Corpus luteum cyst rupture with consequent hemoperitoneum is a common disorder in women in their reproductive age. This condition should be promptly recognized and treated because a delayed diagnosis may significantly reduce women's fertility and intra-abdominal bleeding may be life-threatening. Many imaging modalities play a key role in the diagnosis of acute pelvic pain from gynecological causes. Ultrasound study (USS) is usually the first imaging technique for initial evaluation. USS is used to confirm or to exclude the presence of intraperitoneal fluid but it has some limitations in the identification of the bleeding source. Contrast-enhanced computed tomography (CT) is the imaging modality which could be used in the acute setting in order to recognize gynecological emergencies and to establish a correct management. Magnetic resonance imaging (MRI) nowadays is the most useful technique for studying the pelvis but its low availability and the long acquisition time of the images limit its usefulness in characterization of acute gynecological complications. We report a case of a young patient with hemoperitoneum from hemorrhagic corpus luteum correctly identified by transabdominal USS and contrast-enhanced CT. Acute pelvic pain in women of childbearing age is a common and frequent cause for admission to emergency room (ER), necessitating emergent medical evaluation especially when it is due to hemoperitoneum .In this scenario, the wide range of differential diagnoses that must be considered when assessing abdominal pain represents an issue for the clinical approach.Sometimes it can be difficult to distinguish gynecological from gastrointestinal and urinary tract emergencies because of overlapping symptoms and signs. Various imaging modalities in association with clinical findings play an important role in the characterization of the cause of pain , 3.Early diagnosis is necessary to preserve the reproductive systems and the life of the patient in severe cases. Hemoperitoneum may occur in the context of various gynecological emergencies; in some cases it could be a complication of a ruptured hemorrhagic corpus luteum –5.Suspecting gynecological disease in young women the use of X-ray radiation and computed tomography (CT) is not recommended due to the risks associated with irradiating the pelvis. Ultrasound study (USS) and magnetic resonance imaging (MRI) are the preferred imaging investigations in pelvic diseases; however, in gynaecological emergencies, when a simple and rapid assessment of the patient's condition is required and once pregnancy is excluded, the CT is the investigation of choice .We describe a case of hemoperitoneum from a ruptured hemorrhagic corpus luteum in an adolescent woman in which the use of emergent CT in the ER was necessary in order to obtain the correct diagnosis.A 16-year-old adolescent female presented to the ER with sudden onset abdominal pain, lasting for six hours. Her last menstrual period was 18 days back. The patient did not have previous diseases or surgery.On physical examination she presented tachycardia (pulse rate 115 beats per minute), tachypnea (respiratory rate 22 breaths per minute), and hyperpyrexia (37.8°C) with abdominal distention and severe rigidity on the lower quadrants on palpation. Blood pressure (BP) was 85/60 mmHg and she was anxious but alert and oriented.β-human chorionic gonadotropin (βhCG) level was below 4 UI/L. Gynecological examination excluded abnormalities of the external genital structures in a virgin patient.Laboratory investigations showed mild leukocytosis, slight elevation of C-reactive protein (CRP) (192 mg/L), and discrete anemia (hemoglobin 10 g/dL). Urine output was 26 cc/h and serum; Transabdominal USS revealed a moderate amount of fluid in the abdomen and in the pouch of Douglas and a complex right adnexal mass with signs of peripheral vascularization .An abdominal CT study was indicated and performed using a 64-row scanner , before and after the injection of iodinate contrast medium .The unenhanced CT scan  showed aAfter fluid resuscitation, the patient became haemodynamically stable and the subsequent emergent laparoscopy confirmed a bleeding corpus luteum as the cause of the hemoperitoneum.Spontaneous hemoperitoneum may occur in various gynecological emergencies. The most common gynecological causes of spontaneous hemoperitoneum in women of childbearing age are ectopic pregnancy and ruptured corpus luteal cyst. More uncommon causes are uterine rupture, endometriosis, and ruptured hydropyosalpinx .Corpus luteum is a functional cyst developing in the luteal phase of the ovarian cycle which regresses spontaneously in corpus albicans when pregnancy does not occur . Being aβhCG-levels is necessary to differentiate ruptured corpus luteal cyst from ruptured ectopic pregnancy, which may have a similar presentation [The diagnosis of ruptured corpus luteal cyst is based on a high historical suspicion , clinical features, and laboratory tests. The latter often show anemia, raised CRP, and mild leukocytosis. These signs and symptoms are similar to gastrointestinal tract diseases. Patients may present a wide range of clinical signs, from no signs to severe peritoneal irritation which can be confused with, for example, acute appendicitis. The evaluation of serum entation , 8. A peentation . Variousentation , 4, 9. Bentation , 4. Doppentation .Although MRI is the most adequate technique for the pelvic evaluation, thanks to its high-soft-tissue contrast capability, it is not usually used in the acute setting due to its considerably long acquisition time, limited availability, and high costs , 4.For these reasons, MRI has limited availability in emergency department and it is not currently included in the American College of Radiology Appropriateness Criteria .Therefore, in the ER gynecological diseases are frequently detected with CT examination , 4, 5. TOn CT examination, corpus luteum usually appears like a well-circumscribed unilocular adnexal lesion, rarely bilocular. The cyst walls appear slightly thickened (<3 mm) and show a characteristic inhomogeneous contrast enhancement after administration of contrast medium due to increased vascularity. The cystic content is mixed with a high attenuation component 45–100 HU) and in some cases it presents a “fluid-fluid hematocrit” level [5–100 HU Contrast-enhanced CT may be helpful in excluding other intra-abdominal diseases  that can cause hemoperitoneum in the young female patient , 9.For the management of hemoperitoneum a standard algorithm is not reported in literature. Historically the treatment of corpus luteum hemorrhage was exclusively surgical, while nowadays it can be managed with a conservative approach. In either case the treatment targets at preserving ovarian function as well as at eliminating the source of bleeding .In particular, the conservative approach is the first-choice treatment when the patient is hemodynamically stable (systolic BP > 90 mmHg) with hemoglobin values that keep being constant over 4–6 hours of monitoring . In caseIn summary, USS is a helpful imaging modality in diagnosing some gynecological emergencies in the ER but may not detect the entire range of the disease, whereas contrast-enhanced CT of the abdomen is recommended in the acute ER setting to diagnose gynecological emergencies and to initiate correct management in a timely fashion.


Adrenal hemorrhage (AH) is a rare but life-threatening condition. Small focal hemorrhage may present subclinically, but massive hemorrhage may lead to rapid cardiovascular collapse and ultimately death if not diagnosed appropriately and treated quickly. Most cases reported in the literature have been treated conservatively. In an event of increasing hemorrhage during conservative management, it may be tricky to intervene surgically because of the hematoma around the gland. Here we describe a case where we managed a large spontaneous AH by a combination of angioembolization and laparoscopic adrenalectomy. Etiology includes both traumatic and nontraumatic events. Clinical manifestation depends on the degree and rate of hemorrhage. Small focal hemorrhage may present subclinically, but massive hemorrhage may lead to rapid cardiovascular collapse and ultimately death if not diagnosed appropriately and treated quickly. Most cases reported in the literature have been treated conservatively. In an event of increasing hemorrhage during conservative management, it may be tricky to intervene surgically because of the hematoma around the gland. Here we describe a case where we managed a large spontaneous AH by a combination of angioembolization and laparoscopic adrenalectomy. To the best of our knowledge, it has not been reported in the literature yet.AA 26-year-old, nonhypertensive young lad presented to us in emergency with left-sided acute abdominal pain of 1 day duration. Pain was sudden in onset, severe, continuous, and nonradiating. On initial examination, he was hemodynamically stable with blood pressure of 140/70 mm Hg and heart rate of 110 beats per minute. On abdominal examination, he had a tender vague lump in the left upper quadrant of abdomen. Bowel sounds were normal. First, ultrasound (USG) abdomen and, later, contrast-enhanced CT scan abdomen were performed, which revealed hemorrhagic collection of 9 × 8.2 × 8.2 cm near the left suprarenal region with 4 × 4.5 × 4.2 cm adrenal mass. To evaluate the adrenal mass, MRI was performed, which showed the left suprarenal mass with large hemorrhagic component, suggestive of spontaneous hemorrhage in an adrenal mass . Based oAH may result from acute illness/stress, anticoagulation, coagulopathy, underlying tumor-like angiomyolipoma, trauma, or idiopathic disease. Most of the reported cases of AH in the literature are seen in pregnancy. These are usually intra-AHs. In young adults, idiopathic spontaneous AH is extremely rare and has never been reported. The presenting symptoms usually are hemorrhagic shock, flank pain, and fever.1 Tumors known to cause spontaneous bleed are pheochromocytoma, myelolipoma, metastasis, carcinoma, and rarely adenoma. There are ∼50 reported cases of spontaneous rupture of adrenal pheochromocytoma causing haemorrhage2 in the literature, but there is no reported case of adrenal adenoma causing massive bleeding.AH has been reported in 0.3% to 1.8% of undetected cases in autopsy studies.Once adrenal pathology is suspected in a patient with retroperitoneal hemorrhage, he should be managed in an intensive care unit with close hemodynamic monitoring. Serial hematocrit and USG monitoring of size of hematoma has to be performed. Pheochromocytoma should always be kept in mind while dealing with such a case. MRI is the imaging modality of choice for diagnosis of nontraumatic AH.In patients with active bleeding, angiographic embolization is a valuable tool to achieve hemostasis. If complete hemostasis is achieved, patient is asymptomatic, and hemodynamically stable, then immediate surgical exploration should be avoided. But if patient deteriorates, surgical exploration may have to be performed. All preoperative principles performed in pheochromocytoma should be followed.This is the first case report of such a massive spontaneous AH in adrenal adenoma in a young adult, which was effectively managed laparoscopically.A high index of suspicion is required to make a timely diagnosis of AH. Although acute surgical removal of an adrenal tumor within a large hematoma should be avoided, as proper oncologic resection may not be possible, it sometimes has to be performed in a symptomatic hemodynamically deteriorating patient.


