution to avoid it by precise dissection when using the instruments during surgery. The incidence of iatrogenic diaphragmatic hernia after surgery may be reduced if a final look of diaphragm is systematically realized at the end of each laparoscopic operation.Written informed consent was obtained from the patient for publication of this case report and accompanying images.CT: Computed tomography.All Authors does not have any financial relationship with any organization. No benefits in any form have been received or will be received from a commercial party related directly or indirectly to the subject of this article. All authors have the full control of all primary data and that they agree to allow the journal to review their data if requested. All authors contributed to the realization of this manuscript. The authors declare that they have no competing interests.All of the authors were involved in the preparation of this manuscript.MS write the manuscript coordinated the team, and helped in literature research. HM was an assistant surgeon and made substantial contributions to conception and design. YPL performed the operation and edited the final version of the manuscript. All authors read and approved the final manuscript.


Introduction. Chronic constipation is common in the elderly, and often no underlying pathology is found. Primary colonic dysmotility has been described in children but is rare in the elderly.  Case report. We present an 82-year-old female with long standing constipation presenting acutely with large bowel obstruction. Laparotomy and Hartman's procedure was performed, and a grossly distended sigmoid colon was resected. Histology revealed a primary myopathic process.  Conclusion. Primary colonic myopathy should be considered in elderly patients presenting with large bowel obstruction and a long preceding history of constipation, particularly when previous endoscopic examinations were normal. Primary colonic dysmotility secondary to a myapothic process has been described in children presenting with chronic constipation , 2. OnlyThis 82-year-old female presented with gross abdominal distension and lower abdominal pain as an emergency. Her normal bowel habit was that of longstanding constipation, but she had loose stools over the preceding week. Accompanied with this, she had a six-week history of nonspecific malaise, loss of appetite, and dizziness.She had previously been admitted to a hospital 13 years ago with a 5-month history of abdominal distension, constipation, and faecal incontinence. Abdominal X-ray revealed gross faecal impaction which was managed successfully with phosphate enemas. Follow-up flexible sigmoidoscopy and biopsies revealed nonspecific colitis and melanosis coli.Past medical history included hypertension and impaired glucose tolerance. Past surgical history was a previous caesarean section many years ago.9/L.On examination, the patient was tachycardic (HR 115), hypotensive (BP 80/44), and tachypnoeic (RR 24). She had a nontender, grossly distended abdomen with an indentable mass in the lower left quadrant. Blood tests revealed acute renal failure  and microcytic anaemia . CRP was 55 mg/L, and WCC was 9.1 × 10CT scan revealed massive dilatation of the sigmoid colon with faecal material within, measuring up to 17.9 cms in diameter, and no evidence of mechanical obstruction or perforation , that isAt laparotomy, she was found to have massive colonic dilatation of the sigmoid and descending colon , but theHistology from the resected specimen revealed that the longitudinal muscle layer was degenerate and absent in many areas of the specimen. The degenerate areas consisted largely of elastosis. The myenteric and submucosal plexuses were degenerate where the longitudinal muscle is absent. Accompanied with this, CD117 staining for the interstitial cells of Cajal was reduced. Also, polyglucosan inclusion bodies were present throughout the longitudinal and circular layers of the muscularis propria, with almost total degeneration of the longitudinal layer, suggesting a myopathic process. The occurrence of longitudinal loss was in areas where the overlying muscle and mucosa were intact indicating that this was likely to be a primary myopathy. There was also a replacement of myocytes in both muscularis layers with elastosis and fibrosis. Additional areas of elastosis were found in the submucosa, with widespread smooth muscle metaplasia, thought to be a secondary characteristic of disordered motility.This patient presented acutely with large bowel obstruction but had been suffering for many years from chronic constipation. Whilst it is rare for a patient to present in this way, chronic constipation in the elderly is a common and important problem. Understanding the aetiology may provide useful therapeutic strategies in the future .Primary colonic myopathy should be considered as a potential diagnosis in elderly patients presenting with large bowel obstruction particularly when associated with a longstanding history of constipation as evidenced by this case. Endoscopic examination and biopsies may be unhelpful as the pathology lies in the muscularis layer of the colonic wall, and this diagnosis must be borne in mind when normal mucosal biopsies are seen.


