Chordoma arising from the cervical spine is rare and the traditional long-term prognosis is typically poor. Total en bloc spondylectomy with a wide margin is generally accepted to be the most appropriate management for thoracic and lumbar malignant tumors. However, this method is still challenging for the cervical spine because of the proximity of the tumor to the vertebral arteries and neural elements. Here, we report a 73-year-old man with a C5 vertebral chordoma treated with total piecemeal spondylectomy. Histological examination revealed pathognomonic physaliphorous cells with mucus-filled cytoplasm in the tumor, and the ratio of Ki-67-positive cells within the tumor was high (19.0%), showing active proliferation rate. Local recurrences were found at 9 months, 4 years and 2 months, and 6 years after the initial surgery. All the recurrences were encapsulated and isolated and treated with an additional en bloc resection successfully at each stage. Eight years after the initial total piecemeal spondylectomy, the patient maintained his intact neurological status without local recurrence or metastasis. The prognosis of cervical chordoma depends on the patient's age, surgical procedures, and histological features. In this report, we present that piecemeal spondylectomy is an alternative management for aged patients with cervical chordoma, even for those with high MIB-1 index.  Chordoma is a slow-growing, locally invasive, rare malignant tumor restricted to the axial skeleton that occurs with an overall incidence of less than 1 in 1,000,000 people . The conDepending on the location, chordoma can involve the nervous system. Compression of the adjacent spinal cord can cause neurological symptoms, such as paralysis, ataxia, loss of proprioception, urinary incontinence, and/or constipation . The locTotal en bloc spondylectomy with a wide margin is generally accepted to be the most appropriate management for thoracic and lumbar lesions. However, this method is still technically challenging for the cervical spine because of the proximity of the tumor to the vertebral arteries and neural element. On the other hand, old patients were featured as having significantly longer survival than younger patients with cervical chordoma regardless of the resection methods, either total piecemeal spondylectomy or total en bloc spondylectomy .Here, we report the case of a 73-years-old patient with C5 vertebral chordoma who showed neurological symptoms because of tumor invasion into the neural foramen and canal, compressing the spinal cord and nerve root. We performed total piecemeal spondylectomy and spinal reconstruction. Local recurrences were found at 9 months, 4 years and 2 months, and 6 years after initial surgery. All recurrences were encapsulated and treated with additional resection successfully at each stage. Eight years after the initial total piecemeal spondylectomy, the patient maintains his intact neurological status without local recurrence or metastasis.The patient was a 73-year-old man who was followed up for left shoulder pain for 1 year in another hospital. Magnetic resonance imaging (MRI) was performed because of progression of signs and symptoms, such as backache and lower limb weakness. Muscle weakness level was 4 in the manual muscle test, with slight difficulty of gait and muscle atrophy of the interossei dorsales muscle bilaterally. The patient complained of a moderate glove-and-stocking-type numbness on the four limbs. The MRI scan showed an area of increased signal intensity on T2-weighted images in the C5 region . At discThe pathological diagnosis was chordoma. On pathological examination, the cytoplasm of the tumor cells was acidophil with considerable nuclear pleomorphism . It was Regarding the postoperative course, the patient underwent three additional spinal surgeries, because local recurrences of the initial lesion were found at 9 months, 4 years and 2 months, and 6 years after the first surgery Figures . All thrGenerally, surgical resection with a wide margin is the most appropriate management for sacrococcygeal chordoma . Tomita The present case was treated with total piece meal spondylectomy, because the tumor invaded the neural foramen and spinal canal, compressing the neural element, which resulted in neurological symptoms. The three recurrences might be originated from the contamination of the tumor tissue even after thorough resection macroscopically with MRI confirmation. However, all the recurrences were fortunately located adjacent to the titanium mesh cage, encapsulated without adhesion to the dura. These conditions made the recurrent lesion easy to remove with additional surgery. The prognosis in cervical chordoma depends on not only the surgical procedures, such as total en bloc spondylectomy or total piecemeal spondylectomy, but also the histological features or age of the patient.Microscopic tumor necrosis and MIB-1 index (Ki-67 level of >5%) are histologically associated with a significantly increased risk of local recurrence and metastasis, indicating the impact of certain factors on survival in case of chordoma . The preThe rates of local recurrence and metastasis depend on the quality of the initial surgery, pathological features, and age of the patient. In the present case, tumor resection was performed via anterior and posterior approaches by total piecemeal spondylectomy but not en bloc resection, followed by three additional resections of the recurrent lesions. This resulted in successful spinal stabilization without neurological deficiency, and the patient was able to obtain good performance in activities of daily living without lower and upper limb paralysis, that is, good functional prognosis, at 8 years and 4 months after the initial surgery.Piecemeal spondylectomy is an alternative management for cervical chordoma in aged patient even with high MIB-1 indexes.


Thrombocytopenia is a frequent finding in patients with solid tumors. It is usually caused by bone marrow infiltration or by myelosuppression due to anticancer therapy; however immune thrombocytopenia (ITP) associated with solid tumors is rare. Neuroblastoma is the most common extracranial solid tumor in children. Here we report the case of a two-year-nine-month-old patient with adrenal neuroblastoma who presented with ITP. Paraneoplastic ITP was considered in the differential diagnosis. Bone marrow infiltration and other causes of thrombocytopenia were excluded and the patient was treated with intravenous immunoglobulin and tumor resection. Platelet count increased rapidly after surgery and complete remission of ITP was achieved. Thrombocytopenia is a frequent finding in patients with cancer and is usually caused by bone marrow involvement of malignant cells or by toxicity from anticancer therapy. Rarely immune thrombocytopenia (ITP) is reported at the time of diagnosis. Although this condition could represent incidental finding of two common pathologies, paraneoplastic processes should also be considered. Immune thrombocytopenia has been reported as a paraneoplastic syndrome with several tumors –3. HowevNeuroblastoma is the most common extracranial solid tumor in children . NeuroblHerein, we report a case of neuroblastoma presenting with ITP and we discuss the probability of paraneoplastic thrombocytopenia.A 2-year-9-month-old male patient with a left adrenal mass detected by abdominal ultrasonography was referred to our institution. His chief complaints were paleness, abdominal pain, and back pain of one-week duration. Fever or findings consistent with viral infection, history of vaccination, and drug use during the previous weeks were not reported. His previous medical history and family history were unremarkable.3, mean platelet volume (MPV): 9,1 fL, and white blood cell: 10740/mm3. Lactate dehydrogenase was 392 IU/L ; other laboratory tests including renal and liver function tests, serum electrolytes, prothrombin time, activated partial thromboplastin time, fibrinogen, and ferritin were within normal range. The serology tests for HIV, Hepatitis B, and Hepatitis C were negative.He had mild pallor; his vital signs, growth, and development were normal. Physical examination revealed a mass at the left upper quadrant of the abdomen and several ecchymotic lesions measuring 1 to 1.5 cm in diameter, on the pretibial areas. Hepatosplenomegaly and lymphadenopathy were not detected. Complete blood count (CBC) revealed thrombocytopenia and mild anemia with hemoglobin (Hb): 9.6 g/dl, MCV: 75.8 fL, platelet (PLT): 53000/mmAbdominal MRI was performed. A heterogeneous mass with smooth borders, containing cystic and hemorrhagic areas, measuring 59 × 60 × 74 mm was detected at the left adrenal gland. The tumor did not cross the midline and no vascular invasion, lymphadenopathy, or metastases to other abdominal organs were detected with MRI . NeuroblPatient was admitted to hospital for further evaluation and surgery. Neuron specific enolase was found to be 26 ng/ml ; serum catecholamines were within normal range. Tumor metastasis was not observed in I-131 Metaiodobenzylguanidine and Technetium-99 m scan.3. Bilateral bone marrow biopsy and aspiration were performed for tumor staging and for investigating the etiology of the thrombocytopenia. Cytomorphologic examination revealed normocellular bone marrow with continuous maturation in erythroid, myeloid, and megakaryocytic elements. The number of megakaryocytes was increased. Young forms of megakaryocytes and rare micromegakaryocytes were observed as well as mature forms. There were no tumor cells, pseudorosettes, storage cells, and/or erythrophagocytosis in the bone marrow; thus an immune thrombocytopenia was considered  and subsequently transfused with platelets in order to maintain an acceptable level of thrombocytes for diagnostic and therapeutic surgery . An incrs of ITP .Tumor histopathology revealed a Schwannian-stroma-poor, differentiating neuroblastoma with low mitotic rate and low mitosis-karyorrhexis index (MKI < 100/5000 cells)  Figure . The resThe patient was diagnosed as stage 1 neuroblastoma according to the International Staging System  and immuThrombocytopenia in cancer patients is induced by several mechanisms: 1) decrease in platelet production caused by bone marrow infiltration or by bone marrow suppression due to cytotoxic drugs, radiotherapy, or infections, (2) increased platelet destruction due to immune pathologies or disseminated intravascular coagulopathy (DIC), 3) platelet sequestration as a result of splenic metastasis. Immune thrombocytopenia is mainly a diagnosis of exclusion and all other causes for thrombocytopenia must be ruled out in order to confirm the diagnosis. ITP can be either primary (idiopathic) or secondary. Although ITP with tumors may be a coincidental finding, it has been described as a paraneoplastic syndrome of several tumors [ decrease plateletThe association between ITP and lymphoid neoplasms, particularly chronic lymphoid leukemia and Hodgkin's disease, is well described , 9. The Paraneoplastic syndromes are disorders that are not directly related to the growth, invasion, or metastasis of the tumor but occur as a result of autonomous secretion of hormones, peptides, or cytokines by the tumor. The major paraneoplastic syndromes associated with neuroblastomas are opsoclonus-myoclonus-ataxia syndrome (OMAS), which is believed to have an autoimmune pathogenesis , 12 and In patients with neuroblastoma, although very frequent during chemotherapy and/or radiotherapy, thrombocytopenia at diagnosis is usually related to bone marrow infiltration by the tumor cells, which results in stage IV disease. In the present case, thrombocytopenia was detected at initial evaluation before the commencement of therapy. There was no evidence of infection or DIC. Splenic involvement that could result in thrombocyte sequestration was ruled out by abdominal MRI. Mean platelet volume was normal. Bilateral bone marrow aspiration and biopsy were performed and tumor involvement was ruled out. Bone marrow aspiration revealed normocellular bone marrow with no tumor cells, both young forms and mature megakaryocytes were observed and the number of megakaryocytes was increased suggesting an autoimmune process accompanying neuroblastoma rather than bone marrow infiltration. Although tests for platelet antibodies could not be performed we concluded that thrombocytopenia in this case was immunologic in origin after reviewing his clinical, laboratory, and imaging findings. Since ITP may occur in isolation (primary) or in association with other disorders (secondary), secondary causes were investigated . The patThe mechanism of immune thrombocytopenia associated with tumors remains to be elucidated. Immune dysregulation occurring in the setting of cancer is thought to result in increased platelet destruction . AutoantTreatment of paraneoplastic ITP associated with solid tumors included various combinations of corticosteroids, splenectomy, IVIG, and interferon in addition to primary treatment of the underlying malignancy with surgery, chemotherapy, and/or radiotherapy. Complete remission of ITP after curative surgery was generally low and additional therapeutic agents were needed . In the In conclusion the aim of this case presentation is to emphasize that bone marrow infiltration is not the only cause of thrombocytopenia at diagnosis in neuroblastoma. Although rarely, an immune thrombocytopenia either primary or paraneoplastic has to be taken into consideration. A prompt and appropriate intervention for thrombocytopenia may allow the specific oncological treatment.