Segmental intestinal dilatation (SID) is a rare developmental anomaly of the midgut, characterized by sharply demarcated dilatation of a gastrointestinal segment with clinical findings of intestinal obstruction. Although morphologic criteria for SID are clearly delineated, etiological origin of dilated intestine is unknown. Histology of the resected segment is usually reported to have normal presence of ganglion cells in the myenteric and submucosal plexuses. Intestinal muscle is hypertrophied, and heterotopic gastric mucosa may also be encountered. A 3-day-old male infant presenting with clinical findings of intestinal obstruction was diagnosed to have SID and had supernumerary intestinal muscle coat (SIMC) in the dilated segment, without any evidence of neurological abnormality. Histopathological findings of the resected ileal segment are reported to discuss the role of architectural malformation of muscularis propria as a cause of SID. Segmental intestinal dilatation (SID) is a rare gastrointestinal anomaly introduced by Rossi and Giocomoni . AccordiAlthough diagnostic criteria of SID are well established, the etiology of the disease is unclear. Histology of the involved bowel usually shows normal neurological innervation with a hypertrophied or thin muscle layer . HeterotThe presence of an additional smooth muscle layer in the intestine is a rare congenital anomaly of enteric smooth muscle and considered as a cause of pseudo-obstruction or constipation . SupernuA 3-day-old male infant with intestinal obstruction was diagnosed as SID, and the patient underwent resection of involved bowel and anastomosis. Microscopical evaluation of the resected segment revealed SIMC with normal neurological innervation. We aim to discuss the role of architectural malformation of intestinal muscle coat in the etiology of SID.A 20-day-old male infant, weighing 3,200 g, was delivered vaginally at 37 weeks of gestation. He was admitted to the emergency department with abdominal distention and bilious vomiting. He had inability to pass stool in the first 3 days of his life. Then, he irregularly passed the stools with rectal stimulation. His prenatal history was unremarkable. He has no family history of pseudo-intestinal obstruction.3/μL, and platelet 310 × 103/μL. Biochemical analyses including liver and renal function tests were within normal limits. The physical examination of all systems was normal except abdominal distention. His abdominal X-ray showed a distended large bowel loop with air-fluid levels  immunohistochemical staining (Figure SID is a rare congenital anomaly characterized by sharply demarcated dilatation of gastrointestinal tract without any evidence of mechanical obstruction . The mosin utero vascular accidents, primary aplasia of an intestinal segment, and congenital damage to the nerves in myenteric plexus are considered as possible causes of SID [Although diagnostic criteria are well established, the etiology of the disease is unclear. It has been suggested that transient embryonic structures such as vitelline vessels and omphalomesenteric bands cause extrinsic compression of both ends of bowel ,10. In as of SID . The rols of SID . We haveSID can also be detected as an intra-abdominal cystic mass in prenatal ultrasonography . On abdoThe treatment of SID is resection of dilated segment and end-to-end anastomosis . Most paIn most of the cases, histology of the resected segment is usually normal . HoweverArchitectural malformations of muscularis propria consist of supernumerary and/or haphazard orientation of muscle fibers with an additional neural plexus. Smith et al. have described supernumerary muscle coat in five patients and classified them as diffuse or segmental . SIMC isSID is a congenital anomaly of gastrointestinal tract with clinical findings of intestinal obstruction. We show for the first time that at an extremely rare malformation of muscularis propria, SIMC can be seen in the dilated segment of SID, and we suggest that this additional muscle layer can play a role in the etiology of SID.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.


Tenosynovial giant cell tumour, diffuse type, also known under a variety of other terms including diffuse pigmented villonodular synovitis, tends to be locally aggressive and not infrequently can show multiple recurrences. The differential diagnosis with the extremely rare and somewhat controversial malignant variant of tenosynovial giant cell tumour, diffuse type, is challenging due to overlapping radiologic features of these two entities. Malignant tenosynovial giant cell tumour is defined by the presence of overtly malignant sarcomatous areas. We describe a very unusual case of a 63-year-old man affected by tenosynovial giant cell tumour, diffuse type of the knee that, despite absence of morphologic evidence of sarcomatous transformation, developed inguinal lymph node metastases following multiple surgical procedures. Tenosynovial giant cell tumour, diffuse type (D-TGCT), also alternatively labeled as diffuse type giant cell-tumour, or diffuse pigmented villonodular synovitis, usually occurs in tendon sheaths and in the synovia of the large joint. The 2013 WHO Classification of Tumors of Soft Tissue and Bone classifies D-TGCT as a locally aggressive neoplasm characterized by high rate of local recurrences [CSF1 gene, represent frequent alterations that set the rationale for effective treatment with imatinib [The pathogenetic mechanisms underlying D-TGCT have been source of sharp debate. Some authors have considered it as an inflammatory disorder , 3, howeimatinib .The development of metastatic spread, to the lungs, of histologically benign D-TGCT  represents an exceptionally rare reported event. This occurrence should be differentiated from true malignant variants of this entity , 5, 7, 9In March 2012, a caucasian 63-year-old man was seen in our institution for a painful swelling of the right knee joint present since 2 years. Pain was exacerbated by digital pressure. This patient resulted affected by D-TGCT that had been surgically treated with multiple synovectomies in 1987, 1990 and 1996, respectively. Histologic review of the original specimens of the primary tumor and of all recurrences was performed. All the specimens exhibited overlapping morphology by showing a combination of hyperplastic synovia, a mononuclear cell proliferation organized in a nodular pattern of growth, scattered hemosiderin-laden macrophages, xanthoma cells and multinucleated giant cells. These findings met the diagnostic criteria for D-TGCT and we therefore confirmed the primary diagnosis. Because of pain and persistent joint effusion, the patient underwent another synovectomy at our institution, and the histologic evaluation of the specimen confirmed the diagnosis of D-TGCT without atypical features. In contrast with previous samples a greater amount of fibrosis was seen.Two years later the patient presented again with painful swelling of the right knee associated with pain localized in the right hip. Loss of weight and functional limitation of the right leg was observed. Magnetic nuclear resonance imaging demonstrated extra-articular soft tissue multilobular masses around the posterior part of the knee joint, with erosion of the lateral femoral condyle. Extension into the patello-femoral joint and the proximal tibia with a low signal intensity on T2-weighted image was present. In T1-weighted images, dark signal nodules, compatible with hemosiderin deposition, were readily identified (Figure Macroscopically, the evaluation of the knee disarticulation revealed a multinodular lesion of 30 cm × 25 cm × 21 cm, that was extensively infiltrating both the leg and the thigh with massive destruction of the knee joint. Alternation of white, yellowish and brownish areas with hematic-cystic lesions was seen (Figure No areas of malignant transformation were identified in the specimen. Four months later a CT scan of the pelvis revealed a mass located in the soft tissue of the contralateral buttock, associated with inguinal lymph nodes swelling without evidence of lung metastases. In January 2015, the patient started systemic treatment with imatinib.Metastatic spread to the lungs and/or to locoregional lymph nodes represents a very unusual event in D-TCGT . Such ocThe mechanism responsible for distant metastases in D-TGCT has not been established yet , 4, 7. IRegional lymph node metastases of other benign soft tissue neoplasms have also been reported . UterineWe herein report a case of conventional D-GCT of the knee that, despite lacking any morphologic features of malignancy developed bilateral loco-regional lymph-node metastasis.Even if this represents an extremely rare occurrence, long term follow-up is strongly advised, particularly for those patients experiencing multiple local recurrences.