Endometriosis is defined as the emergence and growth of endometrial tissue (glands and stroma) outside of the uterus. It affects 8–15% of the population and is most frequently found in the ovary. Extrapelvic implantation may occur in any organ or tissue, but is rare in the abdominal wall. When it does occur there, a history of surgery, whether laparotomy or laparoscopy, is usually present.Malignancy of this type of endometriosis in an abdominal wall scar may occur from just a few months until up to 18 years after surgery . The casWe herein present the case of a 49 year-old patient with a history of appendectomy, two births, a cesarean section, and removal of the endometriotic mass in the cesarean scar, 15 years before.The patient was seen for her routine annual gynecologic examination, in which she reported the presence of a painful abdominal mass, which had increased in size during the previous few months. She described the pain as cyclical, corresponding to her menstrual cycles, and that it reminded her of the lesion for which she had previously been operated on. Her check-ups prior to this consultation had been normal and no abdominal nodule had been observed.A physical examination revealed a mass located in the midline of the abdominal wall, 3 cm above the Pfannenstiel scar. The size of the lesion was roughly 6 cm × 6 cm, and it was painful upon movement and was Fothergill sign positive. The rest of the gynecological examination was normal.Ultrasound scan and mammography were normal. Soft tissue ultrasound  revealed a lesion measuring 70 × 60 mm located in the abdominal wall, confined within the mesogastrium, caudal to the umbilical region, compatible with fibrous soft tissue tumor (desmoid tumor). Due to the history of the patient we were not able to rule out endometriotic implants. Fine-needle aspiration was performed with pap smear revealing a benign process compatible with endometriosis .Tumor marker levels were: CA-125: 243 U/mL and CEA: < 0.5 ng/mL. With a diagnosis of abdominal wall endometriosis, surgery was scheduled for removal of the wall tumor and exploration of the abdominal cavity.The intraoperative pathology of the abdominal wall nodule revealed a mass located at the preperitoneal level with histopathologic result of serous papillary adenocarcinoma . During The case was evaluated by the Gynecology and Oncology Committee, which decided that an extension study with a PET/CT was to be performed. Two hypermetabolic images were observed, one at the level of the abdominal wall mass and another one in the splenic flexure. Surgical laparoscopy was performed with removal of the splenic flexure, which was reported as steatonecrosis with chronic inflammation. The fibrotic area in the abdominal wall mass was also resected, with the pathology providing a benign result as well.Subsequently, adjuvant treatment was administered with 6 cycles of carboplatin and Taxol, plus quarterly follow-ups for clinical examination and laboratory tests (CA-125), and semi-annual follow-ups for radiological tests. After a 48-month follow-up the patient is disease-free.The probability of developing endometriosis in a surgical scar is roughly 0.03–1%  and maliAbdominal scar endometriosis presents as a mass in the abdominal wall, usually adjacent to a scar from previous surgery, and is painful, with or without increased size of the scar. The abdominal pain described by the patient is usually cyclical, correlating with her menstrual cycles, if hormonal reserve is still present .Surgical history must be taken into account for the proper diagnosis. Imaging techniques can also be useful. As a first choice, soft tissue ultrasound is recommended, complementing it with ultrasound-guided fine-needle aspiration of the mass. When there is a suspected diagnosis, as in our case (due to the history of the patient), serum levels of CA-125 are requested. Non-invasive diagnostic tests have a very low sensitivity in such cases.In spite of all these tests, the definitive histopathological diagnosis was serous papillary adenocarcinoma.The differential diagnosis with palpable masses in the abdominal wall should include: hernia, hematoma, lymphadenopathy, lymphoma, lipoma, abscess, subcutaneous cyst, neuroma, soft tissue sarcoma, desmoid tumor or metastasis.The most frequent extragonadal sites of malignant endometriosis are (> 50% of the cases): the rectovaginal septum, the colon and the vaginal wall . In our The most interesting aspect of the case is the nodal metastases, since it is the first case described in literature. Being a preperitoneal tumor, the nodal metastases follow the pattern of tumor