However, sometimes a differential diagnosis with an infectious cause such as aspergillosis can be very challenging as both cancer and infection are sometimes difficult to distinguish. Pulmonary aspergillosis can present as an infectious pseudotumour with clinical and imaging characteristics mimicking lung cancer. Even in the presence of cerebral lesions, radiological appearance of abscesses can look like brain metastasis. These similarities can cause significant diagnostic difficulties with a subsequent therapeutic delay and a potential adverse outcome. Awareness of this infectious disease that can mimic lung cancer, even in an immunocompetent patient, is important. We report a case of a 65-year-old woman with pulmonary aspergillosis disseminated to the brain mimicking metastatic lung cancer.In a patient with a medical history of cancer, the most probable diagnosis of an  Bronchoscopic sampling for cytology and microbiological cultures showed neither proof of malignancy nor infection, and follow-up was chosen. Serial follow-up chest CTs up to December 2011  showed no change and follow-up was ended.A 65-year-old woman, never-smoker, was referred for a second opinion in January 2014 because of an abnormal computed tomography (CT) of the chest with a mass in the right lower lobe. Extensive evaluation in the referring hospital had not revealed a diagnosis. A clear overview of the medical disease history is demonstrated in a timeline , but without growth on culture and these were considered contamination or colonization. In March 2014, a multidisciplinary decision was made for a treatment with a higher dose of prednisone 1 mg/kg/day in combination with macrolide antibiotic treatment for 3 months (with slow tapering of the steroids) under suspicion of cryptogenic organizing pneumonia, but without clinical or radiologic response. Additional diagnostics were considered for the growing part of the lesion, but a CT-guided biopsy and surgical sampling were both not possible because of the risk of a massive bleeding. A follow-up 18FDG-PET-CT was scheduled to evaluate whether in the follow-up lesions would become better accessible for further diagnostic work-up. During the pulmonary work-up, in March 2014, the patient developed new complaints of progressive muscle weakness and sensibility loss of the right upper arm. She was hospitalized in the referring hospital. Additional brain imaging with magnetic resonance imaging (MRI) revealed multiple brain lesions in the cortex and watershed region, in the left corpus callosum, the left thalamus and partially in the right semioval center, which were considered brain metastases by the referring hospital , but they are also capable of causing several and severe types of disease as has been described in patients with bronchiectasis (Aspergillus spp. can also mimic cerebral metastasis. Contrast-enhanced CT and MRI are the modalities of choice for imaging of the brain when brain metastasis is suspected, with MRI more appropriate in characterizing lesions (Aspergillus abscess mimicking a solid tumor are sparse; we could only identify two case resports (As a tertiary center, patients are frequently referred to our center with presumed lung cancer. In a retrospective analysis of a tertiary US hospital, the majority of such patients were proven to have a neoplastic process, only 1.3% had an infection . In addiiectasis . Patholo lesions –13. Alth lesions , 15. A c lesions . In our resports .Coexistence of infectious pseudotumours and solid tumors at initial diagnosis have previously been reported , but arePulmonary aspergillosis, even in the presence of cerebral abscesses, can present as an infectious pseudotumour with clinical and imaging characteristics resembling lung malignancy. These clinical and radiological similarities can cause significant diagnostic difficulties, with a subsequent therapeutic delay and a potential adverse outcome. A definitive histopathological diagnosis should always be strived for when malignancy is suspected, but awareness that this infectious disease can mimic lung cancer even in immunocompetent patients is of great diagnostic and prognostic importance.Patient gave written informed consent in accordance with the Declaration of Helsinki.Conception and design and drafting of the manuscript: MV, AD, and LH. Drafting the manuscript for important intellectual content: all authors. All co-authors critically revised the article and gave final approval of this version to be published.MV has nothing to disclose. AD: none related to current manuscript. Outside of the submitted work: advisory boards: Roche, Pfizer, Eli Lilly Boehringer Ingelheim, MSD, Astra Zeneca, BMS; fees for lectures: BMS, Roche, Ely Lilly. VS: none related to current manuscript. KV: none related to current manuscript. AP: none related to current manuscript. Outside of the submitted work: fees for lectures: Bayer. AL: none related to current manuscript. CP has nothing to disclose. LH: none related to current manuscript. Outside of the submitted work: advisory boards: Boehringer Ingelheim and BMS; fees for lectures: MSD, Astra Zeneca, Roche, research grant: Roche.


We report an extremely rare case of ganglioneuroma involving the external auditory canal and middle ear. Ganglioneuromas are rare benign mature tumors thought to originate from sympathetic ganglions, with the highest incidence in the retroperitoneum, adrenal medulla, and posterior mediastinum. We present a case of ganglioneuroma of the external auditory canal and middle ear. At the age of 12 months, the patient was diagnosed with neuroblastoma stage IV with metastasis to the squamous temporal bone, bone marrow, and skull base. He received a high-risk protocol regimen resulting in complete remission. The patient later presented with recurrent right ear discharge at the age of six years and was diagnosed with ganglioneuroma of external auditory canal and middle ear after appropriate investigations. We report in this article the clinical presentation, investigations, surgical intervention, and follow-up. After the literature review and to our knowledge, this is the first reported case of its kind. Ganglioneuroma maturing from neuroblastoma is one of the theories describing pathophysiology of the disease. Ganglioneuroma should be considered in the differential diagnosis of patients presenting with recurrent ear discharge and decreased hearing in treated cases of neuroblastoma with metastases to temporal bone. Ganglioneuromas are tumors originating from the autonomic nervous system ganglion cells. They are slow-growing benign tumors derived from the neural crest cells, which are found at any location where the sympathetic tissues are normally found . The mosAt 12 months of age, our patient was referred to our center with fever, bilateral proptosis, and abdominal distention. He was diagnosed with left suprarenal neuroblastoma (10 × 10 × 8 cm) stage IV with metastasis to skull, skull base and squamous temporal bone plus bilateral femoral, and tibial and bone marrow lesions. Treatment was commenced in the form of a high-risk neuroblastoma chemotherapy protocol in four cycles , adriamycin, cyclophosphamide, ifosfamide, and carboplatin). The patient responded well to treatment, the symptoms resolved, and the tumor reduced in size. He underwent surgical resection of residual tumor a few months later followed by a fifth cycle of chemotherapy and thirteen sessions of localized intensity-modulated radiotherapy (IMRT) radiation protocol to the abdomen and stem cell transplantation was carried out. There was no evidence of residual disease in follow-up CT brain scans in the temporal bone, middle ear, and external auditory canal. Later, at the age of 6 years, he was referred to the ENT clinic with a right ear canal mass associated with recurrent infection and hearing loss. Examination showed a mass originating from the right external auditory meatus and obstructing the ear canal. Computed tomography (CT) of temporal bone with 1 mm slice thickness showed a soft tissue mass in the right external auditory canal with bony remodeling, soft tissue in the mesotympanum, hypotympanum, and middle ear cavity extending into Prussak's space and minimal erosion of the scutum. The ossicles appeared intact. There was also soft tissue seen in the sinus tympani Figures 1.He underwent right ear examination under general anesthesia with multiple biopsies and partial debulking of the lesion. Histopathology showed ganglioneuroma with no malignancy or neuroblastic elements seen. Based on this histopathology, the patient was then taken to theatre for exploration and excision of the tumor with facial nerve monitoring. The tumor was found to be present above the ear canal and beneath the temporalis muscle extending forward almost to the parotid and onto the zygomatic arch passing down the ear canal to the annulus, causing a degree of bone erosion. The tympanic membrane was normal anteriorly, but posteriorly the drum was displaced laterally by tumor in the middle ear, which extended up into the epitympanum and back into some of the mastoid air cells. There was an extension attached to the vertical portion of the facial nerve as well as the long process of incus and stapes. With the assistance of facial nerve stimulation, the tumor was freed from the facial nerve without damage. The perineural lesion was removed from the facial recess and sinus tympani completely . It was Ganglioneuromas show bundles of longitudinal and transverse bundles of Schwann cells that irregularly crisscross each other. The ganglion cells are large and dysmorphic, often without satellite cells (maturing rather than mature ganglion cells) and they tend to cluster in some foci more than others. There are no neuroblasts seen in the current specimen, which was submitted entirely .Immunohistochemistry staining used neurofilaments protein immunostain (showing bundles of axons), synaptophysin immunostain , and neuronal nuclear immunostain (showing the ganglion cells' cytoplasm and nuclei) . HoweverGanglioneuroma is a benign, slow-growing tumor originating from the sympathetic chain. The most common sites of presentation are the posterior mediastinum and retroperitoneum. Rarely do they present in the head and neck region –7. ThereNeuroblastoma, ganglioneuroblastoma, and ganglioneuroma are tumors of varying maturity with malignant or premalignant tendency with immature cells in neuroblastoma and ganglioneuroblastoma and benign histopathology with ganglioneuroma , 2. Our Preferred imaging in cases of ganglioneuroma is CT scan, to assess the extent of disease, origin, calcification, local extension, and vascular enhancement. CT scan with contrast shows low attenuation and calcification, while T2 MRI scans show heterogeneity with high signal intensity .Clinically significant catecholamine secreting ganglioneuroma is rare , 3, 4. OGanglioneuroma is exceedingly rare in the external auditory canal, with only one reported case and no reports of examples involving both the external auditory canal and middle ear , 9. CT s