Primary adrenal lymphoproliferative disorder (LPD) is an extremely rare disease that is widely known to be associated with methotrexate (MTX) use in patients with rheumatoid arthritis (RA).A 70-year-old man was incidentally found to have a tumor at the dorsal part of the liver in a medical check-up. He had a history of RA treated with MTX. Abdominal ultrasonography demonstrated a low echoic mass (30 mm in diameter) at the dorsal part of the liver, located close to the inferior vena cava. Preoperative differential diagnoses included intrahepatic cholangiocarcinoma, adrenal tumor, and hepatic malignant lymphoma, but no definitive diagnosis was reached. On exploratory laparotomy, the tumor seemed to be derived from the right adrenal gland and adhered tightly to segment 7 of the liver. Therefore, right adrenectomy with partial resection of segment 7 of the liver was performed. Pathological findings revealed diffuse inflammatory cell infiltration with a population of small atypical lymphoid cells, with positive immunohistochemical evidence for Epstein–Barr virus (EBV). Final diagnosis was primary adrenal iatrogenic EBV-positive LPD, classified as “other iatrogenic immunodeficiency-associated LPDs: Hodgkin-like lesions.”In this report, we described the possibility of the spontaneous healing of MTX-associated LPD (MTX-LPD) before treatment and the importance of doubting MTX-LPD and doing immunostaining to necrotic tissue. To our knowledge, this is the first reported case of MTX-related EBV-positive LPD, Hodgkin-like lesion, of the unilateral adrenal gland in patient with RA. The first choice of treatment strategy for MTX-associated LPD (MTX-LPD) is typically discontinuation or dose reduction of MTX. However, the pathogenesis of MTX-LPD still remains unclear and the treatment of MTX-LPD has been controversial. This case suggests the one important possibility for considering the mechanism or treatment of MTX-LPD. This report was approved by our hospital's Institutional Review Board results.Primary extranodal lymphomas account for approximately 1/3 of all lymphomas and can affect almost any organ, most commonly the skin and stomach, followed by the thyroid, bone marrow, lung, and so forth.A 70-year-old man who was incidentally found to have a liver tumor on enhanced computed tomography (CT) during a comprehensive medical examination was referred to our hospital in April 2014. He was diagnosed with RA in 1994 and MTX had been administered since 2006 at a dose of 12 mg/week.His family history was unremarkable. Physical examination revealed no specific abdominal findings. The results of routine blood examination and liver function tests were normal, and serum tumor markers  were within normal limits. Serum cortisol was 14.1 μg/dL  and corticotropin was 91.7 pg/mL . Serum epinephrine was 0.02 ng/mL , norepinephrine was 0.048 ng/mL , and dopamine was 0.02 ng/mL . Abdominal ultrasonography demonstrated a low echoic mass (30 mm in diameter) at the dorsal part of the liver adjacent to the inferior vena cava (IVC). The tumor was hypovascular on enhanced CT scan , Epstein–Barr nuclear antigen (EBNA), and latent membrane protein 1 (LMP1). Staining was negative for CD2, CD3, CD4, CD5, CD7, CD8, CD10, CD15, CD21, CD30, CD56, Bcl-1, Bcl-2, Bcl-6, and MUM1 ] IgG antibody, anti-EBNA antibody, and negative for anti-EBV (VCA) IgM antibody.Given these findings, the final pathologic diagnosis was primary adrenal MTX-associated EBV-positive B-cell LPD, classified as “other iatrogenic immunodeficiency-associated LPDs; Hodgkin-like lesions” by the World Health Organization (WHO).11 MTX therapy for RA was discontinued and the patient was followed regularly. He has remained relapse-free for almost 1 year.Postoperatively, we performed whole-body gallium scintigraphy as an additional investigation and no hot spots were detected. LPD was stage IE according to the Ann Arbor classification.4 It represents only 3% of extranodal lymphomas.3 Secondary involvement of the adrenal gland with non-Hodgkin's lymphoma occurs more frequently, in nearly 25% of patients during the course of disease.9 Secondary adrenal involvement generally affects elderly individuals, especially those with a history of cancer, human immunodeficiency virus infection, or autoimmune disorder, and in about 50% of cases it manifests as adrenal insufficiency due to bilateral adrenal involvement.12Primary malignant lymphoma of the adrenal gland is a rare and highly aggressive disease that has been reported only rarely in the literature.4 defined primary adrenal lymphoma as a histologically proven lymphoma that involves 1 or both adrenal glands and has at presentation no prior history of lymphoma elsewhere and unequivocally dominant adrenal lesions in cases with lymph nodes or other organ involvement. In our case, preoperative enhanced CT revealed no other lesions except that in the right adrenal gland and postoperative whole-body gallium scintigraphy showed no hot spots. The tumor involved hepatic segment 7, but the adrenal lesion was unequivocally dominant. Accordingly, we diagnosed unilateral primary adrenal LPD.Rashidi and Fisher5 first reported the association between lymphoma and MTX therapy in patients with RA. Patients with RA have various immune abnormalities, including T-cell dysfunction and B-cell activation associated with autoantigen stimulation.6 Thought the pathogenesis of RA-associated lymphoma remains unclear, it has been speculated that high inflammatory activity associated with RA, immunosuppressive agents including MTX used for treatment, or EBV infection/reactivation.In 1991, Ellman et al7 MTX itself might have strong correlation with LPD in some cases. In addition, it is well known that immunosuppression predisposes patients to EBV-related lymphomas. Suzuki et al8 reported that the developmental mechanism of MTX-LPD may include reactivation and/or persistence of EBV due to immunosuppression by MTX. Indeed, EBV infection was detected in 12% to 44% of lymphomas emerged in patients with RA.7Actual risk elevation for lymphoma among patients treated with MTX is unclear. However, given the reported fact that there were several cases with LPD regressed after discontinuation of MTX therapy,In current case, blood test revealed positive result for serum anti-EBV (VCA) IgG antibody, anti-EBNA antibody and negative for anti-EBV (VCA) IgM antibody. In addition, the atypical lymphocytes was confirmed in pathologic specimen by strongly positive results in immunohistochemical staining for EBER and EBNA. There results suggest that chronic viremia due to reactivation of EBV may have played a strong role in development of lymphoma in the current case. Given the reported evidence, MTX usage might have relation with reactivation of EBV in this case.6 The first choice of treatment strategy for MTX-LPD is typically discontinuation or dose reduction of MTX. However, if tumor size does not decrease after discontinuation, chemotherapy or radiotherapy should be performed as second-line therapy. Kawakami et al reported that chemotherapy as the first-line treatment without discontinuation of MTX did not always achieve good outcomes, as infections could occur as a complication.13 They recommended starting chemotherapy after stopping MTX whenever possible. Recent descriptions of relapsed Hodgkin's lymphoma that were EBV-positive at initial diagnosis but EBV-negative at recurrence raise the possibility that DNA is lost during tumor progression in some individuals.15 Salloum et al reported that 62.5% of patients observed after MTX withdrawal without additional antitumor therapy showed at least partial regression in response, and most of these patients were EBV-positive.16 Further, survival ratios are higher in patients with Hodgkin's lymphoma than in those with Hodgkin-like lesions.17Iatrogenic immunodeficiency-related LPD usually regresses upon withdrawal of the drug; it is thus considered attributable to a toxic/metabolic effect rather than being EBV-related. In a previous study, tumor size decreased in 20% to 30% of cases of MTX-LPD following the discontinuation of MTX.Histopathologically, atypical lymphocytes were confirmed by strongly positive immunohistochemical staining for CD20, EBER, EBNA, and LMP1. This confirmed the diagnosis of primary adrenal MTX-associated EBV-positive B-cell LPD. In our case, the adrenal tumor showed characteristic changes, with predominant geographic necrotic tissue and diffuse inflammatory cell infiltration in the center of the tumor. Typically, MTX-LPD is not accompanied by tumor necrosis. Since necrotic tissue tends to contain few tumor cells, it can be extremely difficult to accurately diagnose a tumor, and hematoxylin–eosin staining alone fails to provide a definitive diagnosis. In the present case, although the primary adrenal tumor contained a mostly necrotic center, immunostaining of tissue assisted in the diagnosis. In the necrotic tissue, the cellular outlines of lymphocytes indicated lymphoma. Further, immunostaining revealed that the necrotic tissue was strongly positive for CD20.Generally, such necrosis is attributed to the efficacy of chemotherapy or another treatment. It was generally regarded as MTX withdrawal will restore antitumor immunity and leads the tumor cell necrosis. However, our patient had not received treatment for the adrenal tumor and so we have no identifiable etiology for the tumor necrosis. In other words, the tumor cell might be healing spontaneously in spite of the presence or absence of MTX withdrawal in the past reports. There was no report in the past that the MTX-LPD became necrotic without treatment. And also, it is extremely difficult to diagnose the necrotic tissue like this case by the normal pathological examination. However, if we will be able to doubt MTX-LPD, we can add to do immunostaining and provide definitive diagnosis.Here, we have reported the case of primary unilateral adrenal iatrogenic MTX-associated EBV-positive LPD with Hodgkin-like lesions.


Primary bone lymphoma involving the pelvic bone is a rare entity. It does not have distinct clinical features or radiological features that are diagnostic. Biopsy is the gold standard investigation. We present a case of primary pelvic lymphoma with initial histopathological features of chronic osteomyelitis. Upon further clinical follow-up, repeat biopsy of the lesion revealed features of B-cell Non-Hodgkin's lymphoma, thus emphasizing the need for a high index of suspicion and close clinical follow-up. This case is presented for the diagnostic dilemma and the unique feature of lymphomatous lesion mimicking chronic osteomyelitis in its early stages. Primary bone lymphoma represents approximately 3% of all malignant bone tumors and 5% of all Non-Hodgkin lymphomas . Most osA 65-year-old gentleman presented to the out-patient department with three-month history of continuous, dull aching pain in the left iliac region, radiating to the left thigh. He did not have constitutional symptoms like fever, weight loss, and loss of appetite. Initial examination revealed a 3 × 4 cm tender swelling with indistinct margins localized over the lateral aspect of the left iliac crest. There was increase in temperature and erythema. General examination was unremarkable except for pallor. There was no localized or generalized lymphadenopathy. Blood investigations revealed normal haemogram, ESR of 20 mm/hour and C-reactive protein of 34 mg/dl. Plain radiographs revealed an osteolytic lesion with indistinct margins, cortical breach, moth eaten appearance, and narrow transitional zone . Bone scAt his follow-up visit, the patient appeared pale and had significant loss of weight. The swelling had increased to size of 10 × 10 cm, with extent up to the left inguinal region. The left lower limb was edematous. There was no localized lymphadenopathy. Hip movements were painfully restricted. Plain radiographs of pelvis showed a permeative lesion with significant periosteal reaction over the left iliac crest . The cheFirst established as a clinical entity in 1939, primary lymphoma of the bone is defined as “lymphoma presenting in an osseous site with no evidence of disease elsewhere for at least six months after diagnosis” . It presThe paucity of systemic “B” symptoms and relative rarity of lymphoma in the ilium made us consider another differential diagnosis. Clinically, lymphoma of the bone can mimic the presentation of other small round cell tumors such as myeloma and rarely present with innocuous symptoms analogous to that of osteomyelitis , 8.The diagnosis of lymphoma presenting as a primary bone lesion can cause a dilemma as there are no pathognomonic radiological findings. In plain radiographs, lymphomas have variable and nonspecific appearance ranging from being near normal to a diffusely permeative lesion with soft tissue extension. The lytic-destructive pattern is the most common 70%) and is usually associated with an aggressive periosteal reaction. Cortical breakthrough, an ominous sign, is present in less than 25% of early lymphomas. About one-third of primary lymphomas present with a blastic-sclerotic pattern (13.6%) or with subtle near normal findings (5.8%) 0% and is. A patteThe radiological picture in our patient at first presentation demonstrated a lytic, radiolucent, pelvic lesion with cortical erosion which represents the possibility of infection. However, radiographs at follow-up demonstrated a permeative lesion with ill-defined margins and soft tissue extension, strongly suggestive of a malignancy. The radiological appearance of a primary bone lymphoma continues to evolve, emphasizing the need for a regular and meticulous follow-up .Closed or open biopsy and histopathological examination are the gold standard diagnostic investigation . Needle The lag time for primary lymphoma of bone to manifest its clinical features poses a diagnostic difficulty, thus emphasizing the need for a high degree of suspicion when clinical and histopathological findings do not tally. Delay in diagnosis can have serious consequences and the need to obtain an urgent diagnosis has been stressed .To our knowledge, this is the first report of an innominate bone lymphoma presenting as chronic osteomyelitis. This has been reported due to the unusual location of this tumor, the ambiguity in clinical symptoms, and the diagnostic dilemma thus caused.