spread that affects the abdominal wall muscles. When the primary lesion is located in the infraumbilical region, these metastases drain to the superficial inguinal chains. When the lesion is in the supraumbilical region, nodal metastatic spread may drain to the axillary chains.CA-125 levels in the blood might be slightly elevated however it is a less specific biomarker. Many studies have evaluated different biomarkers for predicting or excluding endometriosis.Recently, one interesting study showed that CRP serum levels were significantly higher in patients affected by endometriosis than in healthy patients. Similarly, other studies have shown that patients with mild endometriosis had lower anti-Müllerian hormone levels compared to patients without disease.Another interesting and innovative study demonstrated an increase in follistatin serum levels in patients with endometrioma when compared to patients affected by other types of benign ovarian cysts.Studies are also underway regarding other probable and more specific biomarkers of ovarian carcinoma and endometriosis, such as the HE4 protein (human epididymis protein 4) combined with CA-125.Evidently, surgery is the definitive therapeutic approach. In our case, the patient was scheduled for surgery under the assumption of a benign diagnosis  in order to resect the mass and thoroughly examine the abdominal cavity.The majority of carcinomas derived from endometriosis are of endometrioid or clear cell histologic type, in some series accounting for 70% of the subtypes . Our casSeveral molecular factors have been found to be involved in the association of endometriosis with ovarian cancer. They include: p53 alterations, PTEN silencing, K-Ras mutations and even expression of HNF-1β . It's known that the micro-environments of endometriosis and endometriosis associated ovarian cancer share similar cytokines and mediators. Whether this similarity represents a link between endometriosis and endometriosis associated ovarian cancer or simply an employment of common signaling molecules to two separate lesions remains to be seen . The molOne hypothetical model describing the pathogenesis of malignant transformation of endometriosis is from the Japanese Society of Clinical Oncology, which examines three simple and typical mechanisms of carcinogenesis .The first speculates that the endometriotic epithelium serves as a precursor of cancer in the same way that the surface epithelium of a normal ovary gives rise to ovarian tumors and contributes to the emergence of epithelial tumors, epithelial–stromal tumors, both, or borderline tumors.The second mechanism refers to similarities in the carcinogenic process of endometrial neoplasia, which, under the influence of unopposed estrogens, basically leads to an endometrioid tumor.The third describes the idea that this carcinogenesis is strongly influenced by the microenvironment within the endometriotic lesion and that continued exposure to oxidative stress and inflammation promotes a resistant and slow-growing phenotype of malignant tumors such as clear cell carcinoma.Although this hypothesis may be simplistic and require further validation, it remains a useful model of preliminary carcinogenic mechanisms in endometriosis .These patients cannot be treated in the same way as those with ovarian neoplasia, since this is not the lesion to be treated, nor does it have the same dissemination mechanism. The literature primarily describes surgical treatment, the surgical removal of the disease, while ruling out metastasis in possible organs or epithelia at risk. For this reason we decided to remove all the macroscopically visible disease along with the omentum, both ovaries and an endometrial biopsy.As for the adjuvant therapy, the patient was treated with carboplatin and Taxol because serous papillary histologies tend to respond to this combination of chemotherapy.This is a unique case since no other well-documented cases of malignant transformation of endometriosis with preperitoneal lymph node metastasis have been described in literature. Because this is such a rare etiology, there is very little experience in the management of these types of patients to help guide us in considering an ideal treatment strategy. Further molecular studies are needed to shed light on the mechanisms of the pathogenesis of this disease. Nevertheless, we are certain that the proper treatment of endometriosis, or the monitoring of these patients, can either prevent the very occurrence of a malignant lesion or allow for diagnosis and treatment at an early stage.The authors declare that there is no conflict of interest.