Sex cord tumors of the testis in post pubertal men are rare. Mixed leydig-Sertoli-Granulosa sex cord tumors are exceptionally rare. To the best of our knowledge there are only three reported similar cases in the literature. We reported a case of a 27-year-old male who presented with huge left scrotal mass of 6-years duration. The gross tumor specimen after resection measured 11 cm in diameter. Histological examination revealed mixed sex cord stromal tumor. This case demonstrates the limited ability of accurate diagnostic determination preoperatively, with pathologic examination and immune-histochemical staining post-orchiectomy representing the only definitive means of diagnosis. It also highlights the unique radiological appearances of this tumor, which were not previously reported in literature. Sex cord tumors of the testis in post pubertal men are rare ,2. MixedA 27-year-old man presented to our urology clinic with left testicular swelling of 6-years duration. His medical history was negative, particularly for cryptorchidism. On physical examination, there was a large non tender swelling of the left scrotum, which showed partial transillumination. There was no gynecomastia or any systemic symptoms. Accordingly, ultrasound testicular examination was done; this showed large mixed solid and multilocular cystic mass replacing the left testis. The cysts vary in appearance from anechoic to echogenic . MoreoveTesticular cancer is relatively rare, accounting about 1-1.5% of all cancers in men ,5 howeveAlthough mixed sex cord tumors of the testis are rare in post pubertal men, they should be considered in the differential diagnosis of testicular masses in adults. The diagnosis can be challenging, as it can present with a long history of testicular mass reaching large sizes. This case reflects the clinical and radiological features of mixed sex cord tumor. It further illustrates the difficulty of differentiating it clinically and radiologically from other lesions. Also it highlights the tumor potential for assuming extreme proportions. Given the rarity of these tumors, and its challenging imaging features, more data collection will be helpful in the management of future patients.The authors declare no competing interest.


Dear SirCystic hygroma or macrocystic lymphatic malformation is a well-known entity and its diagnosis and management is also well described in literature. Rarely its size and location may pose management challenges.[1-3] We managed a case of humongous macrocystic lymphatic malformation of axilla and chest wall in a newborn. At 25 weeks of gestation, a 16cm mass was reported by antenatal ultrasound around left chest wall and arm. At 34.4 weeks of gestation, the baby boy was born by cesarean section. On examination, a 16x16cm mass was noted at the left chest wall with involvement of the skin and subcutaneous tissue; the tumor was soft, mobile and without adherence to deep layers . The baby had respiratory distress because of the location of the tumor and was transferred to the neonatal intensive care unit where ventilatory and hemodynamic support was provided. Plain and contrast magnetic resonance angiography of the neck and arm reported a large mass with characteristics of a hemolymphangioma in the axilla with multiple macrocysts and thin septa having contrast enhancement; the mass did not invade structures or vessels , surgical resection was performed. An incision was made around the tumor dissecting to the fascia of the pectoral muscle and the serratus anterior. The tumor was gradually dissected to its base to avoid injury to the axillary neurovascular bundle and subsequently removed with skin adnexa. Local skin flaps were made to cover the defect and a closed drainage was placed . Histopathological study reported a cystic hemolymphangioma. Postoperative recovery was uneventful.The index case is management challenge. The huge size of the mass led to respiratory distress which was managed by mechanical ventilation in ICU. After initial respiratory and hemodynamic stabilization, the other challenge was excision. Fortunately, the lymphatic malformation was not invading the neurovascular bundle of the axilla and excision went smooth and meticulous.Source of Support: NilConflict of Interest: None


Urethral duplication and megalourethra are rare urethral anomalies. However, the concomitance of urethral duplication and double megalourethra has not been reported previously.A newborn was presented with penile swelling during voiding. Physical examination revealed a retractable foreskin and two external meatus of a double urethra. Retrograde urethrography demonstrated two complete megalourethras. Urethro-urethrostomy and urethroplasty were performed when the patient was 10 months old. The patient was followed up for one year without any urinary problems and has good cosmetics and urinary continence.The concomitance of these two rare anomalies and more importantly its surgical treatment makes this case report unique and valuable. Megalourethra is a rare congenital urethral abnormality characterized by dilatation of the anterior urethra without any distal obstruction. Urethral duplication (UD) is a rare congenital malformation that is more frequently found in males and often associated with other abnormalities of the genitourinary tract, heart, bowel and bones. Although both megalourethra and UD may present with associated anomalies, to the best of our knowledge, the concomitance of these two anomalies has not been reported in the English literature previously.Here, we report a case of type IIA1 complete UD with megalourethra in both urethras. This is the first reported case with the presentation of duplicated megalourethras.A newborn presented with penile swelling . His motUrethroscopy was performed via both urethras and determined that while the dorsal urethra opened to the bladder neck with a narrow proximal lumen, the ventral urethra opened to the bladder neck with a normal calibre.Urethro-urethrostomy and urethroplasty were performed when the patient was 10 months old. During surgery, both urethras were catheterized with 8-Fr catheters and filled with saline to demonstrate the megalourethras . CirculaThe patient was followed up for one year without any urinary problems and has good cosmetics and urinary continence .Informed consent was obtained from the parents of the child. Patient-informed consent for publication was obtained after the manuscript was shown to the parents.Both the UD and the megalourethra are rare urethral anomalies and the concomitance of these two anomalies has not been reported previously. The embryological development of UD is uncertain. There are likely different causes for the various types of anomalies. A misalignment between the termination of the cloacal membrane and its relationship with the developing genital tubercle and urogenital sinus has been reported . In 1976Megalourethra is a rare congenital urethral anomaly that results from defective development of the corpus spongiosum alone or with hypoplastic corpora cavernosa that occurs in scaphoid or fusiform form, respectively. Megalourethra is more commonly seen in patients with prune belly syndrome and has been reported in association with vertebral defects, anorectal atresia, tracheoesophageal fistula, and renal dysplasia syndrome. The least severe and most common form is the scaphoid variety. The more severe fusiform variety is caused by failure of the penile mesoderm to form spongiosal tissue and corpora cavernosa within the penis.In most cases, a micturating cystourethrography may be sufficient to demonstrate the anatomical and functional features of the urethras in both anomalies . HoweverSeveral surgical techniques have been described to treat UD and megalourethra. Treatment of the UD with megalourethra should be individualized according to the anatomy.In the case of type IIA1 defects, Salle et al.  performeIn our case, we performed a urethroplasty without excising one of the megalourethras. We were able to create a common distal single urethra with a single meatus, resulting in a good functional urethra and nice cosmetic appearance. The proximal urethras were not dissected and thus we were able to avoid damage to the external sphincter.To the best of our knowledge, this is the first reported case of a double megalourethra with complete UD. We believe that our successful treatment plan will be a useful addition to the literature and provides a guideline for future cases.


We report a case of nevus lipomatosus cutaneous superficialis of Hoffman-Zurhelle(NCLS), with multiple lesions, in a ten-year-old child. The NLCS is consideredrare. The classical clinical presentation is characterized by multipleskin-colored or yellowish papules and nodules, which can have a lineardistribution. Histologically, it is characterized by the presence of matureectopic adipocytes in the dermis. The main therapeutic option is surgicalexcision. The classical Nevus lipomatosus cutaneous superficialis is reported inthis case. Clinically, the disease is characterized by multiple non-painfulpedunculated, cerebriform, yellowish or skin-colored papules or nodules, which canhave a linear distribution.2 Histologically, it is characterizedby the presence of mature ectopic adipocytes in the dermis. We report a linearvariant of NLCS in a child.Nevus lipomatosus cutaneous superficialis (NLCS) is a rare entity. It was firstdescribed by Hoffman and Zurhelle in 1921.We report a ten-year-old mulatto patient with a history of asymptomatic lesions,which had been progressing since the age of two. Dermatological examination revealedskin-colored plaque lesions, irregular surface, on the left arm, in the rightinguinal region, mesogastric region, and on left flank in a linear distribution. Histopa3 They are rarely located in other areas. Festa Netoet al. (1984) reported the first case of NLCS in Brazil in a 4-year-old patient withmultiple hairy lesions on the wrist.4NLCS is characterized by multiple yellowish or skin-colored papules or nodules. Theycan affect the pelvis, lumbosacral region, and butocks.1 Its clinical aspect may vary from lobulated tumorlesions – with a pleated cerebriform appearance – to small neoformations covered bynormal skin – producing just a slight elevation that may go unnoticed. Occasionally,these lesions can present with hair growth.6 The present case wascharacterized by multiple lesions with a linear distribution.NLCS exists in two clinical varieties: 1) the solitary form, with a single lesion andvariable location; 2) the linear classic form, with multiple lesions in the pelvicand gluteal regions, as described by Hoffman and Zurhelle.6 Other authorsconsider the hypothesis that the deposition of adipocytes is caused by degenerativealterations of dermal collagen bundles and elastic tissue.7The pathogenesis of NLCS is still not well defined. Jones (1975) suggested thatectopic adipocyte would originate from precursor cells from the dermalvessels.8Histopathologically, there is, in the superficial and middle layers of the dermis,the presence of dispersed or grouped lobules of adipose tissue, composed ofwell-differentiated adipocytes – preferably in a perivascular distribution – andsurrounded by collagen fibers with normal appearance.9 Our patient had lesions that clinically resembled connectivenevus. However, histopathology demonstrated the presence of adipocytes in thedermis, confirming the diagnosis of NLCS. The therapeutic option for this nevus issurgical excision, with no reports of other forms of treatment.10Clinical differential diagnosis of the NLCS includes other tumors or nevi that may belinear or multiple and zosteriform in distribution, such as: connective nevus,sebaceous nevus and verrucous nevus.