Pituicytoma is a rare, low-grade glial neoplasm that arises in the neurohypophysis or infundibulum and usually presents as pituitary gland enlargement. They are often misdiagnosed as pituitary adenomas. Causes have varied for high serum adrenocorticotropic hormone level reported in a few patients with pituicytoma.We report a rare case of pituicytoma accompanied by corticotroph hyperplasia—a challenging diagnosis guided by clinical presentations, radiological signs, and biopsy.We present a case of pituicytoma with corticotroph hyperplasia in a 46-year-old woman with typical Cushing syndrome. Magnetic resonance imaging revealed a lesion in the sellar area with equal T1 and T2 signals and marked homogeneous enhancement. We present detailed analysis of the patient's disease course and review pertinent literature. 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 of this journal. Because of this, there is no need to conduct special ethic review and the ethical approval is not necessary.The patient underwent a surgical exploration and tumor resection through a trans-sphenoidal approach. Pathologic results revealed pituicytoma and corticotroph hyperplasia. As adrenocorticotropic hormone and cortisol levels did not decrease to normal, the patient received radiotherapy and recovered uneventfully. No recurrence was found over 8 years of follow-up.Pituicytoma is a rare type of sellar tumor. Pituicytomas in patients with Cushing syndrome are rarer still. To our knowledge, this is the first report of Cushing syndrome caused by corticotroph hyperplasia in a pituicytoma patient. Up to now, there have been only less than 60 cases reported to match this depiction. Pituicytomas can lead to local tumor-mass effects such as optic chiasm compressive symptoms, pituitary hormone disorders secondary to pituitary gland compression or pituitary stalk compression, and cavernous sinus syndrome caused by possible lateral invasions. Honegger once reported a pituicytoma patient who presented with Cushing syndrome caused by an adrenocorticotropic hormone (ACTH) microadenoma.3 The golden criteria of diagnosis for pituicytomas are histopathological and immunohistochemistry (IHC) examinations, since clinical manifestations and radiological features both are nonspecific.4According to current World Health Organization (WHO) classifications for central nervous system (CNS) tumors, pituicytoma is now reserved for low-grade glial neoplasms (grade I) derived from neurohypophyseal pituicytes, a modified glial cell found in the posterior pituitary gland and infundibulum, which is distinct from pilocytic astrocytomas.In this study, we present a case of a 46-year-old woman suffering from Cushing syndrome. Magnetic resonance imaging (MRI) showed a sellar lesion with equal T1 and T2 signals and marked homogeneous enhancement. We originally supposed the tumor to be an ACTH pituitary adenoma and performed a trans-sphenoidal surgery, but histopathological examination corrected the diagnosis to pituicytoma coexisting with corticotroph cell hyperplasia. To our knowledge, this is the first reported case of pituicytoma with corticotroph hyperplasia presenting in this manner. The relevant literature is reviewed and discussed.2 (obese) and her blood pressure was 147/105 mm Hg in despite of undergoing a daily enalapril maleate therapy. Her muscle strength of lower extremities was grade 4/5. No gross visual field defects were detected. Sensation function and tendon reflexes were almost normal. Pathological signs and ataxia were absent.The patient is a 46-year-old Chinese female who developed symptoms and signs of Cushing syndrome, including supraclavicular and dorsocervical fat pads, a moon-shaped face with increased frequency of acne, thin extremities, severe fatigue and fairly profound muscle weakness, a tendency to bruise easily, memory dysfunction, anxiety, and insomnia for around 2 years before her visit at our hospital. Over the half-year period before diagnosis, she developed rapidly progressive opsomenorrhea and noted enlargement of her abdomen with a weight gain from 65 to 80 kg. She had also complained of different degrees of headache, which was mainly focused on the forehead, and could be alleviated by rest or analgesics. She denied polyuria and nocturnal enuresis, and had no special family history or personal history. Upon physical examination, the patient's BMI was 38.5 kg/mTable An MRI revealed an abnormal enlarged parenchymal lesion in the sellar region , measuring about 15 mm × 10 mm × 7 mm  and tend to occur during the middle decades of life .6 Pituicytomas are hypothesized to arise from the posterior pituitary lobe or in the lower portion of the stalk, and extend gradually upwards; they are believed to originate from the neoplastic transformation of pituicytes, which are glial cells that occupy perivascular zones of the neurohypophysis, which regulates hypothalamic hormone release.6 Some authors propose that pituicytomas originate from folliculostellate cells of the adenohypophysis for completeness sake, despite a lack of convincing evidence. Five different types of pituicytes have been identified histologically: major cells, dark cells, oncocytes, ependymal cells, and granular cells, with each cell type believed to give rise to a distinct tumor type. The first 2 are the most common.7 In the 2007 WHO classifications for central nervous system tumors, pituicytomas are classified as rare, solid, grade I glial tumors.8Pituicytoma was first described by Liss in 1958. It is well-recognized, but exceptionally rare, and commonly appears as a solid, well-circumscribed, noninfiltrative lesion that occupies the sella turcica or the suprasellar region.9 Those located in the sella region usually show symptoms similar to pituitary adenomas. The common endocrinological abnormalities include hyperprolactinemia, hypopituitarism, male hypogonadism, and low testosterone. Lateral expansion of the mass into the cavernous sinus could cause compression against the oculomotor nerve, trochlear nerve, or abducent nerve, leading to symptoms like oculomotor paralysis. Suprasellar lesions often present with visual disturbances due to compression of the optic nerve or chiasm. Although the neurohypophysis is associated with the storage and release of antidiuretic hormone, and is believed to be the origin of pituicytomas, only 5% of all reported cases presented with central diabetes insipidus (DI), for reasons that are unclear.10 In our case, the patient also denied any symptoms of DI; her primary clinical presentation was Cushing syndrome.Generally, the location of the lesions determines the clinical features of pituicytomas.14 Notably, the reasons for high ACTH levels vary among these cases, including a pure mass effect that might disturb the hormone secretion, and coexistence of a microadenoma neglected originally. Whether pituicytomas are associated with other endocrine pathologies is unclear, due to the rarity of described cases. In the present case, pituitary corticotroph hyperplasia confirmed by histopathological and immunohistochemical tests ultimately gave the explanation. To our knowledge, this is the first reported case of a pituicytoma coexisting with corticotroph hyperplasia. Corticotroph hyperplasia as a cause of Cushing disease, although uncommon, has been described in the adult population and has been shown to precede adenoma development, or even to coexist with adenomas.15 However, this seems to be exceedingly rare in patients with a pituicytoma. In addition, Cushing disease is almost invariably due to corticotroph adenomas rather than pituicytomas.16 Therefore, the present unique case has certain educational significance; whether pituicytes can secrete ACTH may warrant some investigation. In fact (as mentioned above), their histogenesis is still debated, with recent reports suggesting that pituicytomas arise from the folliculo-stellate cells of the adenohypophysis, which are nonendocrine spindled cells that express S-100 and Bcl-2. Of note, to minimize extra invasive manipulation, it is really a pity in this case that IPSS was not done before surgery. The IPSS test could not only help to determine whether the elevated ACTH comes from peripheral or central, but also could help to determine whether the source of ACTH tend to be left or right. This is helpful for exploratory during surgery and to remove the lesion completely. In certain cases, especially those where the ACTH source is not clear before surgery, IPSS is highly recommended.High serum ACTH levels were noted in only 5 previous cases, with our case as the sixth one Table .3,11–14 17 Sometimes, they can exhibit sellar enlargement and bony remodeling. Most reported MRI findings describe a solid, homogenous mass, iso-intense on T1-weighted images and hyperintense on T2-weighted images, with homogenous contrast enhancement, closely mimicking pituitary adenoma or meningioma.9 Heterogeneous enhancement, calcification, and cystic changes were rarely seen.17 In our case, the lesion presented with equal T1 and T2 signals, which accords with previous reports.Differentiating pituicytomas from common sellar region lesions such as pituitary adenoma or meningioma is difficult if based only on their MRI appearance, partly because their imaging findings are not specific enough. In fact, most reported pituicytomas were misdiagnosed before surgery.4 Grossly, pituicytomas are discrete, solid, well-circumscribed tumors with a firm rubbery texture; their size may reach several centimeters. Histopathologically, pituicytomas are characterized by a compact architecture of elongated bipolar spindle cells arranged in interlacing fascicles or a storiform pattern.18 Kwon and Suh19 once presented an unusual pituicytoma case with a vague perivascular pseudorosette histological pattern, large multinucleate pleomorphic cells, and scattered Herring bodies, which implies origination from ependymal pituicytes. Generally, pituicytomas are noninfiltrative and immunoreactive for vimentin, S-100 protein, and GFAP , with occasional cytoplasmic EMA positivity. Mitoses are extremely rare; MIB-1 labeling indices are low at 0.5% to 3%.20As the neuroimaging features of pituicytomas are nonspecific, its diagnosis relies on pathological findings.6 As pituicytoma is a slow-growing and rare low-grade glial neoplasm, no recurrence has been reported after gross total resection, although follow-up periods have varied. However, the highly vascular nature of the tumor and its potential for infiltration can make total resection difficult; recurrence is therefore a possibility.21 Under this circumstance, radiotherapy to control residual tumor and increase survival is recommended, although management is uncertain due to the rarity of this tumor.13 In our case, the ACTH and cortisol levels did not decrease to normal by 2 weeks after resection, although postoperative MRI showed a satisfying tumor removal. As preoperative examinations indicated that ectopic ACTH syndrome was unlikely, these results implied an insidious residual tumor, possibly with corticotroph hyperplasia. The patient underwent radiotherapy and her ACTH and cortisol levels eventually decreased to normal levels. Now, after 8 years of outpatient follow-up care, the patient's postoperative condition has remained uneventful. To our knowledge, this is the longest reported follow-up period for pituicytoma.The best treatment of pituicytoma is gross total resection via a trans-sphenoidal approach, both of which release the mass effect and provide histopathological specimens, which are especially valuable in cases with atypical clinical and radiological presentations and unconfirmed diagnoses.We report here a rare case of pituicytoma in the neurohypophysis, coexisting with ACTH-secreting pituitary hyperplasia in adenohypophysis—a combination which has not been previously reported. Pituicytomas are not easily differentiated from other common sellar region lesions; accurate diagnosis relies on pathological examination. Corticotroph hyperplasia is an uncommon cause for Cushing syndrome, but should be considered for patients with both Cushing syndrome and pituicytoma. Postoperative radiotherapy is a possible treatment for such cases where residual tumors are suspected.


