Metastasis to the penis is an uncommon occurrence, with only about 370 cases reported in the literature to date. The majority of the primary tumors are genitourinary in origin. We report on a patient with undiagnosed disseminated rectal adenocarcinoma, who first presented with lesions of the corporal bodies. A review of the literature indicates that corporeal metastasis as an initial presentation of malignancy is an extremely rare occurrence and carries a very poor prognosis. Despite the penis being an organ with rich vascularization, metastasis to this site is an extremely rare event. The majority of cases involve primary tumors from the pelvic region including bladder 30–35%), prostate 28–30%), rectosigmoid colon 13%), kidneys (8–10%), and testes (5%) [3%, kidne8–30%, re0–35%, prAn 88-year-old man presented to his urologist with a new complaint of penile lesion. The lesion was on the dorsum of the penis and midshaft and very tender. It appeared rather abruptly and was not associated with any recent trauma. He had urologic history including left orchiectomy and left groin radiation many years ago, benign prostatic hyperplasia (BPH) treated with transurethral resection of the prostate (TURP) twice, overactive bladder treated with oxybutynin, low testosterone treated with transdermal testosterone replacement, recurrent prostatitis, and irritative voiding symptoms. He had a history of prostate biopsy for elevated PSA and palpable nodule which was negative for malignancy as were all of his TURP specimens. He had also underwent urodynamic studies and multiple cystoscopies. Approximately 18 months prior to the appearance of the penile lesion the patient began complaining of intermittent pelvic pain, which usually resolved with treatment for prostatitis. His medical history also included hypertention, hyperlipidemia, arthritis, and chronic diarrhea.On physical exam the patient had hard nodules in the dorsum of his penis at the midshaft, which were exquisitely tender to palpation. These lesions were not clearly consistent with Peyronie's disease; therefore he underwent magnetic resonance imaging (MRI) of the pelvis which showed multiple nonenhancing low signal lesions in the corpus spongiosum and bilateral corpora cavernosa suggestive of metastatic disease .An incisional biopsy was performed which confirmed adenocarcinoma with colonic origin. He next underwent a computed tomography (CT) to evaluate the extent of disease which showed 11.3 cm sigmoid mass with multiple enlarged lymph nodes adjacent to the sigmoid as well as in the retrocrural, para-aortic, and parailiac regions. It also demonstrated lesions consistent with metastases in the liver, adrenals, and lungs .The patient was referred to hematology-oncology. His overall condition began to deteriorate rapidly including decreased exercise tolerance, decreased appetite, and weight loss. Because he would not likely tolerate systemic chemotherapy he was offered palliative treatment in the form of targeted chemotherapy after testing his tumor for genetic mutations. The patient requested time to consider his options. He passed away at home 5 weeks after his biopsy and 9 weeks after he initially presented with penile lesions.The first report to ever describe metastatic disease to the penis was in 1870 . ColonicInitially one should consider more common diseases which can cause penile or corporal lesions when developing a differential diagnosis for a patient complaining of penile lesions. Benign lesions of infectious  and noninfectious (benign priapism and Peyronie's disease) etiologies should be considered. Primary tumors of the penis are much more common than secondary tumors, and one should consider squamous cell carcinoma, invasive penile carcinoma, sarcoma, melanoma, and extramammary Paget's disease in the differential as well. In two case reports , 7, the Metastatic lesions to the penis or corporal bodies may present with a wide range of complaints. The most common presenting complaint is priapism (40%), followed by urinary retention, penile nodules, ulceration, perineal pain, edema, generalized swelling, broad infiltrative enlargement, dysuria, and hematuria . Most ofSeveral routes of metastasis to the penis have been proposed including retrograde venous spread, retrograde lymphatic spread, arterial spread, direct extension, and implantation secondary to instrumentation . RetrogrIn order to make a diagnosis of the penile lesion, a biopsy or corporeal aspiration is needed. It is important to differentiate primary versus secondary tumors, since this will dictate treatment. After obtaining a biopsy for pathologic diagnosis noninvasive imaging can be performed using PET, CT scan, or MRI in order to evaluate the extent of disease.It has been demonstrated in the literature that most patients with penile metastasis have widespread disease at the time of presentation and treatment is often oriented toward palliation. This usually includes a combination of chemotherapy and radiotherapy. Priapism can be treated with dorsal nerve block or penectomy, if the pain is refractory to less invasive measures. Penectomy can also be performed with intent of cure, in the rare instances when a patient presents with local disease. Such a case was reported on a patient with recurrent rectal carcinoma, who presented with a localized lesion in the corpus spongiosum . UnfortuMetastases to the penis are an uncommon occurrence. Most of the cases reported have synchronous primary malignancies present or primary tumors that were previously diagnosed. Our case was unusual, in which the penile mass was the initial presentation of a nonurologic primary malignancy. While these cases are rare, clinicians should remain aware that such presentations can occur and keep them in their differential diagnosis. Regardless of the site of primary tumor or treatment modality, early referral to hematology-oncology is paramount as the prognosis remains poor for these patients.


A 52 Year old male was admitted with respiratory distress. Radiological examination revealed a large mass in patient’s right hemi thorax with mediastinal shift and partial lung collapse. Biopsies previously done conferred the diagnosis of solitary fibrous tumor; however, in order to avoid a surgical resection, patient didn’t follow the adviced procedure. After thorough counseling, surgical resection was done with few post operative complications and patient recovered well with ability to perform his daily activities with partial support. The histopathology results showed solitary fibrous tumor. Apart from pneumonia and local wound infection, patient status was well for the next six week follow-up.  Their origin is much debated. Though most arise from the mesothelial cell, occasionally some arise from the pleural fibroblast.Over the years this entity has acquired a number of synonyms, including localized fibrous tumor, benign mesothelioma, localized fibrous mesothelioma, submesothelial fibroma, and pleural fibroma. The usage of term ‘mesothelioma’ for this tumor is discouraged because of potential confusion with diffuse malignant mesothelioma, a much more serious disease.2,3 Though they can arise virtually from any site in the soft tissue, pleural fibromas mostly originate in the visceral pleura upto 80%, while 20% arise from the parietal pleura.4The term solitary fibrous tumor (SFT) was first mentioned in scientific literature by Wagner in 1870. However the first discussion of its clinical and pathological properties was explained by Klemperer et al. in 1831. Approximately 78-88% of the SFT’s are benign and 12 to 22% are malignant.Symptoms of SFT’s are non-specific and mostly include chest symptoms like cough, dyspnea or chest pain which mostly correlates with tumor size. CT Scan and MRI remain the choice for determination of the relationship of mass with surrounding tissue in case of large masses. Malignant transformation is also not uncommon.A fifty two year old male was admitted via Emergency department with complaints of shortness of breath. Patient had history of intermittent episodes of shortness of breath for last two years with each episode being worsened from the previous one. The last episode started one month ago restricting his physical activity only to few steps; Shortness of breath is partially relieved by lying laterally. It was also associated with productive cough with white scanty, non-foul smelling sputum, occasionally mixed with blood. Patient also received ATT (anti tuberculosis therapy) for last fifteen days without any betterment in his status. He holds past two year record of admissions, where he was treated for pneumonia initially; later two admissions were done with extensive workup firstly in August 2012 and latter January 2013. Biopsies were done both the time and both confer the diagnosis of solitary fibrous tumor. Patient was advised for followup in both institutes, but never returned and finally landed in our institute in order to avoid a possible surgical resection.Clinically patient was restless, tachycardic and tachypneic but maintained oxygen saturation on 3-4 liter of oxygen. His chest examination showed decrease right sided movements with dull percussion note, decrease breath sounds. Lab investigations further revealed anemia with Hemoglobin of 8.0 gm/dl, blood gases showed mild hypoxia with PO2 of 80mm Hg while rest of his metabolic panel & co-agulation profile were within normal limit.Chest X-ray showed a large homogenous opacity on right side with mediastinal shift. CT Scan of chest confirmed the finding of a large heterogeneous mass with few sparks of calcification occupying almost whole of the right hemi thorax. Medially the mass was abutting and pushing superior vena cava, Aorta, right pulmonary artery and heart. Mass was also causing compression effects on the right hemi-diaphragm. It is displacing the mediastinal contents on to the left and caused compression collapse of right medial and lover lobe. Minimal pleural effusion with pleural thickening was also noted.  and 2After thorough discussion and multiple settings; patient was counselled for surgical removal of the mass and its catastrophic outcome in case of further delay. Explorative thoracotomy was done and a huge extra pleural mass was removed. Right upper lobe of the lung was also adherent to the mass. Careful incision was performed to remove the mass preserving the surrounding vascular structures with minimal blood loss. The size of excised mass 24 x 20cm x 8cm and it was 3150gm in weight. . The lowThe pathological examination revealed a single multi nodular encpsulated pale white firm piece of tissue. On serial sectioning two collapsed cavities were also indentified with larger one measuring 5 x5 cm filled with yellow pleurant exudate. A flap of pleura id identified too measuring 12x6 cm.Microscopic examination revealed demarcated lesion composed of spindloid cells. The nuclei are oval to elongated with evenly dispersed chromatin. Cytoplasm is abundant. Scattered mitotic figures are seen. Areas of hyalinization and fibrosis are noted. Conclude as neoplastic lesion; solitary fibrous tumor as possibility.Patient was kept on mechanical ventilation, but, as per plan, the ICU team could not wean him off in next two days. Patient also developed fever spikes and later developed ventilator associated pneumonia. Tracheal cultures revealed acinetobacter. Intravenous anti-biotics were given as per sensitivity and after six days patient was finally extubated. Drains were removed on third day of surgery. Physical capacity continues to increase slowly and patient was able to walk short distances without any discomfort.5 Consequently, the failure or initial management led to further investigation and diagnosis of the sinister wasting clinically important time period, when the in growing tumor is causing increased pressure effects. The difficulty can further be bundled in developing countries where clinicians solely clinical acumen for diagnosis or due to scarcely available diagnostic resources.5Pleural fibrous tumor are rare tumors and due to their non-specific symptoms are often misdiagnosed as pleural effusions, pneumonia, Tuber-culosis or else.3 However the rate of recurrence is higher in malignant SFT with poor prognosis, most of the patient dying within two year of diagnosis.1Prognosis of solitary fibrous tumor is excellent and only 8% of the disease will reoccur after first resection, with the recurrence usually cured after additional surgery.6 Complete resection yields better long term survival as compared to partial resection and decrease the incidence of recurrence too.6If Malignant SFT is associated with pleural effusion then recurrence and mortality is recorded even higher.7 In our case, though the patient was earlier diagnosed on basis of Chest X ray and treated as Tuber-culosis, but sooner he was diagnosed on biopsy as solitary fibrous tumor. Yet, his surgical resection was delayed for at least seven month indicating lack of counselling; a pivotal role in modern day clinical practice and ethics.CT scan is a valuable tool and contrast enhanced images help not only in diagnosis but also modify surgical decisions. MRI is also comparable in this regard.8 On the contrary, if our patient had been counselled properly, a neoplastic lesion could have been operatively removed earlier with lesser complications and morbidity.Counselling plays a vital role in development of doctor-patient relationship, patient’s satisfaction and course of their disease.In view of it, we emphasize the practicing clinicians especially in developing country like ours that counselling in comparison with therapeutics is equivalent and if not done properly may result in conversion of an amendable disease to latter more morbid state/disease.SH: Critical revision for important intellectual content and final approval of the version to be published.SSH: Concept, Design, Acquisition of Data and Critical revision for important intellectual content.MAA: Concept, Design and Critical revision for important intellectual content.SS: Design and Acquisition of Data.