Intussusception is rarely encountered in adults, accounting for just 5% of all occurrences and 1% of bowel obstructions. In up to 90% of episodes of adult intussusceptions, operative intervention is required secondary to pathological lead points. Prior to the current report, only three cases of total ileocolic intussusception with rectal prolapse in adults have been described in the world literature, making it an important contribution to surgical knowledge. In addition to a discussion of disease etiology, this review outlines sound diagnostic and therapeutic principles in the successful management of this rare emergent surgical condition.zygosis during the embryological period, in which there is persistence of the ascending and descending mesocolons and lack of apposition to the retroperitoneum. The diagnostic work-up, operative strategy and pathological findings are discussed. The three previous cases reported in the English-language medical literature were reviewed.In this case report, we will present a rare case of total ileocolic intussusception with rectal prolapse in a 22 year-old female without antecedent history. She had both a lead point in the cecum, as well as a highly mobile, intraperitoneal colon. Lead points have been found in only half of the reported cases, including this one. In addition, colonic laxity may enable this phenomenon, being attributed to the loss of Adult intussusception, while uncommon, may be encountered in an acute surgical setting and optimal outcomes depend on a high index of suspicion and expeditious management. Embryological divergence may contribute to the even rarer variant of total ileocolic intussusception with rectal prolapse. Intussusception in adults is rare, representing 1% of bowel obstructions and 5% of all intussusceptions . While iA 22 year-old female with history significant only for anemia and no previous surgical history or family history of malignancy complained of abdominal pain and bleeding per rectum. At an outside facility, she was diagnosed with new-onset rectal prolapse which was reduced prior to presentation to our emergency department. On physical examination she was found to have abdominal tenderness without peritoneal signs and evidence of fresh blood on rectal exam without hemorrhoids or prolapse. Rigid proctoscopy confirmed bloody mucosal tissue without a clear source of hemorrhage and no evidence of ischemia. Laboratory values were unremarkable and abdominal films revealed a small bowel obstructive pattern with a paucity of identifiable gas in the colon. Figure  ComputedThe CT scan was concerning for total ileocolic intussusception to the level of the rectum with possible compromised bowel. The patient was brought to the OR for an urgent exploratory laparotomy. The distal small bowel was invaginated into the colon throughout its entire length and could be palpated in the upper rectum Figure . The patWhile abdominal pain, nausea and emesis were the predominant presenting symptoms in 78% of patients in one large institutional series, the preoperative diagnosis was correct in only 33% in this same cohort .The wideIntraoperative strategy in adult intussusception generally favors resection without reduction in adults given the high preponderence of tumoral lesions as lead points. As detailed in this case report, extensive intussusceptions involving the right colon may be selectively considered for careful distal to proximal manual reduction before definitive resection in order to avoid a more extensive resection or two-stage procedure . This shInterestingly, ileocolic intussusception presenting with rectal prolapse is exceedingly rare in adults, with only three cases previously reported in the English-language world literature Table  3-5]. D-5. D3-5]Chen and colleagues describe this presentation in a 36 year-old male who presented with an initial two month history of diarrhea followed by constipation and abdominal pain. While straining to defecate, a mass prolapsed from his anus and he presented for evaluation. His prolapsing mass was reduced but did not relieve his abdominal pain. Barium enema confirmed a filling defect in the sigmoid colon without proximal filling of the colon. At laparotomy, ileosigmoid intussusception was confirmed and could not be reduced, resulting in a subtotal colectomy. Pathology confirmed a 9x6x5 cm benign lipoma at the ileocecal junction .Ongom and colleagues describe an ileocolic intussusception in a 32 year-old female who initially reported colicky abdominal pain and vomiting, associated with straining during defecation and incomplete evacuation of her rectum. Over the next two weeks prior to presentation, she noted continued colicky abdominal pain, bloody-mucoid discharge and a reducible mass protruding from her anus. On physical examination, an abdominal mass was palpated in the umbilical region and rectal mass noted 3cm proximal to the anal verge. Abdominal ultrasound confirmed the presumptive diagnosis of prolapsed intussusception with partial bowel obstruction. The mass was only able to be partially reduced in a distal to proximal direction and a subsequent right hemicolectomy was performed. The authors noted absence of hepatocolic and splenocolic ligaments and lack of retroperitoneal fixation. Although pathology was negative for neoplasm, they theorized the lack of zygosis with persistent ascending and descending mesocolons helped to enable this presentation ,9 and inThus, two principle factors are causative in this case presentation of total ileocolic intussusception with rectal prolapse. The first being the lead point pathology of the villous adenoma, and the second being the increased colonic mobility associated with lack of zygosis.Intussusception is an uncommon etiology of bowel obstruction in adults and can be attributed to benign and malignant pathologies. Despite advancements in diagnostic accuracy, a high index of suspicion and clinical acumen is required for timely diagnosis and therapy of this condition in adults. Total ileocolic intussusception with rectal prolapse, found at the end of the adult intussusception spectrum, may be predisposed by an embryological variant lacking zygosis. For the acute care surgeon who may encounter this rare surgical emergency, the diagnosis should be considered in the differential of a prolapsing rectal mass and be expeditiously managed to optimize patient outcomes. Assessing for the absence of zygosis should be an adjunct to the operative procedure as well.Written infromed 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.The authors do not have any financial or non-financial competing interests to declare.Study concept and design: JF, OB & YK. Acquisition of data: JF, OB. Analysis of data: JF, OB & YK. Drafting of manuscript: JF. Critical revision of manuscript: JF, YK. Study supervision: YK. All authors read and approved the final manuscript.