Isolated rectus abdominis muscle endometriosis is very uncommon with less than 20 case reports being published to date since its first description in 1984 by Amato and Levitt. We report another case of isolated rectus abdominis endometriosis in a 37-year-old patient with a previous caesarian section. We also discuss the diagnostic and treatment particularities in these patients. In our case, the treatment was only surgical and currently the patient is disease-free during the 24-month follow-up.  Endometriosis is defined as a functional ectopic endometrial tissue. Anterior abdominal wall endometriosis is defined as functional extraperitoneal endometrium. Endometriosis is not unusual in reproductive age patients and can be located in almost any organ. The most common affected sites are ovaries, sacrouterine ligament, rectovaginal septum, and pelvic peritoneum . Less coAll medical treatments for endometriosis are equally effective in relieving pain. However, all of them alleviate pain symptoms for as long as they are used, but pain always relapses when medication is discontinued. Therefore, medications need to be used in the long term . The onlWe report an additional case of this very uncommon condition as well as the literature review.A 37-year-old patient presented due to painful mass in the left hypogastric area, in the site of a Pfannenstiel incision scar as a result of a caesarian section 3 years previously. Her past medical history was otherwise insignificant. The pain intensity and the mass size seemed to increase during menses. Upon physical examination, a firm pseudotumorous mass in the left side of the postoperative scar of about 2 cm in diameter was palpated. Her level of CA 125  was within normal limits, 25 units/mL (reference range: 0–35 kU/L). Due to the cyclic pain related to menses and tumorous mass in the site of a C-section scar, anterior abdominal wall endometriosis was suspected and the patient was scheduled for ultrasound and magnetic resonance imaging (MRI). Ultrasound with Doppler revealed hypoechoic, heterogeneous roundish nodule in the left end of the postoperative scar, with single vascular pedicle entering at the periphery of the mass. The MRI exam evidenced a 21 × 23 × 28 mm heterogeneous mass in the lower left rectus abdominis muscle with focal areas of high and low signal intensity, suggesting old haemorrhage or fibrosis Figures . The MRI per primam. The 24-month follow-up showed no disease recurrence.During surgery, straight below the aponeurosis of the rectus abdominis muscle, a dense tumorous mass was observed and its complete excision including a 7–10 mm safety area was performed. Hemostasis was achieved by ligation of the vascular pedicle entering at the periphery of the mass as well as by electric coagulation. A single dose of ceftriaxone (2 g) was given intraoperatively. Due to a significant defect in the aponeurosis, it was decided to close it in a tension-free procedure, so a polypropylene mesh was used . The remSince there was R0 resection and her CA 125 was within normal limits without signs of malignancy upon histology examination, no gynecological oncologist was involved in the follow-up of this patient.Endometriosis is considered a common gynecological pathology and its frequency reaches up to 15% . The mosTo date, there are several theories to explain endometriosis: the implantation or reflux theory, the direct extension theory, the coelomic metaplasia theory, the induction theory, the embryonic rest theory, the lymphatic and vascular metastasis theory, and the composite theory combining implantation and vascular and lymphatic metastasis . The intAnterior abdominal wall endometriosis accounts for up to 1% of all endometriosis localization cases described in the literature . It is cThe diagnosis is challenging since the clinical signs are not specific and the differential diagnosis includes suture granuloma, lymphadenopathy, abscess, inguinal hernia, incisional hernia, primary or metastatic cancer, lymphoma, lipoma, hematoma, sarcoma, desmoids tumor, and subcutaneous and sebaceous cysts . Still, Recent advances in imaging techniques  made the diagnosis of abdominal wall endometriosis easier, although the imaging signs are not specific and are not very informative outside of the clinical context  .Another diagnostic procedure, fine needle aspiration (FNA), was reported to be useful as well for excluding malignancy, but it seems to be inconclusive in up to 75% of cases. Moreover, FNA is associated with an increased recurrence risk . Under tThe treatment of choice for endometriosis of the rectus abdominis muscle is a wide local excision of the lesion with negative margins. The surgical excision should include 5–10 mm of the surrounding healthy tissue as surgical margin and care must be taken not to rupture the mass to avoid reimplantation of microscopic remnants of endometrial tissue . The samTo date, medical therapy for abdominal wall endometrioma is considered updated since it is unsuccessful and permits just temporary symptoms relief . MedicatRecurrence is rare, usually presents within 1 year, and is likely to be the result of an inadequate excision . To dateWe presented a rare case of anterior rectus abdominis muscle endometriosis diagnosed preoperatively and confirmed by histopathology and immunohistochemistry CD 10. The treatment of this exceptional condition should be surgical and this clinical entity must be included in the differential diagnosis of any abdominal mass in fertile female patients with or without surgical history.


Introduction The aim of the study was to evaluate the results of surgery to remove huge mediastinal masses and their pathology. Surgical resection was chosen for accurate diagnosis and treatment of the huge mediastinal masses extending into the pleural cavity.Methods Records were reviewed for eight patients who had the diagnosis of huge benign mediastinal masses and who underwent operation; details of the patients and operations were recorded.Results Mean age was 34.5 (range 22 to 44) years, and male-to-female ratio was 2:6. Computed tomography and magnetic resonance imaging (MRI) were used to evaluate the location and extent of the abnormality and to characterize the tissue components of the mass. Most of the tumors were located in the posterior mediastinum. The most frequent presenting symptom was exertional dyspnea. The majority of cases underwent posterolateral thoracotomy, and complete resection was possible in seven patients. Partial resection could only be performed in one. The mean diameter of the resected masses was 15 × 10 cm. Histopathologic examination revealed 3 neurogenic tumors, 2 teratomas, 1 thymolipoma, and 1 ectopic thyroid, and 1 hemangioma. Minor complication was seen in two cases.Conclusion The presurgical thoracic MRI provided correct diagnosis along with radiologic characterization and topography. Surgery must be the preferred treatment in huge benign mediastinal masses. Most mediastinal masses are primary and asymptomatic. They are diagnosed either accidentally or when they press on the adjacent anatomic tissues. Therefore, the size of the lesion is important because it plays a role in the appearance of symptoms.Eight cases with rarely seen huge mediastinal mass underwent surgical treatment in our clinic. Mediastinal cysts were not included in the study. The cases were evaluated in terms of age, sex, primary diseases, symptoms, mass size, computed tomography (CT) and magnetic resonance imaging (MRI) findings, type of surgical treatment, and histopathologic results. Full blood count, biochemical examinations, bilateral pulmonary function test, and CT were obtained in all cases and in some cases respiratory functional tests, MRI, and echocardiography were obtained. The surgical treatments applied were either median sternotomy or right/left thoracotomy. The complications during the operation and follow-up were recorded .Two of the eight patients diagnosed and treated for huge benign tumor were men and six were women. The mean age was 33 (range 22 to 44). The general features of the patients are given inCases 1, 2, 3, 4, and 8 had respiratory problems and cases 6 and 7 had nonspecific complaints; one case presented to our clinic with trauma.The ecchymosis in case 1 was found especially in the head, thorax, and right hemithorax interior upper side, and a tubercle was seen on the right arm. The right radial pulse could not be taken. Additionally, there was crepitation and pain in the right clavicle area. Respiratory sounds could not be heard in the anterior upper right. There was bleeding from time to time as evidenced by hemoptysis of 50 to 100 mL, diagnosed as maxillofacial injury by the otorhinolaryngology department; however, it was not certain whether the bleeding was from the maxillofacial injury or from the lungs. Tachycardia and blood pressure were not stable. Due to the maxillofacial injury, bronchoscopy was not used; the patient was taken to the operation with the diagnosis of hematoma in the thorax .2: 48 mm Hg) was found. After the tests, the patient was taken to the operating room under elective conditions with mass diagnosis.A respiratory problem was clear in case 2; the patient could not lie back and the respiratory sounds in both lungs were decreased. In blood gas examination, hypoxia , only a biopsy was taken. Anatomic pulmonary resection was not needed for any cases. In cases 3 and 7, the phrenic nerve was excised too because the mass captured the phrenic nerve on the same side. Diaphragmatic elevation developed in both cases, whereas dysphonia was only seen in case 7. No other complication was encountered. There was no recurrence during the long-term follow-up.Because most of the masses originating in the lungs are small outer cell lung cancers, their diagnosis and treatment modalities are relatively uniform, and speak a common language. However, it is more difficult speaking a common language for mediastinum masses. Although the mediastinum is an anatomic area, it contains different tissues and pluripotent cells. For that reason, mediastinum masses can be benign or malignant, and malignant mediastinum tumors can be primary or secondary tumors. Primary malignant tumors are rarely seen; secondary tumors are accepted as metastatic tumors and generally result from lymphatic spread of neoplasms to the mediastinal lymph nodes. Primary mediastinum benign tumors, on the other hand, are a heterogenous group appearing in neoplastic, congenital, and inflammatory cases; they are generally accepted as asymptomatic and can reach large sizes. In a series of 2,431 cases gathered from the literature, the incidences of tumors in the mediastinum were as follows: neurogenic tumors, 21%; thymoma, 19%; lymphoma, 13%; neoplasia with germ cells, 10%; primary carcinoma, 5%; mesenchymal tumor, 6%; endocrine tumor, 6%; cysts, 18%; and others, 3%.It has been stated that 56 to 62% of the cases in the research were symptomatic; 16% had dyspnea, 30 to 43% had chest pain, and 16% had coughing.As with other thoracic cysts, the purpose of radiologic examination and histopathologic typing in mediastinal masses is to obtain a diagnosis that will differentiate mediastinum mass from the masses that have the same prior radiologic image results so that the optimal treatment method can be planned. At the same time, it is necessary to determine compression and invasion into the tracheobronchial tree, pulmonary vascular structures, and superior vena cava and other mediastinal problems to determine the systemic problems to be encountered pre- and postoperatively. For that reason, CT provides vital information on the localization of the lesion in the mediastinum and the density of the mass, its size, and its relation with mediastinal anatomic structures, along with information on whether the mass includes liquid or calcifications and its weight and shape. However, Rendina et al stated that the rate of correct histopathologic diagnosis prediction was not very high with CT (around 68%).The evaluation of the operability of mediastinal masses is very different than that of lung cancer. The appropriate operative procedure and surgical timing depend on the patient's age, the size and type of the tumor, its localization and expansion, accompanying diseases, cardiorespiratory reserve, and the experience of the surgeon.The type of incision used for resection is usually posterolateral thoracotomy and second median sternotomy.Though huge masses in the mediastinum and extending to the pleural cavity are rare, surgery should be performed after a thorough radiologic examination. Thoracic MRI is necessary to obtain a histopathologic diagnosis and eliminate the disease and the symptoms.