Rhabdomyosarcoma is a soft tissue neoplasm that usually arises in the headand neck region and genitourinary tract. Skin metastasis of rhabdomyosarcoma is extremely rare; of thirteen cases reported in the literature, most were children younger than 10 years and only three cases have been reported in adults.A 20-year-old Moroccan man was admitted with a right orbital tumor. The tumor was excised and histopathology examination confirmed a diagnosis of rhabdomyosarcoma. The patient was treated with chemotherapy, but local recurrence occurred one year later. The patient underwent right orbital exenteration followed by chemotherapy and radiotherapy. After 6 months, the patient developed a cutaneous mass in the right lumbar region, which was resected. Immunohistochemical examination of the tumor showed this to be a cutaneous metastasis of rhabdomyosarcoma. The patient was treated by chemotherapy and there appeared to be no recurrence after 9 months of follow up.Skin metastasis from rhabdomyosarcoma is extremely rare, particularly in adults. The purpose of presenting this case report is to raise awareness among clinicians— skin biopsy and immunohistochemistry are needed to distinguish this neoplasm from other cutaneous tumors so that appropriate treatment can be initiated. Rhabdomyosarcoma (RMS) is the most common soft-tissue sarcoma in childhood and adolescence. Approximately 350 new cases occur each year in the US . The mosA previously fit and well 20-year-old Moroccan man presented with a right orbital tumor measuring 2 cm in diameter that had been rapidly increasing in volume. A complete resection of the tumor was made. The diagnosis was made on histological examination, confirming the orbital tumor to be an embryonal rhabdomyosarcoma.The patient received six cycles of adjuvant chemotherapy with alternating vincristine, adriablastine and cyclophosphamide(VAC). One year later, the patient developed a local recurrence. Exenteration was performed,followed by treatment with six cycles of ifosfamide plus etoposide (IE); local radiotherapy was also given at a dose of 45 Gy. However, after 6 months, the patient developed a cutaneous mass in the right lumbar region, which was resected. A computed tomography - scan of the thorax, abdomen and pelvis did not reveal any metastasis. Macroscopically, the tumor size was 4 × 4 × 2.2 cm; the cut surfaces were nodular and fleshy; there was no necrosis and the edges showed an infiltrative pattern. Histologic sections with microscopy showed a proliferation of undifferentiated round cells arranged in sheets with some tapered eosinophilic cytoplasm. High mitotic rate was seen. The tumor cells infiltrating the subcutaneous tissue. Hematoxylin and eosin stained sections revealed loosely arranged round cells with large hyperchromatic nuclei and rare strap cells with eosinophilic cytoplasm Figures  and 2. IPhotomicrographs showing a sheet of undifferentiated round cells. The tumour cells were immunopositive for desmin Figure  and myogRMS is an uncommon neoplasm in the adult population. Metastasis to skin is extremely rare in both children and adults with RMS. Only three adult cases of RMS with skin metastases have been reported in the literature –8. Most Most cases of RMS skin metastasis are of the alveolar subtype, which is most often seen in pediatric patients . It is oCytogenetic analysis plays an important role in confirming the diagnosis, but only for alveolar RMS because there is no specific cytogenetic or molecular markers for embryonal RMS. Alveolar RMS is associated with a specific translocation, t or its variant t .Treatment for this tumor is multimodal, including surgical excision with or without radiotherapy and chemotherapy. The prognosis of this disease depends on the location and number of metastatic sites, and the treatment received . The 13 Embryonal RMS with skin metastasis is quite rare and can readily be misdiagnosed. Skin biopsy with immunohistochemistry is necessary to make the diagnosis and to distinguish this malignancy from others cutaneous conditions, so that adequate treatment can be initiated.Written informed consent was obtained from the patient for publication of this Case Report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.


Epidermoid cysts represent common benign tumors occurring anywhere in the body but very rarely in the penis. Only a few cases of penile localization have been reported in the literature so far, most of them being congenital and/or idiopathic, usually presenting in children as slow-growing, solitary, well-delimited cystic lesions. Here, we describe the case of a patient with a penile epidermoid cyst presenting as an ulcerated lesion of the coronal sulcus, thus mimicking penile cancer.A 36-year-old Caucasian man presented with a three-month history of a rapidly growing asymptomatic ulcerated lesion in the ventral portion of the penile coronal sulcus. At surgical exploration, the area under the ulcerated lesion had a well-demarcated cystic shape; following its wide excision, an intraoperative histological examination revealed an epidermoid cyst. No recurrence had occurred at nine years of follow-up.Rare benign tumors of the penis, like the described epidermoid cyst, may mimic cancer. Nevertheless, penile ulcerated lesions should always be surgically explored as wide excision and intraoperative histological examination remain the only means of obtaining a precise disease definition and, consequently, administering the appropriate treatment. Epidermoid cysts represent common benign tumors arising from the infundibular part of hair follicles, occurring anywhere in the body but very rarely on the penis. In fact, only a few cases of penile localization have been reported in the literature so far, the vast majority being congenital and/or idiopathic lesions diagnosed during childhood. TherefoWe report here the first case, to the best of our knowledge, of a penile epidermoid cyst presenting like an ulcerated lesion of the coronal sulcus, thus mimicking penile cancer.A 36-year-old Caucasian man presented with an asymptomatic ulcerated lesion in the ventral portion of the coronal sulcus Figure , which hEpidermoid cysts of the penis are extremely rare. Most of reported cases occurred in children, thus suggesting the etiology of ‘primary’ penile epidermoid cysts being embryonic developmental defects, particularly abnormal closure of the median raphe. To the Reported cases of both primary and secondary penile epidermoid cysts presented as slow-growing, solitary, well-delimited cystic lesions with a smooth and soft appearance,3. Our cAlmost all reported cases of penile epidermoid cyst occurred on the ventral aspect of the penile shaft, whereas only one case involved the glans penis. Again, It has, however, been postulated that the coronal sulcus may act as a cleft where hairs may collect and are forced to penetrate into the penile shaft and foreskin by the natural movement that occurs between these two surfaces. It coulNeoplastic transformation of epidermoid cysts is rare, and it has never been reported in penile cases-3; in anIn conclusion, ulcerated lesions of the penis should always be suspected of being cancer and, therefore, be surgically explored. In suchWritten informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.The authors declare that they have no competing interests.LC conceived the study and revised the manuscript.VM drafted the manuscript. FS carried out the pathology evaluation. PM carried out the data acquisition and data analysis. FL carried out the pathology evaluation. PB participated in the pathology supervision. TA contributed to the supervision. GC contributed to the supervision. All authors read and approved the final manuscript.