This is a case report of a newborn baby with gastric duplication cyst presented with non-bilious vomiting and upper abdominal distension. The diagnosis was suspected clinically and established by ultrasonography and computed tomography. The cyst was completely excised with uneventful recovery. Gastrointestinal duplications can occur anywhere in the alimentary tract from the mouth to the anus. Gastric duplication is very rare and accounts for only 4% of gastrointestinal duplications. They are usually cystic and are located on the greater curvature; and have no communication with the gastric lumen. They typically present with partial obstruction. An upper abdominal mass is often palpable on examination. Haematemesis and melaena may rarely occur [1-3]. We are reporting a case of gastric duplication cyst presenting with gastric outlet obstruction.A 10-day-old male neonate born by normal vaginal delivery at term, presented with recurrent non bilious vomiting. Antenatal ultrasonography revealed a cystic abdominal mass. On examination, there was mild upper abdominal distension with no palpable mass. Laboratory investigations were normal. Plain roentgenogram of abdomen  showed soft tissue mass displacing the stomach upwards. Abdominal ultrasound revealed a cystic mass measuring 42 X 36 mm arising from the greater curvature of the stomach with well defined thick wall displacing the stomach upwards. Computed tomography scan of the abdomen  confirms the ultrasound findings. Gastric duplication cyst was the working clinical diagnosis.Excision of the cyst was initially attempted laparoscopically, but it had to be converted to open laparotomy owing to the difficulty to separate the cyst from the stomach. A large cystic mass with well-defined muscle coat was adherent to the greater curvature of the stomach and not communicating with the gastric lumen or adjacent structures. The cyst was totally excised without entering the gastric lumen. . Gastric seromuscular defect was approximated by interrupted absorbable suture. Histopathologically, the cyst had smooth muscles in the wall and inner lining of gastric mucosa. The child had uneventful post-operative recovery and discharged on the 5th post operative day. Gastrointestinal (GI) duplication cysts are rare malformations that can occur at any part of the alimentary tract. Although recognized since 1733 by Calder [4], it was not until 1937 that Ladd [5] introduced the term duplication of the intestinal tract to describe a group of congenital anomalies characterized by having a well developed coat of smooth muscle, their epithelial lining is composed of some type of gastrointestinal mucosa and they are attached to the alimentary tract.Gastric duplications are typically spherical cysts which usually develop on the greater curvature of the stomach. The majority are not communicating with the gastric lumen . The mucosal lining is usually of gastric with ectopic pancreatic tissues in about 40% of cases [6]. Our case contained only gastric mucosa.They are generally identified within the first year of life [7]. The clinical manifestation is non-bilious vomiting and abdominal mass . Rare clinical presentations are haemat-emesis, chronic anemia, gastrointestinal bleeding; recurrent pancreatitis and perforation with peritonitis and fistulous communication to the spleen or lower lobe of the lung [8-10]. If left untreated, gastric duplications are associated with malignant degeneration [11].Treatment is complete excision of the cyst. Marsupialization of the cyst wall and stripping of the mucosal lining is the second option if it is not possible to find a plane between the cyst and the stomach. There are few cases in the literature successfully treated by laparoscopic resection [12]. It was not feasible in our case to complete the procedure laparoscopically as the dissection plane was difficult to identify.Source of Support: NilConflict of Interest: None


The occurrence of nasopharyngeal tuberculosis is rare even in areas where tuberculosis is endemic. Here, we report a case of rare primary nasopharyngeal tuberculosis, promptly evaluated by nasolaryngoscopy.Mycobacterium tuberculosis. Histopathological examination from the thickening nasopharyngeal mucosa revealed granulomatous formation with caseous necrosis. Ziehl-Nielsen staining directly could detect acid-fast bacilli. Chest X-ray and CT scan ruled out the pulmonary tuberculosis. Base on these findings, we diagnosed it as primary nasopharyngeal tuberculosis. After six months anti-tuberculous therapy, the patient’s symptoms had completely disappeared. Nasolaryngoscopic examination and CT image after 6 months post therapy revealed a normal nasopharynx with complete resolution of the lesion.A 78-year-old woman presented with postnasal drip and a cough of 1-month duration. Endoscopic examination of the nasopharynx revealed irregular mucosal thickening of the right lateral and posterior wall of the naso (epi)-pharynx, which was covered with yellow discharge presenting as postnasal drip. Computed tomography (CT) demonstrated enhanced soft tissue area in the right lateral and posterior wall of the nasopharynx. Bacteriological examination from a nasopharyngeal swab revealed that staining for acid-fast bacilli was positive and the quenching probe PCR test was positive for We recommend endoscopic examination for patients suffering from chronic postnasal drips to avoid inappropriate diagnosis. Mild symptoms related to the nasopharynx can be easily overlooked, and may present themselves as serious medical conditions at a later stage. When symptoms become chronic, nasolaryngoscopy is used to rule out serious medical conditions such as malignancies. Nasolaryngoscopy is easy to learn, safe, painless, convenient to perform, and readily accepted by patients. Furthermore, it also allows direct visualization and evaluation of both the lesion and the nasopharynx and larynx anatomy, under local anesthesia. It thus allows for early diagnosis and treatment in a primary care center by reducing unnecessary referral , 2.The occurrence of nasopharyngeal tuberculosis is rare even in areas where tuberculosis is endemic. Our literature search for primary nasopharyngeal tuberculosis revealed that very few cases have been published thus far –5. Here,Mycobacterium tuberculosis. Moreover, QuantiFERON-TB Gold was positive. Histopathological examination from the thickening nasopharyngeal mucosa revealed granulomatous formation with caseous necrosis  were not observed. Endoscopic examination of the nasopharynx revealed irregular mucosal thickening of the right lateral and posterior wall of the naso (epi)-pharynx, which was covered with yellow discharge presenting as postnasal drip Fig. . Computesis Fig. . Ziehl-Nsis Fig. . Indigensis Fig. . Base onion Fig.  and h.FiNasopharyngeal tuberculosis is commonly associated with cervical lymphadenopathy , 4. In tWritten 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 of this journal.


On follow-up visit after one month patient was doing very well and had no complications.Gastric perforation is a very rare complication of necrotizing pancreatitis. We present an interesting case of gastric perforation after necrosectomy for necrotizing pancreatitis without generalized peritonitis. Abdominal drain was seen inside the stomach on endoscopy and there were no clinical features of generalized peritonitis even after 10 days of surgery. Patient was re-explored. The drain was removed and stomach was primarily repaired. The patient recovered uneventfully and was discharged home on 6 While the debate and controversies on treatment options for the optimal management of severe necrotizing pancreatitis (SNP) are still ongoing, open surgical debridement remains the “gold standard” for the treatment of infected pancreatitis and pancreatic necrosis. Necrosectomy and subsequent closed continuous lavage of the lesser sac is, among the open necrosectomy techniques, the one with the lowest morbidity.7Along many other complications of necrosectomy, a very rare on is gastric perforation. This case was unique because the gastric perforation after necrosectomy was without symptoms of generalized peritonitis. The perforation was well plugged around the drain in stomach and hence generalized peritonitis was avoided in the presence of gastric perforation.A 56 years old lady from Afghanistan presented in the surgical outpatient department of our hospital with a history of laprotomy and necrosectomy for necrotizing pancreatitis in Afghanistan 10 days back. Her chief complaints were epigastric pain since surgery and fluid draining through the abdominal drain soon after drinking any liquid. This drain was on the left side of the upper abdomen. The color and consistency of fluid in drain was very similar to the fluid she would drink. On clinical examination she had no signs of septicemia and generalized peritonitis. She was afebrile with a regular pulse of 84 per minute and blood pressure of 115/75mmHg. The laprotomy wound dressing was dry and clean. There were two abdominal drains on either side of abdomen. She was admitted in ward and was investigated. Blood tests revealed WBC=1210 and shift to the left, rest of the blood count, serum electrolytes, liver function tests and renal function tests were in normal limit. Her hepatitis serology for HBS and HCV was negative. Her gastrograffin study of upper gastrointestinal tract showed external drainage of the contrast from the stomach through a drainage tube in her left upper abdominal area with suspicion of malposition of drainage tube with its tip in the stomach. CT scan showed abdominal collections in lesser sac and peri pancreatic region approximately about 60ml and 65ml respectively. Drainage tube was noticed entering through the left hemi abdominal wall entering the greater curvature and ending up in the gastric lumen. Upper gastrointestinal endoscopy revealed multiple small gastric ulcers and tip of the abdominal drain inside the stomach along the greater curvature. Patient was re-explored. Per operative findings were:Left sided abdominal drain was seen, piercing the greater curvature of the stomach. About 2cm, from the tip of the drain, was found inside the stomach.Omental mass in the area of the pancreatic bed, adherent to loops of small bowel.Rest of the bowel was normal and no other abnormality was seen.th post operative day. Her post operative recovery was uneventful. She was discharged home on 6th post operative day. Her first follow up was planned two weeks after the surgery. She had no active complaints and was taking normal diet, tolerating well and had normal bowel habits.The abdominal drain from the stomach was removed. The edges of the gastric perforation were refreshed. Primary repair of the gastric perforation with omentopexy was done. Oral fluids were commenced on 4Acute pancreatitis is still associated with significant morbidity and mortality. Current management guidelines are sometimes equivocal, particularly in relation to the surgical treatment of severe disease. Despite a relative shortage of prospective randomized trials, there has been a significant change in the surgical management of acute pancreatitis over the past 20 years. This change has been away from early aggressive surgical intervention towards more conservative management, except when infected necrosis is confirmed. A formalized approach with appropriate use of the various non-surgical and surgical options is feasible in the management of severe acute apancreatitis.8In a study of 88 patients with necrotizing pancreatitis Connor S et al found that almost all patients undergoing necrosectomy developed significant early or late complications or both. Multi-organ failure and post operative hemorrhage were independent predictors of mortality.Another important factor is that placement of abdominal drain plays a vital role in reducing the chances of peritonitis in our patient and recognition of perforation as well. This not only shows the importance of traditional placement of abdominal drain after necrosectomy but contradict a study performed by Kevin C Conlon et al, which showed that surgically placed intra peritoneal drains reduces the rate of either death or complications associated with pancreatic resections.14