Malignant transformation has been reported in approximately 1% of the endometriosis cases; herein, we report a case of clear cell endometrial carcinoma arising from endometriosis foci located within a caesarean section scar.2, biweekly administration), the death of the patient disease occurred 1 month later.In November 2014, a Caucasian, 44-year-old woman was transferred to our institution because of severe respiratory failure due to massive lung embolism and rapid enlargement of a subcutaneous suprapubic mass. Abdomino-pelvic magnetic resonance showed a 10.5 × 5.0 × 5.0 cm subcutaneous solid mass involving the rectus abdominis muscle. Pelvic organs appeared normal, while right external iliac lymph nodes appeared enlarged (maximum diameter = 16 mm). A whole-body positron emission tomography/computed tomography scan showed irregular uptake of the radiotracer in the 22 cm mass of the abdominal wall, and in enlarged external iliac and inguinal lymph nodes. In December 2014, the patient underwent exploratory laparoscopy showing normal adnexae and pelvic organs; peritoneal as well as cervical, endometrial and vesical biopsies were negative. The patient was administered neo-adjuvant chemotherapy with carboplatin and paclitaxel, weekly, without benefit and then underwent wide resection of the abdominal mass, partial removal of rectus abdominis muscle and fascia, radical hysterectomy, bilateral salpingo-oophorectomy, and inguinal and pelvic lymphadenectomy. The muscular gap was repaired employing a gore-tex mesh while the external covering was made by a pedicled perforator fasciocutaneous anterolateral thigh flap. Final diagnosis was clear cell endometrial adenocarcinoma arising from endometriosis foci within the caesarean section scar. Pelvic and inguinal lymph nodes were metastatic. Tumor cells were positive for CK7 EMA, CKAE1/AE3, CD15, CA-125, while immunoreaction for Calretinin, WT1, estrogen, and progesterone receptors, cytokeratin 20, CD10, alpha fetoprotein, CDX2, TTF1, and thyroglobulin were all negative. Liver relapse occurred after 2 months; despite 3 cycles of pegylated liposomal doxorubicin (20 mg/mAttention should be focused on careful evaluation of patient history in terms of pelvic surgery, and symptoms suggestive of endometriosis such as repeated occurrence of endometriosis nodules at CS scar, or cyclic pain, or volume changes of the nodules. Malignant transformation has been reported in approximately 1% of the endometriosis cases, and most frequently this transformation takes place at the ovary, accounting for about 80% of the endometriosis-associated malignancies [Endometriosis occurring in surgical abdominal scar has been mainly documented after caesarean section (CS) or hysterectomy (0.03 up to 0.4%), and its malignant transformation is very rare : clear cGiven the rarity of malignant transformation of abdominal scar endometriosis to clear cell histology, pathogenesis and risk factors of this disease are hardly assessable . NonetheHerein, we report a new case of CCC arising from endometriosis foci located within a CS scar; a systematic review of the available literature relative to this issue is also presented.In November 2014, a Caucasian, 44-year-old woman was transferred to our institution from the emergency unit of another hospital where she had been successfully treated for a severe respiratory failure due to massive lung embolism and cardiogenic shock.Her familial history was uneventful; she had undergone one caesarean section 9 years before without complications and had assumed oral contraceptives until the appearance of symptoms. She had never suffered from signs or symptoms of endometriosis.The patient referred to have documented, since the last 5 months, the slow enlargement of a suprapubic mass at the CS scar  and abdominal swelling.In July 2014, she had already performed abdomino-pelvic magnetic resonance imaging (MRI) showing a 10.5×5.0×5.0 cm subcutaneous solid mass with cystic areas and internal septa involving the rectus abdominis muscle. The mass appeared strictly adherent to the uterus and recto-sigma. Pelvic organs appeared normal, while right external iliac lymph nodes appeared enlarged (short axis maximum diameter = 16 mm).The patient had been already triaged to fine needle aspiration (FNA) of the mass which was suggestive of endometrial tubule-papillary carcinoma.At physical examination, a suprapubic mass of almost 20 cm maximum diameter was documented close to the midpoint of the CS scar Fig. . Laborat18F-FDG (148 MBq), showing irregular/non-homogeneous uptake of the radiotracer in the 22 cm mass of the abdominal wall. Increased 18F-FDG uptake was also seen in enlarged external iliac and inguinal lymph nodes bilaterally  scan was performed about 60 min after the intravenous administration of lly Fig. . Abdominlly Fig. .Fig. 2PrIn December 2014, the patient underwent exploratory laparoscopy showing normal adnexae and pelvic organs; peritoneal as well as cervical, endometrial, and vesical biopsies were negative.2), every week. After 3 cycles (9 administrations) MRI documented only a slight reduction of the mass and lymphadenopathies, thus leading to consider the need of a multidisciplinary approach with gynecologic and medical oncologists, surgeons, and plastic surgeons in order to plan the most adequate patient treatment. After a thorough evaluation, radical surgery with reconstruction was planned, and an extensive counseling was carried out with the patient and her relatives.Considering the results of FNA suggesting primary Müllerian-derived carcinoma and the extent of disease whose radical resection would require widely demolitive surgical procedures, the patient was triaged to neo-adjuvant chemotherapy with carboplatin (AUC = 2), and paclitaxel  as well as inguinal and pelvic lymphadenectomy were performed.At the end of surgery, the abdominal wall defect appeared as a round hole of 14 × 14 cm which involved the abdominal wall thickness completely. The muscular gap was repaired employing a gore-tex mesh anchored directly on the residual rectus muscles and sheets. The external covering had been planned by a pedicled perforator fasciocutaneous anterolateral thigh flap. The perforator vessels from the descending branch of the lateral circumflex femoral artery had been preoperatively located, with ultrasound doppler sonography, at the level of the intermuscular lateral septum of the thigh. The right thigh was chosen as donor site because the perforators were more distal, thus allowing to lengthen the pedicle. The donor site, on the right thigh, was repaired both by direct closure, and a skin graft harvested from the contralateral thigh. Postoperative course of the wounds was uneventful and stitches were removed at 2 weeks. The patient was discharged after 18 days in good clinical conditions, and the cosmetic result was acceptable Fig. .Macroscopically, the surgical specimen consisted of cutis and subcutaneous tissue which contained a capsulated tumor mass measuring 18 cm in maximum diameter, infiltrating the rectus abdominis muscle. The cut surface was whitish and showed areas of necrosis and hemorrhage. Histological examination of tissue samples revealed neoplastic proliferation of large-sized, epitheliomorphous cells with abundant clear or occasionally eosinophilic cytoplasm and prominent nucleoli. A remarkable degree of nuclear atypia with diffuse hyperchromasia and irregular nuclear contours, and cellular pleomorphism was evident throughout the whole lesion. Architecturally, the tumor featured a mixed pattern of growth, both solid and tubulocystic, occasionally including distinctive papillary areas with hyalinized stromal cores. The clear cells lining the papillae and the cystic spaces showed the characteristic hobnail appearance. A few eosinophilic hyaline globules were observed as well Fig. . InteresImmunohistochemical investigation showed positive tumor cell reaction for CK7 Fig. , EMA, CK2, biweekly administration) was started, and CT scan evaluation after 3 cycles showed the progression of hepatic involvement and appearance of ascites. The death of the patient disease occurred 1 month later.After 2 months from surgery, the patient was submitted to total body CT scan in order to plan subsequent adjuvant treatment. Despite the absence of disease in the pelvis, CT scan documented the appearance of six neoplastic lesions (from 2.7 to 5.5 cm maximum diameter) in the liver. Treatment with pegylated liposomal doxorubicin  Table  before tDespite the aggressiveness of surgery and the multimodal treatment approach, of 18 cases with available follow up data, 10 experienced relapse of disease, mostly at distant sites, and 8 patients died of disease.Apart from the long time interval to diagnosis and the wide extension of disease in the abdominal wall, clinical aggressiveness is also sustained by the intrinsic biologic aggressiveness of CCC, which differs from other endometrial cancer histotypes. In this context, proper diagnosis should take advantage of immunohistochemical panels with several markers in order to exclude serous histotype as well as mesothelial tumors such as malignant mesothelioma and papillary mesothelioma which are usually positive for Calretinin, WT1, and keratin 5/6, contrary to what is observed in CCC. In addition, WT1 is the most important immunohistochemical marker to distinguish serous carcinoma from CCC.It has to be acknowledged that of 9 cases undergoing lymphadenectomy or sampling, 8 showed diffuse metastatic involvement of inguinal, and/or pelvic, and/or aortic lymph nodes  , 16, 18;Obviously, also prevention of endometriosis implantation at time of CS is of utmost importance: as recently emphasized, the uterus should not be exteriorized, exposure of endometrial mucosa during uterus suturing should be limited, and peritonization may be advised, although there is no definitive data about these issues .Attention should be focused on careful evaluation of patient history in terms of pelvic surgery, and symptoms suggestive of endometriosis such as repeated occurrence of endometriosis nodules at CS scar, or cyclic pain, or volume changes of the nodules.