A 12-year-old adolescent presented with refractory seizures and was found to have a mesial temporal lobe lesion. The patient underwent biopsy and was diagnosed with an arteriovenous malformation. Supratentorial lesions in the pediatric population can have a large variety of underlying etiologies, which can be challenging to differentiate on neuroimaging. In this report, we discuss the key features on MRI of several neoplastic, vascular, and infectious processes that can aide in the diagnosis. A 12-year-old adolescent presented to his primary care physician with new onset staring episodes. His family stated the patient would be unresponsive during these episodes and would make a “wringing” motion with his hands. The episodes lasted about 1 min and were not preceded by an aura. After the episodes, the patient felt tired. At the time of presentation, the episodes were occurring daily. He was initially started on carbamazepine by his primary care physician who thought the episodes were consistent with seizures and he was referred to neurology. His past medical history was unremarkable. He was a full-term birth and had normal development. He was performing well in school and involved in several athletic activities.On presentation to neurology, it was thought his episodes were consistent with complex partial seizures. His neurologic examination was unremarkable. His seizures were well controlled on the carbamazepine, which was continued and an EEG and MRI brain with and without contrast were ordered. The EEG was unremarkable, but the MRI brain showed a left mesial temporal lobe lesion Figure . At thisA biopsy of the left mesial temporal lobe lesion was analyzed Figure . On micrThis case demonstrates the difficulty in distinguishing neoplastic, vascular, and infectious lesions on imaging. Additionally the patient presented in this case had an initial biopsy that was inconclusive, which further supports the importance of gleaning as much information as possible from imaging studies. In this report, we provide a brief summary of the classic imaging characteristics of a variety of neoplastic, vascular, and infectious etiologies and discuss how this may have helped with the initial work-up and diagnosis of this patient.In the pediatric population, supratentorial cortical based tumors represent 25–40% of all brain tumors . Most ofInfectious and vascular etiologies would also be on the differential for this patient. Infectious etiologies to consider are an abscess and limbic encephalitis. Abscesses can have a range of characteristics on MRI depending on the age of the lesion. Early in the course it will have an ill-defined shape with patchy enhancement . As the The primary vascular lesions that would be considered are cavernous malformations and AVM. The majority of cavernous malformations occur supratentorial and typically present in the third through fifth decades of life, rarely in childhood. Cavernous malformations can have a wide range of radiological appearances on imaging depending on the lesion type (I–IV) . A high 18F]fluoro-d-glucose (FDG)–PET scans analyze the uptake of glucose, which is increased in malignant tumors. PET scans are especially helpful for distinguishing recurrent tumor from radiation necrosis (11C] methionine (MET) and 18F-fluroethyl-tyrosine (FET) are currently available options, but one limitation is that they are not commercially available at all institutions  and functional/metabolic studies . MR spectroscopy identifies markers of cellular proliferation and certain profiles are suggestive of neoplastic lesions versus infection or ischemia , 2. Simiitutions . Additioitutions . AlthougThis case of an underlying AVM resulting in a refractory seizure disorder highlights the challenge in distinguishing neoplastic, infectious, and vascular lesions based on imaging. MRI can provide a great deal of information about a lesion and it is important to carefully analyze available imaging to obtain as much information as possible. Additionally, when uncertainty remains about diagnosis, it may be helpful to utilize other imaging modalities including vascular imaging, MR spectroscopy, and PET scans. Finally, when a diagnosis remains unclear, biopsy needs to be considered, but ultimately it is the entire work-up and clinical picture that will lead to a correct diagnosis.The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.


Renal angiomyolipoma (AML) is the most common type of benign mesenchymal tumor of the kidney. AMLs typically present as benign lesions without local invasion. However, the tumor may exhibit aggressive behavior. Intravascular extension into the inferior vena cava (IVC) and hemorrhagic aneurysm formation associated with AML has rarely been reported in the past. In the current study, the novel case of a 77-year-old female is described who presented with a tumor thrombus extending to the IVC. The patient subsequently underwent a radical nephrectomy and an IVC tumor thrombectomy. In addition, the available literature regarding this unusual complication of a common renal neoplasm has been reviewed. It is essential for radiologists and clinicians to be aware that AMLs may exhibit these types of aggressive behaviors. Renal angiomyolipomas (AMLs) are usually benign lesions, however, liposarcomatous degeneration has previously been described . AMLs maA 77-year-old female patient was incidentally diagnosed with a tumor of the right kidney during a health examination by ultrasound at the Department of Urology, First Affiliated Hospital  on September 10, 2013. There was no history of right flank pain, dysuria, gross hematuria, abdominal distention, pyrexia or lower extremity edema. The patient had been diagnosed with hypertension five years previously and was receiving regular medication. The family history of TS was negative and a physical examination and laboratory tests revealed no abnormalities. Ultrasonography revealed a hyperechoic mass at the lower pole of the right kidney, which extended into the IVC. Furthermore, a contrast-enhanced abdominal computed tomography (CT) scan revealed an extensive mixed hypodense mass, which was predominantly composed of fat , a subtype of AML, is considered to be a unique type of renal tumor with malignant potential . Luo et et al  reportedEarly diagnosis of AML predominantly relies on imaging techniques. Ultrasound is an effective screening method and CT provides the greatest diagnostic value. Typically, AMLs are rich in fat and represented on CT scans by a low signal, rendering them easy to diagnose . Approxiet al (et al (AML is considered to be a benign neoplasm and is associated with a favorable prognosis. However, a rare case of renal AML presenting with liposarcomatous transformation has previously been reported . Yiu et et al  summarizet al  identifil (et al  hypothesIn conclusion, it is essential that urologists consider the potential presentation of AML involving the renal vein and/or IVC. In addition, it is proposed that the risk and benefits of surgical treatment are discussed prior to surgery. However, increased attention on establishing a variety of methods for treating this type of lesion is required.


Alpha-fetoprotein is produced by a variety of tumors such as hepatocellular carcinoma, hepatoblastoma, and germ cell tumors of the ovary and testes. However, we present a case of significantly elevated serum alpha-fetoprotein without evidence of malignant disease in a patient who is a carrier of chronic hepatitis B.A 60-year-old Korean man presented with markedly increased alpha-fetoprotein . Various diagnostic evaluations, including computed tomography of the abdomen and thorax and ultrasonography of the abdomen and testes, showed liver cirrhosis and mild splenomegaly; however, no mass was detected in the liver, testes, or other organs scanned. The laboratory findings showed elevated liver function, positivity for hepatitis B e antigen, and a marked increase in hepatitis B virus deoxyribonucleic acid copy number (>7 × 105 IU/mL). Our patient was diagnosed with acute exacerbation of chronic hepatitis B, and we presumed that this condition might be related to extremely elevated alpha-fetoprotein. When our patient was treated with entecavir, the serum alpha-fetoprotein level immediately decreased, in parallel with the hepatitis B virus deoxyribonucleic acid copy number.We report a rare case of extremely elevated alpha-fetoprotein due to acute exacerbation of chronic hepatitis B without any malignancy, and a decrease in this tumor marker simultaneous with a decrease in hepatitis B virus deoxyribonucleic acid copy number on entecavir treatment. This case report is important due to the rarity of the case; furthermore, it provides details of a diagnostic process for a variety of benign diseases and malignant tumors that should be considered in patients with elevated alpha-fetoprotein. Thus, we present a case report, along with a review, that will be helpful for diagnosis and treatment of patients with elevated alpha-fetoprotein. Serum tumor markers (STMs) are widely used in clinical settings as effective indicators for screening of malignant tumors, diagnosis of tumor type, establishing a prognosis, analyzing treatment effects, and early detection of relapse. Although the roles of STMs are limited in many cases, owing to their varying sensitivity and specificity, when STM values are markedly elevated, the corresponding cancer is strongly suspected . In partA 60-year-old Korean man was admitted to our institution due to elevated α-FP discovered during a health screening examination. He had been diagnosed with chronic hepatitis B 6 years prior and received regular follow-up observations; however, he had not been prescribed any medication, as no abnormal findings associated with indicators of active hepatitis, cirrhosis, or liver cancer were discovered. In addition, our patient had not received an abdominal ultrasound examination or a blood test in the past 2 years.Our patient showed a highly elevated α-FP level of 2350 ng/mL , along with elevated aspartate aminotransferase (AST), 201 U/L (5–40 U/L), alanine aminotransferase (ALT), 209 U/L (5–40 U/L), total bilirubin, 1.91 mg/dL (0.2–1.2 mg/dL), direct bilirubin, 1.3 mg/dL, alkaline phosphatase, 100 U/L (35–123 U/L), and gamma-glutamyl transpeptidase, (r-GTP) 256 U/L (15–73 U/L). Our patient had no history of taking herbal or traditional Korean medicines or western medicines, and no history of infection or travel.Although our patient was considered to be at high risk for liver cancer because he was a carrier of chronic hepatitis B, no liver tumors were observed on the abdominal ultrasound image; however, splenomegaly accompanied by moderate cirrhosis was found. Interventional dynamic computed tomography (CT) was performed on his chest, abdomen, and pelvis for precision testing of the liver parenchyma and assessment of cancers that typically show elevated α-FP, such as testicular cancer and germ cell cancer, along with paraneoplastic syndrome, which also results in secretion of α-FP. However, the findings again indicated only cirrhosis, as no tumor or lymphadenopathy was observed in the liver parenchyma, chest, abdomen, or pelvis. Moreover, there were no specific findings from a precision urological physical examination and a testicular ultrasound scan. In addition, gastric endoscopy was performed to test for α-FP-secreting hepatoid gastric adenocarcinoma, a common gastric cancer in Korea that is known to have a poor prognosis; however, no malignancy was detected. Other tumor markers, such as beta-human chorionic gonadotropin (β-HCG) <1.20 mIU/mL (0–1.25 mIU/mL), protein induced by vitamin K absence or antagonist-II (PIVKA-II) 11 mAU (0–39 mAU), and carcinoembryonic antigen (CEA) 2.8 ng/mL (0–5 ng/mL), were all within the normal range.5 IU/mL), he was diagnosed with acute exacerbation of chronic hepatitis B. Although it is extremely rare for α-FP to be elevated above 2000 ng/mL  due to acute hepatitis B, there was a case study reporting decreased α-FP following treatment for acute hepatitis B. Treatment for hepatitis B was administered first and a follow-up imaging test was scheduled for 2 months later.Precision testing for hepatitis was performed because our patient had a history of chronic hepatitis B and showed elevated AST and ALT. The test results were hepatitis B surface antigen positive (HBsAg+), hepatitis B e antibody negative (HBsAb−), and hepatitis C virus antibody positive (HCV Ab−), and additional tests showed hepatitis B e antigen positive (HBeAg+) and hepatitis B e antibody negative (HBeAb−). Moreover, since our patient also showed a marked elevation in hepatitis B virus deoxyribonucleic acid (HBV DNA) copy number  significantly decreased. For AST/ALT and α-FP, these values further decreased to 53/47 U/L and 370 ng/mL, respectively, after 2 months, and to 39/36 U/L and 163 ng/mL, respectively, after 3 months. After treatment with entecavir, the HBV DNA copy number decreased to an immeasurable level after 3 months and α-FP was normalized after 11 months  for prostate cancer, cancer antigen 125 (CA125) for ovarian cancer, and alpha-fetoprotein (α-FP) for hepatocellular carcinoma (HCC) is used as a minimally invasive screening test using blood samples [et al. purified human α-FP in 1971 and developed a radioimmunoassay technique to quantify it, which has since been used in clinical settings. Currently, α-FP is traditionally and widely used as a tumor marker for HCC. However, the sensitivity and specificity for using α-FP to detect HCC is only in the range of 41–65 % and 80–90 %, respectively, as serum α-FP is normal in up to 35 % of small hepatocellular carcinoma cases [Among these, α-FP is a glycoprotein secreted by fetal cells during the embryonic stage and in some malignant diseases. In 1944, Pederson first discovered a fetus-specific serum, and in 1964, Tatarino reported α-FP in the serum of a patient with hepatocytic cancer. Ruoslahti ma cases –4.Several tools used alone or in combination with α-FP have been evaluated, as there are many different views regarding the use of α-FP as a biomarker. For example, a recent Japanese guideline indicates the combined use of tumor markers  for the diagnosis of HCC .Although α-FP is widely known as a major marker for primary liver cancer, it is also known to increase with a variety of other diseases such as various malignant tumors, hepatic necrosis, acute hepatitis, colitis, and gynecological diseases . GeneralAs the name fetoprotein indicates, levels of this protein are elevated during pregnancy, but can also be elevated, along with ß-human chorionic gonadotropin (ß-HCG), in ovarian, endometrial, testicular, and extragonadal germ cell cancers, depending on gender. Therefore, in addition to testing for serum ß-HCG and imaging the uro-reproductive system, a close examination by a urologist or gynecologist is necessary. In particular, extragonadal germ cell tumors often develop in the retroperitoneum and mediastinum, whereas teratomas are observed in a variety of areas, and have been reported to become more frequent following lung cancer; hence, chest imaging is also required , 7. In KFew cases have been reported in which α-FP was elevated without any evidence of a malignant tumor; however, studies have reported values elevated to 2002 ng/mL in young patients with autoimmune hepatitis, and up to 371.51 ng/mL in adults with cavernous hemangioma , 10.A case similar to the current case, in which α-FP was elevated to >2000 ng/mL due to acute exacerbation of hepatitis, but normalized following anti-viral therapy, as well as a few other cases have been reported internationally. However, cases like these are extremely rare, and even in the above-mentioned cases, the authors only discussed proof for the absence of liver cancer and the treatment process for acute hepatitis; they did not discuss ruling out other diseases that could have potentially increased α-FP –13.Here, we present a case of α-FP elevation due to acute exacerbation of chronic hepatitis without any evidence of a malignant tumor, along with a literature review.This case report is important because of the rarity of the case, and provides details of a diagnostic process for benign diseases (acute hepatitis and hemangioma) and malignant tumors  that should be considered in patients with elevated α-FP. We believe that the findings presented in this report will be helpful for the diagnosis and treatment of patients with elevated α-FP.