Esophageal submucosal tumors are occasionally detected incidentally during a gastrointestinal survey. In the present study, a case of esophageal leiomyoma is reported, which was incidentally detected by chest radiography during an annual survey of mass-screening for lung cancer. The patient underwent a laparoscopic lower esophagectomy, a proximal gastrectomy and a gastric tube reconstruction. Macroscopic examination revealed a 50×40×28-mm mass, while microscopic examination identified submucosal smooth muscle tissue without mitotic activity or necrosis. The tumor was diagnosed as an esophageal leiomyoma. The patient was asymptomatic during the three-month follow-up period. However, when a mass lesion adjacent to the gastrointestinal tract is detected during chest radiography, the possibility of a rare disease should be considered. Therefore, further investigation with upper gastrointestinal radiography and gastroendoscopy should be performed. Leiomyomas are the most common esophageal mesenchymal neoplasms, even though their occurrence is rare. Patients with esophageal leiomyomas are usually asymptomatic. The most common symptoms, when present, include epigastric discomfort, dysphagia, regurgitation, gastrointestinal bleed, diarrhea and weight loss. Esophageal leiomyomas are occasionally detected incidentally during the examination of other gastrointestinal diseases, of which the majority are identified during endoscopic examination or upper gastrointestinal radiography –4. ConsiA 55-year-old female was referred to the Mito Medical Center  following the detection of a nodule on a chest radiograph performed during an annual survey of mass-screening . The masEsophageal SMTs are occasionally detected incidentally during the examination of other gastrointestinal diseases –4. In thCommon symptoms of an esophageal leiomyoma include dysphagia, epigastric pain and retrosternal pain or burning. However, the leiomyoma grows slowly and half of the patients do not present any symptoms unless the tumor is intramural ,7. BleedThe diameter of esophageal leiomyomas has been reported to be <5 cm in 49% of cases, 5–9 cm in 33.7% of cases and >10 cm in 17.3% of cases . SeveralCommunity-based lung cancer screening using chest radiography is well-established in Japan –15, alloTo the best of our knowledge, the current study presents the first case of an esophageal leiomyoma detected on a chest radiograph during a mass-screening program. In conclusion, a mass lesion adjacent to the gastrointestinal tract that is detected on a chest radiograph may potentially indicate a rare disease. Therefore, further investigation with upper gastrointestinal radiography and gastroendoscopy should be performed.


Sclerosing angiomatoid nodular transformation (SANT) is a relatively new entity in the spleen, which usually presents in the form of single nodule. Only 5 multifocal SANT cases have been reported in English literature. The present case is the first report of a 38-years-old male patient with SANT in the form of multiple nodules, who has been cured via laparoscope. In comparison to solitary SANT, multifocal SANT occurs more likely in males than females and association with malignant neoplasm has not been described yet. Multifocal SANT as well as solitary SANT show some relationships with IgG4-related sclerosing disease.The online version of this article (doi:10.1186/s13000-015-0312-2) contains supplementary material, which is available to authorized users. Sclerosing angiomatoid nodular transformation (SANT) is one kind of very rare benign lesion in the spleen, which may present as ., Solitary type or Multifocal type SANT. To date, a total of 127 cases of SANT have been reported in the English literature, of which only 5were multifocal . In thisA 38-years-old man suffered from dull pain in left upper quadrant of abdomen in December 2011. The pain became sharp when the body turns right lateral flexion, without other symptoms such as nausea, vomiting, abdominal distention, diarrhea. The patient denied referring to the physician for any consultant and treatment further.On August 2012, abdominal CT scan was performed for the patient, which showed an enlarged spleen, with multifocal lobulated or irregular nodules in the spleen parenchyma. These nodules were isodense or hypodense, well-defined boundary, which were 3.5 cm, 5.5 cm and 7.6 cm in diameter respectively Fig. . The artThe patient change given laparoscopic splenectomy, and a new upper midline abdominal incision was made for removing the large spleen. The spleen was sectioned into pieces and placed in a specimen bag and were sent to Department of Pathology. The patient did well during the post-surgical period and discharged 14 days after the surgery without any complications.Macroscopically, the spleen was 18 cm × 11 cm × 8 cm in bulk volume, with three well-circumscribed round tumors on the cut surface which measured 6.5 cm, 5.0 cm and 3.5 cm in diameter, respectively, which was consistent with the impression of multifocal lesions on CT findings. Every mass was non-encapsulated and grey brown, focal calcification with a grey white stellate scar in the center of the lesion Fig. . MicroscSANT of the spleen, as a new entity, was first described in detail in a series of 25 patients by Martel et al.  in 2004.Of all 128 cases, 122 were solitary SANT, of which 53 were males and 69 were females; whereas, only 6 cases(include this case) were multifocal SANT, of which 4 were males and 2 were females, as shown in Table In this work, review of available documents in English literature, 30 of these 127 cases (23.6 %) coexisted with other diseases, such as idiopathic myelofibrosis, bile duct cancer, pancreatic cancer, acute pyelonephritis, and so on, most of which were cases of malignancy. Those results were matched up with Martel’s report  further Of 6 cases of multifocal SANT, 4 cases coexisted with other diseases, such as idiopathic myelofibrosis, multifocal calcifying fibrous pseudotumors, pancreatic mucinous cystadenoma and hepatic cyst, as shown in Table Macroscopically, the 5 reported patients with multifocal SANT presented multiple nodules (2–8 nodules), ranging in diameter from 1 to 6.5 cm. The characteristics of the present case were similar to those 5 patients. In addition to the differences in typical characteristic of multiple nodules and the clinical features mentioned above, the traits in terms of imaging , patholoP = 0.705), but they were statistically significant (P = 0.009) with the differences between multifocal SANT and control spleens. Compared with normal spleens, the data showed some relationships between multiple SANT and IgG4-related sclerosing disease as well as solitary SANT, which presented evidence to support the hypothesis that this disease entity was associated with IgG4-related sclerosing disease as previous study reported by other literature [Meanwhile, we found that a variable number of plasma cells were present in the internodular sclerotic stroma and a few of which showed IgG4 positive with immunohistochemistry. On average, the number of IgG4+ and IgG+ cells were 22/HPF and 95/HPF, respectively. The IgG4/IgG ratio was 23.1 %. Kuo et al. [terature , 13.SANT is a benign lesion, for which the curative treatment is splenectomy. Review of the existing literature revealed that only 8 cases of solitary SANT were treated with laparoscopic splenectomy. The treatment effect was similar to that by open operation according to the experience of follow up , 13, 14.In summary, we reported a unique patient with multiple nodules of SANT of the spleen who underwent laparoscopic splenectomy successfully. In comparison to solitary SANT, multifocal SANT occurs more likely in males than females and incorporates with other diseases, in which malignant neoplasm has not been described yet. Multifocal SANT as well as solitary SANT show some relationships with IgG4-related sclerosing disease. When multiple lesions are found in spleen in the imaging examination, the clinicians and radiologists should be conscious of the possibility of multifocal SANT.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 of this journal.


Sarcoidosis is a granulomatous disease that may affect any organ of the body. The most frequent loci of manifestation are the lungs. However, there are individual cases where bones are affected. The literature describes cases in which swelling or fistula were the first findings of a bone lesion. This is the first case reporting an osteolysis in both angles of the mandibles which led to the diagnosis of sarcoidosis with multi-organ involvement.The authors present a 74 years old European female patient without previous diagnosis of sarcoidosis who presented with pain in the area of the jaw angles. There were no further clinical symptoms. Bone biopsy following radiological investigation demonstrated non-caseating granulomas consistent with sarcoidosis of the bone. Further evaluation confirmed multi-organ disease with involvement of lungs, intrathoracic lymph nodes, and the central nervous system.This case report shows that diagnosis of a severe disease can be missed if systematic clinical signs are not given. Furthermore, an accurate anamnesis and examination is required to receive an early diagnosis which often needs an interdisciplinary approach. Sarcoidosis is a chronic granulomatous disease of unknown origin with clinical manifestations being highly variable between affected patients. Sarcoidosis was first described by the surgeon and dermatologist Jonathan Hutchinson in London more than 100 years ago . An exagThe clinical appearance is highly variable, involving sustained cough, erythema nodosum, periarticular inflammation, fever and occurring fatigue. The diagnosis is based on three pillars: (1) clinical assessment, (2) radiographic signs and (3) histological evidence of non-caseating granulomas . A biopsA 74 year old European female patient approached her dentist due to pain in the right lower jaw. A panoramic X-ray was performed, presenting an osteolysis of unknown origin in the mandible on both sides, right more than left Fig. . The patAt the day of first presentation there were no signs for locale infection on the oral and extraoral examination, and no palpable lymph nodes in the cervical region. Only the tooth 47 showed percussion sensitivity and was not sensitive to cold. There were no abnormal laboratory findings, specifically normal C-reactive protein, serum calcium and alkaline phosphatase.A computed tomography with 3D reconstruction of the osteolysis was performed. The radiography showed loosening of cancellous bone with extension of the mandibular canal and partially interrupted cortical bone on the right side. The findings were suspicious for osteomyelitis or a malignant lesion. Additional Single Photon Emission Computed Tomography (SPECT-CT) examination was completed presenting increased bone metabolism in the area of the vertex and the jaw angles of both sides, right more than left Fig. . FurtherDue to relevant multi-organ disease with symptomatic bone involvement and strong suspicion of neuro-sarcoidosis immunosuppressive therapy with azathioprine was installed. A follow-up SPECT-CT scan after six months of treatment revealed regressive bone metabolism of all previously described lesions, and no new lesions were detected. The patient was free of pain and the immunosuppressive treatment was well tolerated.A couple of differential diagnosis should be considered when evaluating intrabony lesions of the jaws, in particular of the lower jaw: osteomyelitis, metastatic tumors, multiple myeloma, Langerhans histiocytosis. All these findings may present a similar radiographic image and could be confounded with a sarcoidotic lesion and had therefore to be considered in our patient.There are a few cases in literature reporting sarcoidosis with manifestation in the lower jaw. Monasebian et al. described the diagnosis of sarcoidosis by means of a recurrent swelling of the chin in 1997 . Here, lOur case differs from aforementioned studies as it began with an osteolysis of unknown origin in the mandible on both sides. To our knowledge there is no scientific literature about the onset of sarcoidosis on the lower jaw.Systematic signs could be even missed in some cases. This fact may not lead astray. We suggest considering a chronic granulomatous disease in cases of intra-bony lesions. Each obscure bone lesion has to be biopsied to get the accurate diagnosis followed by the adequate therapy.The first symptoms of multi-organ sarcoidosis can be variable and symptomatic bone involvement is a rare presentation of this disease. Histologic confirmation is mandatory, followed by further diagnostic assessments with regard to other organ involvement. This case is of utmost clinical importance as it demonstrates that an accurate anamnesis and clinical and radiological examination are required for an early diagnosis of this rare disease.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 of this journal.