Osteosarcoma metastasis causing intussusception is a very rare entity, with a pejorative prognosis. Based on a case, we performed a literature review in order to better assess this situation. We conclude that, in patients with a history of osteosarcoma lung metastasis, echographic and/or computed tomography scan evidence of a small bowel obstruction with intussusception should lead to an open surgical procedure if the laparoscopic approach does not allow to accurately explore and resect the lesion, in order to prevent misdiagnosis and to avoid further delay in the management. Patient management of children with metastatic osteosarcoma has improved dramatically over the last few decades. This is thanks to an effective multidisciplinary approach associating multiple regimens of chemotherapy with surgical resection of primary and secondary lesions. Indeed, patients are living longer, and typical tumour evolution has changed. Hence physicians are having to deal with the involvement of different organs, even though such cases were considered exceptional in the 20th century ,2. MetasA 17-year-old female patient was admitted having suffered 2 days of abdominal pain. She was in the 5th year of follow-up after an osteogenic osteosarcoma of the distal right femur. The primary tumor had been treated with three courses of induction chemotherapy using doxorubicine, methotrexate and cisplatin (following the AOST 0331 protocol) before she had undergone an amputation of the upper femoral shaft. Over the following years, she required two clam-shell thoracotomies for bilateral lung metastasis, along with chemotherapy . The most recent intravenous treatment was administered 2 years ago; the last thoracic surgery was performed 36 months ago.The patient was admitted for mild abdominal pain; there was no vomiting or weight loss. Clinical examination revealed a slight tenderness of the right lower abdomen, without symptoms of intestinal obstruction. An initial ultrasound demonstrated the presence of an ileo-ileal intussusception Figure A. After The initial postoperative course was uneventful until the 4th day, when an acute bowel obstruction occurred. A nasogastric tube was placed. The persistence of symptoms led to another CT scan Figure B that reAn open surgery was performed as the previous attempt at laparoscopy had failed to demonstrate the cause of the intussusception. The intussusception was easily found, but was irreducible due to a focal lesion of the intestine wall. A resection with end-to-end anastomosis was performed. The postoperative course was uneventful. Histopathology confirmed the metastasis from the initial tumor Figure ., such as carcinomas or lymphomas, whereas others are secondary and rare. The characteristic origins of these metastases are lobular breast cancer, lung cancers, renal carcinomas and melanomas [Intussusception is frequent and typically occurs in young children; it is mainly idiopathic or due to the reaction of lymphatic mesenteric nodes . Less frelanomas . Osteosaelanomas . Charactelanomas . Once thelanomas . In our elanomas . It has elanomas . In thesWhatever the management of this patient, the prognosis is poor according to previously reported cases. This is probably due to the simultaneous or previous presence of lung metastasis in all cases, as reported by Horiuchi and colleagues . A seconOsteosarcoma metastasis causing intussusception is a very rare entity, with a pejorative prognosis. In patients with a history of metastatic osteosarcoma of the lungs, the presence of a small bowel obstruction, together with echographic and/or CT scan evidence of intussusception, should lead to a manual exploration eventually associated with a preliminary laparoscopic approach, because of the need for a meticulous palpation of the lesion, potential resection, and the risk of misdiagnosing this entity.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.CT: Computed tomography.The authors declare that they have no competing interests.All authors have been involved in the management of the patient and in the conception of the manuscript. OA, KP and JMJ have been involved in drafting the manuscript or revising it critically for important intellectual content. All authors read and approved the final manuscript.