Symptomatic presentations of colonic lipomas are very rare in clinical practice, and may mimic colonic malignancy. The likelihood of presenting symptoms has been shown to depend on the size of the lesion.We describe the case of a 72-year-old Sinhalese man presenting with worsening mucoid diarrhea who was subsequently diagnosed to have a lipoma of the sigmoid colon. His disease was successfully managed with endoscopic resection.Confidently establishing the rare diagnosis of a colonic lipoma usually requires a combination of endoscopic, radiological, and histological evaluation, and is therefore very challenging. With the advancement of endoscopic procedures, endoscopic resection is widely practiced as the definitive management of these cases. Colonic lipomas are rare mesenchymal tumors that are usually asymptomatic. However, large lesions have been described to cause symptoms like abdominal pain, constipation, diarrhea, and even obstruction due to intussusception . The epiA 72-year-old Sri Lankan Sinhalese man presented to our clinic with a 3-month history of worsening mucoid diarrhea. Initially his diarrhea was infrequent and mild, but had gradually worsened to 15–20 episodes per day with associated tenesmus and occasional blood staining, significantly limiting his daily activity. He had no loss of appetite or weight loss. His medical history was unremarkable except for dyslipidemia, for which he was taking atorvastatin (10 mg daily). He had no history of past surgery. Results of an abdominal examination were normal and a digital rectal examination did not reveal any masses or blood. Results of laboratory investigations, including a full blood count, basic biochemistry, and tests for inflammatory markers, were within normal limits.Subsequently, our patient had a colonoscopy, which revealed a single 3 × 2 cm pedunculated, spherical, smooth mass with minimal surface ulceration in his lower sigmoid colon Fig. . InitialThe procedure was uncomplicated and resulted in a dramatic improvement in our patient’s symptoms. A histological examination confirmed the presence of mature adipose tissue in a submucosal location. Sigmoidoscopy 2 weeks after the procedure showed a well-healed excision site.et al. estimates that colonic lipomas constitute about 0.3 % of cases involving colorectal problems and 1.8 % of cases of benign colorectal disease [Although colonic lipomas are the second most common benign colonic tumor, they are surprisingly rare. Since the first case was reported by Bauer in 1757, only about 300 cases have been recorded according to the literature . A recen disease .Lipomas are usually found incidentally during imaging or colonic surgery for other indications. The probability of being symptomatic correlates with the size of the lipoma; lesions larger than 4 cm become symptomatic in 75 % of cases . The symRadiographical investigations like barium enema, computed tomography (as in our case), or magnetic resonance imaging may assist in the diagnosis. However, lesions smaller than 2 cm are difficult to visualize radiologically and cases with complications like intussusception or bowel wall thickening may be misdiagnosed as malignancy . EndoscoColonoscopy usually demonstrates a pedunculated or sessile smooth lesion that may demonstrate the “pillow” or “cushion” sign when indented with closed biopsy forceps—that is, it promptly reassumes its previous shape on release . SuperfiSmall <2 cm) asymptomatic lesions, if unequivocally diagnosed, can be simply followed up as there is no significant malignant degeneration  cm asymp. Staged Establishing the rare diagnosis of a symptomatic colonic lipoma can prove challenging, requiring multidisciplinary input. However, once the tumor is confidently diagnosed, modern endoscopic techniques have revolutionized the management, making surgery necessary only in complicated cases.Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.


Hemangioma is the most common benign tumor of the liver. Liver hemangioma (LH) usually remains asymptomatic, but the most common symptoms associated with LH are abdominal pain and discomfort. LH is an uncommon cause of bile duct dilatation and obstructive jaundice. An 83-year-old Japanese woman who received hemodialysis at another hospital was referred to our hospital because of abnormal liver function and obstructive jaundice. Abdominal computed tomography and magnetic resonance imaging revealed a 13-cm tumor in liver segments IV–V and intrahepatic bile duct dilatation. Endoscopic retrograde cholangiopancreatography revealed extrinsic compression of the bile duct at the hepatic hilar region. Laboratory tests showed that the patient had low platelet counts and low fibrinogen levels. Because the patient had hyperbilirubinemia and Kasabach-Merritt syndrome, we performed a segmentectomy of liver segments IV and V. Histological examination showed hemangioma of the liver. The patient’s thrombocytopenia and coagulopathy improved immediately after surgery. In conclusion, LH is a very rare cause of obstructive jaundice. LH has the potential to compress the bile duct and cause obstructive jaundice. Liver hemangioma (LH) is the most common benign tumor arising in the liver, with an estimated prevalence of 0.4 to 7.3 % in the general population , 2. MostWe present a case with obstructive jaundice due to LH coexisting with Kasabach-Merritt syndrome, together with a discussion of the related medical literature.An 83-year-old woman with diabetes mellitus, hypertension, and spinal canal stenosis who was undergoing hemodialysis for chronic renal failure was admitted to another hospital, with itching as the chief complaint. She had notable hyperbilirubinemia , 4.6 mg/dl; direct bilirubin (D-Bil), 3.7 mg/dl; alkaline phosphatase (ALP), 1333 U/L; gamma glutamyl transpeptidase (γ-GTP), 350 U/L) in blood examination. Abdominal echography examination revealed intrahepatic bile duct dilatation and computed tomography (CT) showed a 13-cm mass in the right lobe of the liver. Endoscopic retrograde cholangiopancreatography (ERCP) revealed extrinsic compression of the bile duct at the hepatic hilar region.To improve the jaundice, endoscopic nasobiliary drainage was performed and yielded gradual improvements in the jaundice. At the same time, biliary cytology was performed. The architecture was disturbed, characterized by variation in internuclear distances and dyskaryosis. Therefore, the liver tumor was diagnosed as suspicious for malignancy. The patient was transferred to our hospital for hepatic tumor therapy.3 per cubic millimeter; fibrinogen, 122.5 mg/dl; fibrinogen and fibrin degradation products (FDP), 200 μg/ml; D-dimer, 88.8 μg/ml).Laboratory tests of biliary enzymes showed an improvement  but revealed blood coagulopathy  Fig. .A stricture of approximately 2 cm was observed in the common bile duct on ERCP and suspected to be a result of compression from the outside wall, causing partial expansion of a right lobe bile duct and high expansion of the left lobe bile duct Fig. .The preoperative diagnosis was suspected to be cavernous hemangioma, but differential diagnosis suggested hemangioendothelioma and hepatic echinococcosis. Because of the malignant potential of the tumor and the coexistence of Kasabach-Merritt syndrome, surgery was performed.Operative findings macroscopically showed a normal liver with a soft and multilocular 13-cm tumor localized in Segment IV–Segment V. A segmentectomy of the liver segments IV and V was performed with resection of the middle hepatic vein. We confirmed that a stricture of a bilateral hepatic duct was removed using intraoperative cholangiography. The intraoperative bleeding volume was 1930 ml. She received transfusion of 4 units of red blood cell transfusion, 5 units of fresh frozen plasma, and 10 units of platelet transfusion during the intraoperative period. She did not need transfusion before and after operation.Pathology revealed that the tumor was composed of cavernous vascular spaces of varying sizes lined by a single layer of flat endothelium. There was focal stromal fibrosis, calcification, and hyalinization Fig. . Our finPostoperative cholangiography did not show a biliary stricture. After surgical resection of the hemangioma, blood coagulopathy improved Fig.   tumor spread to and sheathing of the bile ducts in cholangiocarcinomas, (2) growing cast within the bile ducts in hepatocellular carcinomas, and (3) metastatic infiltration of the bile ducts, particularly in colon adenocarcinoma . Bile duUntil now, only two resected adult cases with obstructive jaundice by cavernous hemangioma have been reported in the English literature Table  7, 8]. , 8. 7, 8The incidence of Kasabach-Merritt syndrome in LH is around 1.7 % , but KasThere are some reports that estrogen contributes to hemangioma enlargement , 11. OurLHs that are large and symptomatic are usually treated surgically, either by enucleation or by resection. LHs require some form of treatment, such as surgical resection, liver transplantation –14, arteLHs may be enlarged, and those close to the hepatic hilum may potentially compress the bile duct and cause obstructive jaundice. Therefore, there is a need to carefully observe hemangiomas.Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.