Osteoid osteoma is a benign tumor that usually occurs in the long bones of young adults. Its symptoms can be diverse depending on the location of the tumor and especially difficult to diagnose when occurring in an atypical location. Osteoid osteoma arising in the sacrum is extremely rare, and here, we present a case that was treated successfully in a minimally invasive fashion under computed tomography guidance.A 25-year-old Asian man was referred to our institution due to persistent pain in the buttock after 12 months of conservative treatment. Computed tomography and magnetic resonance imaging scans revealed a ring-shaped radiolucency consistent with a nidus of osteoid osteoma in the sacrum. The lesion was subsequently resected under computed tomography guidance and the histological diagnosis was compatible with osteoid osteoma. His postoperative course was uneventful, and at two years after surgery our patient is symptom-free with no evidence of recurrence.Computed tomography-guided resection of osteoid osteoma in the spinal column is feasible and accurate if there is adequate margin from vital organs. Although rare, it is important to always bear in mind the possibility of osteoid osteoma occurring in the sacrum when no other apparent lesion is detected. Osteoid osteoma is a benign tumor of the bone that occurs predominantly in the lower extremity in young adults. The tumor can arise in any part of the skeletal tissue; however, it is rarely found in the spine. The lesion is commonly characterized as a small calcified lesion (<2cm) on X-rays with disproportionate pain that aggravates at night. Usually, the nocturnal pain is effectively alleviated by the administration of nonsteroidal anti-inflammatory drugs. The tumor sometimes causes pain even before characteristic findings on X-rays are discernible and is commonly associated with referred pain in a nearby joint. Because of these obscurities of osteoid osteoma, in addition to its relatively low occurrence, the correct diagnosis is often very challenging. In this report, we present a rare case of osteoid osteoma arising in the sacrum that was successfully treated by computed tomography (CT)-guided resection, with a review of the cases of osteoid osteoma arising in the sacrum.A 25-year-old Asian man complained of a persistent pain in the buttock, which worsened at night. He went to a nearby hospital; however, neither physical examination nor X-rays showed apparent abnormalities. A nonsteroidal anti-inflammatory drug was prescribed for a pain of unknown cause. The medication was quite effective, but after a year of prescription, the pain gradually deteriorated and our patient began to experience night pain that interfered with sleep. Because the origin of the pain was still unknown, he was referred to our institute for further examination. Our patient had no previous incidence of trauma or any relevant family or medical history. On physical examination, there was no redness or swelling, but there was tenderness on the right lower sacrum. The overlying skin was smooth and nonadherent, and there was no neurological deficit. On X-ray, no abnormal finding was detected. On the magnetic resonance imaging (MRI) scan, there was an approximately 1cm lesion in the S4 ala of sacrum that extended into the posterior soft tissue, which showed low intensity on T1-weighted images and high intensity on both T2-weighted images and T2-weighted selective partial inversion recovery (SPIR) images with unclear margin, indicative of bone edema or inflammation Figure . Based oOsteoid osteoma is a benign osteogenic tumor first reported by Jaffe in 1935 . Pain soOccurrence of an osteoid osteoma in the spinal column is rare; the incidence has been reported to be approximately 13 percent of all osteoid osteomas -9. When et al. in 1990 [Most of the osteoid osteomas that occurred in the sacrum were treated by open resection and only four cases were treated by CT-guided resection. CT-guided resection of the nidus was first reported by Voto  in 1990 . Several in 1990 ,12. Howe in 1990 . When th in 1990 ,15. AlthHere, we describe an osteoid osteoma of the sacrum treated by CT-guided resection. The present report shows that osteoid osteoma arising in the sacrum can successfully be treated by CT-guided resection without any noticeable side effects or local recurrence. Osteoid osteoma is sometimes difficult to detect especially in the early stage where there is no apparent radiographic findings. It is important to always bear in mind the possibility of osteoid osteoma for persisting pain, and to perform a thorough CT scan whenever the tumor is suspected.Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.CT: computed tomography; MRI: magnetic resonance imaging; SPIR: selective partial inversion recovery.The authors declare that they have no competing interests.SF carried out the data analysis and manuscript writing. MS completed the draft and critically revised the case report. IW and KN carried out the collection and assembly of data. KH contributed to the manuscript writing. YT provided administrative support. HM was responsible for the provision of study material of patients and final approval of the manuscript. All authors read and approved the final manuscript.


Primary ovarian leiomyosarcoma is a very rare tumour which is most commonly seen in postmenopausal women. A case is described here involving a 30-year-old female who presented with pain in the abdomen. The patient underwent laparotomy with peritoneal lavage, bilateral salpingo-oophorectomy, and total abdominal hysterectomy. Microscopic and immunohistochemical findings established the diagnosis of primary ovarian leiomyosarcoma. Similar to sarcoma of other tissues, an adjuvant chemotherapy regimen consisting of vincristine, epirubicin, and cyclophosphamide was given for this present case. Six months after treatment completion, the patient is on regular follow-up and disease-free on clinical and radiological examination. Sarcomas originating primarily in the ovary are rare and make up less than 2% of all ovarian malignancies . PrimaryA 30-year-old lady presented with complaints of right lower abdominal pain since seven days which was insidious in onset and gradually progressive in nature. There was a history of difficulty in micturition and also a history of intermenstrual bleeding which were on and off for the past one year. There was no history of altered bowel habits. An abdominal examination did not reveal any abnormalities. On preoperative perivaginal examination when the cervix was pulled up, a mass was felt through the posterior fornix. Whereas on postoperative perivaginal examination, a small cystic mass was felt in the left fornix. Ultrasonography of the whole abdomen showed a large mass occupying the whole of the pelvis just superior to the fundus of the uterus arising from the right adnexa (not arising from the uterus) measuring 12.5 x 13.2 x 14.3 cc. There was also moderate free fluid seen in the peritoneal cavity with moving internal echoes . The chePrimary ovarian sarcomas are relatively uncommon representing less than 2% of all ovarian malignancies . PrimaryVarious pathogenesis related theories include malignant degeneration of smooth muscle tissue present in the ovary and malignant transformation of uterine leiomyoma to the ovary .Most leiomyosarcomas present in advanced stages with vague symptoms of per vaginal bleeding, pelvic region pain, and altered bowel and bladder habits.Grossly the tumour appears as solid masses with or without cystic degeneration which depends on the size of the tumour . MicroscImmunohistochemical markers used in the diagnosis of leiomyosarcoma of the ovary include desmin, vimentin, smooth muscle actin, S-100 , 7. It iBecause of the rarity of the disease and late presentation, there is no established standard treatment. The main treatment modality is surgery which ranges from fertility sparing surgery to debulking surgery consisting of total abdominal hysterectomy, bilateral salpingooopherectomy, and excision of the tumour masses. Cytoreductive surgery can be performed during relapses and prognosis is good if the total tumour surgical resection is possible , 8, 11.Even though chemotherapy and radiation therapy have been used in the adjuvant setting they do not provide any additional benefits , 13, 7. Leiomyosarcoma of the ovary is a rare malignancy. Because of the rarity of the disease and late presentation, there is no established standard treatment. The main treatment modality is surgery. Even though chemotherapy and radiation therapy have been used in the adjuvant setting often, there is no strong evidence of any additional benefit.