Polycystic (dysgenetic) disease of the salivary glands is a rare entity that has only recently been described in the literature. The disease is more commonly seen in females and majority of the cases have presented as bilateral parotid gland swellings. This case presenting in a 21-year-old male is the first of this unusual entity involving solely the minor salivary gland on the lower lip. This case report highlights the importance for the clinician to be aware of this differential diagnosis, when treating an innocuous lesion like a mucocele.  Salivary gland pathologies present with overlapping histopathological features making it a challenge for the histopathologist as well as the surgeon to diagnose and plan the treatment. Amongst all lesions causing enlargement of the salivary glands, nonneoplastic cysts account for approximately 6% of which one of the rarest cystic condition is dysgenetic polycystic disease (DPD) of salivary gland with distinctive histopathology . It is od glands . dysgenetic polycystic disease of the lower lip with review of the reported cases.In the current article, we describe a unique case ofA 21-year-old male patient reported to the Department of Oral and Maxillofacial Surgery with the chief complaint of swelling on the lower lip of 10 days' duration. The patient gave a history of an increase in swelling in the morning and before meals and the swelling usually subsided by evening. On general physical examination the patient was well built and nourished with no relevant past medical history. None of the family members of the patient had reported a similar lesion.On intraoral examination, a fluctuant swelling measuring approximately 1 × 1 cm in diameter was seen on the lower lip. On palpation, the swelling was nontender, mobile, and soft in consistency. Based on the clinical presentation, a provisional diagnosis of mucocele was rendered. The lesion was surgically excised under local anesthesia and sent for histopathological examination as three soft tissues, white to brown in colour, smooth to rough surface, fluctuant to firm in consistency, and collectively measuring approximately 1.4 × 1.2 × 0.4 cms .Microscopic examination in a low power revealed the presence of numerous ectatic, variable-sized ducts interspersed in a fibrocellular connective tissue stroma . On high Thus, this classical histopathological picture aided in diagnosing the case as a “polycystic dysgenetic disease of minor salivary glands.”Considering the location and the benign clinical appearance of the lesion, the differential diagnosis was narrowed down to four possible lesions: namely, mucocele, lipoma, sclerosing polycystic adenosis, and polycystic dysgenetic disease in order of their frequency of occurrence. These can be easily distinguished histopathologically. Mucocele is unicystic in nature with watery spinnbarkeit nature of material in aspirate, lipoma presents as an encapsulated mass of mature adipocytes, and sclerosing polycystic adenosis exhibits fibrosis and hyperplastic ductal and acinar epithelial elements.The patient underwent assessment of all glandular tissues in the whole body including other salivary glands, liver, kidney, and pancreas and no significant findings were elicited. The lesion in the lower lip healed uneventfully and a review at 18 months showed no recurrence.Dysgenetic polycystic disease (DPD) is a rare benign cystic condition affecting the parotid glands with only 18 well-documented cases reported in the literature . The lesA systematic review of the literature revealed 18 documented cases of dysgenetic polycystic disease (DPD) of salivary glands. The mean age of diagnosis is 31.2 ± 22.28 years and median age is 23 years (range 6–83 years). The lesion is twice as common in females with the male to female ratio being 6 : 13. The lesion is predominantly seen in the parotid. 74% of the cases occurred in the parotid gland . One case showed bilateral submandibular gland involvement and one case showed involvement of bilateral parotid, submandibular, and minor salivary gland. The present case would be the 19th to be documented and the first to be occurring only in the minor salivary gland.history of a recurrent, asymptomatic swelling of the involved gland and no association with any clinical abnormality of salivation or anomaly of salivary gland,presence of enlarged, nontender bilateral parotid swelling for months or years: Batsakis et al. (1988) described the histologic findings in three female patients with this disorder, two of which had bilateral parotid swelling since childhood, and one had unilateral swelling since puberty and subsequently developed swelling of the other gland during young adulthood . Garcia recurrent parotid swelling often occurring in childhood, while symptoms of this disorder might be delayed until adulthood .Certain clinical characteristics are deemed to be unique to this lesion when it presents in the major salivary glands. These includeGenetic susceptibility: five of the cases reviewed gave a positive family history of similar lesion in mother, maternal grandmother, or father; however, in the present case there was no relevant family history. Smyth et al. (1993) reported this condition to be familial with mother and daughter diagnosed with DPD. Moreover, the female predilection exhibits sex linked inheritance . FicarraHistologically the lesion shows multiple ductal ectasias resembling a mucous retention cyst. The presence of microliths/spheroliths histologically has been reported by Seifert et al. 1981), Batsakis et al. (1988), Brown et al. 1995), and Layfield and Gopez (2002) [, and Lay1, BatsakHormonal influence is also considered to exacerbate the underlying condition. Brown et al. (1995) reported a bilateral parotid swelling in a 31-year-old, 4-month pregnant woman which regressed within 4–6 months after parturition establishing a hormonal influence .Alternative theory includes the developmental malformation of the intercalated duct system . It has Many concepts have been proposed regarding the etiology of this condition.Grossly, the cut surface of such lesions usually presents with an exaggerated lobularity and yellow to ivory nodules with a spongy consistency . These nBrown et al. 1994) reported seeing amyloid-like material on aspiration which showed positivity with Congo red stain. However, an open biopsy of the same case showed the amyloid-like material to represent eosinophilic spheroliths . These f reportedAs the lesion is microscopically benign, the current choice of treatment is complete excision. Cases that have involved the parotid gland have been successfully treated by lobectomy or superficial parotidectomy. As multiple salivary glands may be involved, examination of the other glandular structures like the other salivary glands, pancreas, kidneys, spleen, and so forth, must be carried out. However, in view of the paucity of reported cases, long-term follow-up is essential to eliminate the possibility of recurrence and to screen for the potential involvement of other salivary glands . Genetic


Case Summary. A 37-year-old male was admitted due to severe abdominal crampy pain, nausea, vomiting, and obstipation. He had chronic and intermittent abdominal pain from 2 years after OAGB. With high suspicion of complete obstruction, the exploratory laparoscopy was performed. Intraoperative findings showed incarcerated bowel hernia from Petersen's defect. The incarcerated bowel was reduced, and the defect was repaired. The patient was discharged 2 days after operation.  One anastomosis gastric bypass (OAGB) is now considered as an appropriate alternative for Roux-en-Y gastric bypass (RYGB) with some advantages such as absence of risk for internal hernia (IH). But, is really the risk of IH equal zero after OAGB?  The incidence of IH after OAGB is rare but not zero. In any suspicious signs and symptoms for IH, the early exploratory laparoscopy is mandatory to diagnose and treat. One anastomosis gastric bypass (OAGB) or mini-gastric bypass is now accepted around the world as an alternative method for Roux-en-Y gastric bypass (RYGB), the gold standard method of bariatric surgery, with comparable efficacy and safety and some advantages to RYGB , 2. Abse2, was admitted in emergency department with severe crampy abdominal pain, nausea, vomiting, and obstipation from 2 days before admission. He had a history of OAGB (with concomitant Braun jejunojejunostomy 30 cm below the gastrojejunostomy) 3 years ago. Twenty-seven months after OAGB, he had complaints of crampy and intermittent left upper quadrant (LUQ) abdominal pain episodes, which led to two times hospital admissions before. Clinical and paraclinical evaluations such as upper endoscopy, abdominal ultrasonography, abdominal radiographies, and computed tomography (CT) scan with IV/PO contrast had reported normal findings. One of his abdominal plain X-rays is shown in 2 during 3 years after surgery.A 37-year-old male, with body mass index (BMI) of 27 kg/mDuring this admission, on physical examination, he had normal temperature, blood pressure, and respiratory rate; however, he had tachycardia (HR: 110/min). The abdomen was distended without any tenderness and guarding.Due to high suspicion of complete obstruction, after initial resuscitation, the patient underwent exploratory laparoscopy. During laparoscopy, most of the small bowel had been passed through the Petersen's space from right to left and was incarcerated with few patchy lesions in some sites of the small bowel without any sign of severe ischemia and gangrene Figures  and 3. TInternal hernia (IH) occurs after some malabsorptive surgical procedures such as RYGB , with anDue to longer pouch creation in OAGB in comparison to RYGB and therefore larger Petersen's space, the risk of incarceration and strangulation is very low , as in tThe most important lesson is that chronic and intermittent abdominal pain, especially in LUQ, and also nausea especially after meal, can be a presentation of IH after RYGB and OAGB –5. Up to


Background: Non-functional neuroendocrine tumors of the pancreas (NF-pNETs) are a varied group of extremely rare malignancies. The majority of patients already have liver metastases at the diagnosis moment, thus, treatment options are restricted, and the survival rate is reserved.Case report: We presented the case of 59-year-old patient, diagnosed with non-functional well-differentiated pancreatic neuroendocrine tumor grade II (NET G2) with the presence of chromogranin A, synaptophysin and somatostatin receptor 2, together with liver and bone metastases. Patient underwent a surgical excision of the pancreatic tumor, started long-acting somatostatin analogues (octreotide), interferon therapy for liver metastases and local radiotherapy for bone metastases. After one year, the patient developed diabetes, needing insulin therapy. At approximately three years after the diagnosis, the patient was still living, had a good quality of life, and was free of local recurrence of the tumor or other metastases.Conclusion: Our case report presented a rare case of metastatic non-functional well-differentiated pancreatic neuroendocrine tumor, involving a multidisciplinary therapeutic approach in order to obtain a good long-term survival. Pancreatic neuroendocrine tumors (p-NETs) are a captivating and diverse group of rare neoplasms, which arise from pancreatic islet cells. In this article, we presented the case of a female patient with non-functional pancreatic neuroendocrine tumor with multiple liver and bone metastases, whose therapy included a multidisciplinary team of medical specialties: surgery, endocrinology, oncology, radiology, and gastroenterology. After treatment, the metastases were monitored, with no local recurrence of primary tumor. One year after the excision of the pancreatic head and tail, the patient developed diabetes, needing insulin therapy.Fig. 1 a,b).In 2013, a 59-year-old white Caucasian non-smoking woman presented to the surgery department describing symptoms of diffuse abdominal pain, weight loss (18 kilograms/1 year) and unselective anorexia with the insidious onset of symptoms in the previous 6 months. The patient reported a personal history of chronic hepatitis C treated with pegylated interferon and ribavirin 16 years before and no particular familial history.Laboratory tests were within normal ranges, except for an elevated alkaline phosphatase of 158 u/l , a moderate hepatic cytolysis with a small elevation of transaminases, alanine transaminase (ALT) of 36 U/L  and aspartate transaminase (AST) of 37 U/L  and increased carcinoembryonic antigen of 9.75 ng/mL . Pelvic and abdominal computer tomography (CT) with contrast substance revealed hepatomegaly with multiple hypodense formations, the largest with a diameter of 3 cm and a pancreatic tumor of 4/ 3.8 cm diameter, with distal development, involving hilum splenic . A surgical laparoscopic intervention, which revealed numerous whitish tumor formations of 5-30 mm at both liver lobes, without peritoneal carcinomatosis, was performed. A conversion to median celiotomy was decided, finding a 4/4 cm pancreatic tumor located in the distal part of the pancreas, involving peripancreatic tissues and the vessels of splenic hilum. Then, a distal pancreatectomy and splenectomy was performed. Postoperative CT scan performed at 2 weeks after surgery revealed the presence of a pseudocystic collection in the pancreatic stump, of 3.8/ 3.4 cm, which was effectively drained through the locally installed drain. Histopathological and immunohistochemical results diagnosed well-differentiated pancreatic neuroendocrine tumor grade II (NETG2) with liver metastases and no spleen invasion, with the presence of diffuse positive chromogranin A and synaptophysin. The cellular marker for proliferation Ki67 was 15 to 20% and the tumor cells also expressed somatostatin receptor 2 (SSR2)  and serum neuron specific enolase of 56 ng/mL  with normal values of plasmatic serotonin and urinary 5-hydroxyindoleacetic acid. Furthermore, endocrine investigations including pituitary, thyroid, parathyroid, and adrenal hormones were normal and even though genetic testing was not possible, MEN1 syndrome was excluded. Moreover, a bone scintigraphy with technetium 99 was performed and it revealed multiple bone metastases in the vertebral body L3, L4, coccygeal region, posterior rib 10 and 6, left iliac wing, right clavicle and left femoral diaphysis. Based on these results, after a multidisciplinary cooperation between the oncologist, endocrinologist and radiotherapist, the patient started treatment with long-acting somatostatin analogues (octreotide) 30 mg at 4 weeks, interferon alfa 2B – 3 million units three times a week for 6 months and a single local external radiation therapy (dose of 8 Gy) for the relieve of lumbar pains. After completing treatment with interferon, the patient began systemic chemotherapy with 5-fluorouracil. The patient was monitored at short intervals, with no local recurrence of the pancreatic tumor or other metastases and the optimal control of symptoms was obtained. The follow-up examination, one year later, revealed elevated glycemic values, diagnosed as a type 2 diabetes and the patient was started on therapy with long-acting insulin.1,2]. Most p-NETs are sporadic, but in 10% of cases they can occur in association with the hereditary syndromes such as MEN-1 syndrome, Von Hippel Lindau syndrome, tuberous sclerosis and neurofibromatosis type 1 . P-NETshave a low incidence of less than 1 per 100,000 persons, representing 1.3-2% of all types of pancreatic tumors.They are classified as functional p-NETs, associated with a particular clinical syndrome such as Cushing syndrome and non-functional p-NETs, frequently asymptomatic, associated with no particular medical sign. Non-functional p-NETs frequently secrete chromogranin A, neuron-specific enolase, human chorionic gonadotrophin subunits, pancreatic polypeptide or other peptides, but they do not manifest with particular clinical symptoms and thus they are referred as non-functional tumors. At the same time, approximately 10–30% of pNETs are functional and the most common functional p-NETs are gastrinomas and insulinomas, with the rare functional p-NETS represented by vasoactive intestinal peptide tumor (VIPomas) and somatostatinomas . Non-functional p-NETS frequently present with distant metastasis, especially to the liver and bones, as in our patient’s case, due to their asymptomatic nature. Although the majority of p-NETs occur in the head of the pancreas, in our patient’s case, the tumor affected the body and the tail of the pancreas . In our patient’s case, the first choice of treatment was the surgical excision of the pancreatic tumor, followed by treatment with somatostatin analogues and medical therapy for liver metastases, noting that the patient had diffuse bilobed involvement with associated bone metastases. In addition, systemic chemotherapy and a single dose of radiation therapy have been recommended. Furthermore, in our case, the occurrence of diabetes might have been either due tothe partial excision of the pancreas or due to the treatment with somatostatin analogues, which are known to reduce theinsulin levels secreted by the pancreatic cells. The diagnostic strategy includes localization of the tumor  and laboratory tests, in order to confirm the functional or non-functional status. Therapeutic management includes surgery as first choice therapy in resectable tumors, somatostatin analogue or specific molecular targeted therapy,systemic chemotherapy and liver-directed therapies  in patients with a metastatic disease . Our patient is still living, having a good quality of life of approximately 34 months since the diagnosis moment. Patients with p-NETS with metastatic disease have a worse prognosis, with a median survival rate of 22 months [