Hereditary nonpolyposis colorectal cancer (HNPCC), or Lynch syndrome, accounts for 3 % of newly diagnosed cases of colorectal cancer. While a partial or subtotal colectomy is indicated for early stage disease, there is a paucity of data addressing locally advanced disease involving the foregut.We report two patients with hereditary nonpolyposis colorectal cancer presenting with locally advanced colon cancer surgically managed by pancreaticoduodenectomy with en bloc partial colectomy and a review of the literature.Locally advanced colorectal cancer in HNPCC is a rare clinical entity that requires special surgical consideration. Multidisciplinary treatment, including multi-visceral resection, offers the best long-term outcome. Colorectal cancer (CRC) associated with hereditary nonpolyposis colorectal cancer (HNPCC) accounts for 3 % of newly diagnosed cases. It is genetically driven by an autosomal dominant mutation of DNA mismatch repair (MMR) genes and has a phenotypic penetrance of 70–80 % . While aA healthy 56-year-old male presented with a 6-month history of early satiety, fatigue, and 80-pound weight loss. His mother and brother died from CRC at ages 36 and 44, respectively, and sister died from endometrial cancer. Physical exam revealed a firm, right upper quadrant mass.Laboratory data included a hematocrit level of 32.7 %  and carcinoembryonic antigen (CEA) level of 50.28 ng/dL . Computed tomography (CT) of the chest, abdomen, and pelvis revealed a 13.0 × 10.0 cm localized right upper quadrant mass arising from the ascending colon proximal to the hepatic flexure, indistinguishable from the duodenum, and invading the right lateral abdominal wall. Colonoscopy revealed a large, nearly obstructing mass proximal to the hepatic flexure, and esophagogastroduodenoscopy showed a mass in the duodenum. Biopsies from both endoscopies revealed mucinous lesions suspicious but not diagnostic of adenocarcinoma. Neither the positron emission tomography (PET) nor the pre-operative laparoscopy showed evidence of metastatic disease. Neoadjuvant chemotherapy was not pursued due to lack of histologic confirmation of malignancy. Prior to surgery, he was admitted with a lower extremity deep vein thrombosis (DVT), for which he received an inferior vena cava (IVC) filter and therapeutic anticoagulation with enoxaparin.The patient underwent a right hemicolectomy with en bloc classic pancreaticoduodenectomy and resection and reconstruction of the anterior abdominal wall. Reconstruction involved an end-to-side pancreaticojejunostomy, hepaticojejunostomy, gastrojejunostomy, and ileocolostomy. He had an unremarkable post-operative course. Histopathology of the surgical specimen revealed moderately differentiated mucinous adenocarcinoma arising from the hepatic flexure of the colon with focal invasion into a segment of the adjacent small bowel, peripancreatic soft tissues, and duodenum. Seventy-six lymph nodes and all surgical resection margins were negative for tumor. Immunohistochemistry of the surgical specimen showed absence of DNA mismatch repair enzymes hMLH1 and hPMS2 with retention of normal hMSH2 and hMSH6. Polymerase chain reaction (PCR) was consistent with high microsatellite instability (MSI-H) in the tumor. Genetic testing of peripheral blood leukocytes was consistent with mutated hMLH1. He underwent adjuvant chemotherapy with 12 cycles of oxaliplatin, fluorouracil, and folinic acid (FOLFOX) chemotherapy. He remains free of disease 4.5 years after surgery.A healthy 36-year-old male presented with fatigue, vague abdominal pain, diarrhea, and unintentional 40-pound weight loss. He was profoundly anemic with hemoglobin 4.2 g/dL , and CT demonstrated a 14-cm heterogeneous mass in the right hemi-abdomen Fig. , biopsieThe patient underwent a pylorus sparing pancreaticoduodenectomy with en bloc right hemicolectomy. Reconstruction involved an end-to-side pancreaticojejunostomy, end-to-side hepaticojejunostomy, end-to-side duodenojejunostomy, and anatomical side-to-side and functional end-to-end ileocolostomy. Examination of the specimen revealed mucinous adenocarcinoma arising from the right colon with direct extension to the duodenal mucosa Fig. , and hisMutY human homologue-associated polyposis (MAP), Peutz-Jeghers syndrome, and Juvenile polyposis syndrome [Colorectal cancer (CRC) is the third most common malignancy and third leading cause of cancer death in the USA, with around 136,830 estimated new cases in 2013. There are several well-defined inherited CRC syndromes that include HNPCC, familial adenomatous polyposis (FAP), MLH1, MSH2, MSH6, PMS2) secondary to mutations is a characteristic of HNPCC and accounts for over 97 % of mutations [EPCAM gene that inactivate MSH2 have also been identified in families of HNPCC patients [HNPCC is the most common of all identified hereditary CRC syndromes with a mean age of colon cancer diagnosis of 61 years. Loss of expression of mismatch repair genes  guidelines, all patients diagnosed with colorectal cancer under the age of 70, or over the age of 70 and who also meet the Bethesda Guidelines, should undergo tumor testing by immunohistochemistry or MSI testing. Greater than 90 % of HNPCC tumors are MSI-H and/or lack expression of at least one mismatch repair protein, so while positive screening tests increase the possibility of HNPCC in younger patients, a definitive diagnosis cannot be made without identifying a germ line mutation . Workup With regard to surgical management, a subtotal colectomy or segmental resection should be offered to patients. Given the measurable compromises to long-term bowel function and quality of life, without clear survival advantage, our group favors a segmental colectomy and regional lymphadenectomy as opposed to subtotal colectomy , 6. In tp = 0.007)); 5.6 % of the total lymph nodes were positive for metastasis [The current standard for adequate staging of right-sided colon cancers remains at least 12 lymph nodes, as espoused by the NCCN [tastasis  comparedtastasis . HoweverThe paradigm of chemotherapy for locally advanced CRC is changing. Recommendations for the use of pre-operative neoadjuvant chemotherapy to provide better local control for R0 resection have been increasing, citing the theoretical eradication of micrometastases and reduced perioperative tumor shedding, as well as downstaging with better progression-free and overall survival. More recently, there is evidence of added benefit in combining neoadjuvant and adjuvant treatment for CRC, as was performed in case 2 . HoweverOur two cases have several striking similarities that highlight important issues in the surgical management of HNPCC. Each patient presented with proximal, locally advanced, non-metastatic CRC. Careful pre-operative planning was required to perform multi-organ, en bloc resections with negative surgical margins. Despite aggressive lymphadenectomies, neither patient had lymph node metastases arising from their MSI-H tumor. After completing adjuvant chemotherapy, each patient remains disease free at 4.5 and 2.0 years from surgery, respectively. Intriguingly, both patients were also diagnosed with DVTs prior to surgery. However, there is little information available on whether HNPCC confers a risk of hypercoagulability.Locally advanced CRC involving the foregut is a rare presentation of HNPCC. A well-planned, multi-modality treatment approach is necessary for treatment. A review of the literature, summarized in Table We have consent to publish from the individual patients whose cases were presented in this report.


Gossypiboma refers, as a term, to a retained surgical sponge. It is considered as a rare surgical complication which can occur despite precautions. We report a case of a 36-year-old woman who was admitted to our surgical department with symptoms of abdominal pain associated with episodes of nausea and vomiting that lasted for 2 months. Six months ago she had undergone a cesarean section in a private clinic. Computed tomography revealed a high-density mass occupying a portion of the intestinal lumen, which was reported as a “calcified parasite.” The patient was subjected to laparotomy. The intraoperative findings included signs of obstructive ileus and ileosigmoid fistula and a large sponge was found at the resected portion of the small intestine. Although gossypiboma is a rare entity, it should be included in the differential diagnosis. Gossypiboma is a medical term that has been used to describe a serious postoperative complication. A retained gauze/sponge in the abdominal cavity after a surgical procedure has been mainly related with this entity. Terms that have also been used to describe this situation are textiloma, cottonoid, cottonballoma, or gauzeoma . The terA 36-year-old woman was admitted to our surgical department with abdominal pain associated with episodes of nausea and vomiting over the last couple of months. She denied any history of fever, while she reported a weight loss of around 6 kilos over the same period. She also mentioned that she had undergone a cesarean section in a private clinic 6 months ago. There were no significant findings from the clinical examination of the abdomen. Before having been reviewed by our team, the patient consulted two different general practitioners. The first one set the diagnosis of viral gastroenteritis, while the second advised her to have a computed tomography (CT) scan of her abdomen. The scan was carried out on outpatient basis and revealed a longitudinal, high-density mass inside the intestinal lumen Figures , which wSubsequently, the patient was transferred to our unit. As an inpatient, she had further imaging studies: an abdominal sonography that was normal and a plain abdominal radiograph which revealed a high-density mass in the left iliac fossa mimicking a retained radiopaque body .Blood tests were within normal values. The case was discussed with the patient and her family and a decision was made for the patient to have an explorative laparotomy. Intraoperative findings included dilatation of the small intestine, secondary to obstruction, material in the lumen of an ileal loop, and an ileosigmoid fistula. A small bowel resection en bloc with the affected part of the sigmoid  and a loThe patient had an uneventful recovery and was subsequently discharged on the eighth postoperative day. Two months later the patient had a successful restoration of the bowel continuity following a minimally invasive closure of the loop sigmoidostomy.The first case of a retained cotton-matrix item, a gossypiboma, was described by Wilson in 1884 . The abdIntra-abdominal gossypiboma is a rare postoperative complication with potentially severe gastrointestinal manifestations and in most cases the diagnosis may be delayed. The intraluminal location of the retained body has been extremely rarely reported, especially as a cause of intestinal obstruction. Prevention measures and precautions should be our primary goal to avoid such an event, which is associated with significant morbidity and medicolegal implications. The possibility of an existing gossypiboma should always be kept in the physician's mind, when a patient presents with abdominal pain following a recent abdominal surgery.