Pancreatic adenocarcinoma is extremely rare in childhood. We report a case of metastatic pancreatic adenocarcinoma in a 13-year-old boy, revealed by jaundice.A 13-year-old Moroccan boy was admitted with obstructive jaundice to the children's Hospital of Rabat, Department of Pediatric Oncology. Laboratory study results showed a high level of total and conjugated bilirubin. Computerized tomography of the abdomen showed a dilatation of the intra-hepatic and extra-hepatic bile ducts with a tissular heterogeneous tumor of the head of the pancreas and five hepatic lesions. Biopsy of a liver lesion was performed, and a histopathological examination of the sample confirmed the diagnosis of metastatic ductal adenocarcinoma of the pancreas. Our patient underwent a palliative biliary derivation. After that, chemotherapy was administered (5-fluorouracil and epirubicin), however no significant response to treatment was noted and our patient died six months after diagnosis.Malignant pancreatic tumors, especially ductal carcinomas, are exceedingly rare in the pediatric age group and their clinical features and treatment usually go unappreciated by most pediatric oncologists and surgeons. Beyond infancy obstructive jaundice is rarely encountered among children , and theA 13-year-old Moroccan boy was admitted with obstructive jaundice to the Children's Hospital of Rabat, Department of Pediatric Oncology. He had an illness of two months' duration that presented with jaundice, weight loss, melena and hematemesis. On physical examination, our patient had jaundice with hepatosplenomegaly. Laboratory study results showed a total bilirubin level of 26 mg/dL with a conjugated bilirubin of 18 mg/dL , aspartate aminotransferase and alanine aminotransferase levels of 220 and 250IU/L, respectively , and a hemoglobin level of 9 g/mL. Abdominal ultrasonography showed a dilatation of intra-hepatic and extra-hepatic bile ducts and hepatomegaly. Computerized tomography of the abdomen showed a dilatation of intra-hepatic and extra-hepatic bile ducts with a tissular heterogeneous tumor of the head of the pancreas measuring 49/37 mm Figure  and fivePancreatic tumors in childhood most commonly present with a palpable mass or with pain . JaundicMalignant pancreatic tumors especially ductal carcinomas are exceedingly rare in the pediatric age group and their clinical features and treatment usually go unappreciated by most pediatric oncologists and surgeons.Written informed consent was obtained from the patient's next-of-kin 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.The authors declare that they have no competing interests.All authors have made significant contributions by making the diagnosis and through intellectual input in the case management and writing the manuscript.


Localized Nodular Lymphocyte Predominant Hodgkin Lymphoma is a rare disease with an overall good prognosis but frequent late relapses. Due to it's rarity there is no standard therapeutic approach and pathological diagnosis may be hard. In this paper we discuss the technical aspects of the radiation therapy and histological issues. The new fields reductions proposed for classical Hodgkin lymphoma cannot be applied to early stages Nodular Lymphocyte Predominant Hodgkin lymphomas which are usually treated with radiation therapy without systemic chemotherapy. In this paper we describe two cases of failure in the management of nodular lymphocyte predominant Hodgkin lymphoma (NLPHL). Both treated outside our center, they were referred back to our unit for early relapse after “involved node” radiotherapy (INRT).NLPHL is a rare disease  with distinct histology and clinical course. Despite frequent late relapses, NLPHL have paradoxically an overall good prognosis. Due to the rarity of the disease there is no standard therapeutic approach. In this short paper, we discuss the technical aspects of the radiation therapy and histological issues correlated with these two early relapses.The patient is a 53-year-old male who presented a submaxillary right tumefaction (IIA area).The lymph node biopsy (June 2011) demonstrated NLPHL. The Ann Arbor classification was stage IA.The PET scanner confirmed the unique uptake in the cervical area IIA.An INRT started in August 2011 and delivered 36 Gy within 18 fractions using conformational technique and was well tolerated .During the followup the evaluation was assessed using PET scanners. The first PET scanner (January 2012) showed a complete response in the IIA area but a suspicious uptake in the subclavicular area (outside treated fields), confirmed as a relapse on the PET of July 2012 .The biopsy of this early recurrence (5 months) demonstrated a relapse of NLPHL, confirmed in our hematological malignancies expert laboratory. The performance status (PS) of the patient was still 0; biology and clinical examination were normal.The patient is a 46-year-old male who presented a left cervical (III area) tumefaction.After biopsy he was diagnosed with a NLPHL (October 2011) confirmed after a second analysis in an expert center outside our institution.The Ann Arbor classification was stage IA. The initial PET scanner showed a unique left jugular-carotid fixation.An INRT started in January 2012 and delivered 36 Gy within 18 fractions using conformational technique .During the followup the evaluation was assessed using PET scanners.The first PET scanner (October 2012) showed a unique FDG uptake in the IIB territory (outside treated fields), confirmed on a 2nd PET in November 2012 .The biopsy of this early relapse (9 months after treatment) revealed a classical Hodgkin lymphoma (cHL). That unusual histological transformation led to a third analysis of the initial