Large, atypical peripheral ossifying fibromas are known as giant peripheral ossifying fibromas. These lesions have often been associated with heterogeneous clinical and radiographic characteristics subsequently leading to their misdiagnosis. Biopsies have been the gold standard for the diagnosis of such lesions. This study reports on an acute presentation of giant peripheral ossifying fibroma, clinically mimicking a malignant lesion due to its atypical presentation along with its characteristic histological features, which led to the establishment of the diagnosis. Peripheral Ossifying Fibromas (POFs) are focal overgrowths, occurring in the gingiva .They areThis report presents a case of rapidly growing GPOF, clinically mimicking a malignant lesion due to its atypical presentation along with its characteristic histological features, which led to the establishment of the diagnosis.A 55-year-old North Indian male reported to theout-patient service of the current study with a complaint of a swelling in the right side of hisface for 20 days. History revealed that the swelling was initially the size of a pea, and grew rapidly to attain the present size. He also complained of mild pain and discomfort during mastication associated with the swelling. The patient’s medical history revealed that he was not currently under the care of a physician, had no known medical problems, and was not currently taking any medications. He had undergone dental extraction in the area associated with the swelling 6 months prior to referral. He reported that he wasa cigarette smoker, tobacco chewer, and alcoholic for 30 years. Extra oral examination revealed a diffuse swelling of the right side of the face, measuring approximately 5x4 cm in posterior body and angle region of the mandible . There wPeripheral Ossifying Fibromas are relatively common gingival growths, which are considered to be reactive in nature . In 1982Although POFs are generally less than 2 cm in theirmaximum dimension , 2, largThe clinical and radiographic features of GPOFs against that of conventional POFs have been tabulated in The clinical, radiographic, and histological features of GPOFs reported in literature have been tabulated in This atypical clinical presentation led to the impression of an aggressive or malignant lesion leading to the wide range of differential diagnoses, which were considered before the histopathological confirmation was obtained.While it is well accepted that POF is most likely a reactive lesion, few cases of GPOF are known to clearly establish the pathologic process . Amongst reactive lesions of the oral cavity, POFs show higher mast cell count compared to pyogenic granuloma and peripheral giant cell granulomas . It has More studies of GPOF have to be conducted to clarify recurrence, pathologic process, and establish whether GPOF has sufficiently distinct characteristics from POFs. Although GPOFs are rare, such lesions have to be considered during differential diagnoses even for huge lesions with acute onset similar to the present case. Incisional biopsies should be performed for such lesions in order to obtain a confirmatory diagnosis, which will aid abundantly for treatment planning. Biopsy technique for the sampling of such lesions can be critical. Care should be taken to harvest tissue fromthe deepest possible part of the lesion, as thisis necessary for diagnosis of such lesions.


Intracranial hemangiopericytomas (HPCs) are solitary fibrous tumors of the smooth muscle and the mesenchymal origin. While meningiomas located within the olfactory groove are common, an HPC in this location has never been reported previously. Here we describe the rare presentation of a differentiated HPC masquerading as an olfactory groove meningioma in a 33-year-old female presenting with the progressive headaches, anosmia, and visual field disturbances. Following resection, the histopathological analysis confirmed a grade II HPC. A preoperative understanding of the radiographic differences between the meningiomas and HPCs may confirm the treatment planning. An HPC must be considered in the differential diagnosis of the tumors located within the olfactory groove. Intracranial hemangiopericytomas (HPCs) are rare and constitute less than one percent of all the intracranial tumors . DerivedGiven the propensity of these lesions to bleed profusely during the surgical resection and their potential to metastasize distally, early identification is desirable and an aggressive multimodal treatment regimen is warranted. This can include a combination of one or more of the following: preoperative embolization, surgical resection, and/or radiosurgery. The HPCs are most commonly supratentorial dura-associated tumors; they can develop from the dural sinuses, skull-base, falx cerebri, and cerebellar tentorium . While mA 33-year-old Caucasian female presented with progressive headaches, ataxia, anosmia, diminished sense of taste, and visual field disturbances. The ophthalmology examination found papilledema. A comprehensive review of the systems and the physical examination were otherwise negative. The magnetic resonance imaging (MRI) demonstrated an irregularly shaped 6.3 X 4.8 X 4.8 cm intra-dural mass, suggestive of a meningioma arising from the olfactory groove. Intraoperatively, an origin from the olfactory groove was confirmed. Bi-coronal frontal craniotomy accomplished gross total resection of the highly-vascularized tumor Figure . ResectiPost-operatively, the computed tomography (CT) scan demonstrated removal of the tumor and a small amount of blood in the resection cavity. At discharge, on postoperative day (POD) eight, the patient was neurologically intact to the exam and ambulated independently. She was referred to a radiation oncologist for adjuvant therapy. At her six-week postoperative visit, her previous visual symptoms and headaches had subsided. The analysis of the tumor specimen revealed a spindle cell neoplasm associated with perivascular collagen, positive signal transducer and activator of transcription 6 (stat6) expression, normal mitotic activity (four mitoses per 10 high-powered fields), and no necrosis, all of which were consistent with the World Health Organisation (WHO) grade II lesion. An HPC had not been considered in the differential prior to the surgical resection, given the location of the lesion.BackgroundHemangiopericytomas form a small subset of all intracranial neoplasms and are typically diagnosed in the young males. The surgical management is challenged by the high vasculature nature of these tumors. Their ability to metastasize to other organs, including the lung and liver and their high rates of recurrence following gross total resection (GTR) require multimodal treatment.Although initially termed “angioblastic meningiomas,” these tumors were reclassified as HPCs by the WHO. Distinguishing an HPCs from a benign meningioma is particularly important as both tumor types are frequently found attached to the dura of the anterior, middle, and posterior cranial fossas. Meningiomas, however, are more common than HPCs, follow a more benign clinical course, and are generally less vascular during the surgery. While a subset of intracranial meningiomas arises from the olfactory groove, an HPC in this location has not been previously reported. Our unique case demonstrates the possibility of occurrence of an HPC in the olfactory groove and warrants effort to distinguish this more aggressive tumor from a benign meningioma, preoperatively.Hemangiopericytoma vs meningiomas: The radiographic differencesThe histopathological analysis is needed to confirm an HPC as opposed to a meningioma. Nevertheless, the subtle CT and MR imaging features of each entity may help distinguish between them. Chiechi, et al., in their series of 34 HPCs, found that both CT and MR demonstrated a narrow base of dural attachment, in contrast to the broad base of meningiomas. This was observed in our case. The hemangiopericytomas typically have a multi-lobulated, well-marginated contour. Unenhanced CT scans of HPCs may demonstrate adjacent bone erosion; this was found at surgery in our case and necessitated repair of a potential fistula, created by aggressive resection, with a pericranial graft. Unlike meningiomas, the HPCs rarely contain intratumoral calcification or evoke adjacent hyperostosis. The T-1 weighted and T-2 weighted MR images of the HPCs demonstrate heterogeneous, isointense masses with internal vessel flow voids, consistent with the intraoperative finding that these are highly vascular tumors (Figure Differentiation between the World Health Organisation grade II and grade III intracranial hemangiopericytomasThe recently revised WHO guidelines distinguish grade II and III tumors based on mitotic count and cellular phenotype. The grade II tumors have fewer than five mitoses per 10 high-powered fields. The grade III tumors have five or more mitoses per 10 high-powered microscopic fields, nuclear atypia, high cellularity, or a haphazard, cross-leafed growth pattern. In our case, the pathological analysis revealed a spindle cell neoplasm with low mitotic activity and irregular growth in association with thin vessels and collagen. A WHO grade II differentiated HPC was diagnosed.Zhou, et al. described the radiological criteria for separating grade II and III lesions, with grade III lesions being irregular in shape and having necrotic, hemorrhagic, and/or cystic components . These fIntracranial HPCs are rare, malignant, aggressive neoplasms derived from the smooth muscle cells. A poor clinical prognosis is often associated due to frequent local recurrence of the tumor after gross total resection and distal metastasis. Whereas the olfactory groove is a common site for a meningioma, this is the first reported case of the radiographically similar HPC in this location. As HPCs are highly vascular tumors, the surgical removal can carry significant risk. Appreciation of subtle radiographic differences between HPCs and meningiomas should inform the surgical preparation and performance. The WHO grade II and grade III HPCs should be distinguished from one another as they carry different prognoses and may warrant different treatment regimen.