A 15 days old male newborn presented with four exophytic swellings arising from the skin of right lower limb; not restricting its mobility . The lesions were sequentially excised and pathological examination revealed infantile myofibromatosis in these growths. No evidence of malignancy was found . The swelling in the thigh recurred after 6 months and suspecting sarcomatous change, a biopsy was done which confirmed the sarcomatous change. Amputation and chemotherapy was offered, and the poor response to chemo-radiotherapy was explained. Parents refused further treatment and child succumbed to the disease after developing inguinal nodes and visceral metastasis.Infantile myofibromatosis is a rare tumor of infancy. It is mesenchymal in origin and involves superficial structures or may be visceral. The lesions have been found in nearly all kinds of tissues, including the orbit, bone, lip, oral cavity, central nervous system, gastrointestinal tract, lungs, myocardium, liver, and biliary tree. It may present at birth or appears during the first year of life. The exact etiology is unknown and most cases have been reported as sporadic. Both, autosomal dominant and recessive inheritance modes of transmission have been suggested. The prognosis is excellent in solitary or multicentric lesions without visceral involvement, with possibility of spontaneous regression of lesions confined to the skin, soft tissue and bone, and a very low recurrence rate after surgical excision. The prognosis is poor with visceral involvement [1-3]. In our patient the lesion recurred with a sarcomatous change which is an unusual behavior for a lesion known to be benign with a capacity for spontaneous regression.Source of Support: NilConflict of Interest: None declared


Solitary fibrous tumours (SFTs) are rare neoplasms that in the majority of cases are benign. We present the case of a 52-year-old male, with a 23-year history of a slow growing pleural mass, presenting to our department with worsening dyspnoea and localised chest discomfort. The purpose of this case report is to highlight the potential malignancy of a solitary fibrous tumour of the lung along with the key features in diagnosis and management. Solitary fibrous tumours (SFTs) are rare neoplasms with a reported incidence of 2.8 cases per 100,000 persons per year . 80% of β-catenin, and Bcl-2 on subsequent immunohistochemistry assessment. Areas of hypercellularity, nuclear atypia, 18 mitoses per 10 high-power fields, and haemorrhagic and necrotic transformation were also identified, which confirmed that malignant transformation of a benign tumour had occurred  scan confirmed the presence of a solid mass measuring 14 × 7.5 × 9 cm occupying the fissure between the right middle and lower lobe . The mas Figures . The patIt is widely recognised that SFTPs are rare neoplasms that are predominantly benign with no gender predilection , 8, 11.  moderate risk for metastasis. Accordingly, the follow-up for this patient included 6 monthly assessments for the first 2 years after surgical resection, which is comprised of full history, clinical examination, and CT scan. In the absence of recurrent disease at 2 years' follow-up, CT scans are performed yearly thereafter for 5 years. This case demonstrates the importance of maintaining awareness of the malignant potential of SFTPs. Furthermore this case poses an interesting question regarding the value in performing surgical excision early in the disease process in order to obtain early histological diagnosis and therefore allowing for early oncological referral and assessment.Prognosis of malignant SFTPs is often related to tumour size and remains poor with a 5-year survival rate reported to range between 45 and 68% , 15. DemWe present a case of a large malignant solitary fibrous tumour of the pleura that was successfully resected. Based on a risk model for metastasis developed by Demicco et al. , this pa


Lymphangioleiomyomatosis (LAM) is a rare idiopathic disorder that occurs in women of childbearing age, and consists of a diffuse proliferation of abnormal smooth muscle cells along the thoracic and abdominal lymphogenous route.We experienced a case of a 47-yo woman with recent history of systemic lupus erythematosus (SLE) diagnosed with endometrial cancer, initially suspected to have metastasized to pelvic and para-aortic lymph nodes based on preoperative diagnostic imaging. Subsequent pathological diagnosis revealed stage IB endometrial cancer without evidence of lymph node involvement. Instead, enlarged pelvic and para-aortic lymph nodes were found to be due to extrapulmonary LAM, from a primary lesion found inside the uterine myometrium. SLE improved after surgery.This is the first reported case of comorbid endometrial cancer, SLE, and aggressive LAM metastasizing to regional lymph nodes, and strengthens the clinical evidence for a common role of mTOR pathway hyperactivity and estrogen responsiveness in the pathophysiology of metastasizing lesions of the genital tract. TSC1 or TSC2 genes, as well as estrogen (E2) responsiveness [Lymphangioleiomyomatosis (LAM), a rare disorder of unknown incidence and prevalence, is characterized by abnormal proliferation of smooth muscle cells in the family of perivascular epithelioid cells (PEC) expressing human melanin black (HMB)-45. Estimated to affect up to 34 % of women with tuberous sclerosis complex (TSC) , the lunsiveness , often msiveness . Despitesiveness –15, imagIn this report, we describe the case of a woman with SLE, found to have uterine LAM with lymphadenopathy, incidentally found on lymphoadenectomy for treatment of advanced uterine endometrial cancer. To the best of our knowledge, this is the first report of LAM with SLE and endometrial cancer in a single patient. This case report strengthens the possibility that mTOR abnormalities may trigger a variety of autoimmune and developmental disorders in women.A 47-year-old Japanese woman, gravida 2, para 0, was referred to our institution for abnormal uterine bleeding. She had an extensive past medical history which included SLE, anti-phospholipid antibody syndrome (APS), idiopathic thrombocytopenic purpura (ITP), as well as previous dissecting right vertebral artery aneurysm and left cerebellar infarction. Her body mass index (BMI) was 18.1. Workup revealed severe anemia requiring an 8-unit red cell transfusion, and subsequent to gynecological examination, endometrial sampling cytology with conventional biopsy revealed Grade 1 endometrioid adenocarcinoma; serum tumor markers were obtained for evaluation and anticipated ongoing management of endometrial cancer; CA-125, CA19-9, and CEA levels were 176 U/mL, 27U/mL, and 1.8 ng/mL, respectively. Given her intravascular hypercoagulability due to ITP, we administered continuous heparin along with methylprednisolone (mPSL) pulse therapy to bring her hematology profile within an acceptable range of tolerability for surgery. The patient’s laboratory results and serologies are summarized in Table Magnetic resonance imaging (MRI) studies revealed cancer invasion into the uterine myometrium Fig. . On compOn the basis of preoperative findings, we initially diagnosed the patient as International Federation of Gynecology and Obstetrics (FIGO) stage IIIC2 uterine endometrial cancer, with 70 % myometrial invasion, lymphovascular invasion, and metastasis to the pelvic and para-aortic lymph nodes. The patient subsequently underwent adjuvant chemotherapy with paclitaxel (175 mg/m2) and carboplatin . However, no change in size of lymphadenopathy was observed after 3 cycles of chemotherapy. As her overall condition improved, with platelets stabilizing at >80,000/μL, and the patient wishing to avoid radiation therapy due to the considerable complications, we then performed dissection of retroperitoneal (pelvic and para-aortic) lymph nodes. On gross exam, we found well-circumscribed lymph node masses growing along the lymph vessels Fig. , which wMicroscopically, masses was composed of neoplastic smooth muscle leiomyoma-like tumor cells with clear to eosinophillic cytoplasm arranged in alveolar pattern without necrosis Fig. , and sliLAM is an idiopathic and intractable disease predominantly affecting females of childbearing age. Classified into pulmonary and extrapulmonary types, most patients are identified by onset of pulmonary complications, such as respiratory failure after pneumothorax . SporadiPTEN and/or PIK3CA [As noted above, the loss of function mutations identified in TSC1 and TSC2, have been broadly detected in pulmonary LAM cells, and likely explains the high correlation found between pulmonary LAM and TSC . These Tr PIK3CA ; knowledr PIK3CA . Taken tClinicians should also be aware of a previous report suggesting that extrapulmonary uterine LAM may precede both TSC and pulmonary LAM by several years , 6, a reAs mentioned above, the gold standard for diagnosis of LAM is made by histopathological findings. However, as in this case, decision to pursue and timing of surgical treatment is patient-specific. CT and MRI imaging remain the most accessible modalities for diagnosing lymphadenopathy in the absence of surgical exploration. Nonetheless, this case also highlights the appropriate controversy that exists regarding the accuracy of identifying lymph node metastasis from endometrial cancer solely by integrated imaging techniques, which more recently also include PET-CT imaging , 21. In APS, anti-phospholipid antibody syndrome; CT, computed tomography; E2, estrogen; ER, estrogen receptor; FIGO, federation of gynecology and obstetrics; HMB, human melanin black; ITP, idiopathic thrombocytopenic purpura; LAM, lymphangioleiomyomatosis; mPSL, methylprednisolone; MRI, magnetic resonance imaging; mTOR, mammalian target of rapamycin; PEC, perivascular epithelioid cells; PET-CT, positron emission tomography-computed tomography; PIK3CA, phosphatidylinositol-4,5-bisphosphate 3-kinase, catalytic subunit alpha; PTEN, phosphatase and tensin homolog; SLE, systemic lupus erythematosus; SMA, smooth muscle actin; SUV, standardized uptake value; TSC, tuberous sclerosis complex