The algorithm of treatment of septic lateral sinus thrombosis (SLST) has undergone a paradigm shift with the understanding of the natural history of sigmoid sinus thrombosis. Thus, the recent medical literature promulgates the management of these cases with no sinus exploration. However, in view of marked paucity of literature on the cited subject, not much is known about this form of treatment. We present our experience of treating two paediatric cases of SLST with mastoid surgery and no sinus exploration: both cases had excellent recovery. Finally, conclusions are drawn in light of contemporary literature on this subject. Septic lateral sinus thrombosis (SLST) is a rare complication of chronic suppurative otitis media (CSOM) in paediatric population  . InteresManagement of SLST varies considerably in the literature. The traditional teaching has been to do a mastoid surgery with sinus exploration under antibiotic cover –3. Some An 8-year-old boy reported to the ENT Emergency with chief complaints of ear discharge and pain in the left ear with postauricular swelling. Patient also had a history of fever, headache, and nausea and vomiting. The patient had been suffering from CSOM left ear for the past 3 years and had been taking antibiotics for the same off and on. However, no medical records were available for the antibiotic treatment. Examination of the ear showed a perforation in pars flaccida, erosion of scutum with foul smelling discharge, and cholesteatoma flakes. In the postauricular region 4 × 2 cm boggy inflammatory swelling was also seen, which had pus on needle aspiration. In view of intracranial symptoms a medical opinion was sought and subsequent CECT scan (Contrast-Enhanced Computed Tomography) revealed lateral sinus thrombosis . Hence, Surgical PathologyPostauricular Abscess. 5 mL pus was drained.Cholesteatoma. Cholesteatoma was seen in aditus, antrum, attic, epitympanum, PSQ of tympanic cavity involving facial recess, and sinus tympani.Ossicles. Status of ossicles: long process of incus, handle of malleus, and stapes suprastructure were necrosed.An 11-year-old girl reported to the ENT OPD with the complaint of persistently discharging left ear for the past 3 months. In addition, for the past 10 days patient had also developed ear pain and persistent headache. However, patient had no other clinical features of raised intracranial tension. Past history revealed that this girl had been suffering from CSOM left ear for the last 5 years with gradually deteriorating hearing loss. History of rampant use of antibiotics with no medical records was also present. Examination of the ear revealed posterosuperior perforation in the tympanic membrane (involving both pars tensa and the adjoining pars flaccida) with granulations and cholesteatoma flakes. The Griesinger sign was positive. A CT scan of this patient revealed destructive bony changes with dilated sigmoid sinus with partial hypodense filling defect on left side: lateral sinus thrombosis . Thus, aSurgical PathologyGranulations with Cholesteatoma. Antrum, aditus, attic, and posterosuperior quadrant of tympanic cavity involved facial recess and sinus tympani.Ossicles. Long process of incus and stapes suprastructure were necrosed.It would be prudent to note that “ear swabs” were sterile and “hypercoagulability” was absent in both cases. Both patients were treated initially with a combination of amoxicillin and clavulanic acid and metronidazole intravenously for duration of 2 weeks, followed by oral antibiotics for the next two weeks.Some clinical observations regarding these cases merit discussion. Both of our cases had a history of protracted CSOM with abuse of antibiotics. The medical literature cites antibiotic resistance as an important cause for aetiopathogenesis of SLST . FurtherAlthough we diagnosed these cases by CT scan, “Magnetic Radio Imaging (MRI)” is regarded as a more sensitive investigation to diagnose SLST. However, this should be performed in conjunction with CT scan to evaluate associated otologic and cerebral pathology , 5. DespThere is considerable controversy regarding surgical management of SLST: the role of mastoid surgery is also not fully characterized. Further, there is marked surgical dilemma regarding the involved thrombosed sinus: many advocate incision of sinus with removal of the clot, while other simply unroof the sinus and confirm the presence of thrombus with needle aspiration. Both of our cases of SLST had excellent recovery after mastoid surgery only. In no case the involved sinus was explored. This treatment is based on the principle that thrombus formation is secondary to infection, and thus treatment of infection is the core management issue (not the thrombus). It is reasoned that formation of thrombus is a protective mechanism attempting to localize infection, and the natural history of SLST is of resolution. The venous occlusion resolves by 4 to 6 weeks with adequate antibiotic treatment only (no adjuvant surgery or anticoagulant therapy is required). Thus, once the source of infection is eradicated, the thrombus resolves , 4, 7. HIn accordance with aforesaid principle, some otologists have also reported a favourable outcome in patients of SLST by removal of surrounding granulation tissue and inflammation around the sinus (reducing the thrombophilic nidus) with clot left untouched –10. AlsoGiven the success of mastoid surgery under antibiotic cover in both of our cases, we conclude that SLST in CSOM-cholesteatoma disease requires mastoid surgery with no sinus exploration. However, many questions regarding management of SLST still remain unanswered. We cite our modest experience in two cases as a template for future research and modification in the best interest of patient care.In summary, we present this clinical record on account of (i) rarity of the cited complication, (ii) its unique management by excluding sinus exploration, and (iii) underreporting of this management in English medical literature, which limits conclusions to be drawn on the treatment of OLST in accordance with evidence based medicine.


Although colon cancer is the third most common cause of cancer-related death worldwide, the prevalence of gastroenteropancreatic neuroendocrine tumours (GEP-NETs) remains rare. To date, very few cases of GEP-NETs within Meckel’s diverticulum and synchronous colorectal cancer have been reported. Although the coexistence of these two tumour types is uncommon, it is important to be aware of their disease patterns. We present a rare case of a patient with an intestinal GEP-NET arising in Meckel’s diverticulum coexisting with metastatic colon adenocarcinoma, and we discuss the clinical manifestations and the diagnostic procedures and treatment modalities used. This case report underlines the importance of being aware of this particular coexistence, as well as the unlikely metastatic spread of GEP-NETs and the importance of a multidisciplinary approach to cancer treatment. Finally, individualizing the treatment according to the stages of the primaries will result in durable cancer control, particularly in synchronous double malignancy. Neuroendocrine gastroenteropancreatic tumours (GEP-NETs) constitute a heterogeneous group of genetically diverse neoplasms arising from the secretory cells of the neuroendocrine system, with the primary tumours located in the digestive tract. Their iKRAS gene was negative. Moreover, a mass in Meckel’s diverticulum, 8.7 cm proximal to the ileocaecal valve, was encountered. The histological examination revealed a firm, grey-white tumour composed of neoplastic neuroendocrine cells in the submucosa arranged in a nesting pattern measuring 3 × 2 × 2 cm. There was penetration of the muscular layer and lymphatic vessels, but without serosal, neural or vascular invasion. The resection margins were free of tumour. Immunohistochemical staging confirmed a well-differentiated, low-grade GEP-NET (low grade (grade G1), pT3pN0pM0, AJCC stage IIB, CKAE1/AE3–positive, CgA–positive, synaptophysin-positive, Ki-67 at 3%, with 2 mitoses/10 hpf, without necrosis) , carcinoembryonic antigen (CEA) , carbohydrate antigen 19-9 (CA 19-9)  and hepatomegaly, all other systems and laboratory findings were normal. A colonoscopy revealed intraluminal stenosis with a reddish, irregularly shaped mass located in the ascending colon, 15.5 cm from the ileocaecal valve, so biopsy specimens were obtained. An open right hemicolectomy with ileotransverse anastomosis was performed. Intraoperatively, the interior surface of the colon demonstrated an invasive, exophytic, crater-like tumour. The clinical and pathological information about the patient is summarized in Table The patient was seen by a medical oncologist with a view to starting chemotherapy. Because there was only evidence of colon cancer metastases, we decided to start with a FOLFIRI  chemotherapy regimen, which has been reported to induce major tumour responses in 40% to 50% of patients with stage IV colon cancer–9. Our pMeckel’s diverticulum is a vestigial remnant of the omphalomesenteric duct. It is the most common embryonic malformation of the gastrointestinal tract and is present in approximately 2% of the population, with men experiencing symptoms more frequently than women. Its preGenerally, the NETs arise from the dispersed endocrine system and may originate from almost any location in the human body. They are most commonly found in the gastrointestinal tract (such as GEP-NETs) and the respiratory system. As neuroendocrine phenotypes and genetics have become better understood, the definition of neuroendocrine cells has changed and is now accepted as referring to cells with neuromodulator, neuropeptide or neurotransmitter hormone production; dense-core secretory granules; and absence of axons and synapses. GEP-NETs have attracted much attention in recent years because they are often slow-growing neoplasms which are relatively easy to palliate. Moreover, pharmacological therapy has dramatically improved symptom control, thus offering different targeted therapies for metastatic or inoperable disease. This kiIn the past, the phenomenon of multiple primary cancers was considered a medical curiosity. As anticipated, multicentric cancers in a single organ, in paired organs or in contiguous tissues are now known to occur with increasing frequency. Generally, they fall into two categories: (1) synchronous, in which the tumours occur at the same time; and (2) metachronous, in which the tumours follow in sequence. In the 1930s, Warren and Gates, in a stet al.[According to a previous study, gastrin and cholecystokinin were associated with NETs; this resulted in tissue growth in the gastrointestinal tract and carcinogenesis that led to colorectal and gastric cancers. In addiet al. found thet al., 22.Surgery remains the best method of treatment for NETs, regardless of their localization. It is the preferred option for patients with resectable disease, but palliation with tumour debulking, chemotherapy and targeted radionuclide therapy is often needed. For localized disease, only surgery provides the possibility of complete remission, 24. HowThe prognosis for, and long-term survival of, patients with NETs has improved with the advent of more aggressive surgical intervention and the use of long-acting somatostatin agonists and targeted second-line therapy. Recent studies have demonstrated that malignant disease, defined by direct invasion of adjacent organs by tumours, lymph node metastases or distant organ spread, may have 5-year survival rates as high as 77% to 95% when treated aggressively with resection of primary tumours and adjunctive therapy, 34. ForIn addition, surgery is the only curative modality for localized colorectal cancer (stages I and II). Chemotherapy is standard management for patients with high-risk stage II, stage III or stage IV disease . Biologic agents  have assumed a major role in the treatment of metastatic cases, with selection increasingly guided by genetic analysis of the tumour. At present, the role of radiation therapy is limited to palliative therapy for selected metastatic sites, such as bone or brain–40.All patients with synchronous colorectal cancer and GEP-NETs must be extensively evaluated and clinically monitored during the workup and follow-up period in order to detect disease progression or relapse. Typically, patients are seen every 3 to 6 months during the first 3 years and every 6 to 12 months thereafter. Routine laboratory tests, tumour markers , endoscopic investigation of the gastrointestinal tract, abdominal ultrasound, ultrasound of lymph nodes, chest radiography and CT or whole-body PET/PET-CT scans may be used during follow-up. We consider that early diagnosis with complete preoperative examination, careful intraoperative exploration, periodic postoperative surveillance and radical resection can increase survival time. The treatment strategy depends on many factors, such as the surgical approach, the patient’s general condition, the tumour grade, the extent of the disease and the patient’s response to therapy. The treatment strategy needs to be individualized for each patient, 42. HowThe occurrence of synchronous primary cancers remains an issue of great interest to surgeons, and oncologists in particular, as well as the field of medicine in general. The question of common genetic pathways in the pathogenesis of such tumours is always raised when such associations are seen. Because multiple primary cancers can no longer be considered rare, the physician should be alert to the possibility of their occurrence. However, NETs arising in a Meckel’s diverticulum represent a significant clinical challenge because they have varied presentations, and initial imaging studies to locate the tumour may be inconclusive. Our case addresses several important aspects of clinical interest. First, only a very small number of cases have been reported with synchronous GEP-NET within Meckel’s diverticulum and colon adenocarcinoma. Second, there are no clear guidelines for appropriate treatment and follow-up of patients with GEP-NET with a synchronous secondary malignancy; therefore, our experience may help in the consideration of treatment for similar patients. The case that we report also emphasizes the importance of a thorough exploration of the abdomen when carrying out elective laparotomies. The impact of synchronously existing cancers on the overall prognosis of a patient must always be considered when planning therapy in such instances as well. Finally, patients who manifest multiple primary cancers represent a unique experiment of nature that may challenge new avenues of investigation into the complex aetiology of tumours.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.