With a prevalence of 0.4-3.5%, subepithelial lesions of the upper digestive tract are discovered during endoscopic procedures. Treatment depends on etiological and pathophysiological information, ability to diagnose and the different technical resources available.  To demonstrate the effectiveness of a surgical technique that combines endoscopy and videolaparoscopy in the transgastric resection of subepithelial juxtacardic lesions. The patients were assisted with a technical combination between endoscopy and laparoscopy. After diagnosis of subepithelial tumor, intraoperative endoscopy was performed after pneumoperitoneum and placement of laparoscopic tweezers. Through endoscopy, the following steps were performed: demarcation of surgical margins, visualization of the intragastric image for the laparoscopic procedure and removal of the surgical specimen. By laparoscopy the following steps were performed: intragastric intra-abdominal access, resection of the part and closure of the gaps.  This technique was applied in two cases in order to evaluate its initial results. There were two videolaparoendoscopic resections of juxtacardiac gastric tumors of the posterior wall. Both had their endoscopic diagnosis confirmed. After laparoendoscopic and tomographic and/or ecoendoscopic diagnostic complementation and preoperative performance, the laparoendoscopic procedure was indicated. The patients had a good recovery, with a short hospitalization time and no complications.  The combined use of videolaparoscopy and endoscopy is a safe and effective technique for transgastric resection of juxtacardiac subepithelial lesions. It may be important for definitive diagnosis of the tumor. Meanwhile, the surgical team initiates laparoscopy by installing two 5 mm subcostal trocars and one 10-11 mm supraumbilical, respectively, for access the Ultracision® (Ethicon) harmonic scalpel, forceps and 30° videocamera into cavity. The scalpel and the forceps are introduced into the stomach, already inflated by the endoscope, by two orifices of about 5 mm in the anterior wall of the organ, about 10 cm from the cardia. We then proceed to the resection of the demarcated area, guided by an endoscopic image. The gastric segment containing the lesion is removed by the mouth, using the Olympus® tripod, minimizing cavitary contamination. The laparoscopic time closes the resection site and the gastric lumen access holes, with continuous sutures in total plane with Ethibond® 3.0. At the end, the suture is tested by means of the “maneuver of the tire repairman”. The technical steps of the procedure are seen in By endoscopy , but in 34 (3.6%) they increased in diameter by at least 25%,Studies have suggested that videolaparoscopy is a method capable of providing a curative approach for almost all gastric subepithelial lesionsThe greatest technical difficulty encountered in this study was the small field to work in. However, this technique allowed the resection of tumors without the need for large resections, maintaining the stomach with its preserved capacity and without the need for anastomoses.The combined use of videolaparoscopy and endoscopy was shown to be safe and effective in the resection of posterior wall subepithelial lesions. It may be important for definitive diagnosis of the tumor.


One of the most commonly encountered medical problems affecting all age groups in health care is abdominal pain. There are many surgical and medical causes behind this symptom; however, a rare cause of abdominal pain and other unspecific abdominal complaints are gastric bezoars. Gastric bezoars are defined as the accumulation of undigested or partially digested foreign materials in the stomach. They are typically found incidentally during upper endoscopy. Patients may present with abnormal behavior or eating disorders such as pica. Therefore, proper history taking and establishing a good rapport with the patient play a key role in diagnosis. We present a case of trichobezoar-induced heartburn in an 18-year-old female. In this article, we will discuss the types, risk factors, clinical picture, diagnosis, and treatment of this condition. Bezoars are a rare cause of abdominal pain and other unspecific abdominal complaints such as heartburn, constipation, and anorexia. Therefore, thorough history taking in cases with unspecific gastrointestinal symptoms in the absence of a probable underlying cause should include inquiring about excessive ingestion of such materials.Gastric bezoars are defined as the accumulation of undigested or partially digested foreign materials in the stomach. Bezoars are termed trichobezoar when they are composed of hair.This is a case of an 18-year-old Caucasian female who was referred to us from an outlying facility for evaluation of heartburn and chronic abdominal pain. The patient started experiencing the pain several months before presentation. The pain was cramping in nature, intermittent, occurring approximately 3 times per week, lasting for a few hours with no relieving or aggravating factors. There was no history of altered bowel habits. The patient gave no history of previous surgeries. On further questioning, a positive history of hair eating (trichophagia) was obtained but with no history of alopecia, eating pattern change, thoughts of body image distortion, or previous psychiatric disorders.General examination of the patient was unremarkable with no signs of dehydration, halitosis, or lymphadenopathy. Local abdominal examination revealed diffuse mild tenderness of the abdomen. No palpable masses were appreciated. Normal bowel sounds were present on auscultation.Laboratory investigations detected microscopic anemia and mild leukocytosis. An upper gastrointestinal X-ray with air contrast was performed at an outlying facility and showed a distended stomach that appeared to be filled with debris, along with a normally filled duodenum. Esophagogastroduodenoscopy was performed at our institution, which demonstrated a trichobezoar occupying 75% of the gastric lumen extending down through the pylorus and into the duodenal bulb .A few attempts to remove the hair with scissors and forceps were made, but none were successful. Exploratory laparotomy and gastrostomy were performed afterward in which the bezoar was removed successfully and without any complications. It measured 17 × 6 × 7 cm and weighed 2.33 lbs. The patient recovered well and was discharged home with psychiatry clinic follow-up.1Heartburn and abdominal pain are common medical complaints seen in all ages including teenagers. History is helpful in identifying the underlying cause. Every so often, however, a case may present itself with a history that does not point to a favorable diagnosis; such is the case with gastric trichobezoars. It is a rare medical condition with clinicians encountering it around 0.3% of performed upper gastrointestinal endoscopies.17A gastric bezoar is defined as the accumulation of undigested or partially digested foreign materials in the stomach. Bezoars are classified based on their composition and/or location. Materials that compose bezoars include but are not limited to vegetable matter (phytobezoars), which are the most common type of bezoars, persimmon fruit (diospyrobezoars), which are the most common subtype of phytobezoars, hair (trichobezoars), and ingested medications (pharmacobezoar), for example, enteric-coated aspirin, nifedipine, theophylline, and sodium alginate. Other materials that can form bezoars include fungi, cement, milk curd, tissue paper, and vinyl gloves.8 Eventually, bezoars will grow with the accumulation of food, especially cellulose-rich foods and other indigestible materials, and form a conglomerate of various materials. Bacteria may colonize this collection resulting in halitosis.1 Hair accumulation occurs in the stomach but can also extend to the intestines, which may lead to Rapunzel syndrome.9The process of formation of trichobezoar begins with hair accumulation in the gastric folds. Human hair by nature is indigestible and resists peristalsis. Hair proteins get denatured by gastric acid and become oxidized, which in turn causes the change of hair color to black, regardless of the original hair color.8 People at a higher risk for developing bezoars are those with altered gastrointestinal motility or anatomy such as diabetics, patients with neurological disorders, or patients with previous gastric surgeries. Psychiatric illnesses and mental retardation are associated with bezoars due to pica, compulsive and excessive ingestion of food, or inedible substances. Trichobezoars, in particular, are more prone to develop in females and adolescents.10Previously, it was thought that bezoars were formed due to delayed gastric emptying. However, studies have shown that patients with normal gastric emptying can also develop them.8Patients may remain asymptomatic for extended periods of time. If symptomatic, typical clinical presentation includes upper abdominal pain, nausea, vomiting, weight loss, anorexia, early satiety, and symptoms of anemia such as dizziness, palpitations, and easy fatigability.8Clinical examination is usually unremarkable. Occasionally, an abdominal mass may be appreciated on palpation. Halitosis may also be present. Alopecia characterized by areas of patchy hair loss with broken hair strands of variable lengths indicate an association with trichotillomania and trichophagia.8Diagnosis is made using radiological studies such as abdominal X-ray with or without contrast and computed tomography scan or ultrasonography that reveal calcified, granular, or swirl-like structures of solid and gaseous material or filling defects within the stomach. Computed tomography with an orally administered contrast may reveal free-floating filling defects. It is necessary to exercise caution when diagnosing trichobezoars as they may easily be mistaken for malignancies of the gastrointestinal tract. On upper gastrointestinal endoscopy, trichobezoars appear as a black, green, or brown mass found in the funds or antrum of the stomach. Effort should be made to search for coexisting gastric ulcers, perforation, or any other gastric pathology.8A gold standard treatment plan is controversial due to the lack of studies that compare the different treatment modalities. Chemical dissolution of the mass can be used to treat patients with mild symptoms. Patients with two failed trials at chemical dissolution, resistant materials to chemical dissolution such as trichobezoar or moderate to severe symptoms should undergo upper endoscopy and have the mass endoscopically removed. The trichobezoar, however, may break and fragments may get displaced into the small bowel and cause obstruction. Surgery is reserved for cases with failure of the aforementioned treatment methods or complicated trichobezoars. Laparotomy with gastrotomy is the usual method of choice in such patients.12Bezoars may be complicated by pancreatitis, obstructive jaundice, steatorrhea, gastrointestinal perforation, peritonitis, protein-losing enteropathy, intussusception, appendicitis, constipation, pneumatosis intestinalis, and rarely gastric outlet obstruction.8Prognosis after removal is good. Recurrence can occur in 20% of patients and can be prevented by psychiatric evaluation, controlling precipitating habits, increasing water consumption, and assessment of possible underlying causes.Gastric trichobezoar, a bezoar composed of hair in the stomach, could be the cause of chronic abdominal pain or heartburn. It should be suspected when a likely underlying cause is not established particularly in patients with psychiatric disorders or with a history of trichotillomania and trichophagia. Good history taking plays a key role in diagnosing such cases. Diagnosis depends on upper gastrointestinal endoscopy. A gold standard treatment plan is controversial due to the lack of studies that compare the different treatment modalities. After successful management, psychiatric evaluation should be considered and the patient should be educated to prevent recurrence.