Objective. A rare case of myeloid sarcoma (MS), previously referred to as granulocytic sarcoma or chloroma, is presented. Representing a unique form of acute myeloid leukemia (AML), MS may rarely occur in adults. Even rarer, MS may occur as the initial presentation of AML. Methods. We report a singular and illustrative case of an orbital pseudotumor mimicking mass in a 65-year-old male as the initial presentation of AML. Results. Neurosurgical intervention was required to establish the definitive diagnosis via right modified orbitofrontozygomatic craniotomy as well as to decompress the optic canal, superior orbital fissure, and orbit. Conclusion. Postoperatively, he reported decreased pain and improvement of his vision. Further examination revealed decreased proptosis and improved extraocular mobility. Pathological findings demonstrated MS. We review the literature and discuss the neurosurgical relevance of MS as the initial presentation of AML. Acute myeloid leukemia (AML) is a rare type of leukemia whose frequency increases with age. Myeloid sarcoma (MS) represents a unique form of AML in which a tumor-like proliferation of blast cells occurs outside of the bone marrow . PreviouThis 65-year-old male presented with a two-month history of right-sided headache, photophobia, double vision, and intermittent eye pain. His presenting visual acuity was 20/50 OD and 20/20 OS. Examination revealed mild proptosis and chemosis of the right eye with restrictive eye movement. CTA demonstrated no evidence of carotid-cavernous fistula. The presence of his cochlear implant made MRI not advisable. Head CT demonstrated right retrobulbar inflammatory stranding associated with mild proptosis and enlargement of the right inferior rectus and medial rectus muscles. Thyroid profile was normal. Peripheral blood work, bone scan, and CT of the chest and neck were also normal. To evaluate for possible infection or malignancy, ophthalmology service performed a needle biopsy. After the needle biopsy was negative for suspicious histology and negative on flow cytometry for lymphoma and leukemia, IOP was tentatively diagnosed and steroids were started. Despite corticosteroids, his clinical condition continued to deteriorate and he soon developed constant holocranial pain, proptosis, chemosis, disc edema, and decreased visual acuity to 20/400 OD without change in the left eye. Because of his rapid clinical decline, he underwent an MRI evaluation despite his cochlear implant. MRI demonstrated a marked increase in size of the mass in comparison to the original CT, as well as findings indicative of extension of the mass into the right cavernous sinus, involvement of all of the extraocular muscles, and marked compression of the globe with stretching of the optic nerve Figures  and 2. HMS is a rare extramedullary tumorous aggregation of malignant myeloid precursor cells. It usually occurs in the setting of acute myelogenous leukemia, myelodysplastic syndrome, or myeloproliferative disorder. Although first described by Burns in 1811 , King inEstablishing the diagnosis of MS presenting de novo continues to challenge clinicians. In a review of 26 cases of MS, 14 patients presented de novo and all such patients were initially misdiagnosed by the referring pathologist after examination of haematoxylin and eosin (H&E) stained sections. The initial diagnosis was non-Hodgkin's lymphoma in 12 . Even inImaging of these tumors is essential to understand the extent and/or spread of this disease; however, this lesion can share features on MRI and CT similar to carcinomas, metastasis, abscess, and lymphoma . On bothIdiopathic orbital pseudotumor (IOP) is a benign, noninfective inflammatory disorder of the orbit with no known cause. Accounting for the majority of cases of painful orbital mass in adults, it is the third most common orbital disease after thyroid orbitopathy and lymphoproliferative disorders . IOP accThe diagnosis of orbital MS is usually accomplished by ophthalmological needle biopsy. However, because the initial biopsy in our case was negative and the patient was rapidly losing vision, neurosurgical intervention was warranted. A bony decompression of the orbit, optic nerve, and superior orbital fissure was performed with opening the falciform ligament and periorbita. Although the external appearance of the extraocular muscles appeared normal, further dissection demonstrated significantly abnormal tissue interspersed with normal appearing muscle fibers. This may explain why the initial biopsy was negative.Neurosurgeons are commonly consulted for orbital masses that are suggestive of meningioma or optic nerve glioma. However, rarely they may be asked to evaluate patients for orbital decompression.Orbital decompression is usually undertaken in cases of thyroid orbitopathy or acute elevation of orbital pressure due to hematoma or infection , 24. RarKorinth et al.  describeIt remains debatable whether MS confers a poorer prognosis in patients with AML than AML alone. A number of larger case reports, however, have demonstrated survival rates similar to that of AML in both the adult and pediatric populations with 3- and 5-year survivals at 30% and 21%, respectively , 27–32. In summary, we present a unique case of MS where initial biopsy was negative. Because of the rapidly declining vision and severely progressive proptosis in our patient, orbital decompression along with open biopsy was carried out. Such neurosurgical intervention resulted in prompt clinical improvement and established an accurate diagnosis.


At present, no effective therapy exists for treating pure primary ovarian SCC. The present case study describes a patient that presented with progressive coughing, who was diagnosed with an International Federation of Gynecology and Obstetrics stage IV pure primary ovarian SCC with lung metastases. The patient received postoperative chemotherapy, however, the patient succumbed to the disease. The current study also presents a review of the literature.Pure primary ovarian squamous cell carcinoma (SCC) is a rare lesion that usually arises from the malignant transformation of an existing ovarian dermoid cyst. The  The incidence of a pure primary ovarian squamous cell carcinoma (SCC) is extremely rare when not associated with pre-existing ovarian lesions, such as dermoid cysts, Brenner tumors or endometriosis  . To dateA 46-year-old female  was referred to the Department of Pulmonary Medicine  with a history of progressive coughing that had been apparent for three months. Upon chest computed tomography (CT), performed at a local clinic on February 11, 2012, a diagnosis of lung cancer was suspected. The patient was immediately hospitalized and underwent bronchoscopy. A transbronchial lung biopsy revealed an SCC of unknown primary site.To locate the primary site of the metastatic lung cancer, an abdominal CT and positron emission tomography-CT scan was performed. The results of the scans revealed a left-sided pelvic mass, a left hydronephrosis and multiple regions of lymph node metastasis in the pelvic, abdominal, mediastinal and supraclavicular areas.The patient was referred to the Department of Obstetrics and Gynecology  for gynecological treatment. Abdominal exploration was performed on February 24, 2012, which revealed that the solid mass arose from the left adnexal area, was densely adhered to the sigmoid colon and external iliac vessel, and was encapsulating the left ureter. A total abdominal hysterectomy, bilateral salpingo-oophorectomy, adhesiolysis between the tumor and sigmoid colon, segmental resection of the sigmoid colon and reanastomosis were performed. Following segmental resection, the patient underwent an end-to-end anastomosis of the left ureter.2) and carboplatin (5 mg/ml/min) at three-week intervals. Despite the initiation of first-line adjuvant chemotherapy, clinical and radiographical evidence identified tumor progression and aggravation of the lung metastasis. Therefore, a second-line three-course chemotherapy regimen, consisting of topotecan (1 mg/m2) and cisplatin (50 mg/m2) at three-week intervals, and a third-line three-course regimen, consisting of etoposide and ifosfamide at three-week intervals, was administered. Despite this, treatment was unsuccessful and the patient succumbed to the disease on February 12, 2013, following cardiopulmonary arrest.Histopathological analysis confirmed a pure SCC arising from the left ovary. The pathological results were notable for the absence of any associated dermoid cyst or features suggestive of endometriosis, . The patPrimary ovarian SCC is rare, with the majority of cases preceded by dermoid cysts. Alternatively, cases of primary ovarian SCC may be associated with Brenner tumors and endometriosis . Ovarian