histological specimen in our expert center. The conclusion was in favor of a misdiagnosed lymphocyte-rich cHL, based on the positivity of CD30 and inconstant positivity of CD20.Those two cases of early failure (treated in 2 different centers) raise two problems in the management of NLPHL: first, the choice of the irradiation technique and, then, the difficulties of the histological diagnosis.The natural history of NLPHL is very different from cHL's, and an extrapolation from one to another for both clinical and therapeutic features would be hazardous.Nogová et al.  describeP = 0.0092) and overall survival  which the authors attribute, in part, to the earlier stage at presentation of NLPHL.In this large GHSG studythe authors evaluated treatment outcomes for NLPHL versus cHL with a median followup of 50 months. The NLPHL patients experienced significantly better freedom-from-treatment failure  and more late relapse  justifying a long followup.Concerning the timing of the relapses, they reported less early relapse . Median time to relapse (MTR) was 3–6 yrs in the review of the literature reported by Lee and LaCasce –6 and evTo sum up NLPHL is an indolent disease with late relapses requiring a long followup.The toxicity of treatments concerning both extended field RT (EFRT) and chemotherapy was studied in several papers , 5, 9–11Based on these observations, the management of NLPHL has progressively evolved to less aggressive modalities, as for the treatment of cHL , 14.However, no randomized trial has been conducted comparing EFRT versus in-field radiotherapy (IFRT) among patients with favorable cHL. IFRT was adopted as the standard arm in many European trials .As previously mentioned, radiation therapy in the treatment of NLPHL has evolved from EFRT to IFRT , 10, 15.A recent study (H10 EORTC/GELA) proposes a further reduction to INRT for cHL with a good response on a PET scanner after 2 courses of chemotherapy, selecting by this way patients with a good prognosis. Involved fields are described in a paper from Girinsky et al. .The choice of that technique was based on the concept that chemotherapy is effective for microscopic disease. Therefore larger fields would not be necessary anymore.Moreover the advent of the PET scanner enables a better identification of the pathological nodes.The last justification was that radiations complications were related to the irradiated volume and total radiation dose , 10, 12.Assuming similarities between cHL and NLPHL, some teams would apply that deescalation to the management of NLPHL, especially knowing that the prognosis was even better.But there is no rational in the literature for using this “involved node” technique for the treatment of localized NLPHL without chemotherapy. The usual treatment is IFRT alone.This technique (INRT) has probably been chosen in order to reduce toxicity in front of a good prognosis disease. For a cervical irradiation we care about salivary sparing even if the dose used for NLPHL does not induce late effects on the parotids. The dose commonly used is 30–36 Gy based on what is done for cHL, but no dose study exists for NLPHL.Nevertheless INRT has never been described for NPLHL: it is an option for cHL treated with combined modalities.In those 2 cases the use of INRT has led to early relapses in sites which would have been irradiated in an IFRT technique. Two aspects of these relapses are relevant.Firstly the timing of the relapses is striking (5 and 9 months): it is far earlier than reported in literature –7. This Secondly their location would have been included in an in-field radiation Figures  and 4. TIndeed, most of the literature describes, when done, out-field recurrence after IFRT –7, 9 conWe have reported in our 2nd case a revision of the histological initial diagnosis in light of an unusual anatomopathology of the relapse.Transformation may occur in the natural history of NLPHL , 18. ButThat outcome highlights the necessity of a new biopsy when a relapse occurs.That also reminds us of the real complexity of pathological diagnosis in those border cases.Distinction from cHL, especially from lymphocyte-rich cHL, has been shown to be a very common misdiagnosis (32% of errors in the European Task Force on Lymphoma study ). The diIn our case the relapse was characterized by a lymphocytic proliferation with a nodular architecture. The atypical cells had a large nucleus and the immunophenotype responded CD4+, CD15−, CD21+, low CD20+, CD30+, Bcl6+. The reanalysis of the primary disease sample described atypical cells with large nucleus and lobulated nucleolus, CD30+, and inconstant CD20+.Commonly NLPHL is characterized by the lobulated nature of the nucleus “popcorn” even if some cells may have large nucleoli as the Sternberg cells. If those last cells are too numerous, it raises the possibility of the diagnosis of lymphocyte-rich cHL , 20.The immunophenotypic analysis usually respond CD45+; CD20+, CD79a+ (pan-B markers); bcl6+, CD10−, bcl2−, CD30−, CD15−, EBV−. On the opposite, a cHL would be CD30+, CD15+, CD20+ (for 40%), CD79a− and bcl6− .Our case does not fulfill every criterion; nevertheless the pathologists of the expert center concluded that it was a cHL.There is no evidence for treating localized NLPHL with “involved node” radiotherapy. We have reported here 2 cases of early relapses for such treatment in areas which would have been included in IFRT.The aim of this paper is to warn our community not to undertreat those patients who could be cured by optimal local treatment and to highlight once again the difficulty of the pathological diagnosis such as the necessity of biopsying again relapses and reanalyzing samples in expert centers when possible.