Acute obstructive hydrocephalus due to infratentorial extra-axial fluid collection (EAFC) is an extremely rare complication of foramen magnum decompression (FMD) and durotomy for Chiari malformation type I. Presence of infratentorial  EAFC invariably causes obstruction at the level of the fourth ventricle or aqueduct of Silvius, thereby indicating its definitive role in hydrocephalus. Pathogenesis of EAFC is said to be a local arachnoid tear as a result of durotomy, as this complication is not described in FMD without durotomy. Controversy exists in management. Usually EAFC is said to resolve with conservative management; so hydrocephalus doesn’t require treatment. However, in this case EAFC was progressive and ventriculo-peritoneal shunting (VPS) was needed for managing progressive and symptomatic hydrocephalus. Herein we discuss one such case and review the literature regarding the current knowledge of such a rare complication in the posterior fossa.Chiari malformation type I is not associated with other congenital conditions. Typically it presents in adulthood with varying features of increased intracranial pressure, headache, progressive cerebellar ataxia, progressive spastic quadriparesis, segmental amyotrophy and sensory loss, with or without pain. The latest management protocol is foramen magnum decompression (FMD) with or without durotomy. Several complications such as cerebrospinal fluid (CSF) fistula, cerebellar hematoma, and worsening neurological status have been described, but acute obstructive hydrocephalus due to infratentorial subdural hygroma is very rareA 30-year-old Tharu lady from Birgunj, Nepal was referred to the outpatient clinic at the College of Medical Science  with symptoms of chronic dull neck pain without radiation for the previous two years. There was no history of trauma. There was no significant past medical or surgical illnesses. The patient was taking over the counter pain medications (Diclofenac 75 mg PO SOS) for the pain. Due to further aggravation of her symptoms, she opted for medical review. On general examination, she had short neck  with low hair line. There was cape-like dissociative sensory loss starting from C2-T4 level. Muscle tone was of Ashworth grade I. She had exaggerated deep tendon reflexes in upper and lower limbs without appreciable clonus. Magnetic resonance imaging (MRI) of the cervical and thoracic spine was suggestive of Chiari malformation type I with syringohydromyelia extending from C2 to T8 level . There wst postoperative day. The patient’s hospital stay was uneventful and she was discharged after suture removal on the 7th postoperative day.FMD and durotomy were performed after receiving patient consent. Improvement in spasticity was seen immediately from the 1th day of her admission, she deteriorated with severe headache and persistent vomiting Early papilloedema was evident on fundoscopy and a repeat CT scan showed an increase in EAFC and triventricular hydrocephalus .Three weeks following surgery, the patient returned with complaints of headache and dizziness. She had no added neurological deficits and fundus examination was normal. A CT head scan showed minimal infratentorial EAFC and rounded third ventricle and prominent temporal horn. Initially she was managed conservatively with tablet acetazolamide 250 mg PO every 8 hours and strict monitoring for features of raised intracranial pressure such as persistent vomiting, hypertension and bradycardia. On the 6ephalus . Ventric1. Some surgeons prefer to perform a wide midline suboccipital craniectomy and not to open the dura with a view that duramater can slowly stretch so as to accommodate posterior fossa structures. Although some authors advocate performing a simple lax duraplasty alone, others provide a rationale for performing arachnoidolysis subsequent to duraplasty2.FMD with or without durotomy is the most widely used surgical technique for Chiari malformation type I1. Among these complications subdural hygroma or EAFC causing acute obstructive hydrocephalus is very rare6. The exact cause of subdural hygroma is still not clear. It is believed that during durotomy or arachnoidolysis, a small hole may be created on the arachnoid which will cause CSF egress in the subdural space. This tear acts like a one way valve causing more CSF to collect in the subdural space. Initially, CSF accumulates in and around the foramen magnum. With pressure created, CSF will slowly flow towards the cisterna magna, bilateral cerebellar convexity, below tentorium cerebelli and finally through the tentorial hiatus to the supratentorial subdural space3. Some authors have advocated the probable role of increased permeability of the intracranial vessels, which seems to be logical in cases of traumatic brain injury only7. Subdural hygroma under tension causes compression at the level of the fourth ventricle or aqueduct of Silvius, thus causing acute hydrocephalus5.With our improved insights on the pathogenesis of Chiari malformation, our surgical nuances and techniques on the management of the condition have improved significantly. Complication rates are reported to be as low as 2.3%As seen from the literature review, this complication is usually seen in patients above 10 years of age and has special preponderance to females. No clear hypothesis or mechanisms has been proposed for this, however a tear or breach in the dura seems to be the most likely. Age varied from 10 years to 55 years. Until now, including our study, nine cases have been reported, of which seven describe female patients.The site of maximal collection of extra-axial fluid collections differ between case reports. Such extra-axial collections may only be confined to infratentorial area, may extend to supratentorial area or only be prominent in supratentorial areas. Infratentorial extra-axial fluid collections usually cause compression at the level of fourth ventricle or aqueduct of Silvius and cause triventricular hydrocephalus. It may also result in direct compression of the brain stem and cranial nerves, causing acute symptoms. Supratentorial subdural effusion (EAFC) acts like decompensated chronic subdural hematoma thus causing relevant symptoms such as hemiparesis or signs of raised intracranial pressure.EAFC may resolve spontaneously or aggravate causing acute hydrocephalus. Acute hydrocephalus may be transient as EAFC might slowly resolve. Patients usually are symptomatic requiring hospital admission from 3–21 days after FMD.10. Widely opening the arachnoid routinely during surgery might prevent this complication as pinhole dural tear may even occur during closure3.This is a rare complication so the ideal management protocol is not known. Effort should be made to prevent it by avoiding durotomy and arachnoidolysis whenever possible and opening the arachnoid widely whenever deemed necessary. Some authors suggest re-surgery for closing the tiny hole in the arachnoid or opening the arachnoid widely and suturing it to the dura10. However, if there is evidence of significant mass effect and raised intracranial pressure, such patients should be managed surgically either with placement of appropriate burr holes or by performing VP shunting for CSF diversion. VPS should be considered for progressive neurological deterioration and increasing ventricular size. A subduroperitoneal shunt should be employed for recurrent supratentorial EAFC. There is inadvertent risk of slit ventricular syndrome due to over-drainage of CSF following low pressure VP shunting. Repeated EVD (more than twice) increases the risk of infection as well as the odds of upward central herniation in case of CSF over-drainage. So, VPS should be considered in such cases.After complication has already occurred, and is not life threatening, minimal measures such as burr hole and external ventricular drain (EVD) should be undertaken. In most cases reported till date, permanent VPS is not usually required as EAFC usually resolvesEAFC complicating acute obstructive hydrocephalus is a very rare complication following FMD and durotomy for Chiari malformation type I; but should be suspected in post operative cases presenting with headache and vomiting. We can avoid this by only undertaking osseous decompression or in cases where there is need for durotomy, widely opening the arachnoid and suturing it to the duramater. Whenever applicable, attempts should be made to manage these cases without VPS.Both written and verbal informed consent for publication of images and clinical data related to this case was sought and obtained from the patient. The case report is informative about obstructive hydrocephalus development secondary to extradural fluid collection in the posterior fossa following decompression surgery for Chiari malformation type I. The authors mentioned about presence of scarce amount of cases in the literature. It would be better to delineate the prior cases in a table to give more information about the nature of the disease.I have read this submission. I believe that I have an appropriate level of expertise to confirm that it is of an acceptable scientific standard. The authors attempted to demonstrate a rare manifestation of operative intervention on adult type Chiari malformation. This kind of case report would undoubtedly help surgeons to understand the current status of managing this, which is still controversial. They have highlighted the importance of post operative headache and vomiting to identify a rare yet a treatable cause like EAFC. Please mention how you defined the low hair-line.et al. 20051)It would be better for authors to mention why they chose ventriculoperitoneal shunting and not external ventricular drain to deal with the complication, as we know they may resolve spontaneously. (MarshmanAs the literature review seems less, I would rather say "Herein we report a case of Chiari malformation type I with postoperative infratentorial subdural hygroma"Comments:I have read this submission. I believe that I have an appropriate level of expertise to confirm that it is of an acceptable scientific standard.


Teratomas are unusual tumours that derived from totipotent cells with their origin from more than one or usually all three germ cells. Here authors are presenting a case of primary retroperitoneal tumour that is a rare clinical entity. A 19-year-old male presented with right lumbar pain and was found to have complex cyst with large calcification in right adrenal gland on imaging. Intraoperatively, he was found to have a solid mass with areas of soft consistency, which was excised en bloc. On gross examination, the cyst contained pieces of bone, few teeth, and hairs entangled in mucinous material. On histological evaluation, it was confirmed to be mature teratoma arising from the right adrenal gland. He made uneventful recovery and was kept well on annual follow-up. Teratomas are rare tumours that shares radiological features with many other benign conditions. Although definitive diagnosis is possible on histopathological evaluation only, pertinent radiological finding mentioned in this report can help others to suspect this rare yet important clinical entity.This case highlights the importance of correct and meticulous interpretation of the radiological investigation.A 19-year-old male with no prior health issues presented with complaint of vague pain in right flank for the past 9 months. He described the pain as intermittent, nonradiating with no specific aggravating or relieving factors. Past history was unremarkable. On examination, he appears healthy with normal blood pressure and pulse rate. Abdominal and rest of the systemic examinations did not reveal anything significant.His baseline workup including complete blood count, renal profile, and liver function tests was within normal limits. His haemoglobin was 128 g/L  and serum creatinine was 0.8 mg/dL . Based on the findings of imaging, investigations were ordered to exclude functional adrenal tumour although the patient was completely asymptomatic. 24-hour urinary cortisol was 89 mic gm/24 hours  and serum cortisol (dexamethasone suppression) was 0.9 mic gram/dL .Contrast enhanced computed topography  revealedThe most important differential diagnosis based on the imaging could be teratoma, angiomyolipoma, or myelolipoma that shares few radiological features and causes diagnostic difficulty. Confirmation of diagnosis in this case is only possible by histopathological evaluation of resected tissue; however, in this particular case the presence of large calcification  on CT scan was an important finding that is not seen with the above-mentioned clinical entities.The patient underwent en bloc excision of the mass through flank approach with resection of 11th rib. Mass of about 8 × 6 × 4 cm was found just above the kidney having bosselations over its surface with different areas of soft and hard consistency. It was densely adhered to the surrounding structures, so careful dissection was done to define the planes. Tumour was removed and sent for histopathological evaluation.The patient made excellent postoperative recovery and was discharged home. On gross evaluation, multiple structures including teeth, small piece of bone, a piece of cartilage, and few hairs were found within the cyst entangled in mucinous material . HistoloOn follow-up at the 12th postoperative day, the patient was doing well and the wound was completely healed. Histopathology was discussed with the patient and he was advised about follow-up after 6 months. He was last seen after a year of surgery and was completely asymptomatic. His repeated ultrasound was unremarkable as well.Teratoma is a rare neoplasm with incidence of 0.9/100,000 population . TeratomSince teratoma originates from pluripotent cells of two or more than two germ cell layers, the contents can be different. In mature teratoma, range of adult tissue types can be found including skin, muscle, nerve, fat, and tooth structures .The majority of patients are asymptomatic while others may present with nonspecific complaints like backache or flank or generalized abdominal pain although cystic teratomas may rupture and cause sudden onset of abdominal pain, ascites, and peritonitis , 6. HighThis case is one of the very few cases reported in the literature so far, latest by Sasi and coworkers  who repoThese tumours are likely to be benign and carry excellent prognosis after treatment with overall 5-year survival nearly 100% [Teratoma is a rare but important differential diagnosis of retroperitoneal masses. High index of suspicion and radiological investigation are the mainstay of diagnosis. The presence of large calcification is an important finding that may help distinguishing teratoma from other masses. Histopathological evaluation is mandatory to exclude any component of malignancy.