A 70-year-old female presented to the emergency department with a 3-day history of intermittent dysphasia and right facial droop. Computed tomography (CT) and magnetic resonance imaging (MRI) were obtained, and the patient was found to have meningeal carcinomatosis, also known as leptomeningeal metastases. Meningeal carcinomatosis is a rare metastatic complication of some solid tumors and hematopoietic neoplasms, and has a median survival rate of 2.4 months. The role of the emergency physician is to appropriately diagnose this condition, treat emergent side effects, provide symptomatic relief, and ensure multi-disciplinary management. Meningeal carcinomatosis (MC), also known as leptomeningeal metastases, is a rare metastatic complication of some solid tumors and hematopoietic neoplasms . Inciden2 Sat 100% RA. The patient was post-ictal, but became arousable and alert during the initial evaluation. Cardiac exam showed an irregularly irregular rhythm. She had an expressive aphasia with significant weakness to the right upper and lower extremity and right facial droop. The patient was unable to name objects or answer yes and no questions. Laboratory tests were unremarkable. A non-contrast CT head revealed lytic lesions of the skull and an abnormality of the brain. MRI of the brain with contrast showed vasogenic edema in the left frontoparietal region, dural thickening, and lytic/blastic lesions in the skull.A 70-year-old female presented to the emergency department with a 3-day history of intermittent dysphasia and right facial droop. The patient had just returned from an overseas flight the day prior to the onset of symptoms. There was no history of headache, nausea/vomiting, or dizziness. Upon arrival, the patient had a generalized tonic-clonic seizure that responded to benzodiazepines. On examination, vital signs were blood pressure 138/76, P 124, R 23, and OThe approach to the patient with altered mental status includes a broad differential diagnosis including infectious, neurologic, and toxicologic causes. In this case, the presentation and history direct the physician to a neurologic etiology. The CT of the head confirmed the presence of a structural brain abnormality. MRI of the brain confirmed the diagnosis of meningeal carcinomatosis, and the edema finding provided some evidence of a brain tumor as the primary neoplasm.In light of the MRI findings and her seizure activity, she was given an anti-epileptic drug to prevent further seizures and dexamethasone. Dexamethasone has been shown to decrease intracranial pressure and cerebral edema in cases of brain tumors . PatientThis case of meningeal carcinomatosis is somewhat unique in its presentation. The patient was not known to have cancer at the time of her presentation, and her symptoms were focal and stroke-like. It is uncommon for MC to be diagnosed in patients without a previous diagnosis of cancer . In the Although MC is usually a secondary metastatic disease from solid tumors, like cancer of the breast or lung, direct spread from primary CNS tumors is possible . Focal fComputed tomography is 1.5-2 times less specific and sensitive than MRI. Contrast-enhanced MRI is preferred, and larger doses of gadolinium are thought to reduce the false-negative rate [Emergency department management of seizure includes benzodiazepines and AEDs. However, AEDs are not thought to be needed on a prophylactic basis. Treatment of MC-associated headache, neck, and back pain should include analgesics, but may also include steroids. Alternatives to standard analgesics may be appropriate in patients who can be managed as an outpatient. Alternative pain management drugs like amitriptyline, gabapentin, carbamazepine, or benzodiazepines may be prescribed for chronic pain. Acute worsening of headache, neck pain, or back pain could be related to worsening complications, such as obstructive hydrocephalus, edema, or impingement of nerve roots or the spinal cord. Careful exam and history should be used to guide the clinician in determining the need for additional imaging or other diagnostics.Despite intra-reservoir or intravenous chemotherapy, survival is merely 20-23 weeks . ExternaMeningeal carcinomatosis is a malignancy with poor survival rates. The primary sites of this type of metastatic cancer are typically breast and lung, but may include other solid tumors as well as hematopoietic tumors. Most patients diagnosed with MC will already be known to have cancer, but around 9-16% will not, until the time that MC is diagnosed. Despite aggressive chemotherapy and radiotherapy, survival is limited. The role of the emergency physician is to appropriately diagnose the condition and arrange for multi-disciplinary management after stabilization and pain management.Consent was obtained for publication of the details of this case and for publication of associated radiographic images.The authors declare that they have no competing interests.NC participated in the care of the patient and provided case details. DC prepared images, reviewed reports, and performed literature searches. Both DC and NC reviewed the literature and provided authorship of the text of this manuscript.