Background. Eosinophilic angiocentric fibrosis (EAF) is a rare benign condition of unknown aetiology that causes stenosis of the upper respiratory tract. It is most commonly found at the nasal septum and sinus mucosa causing mucosal thickening and nasal obstructive symptoms. The diagnosis is mainly based on characteristic histologic findings. Case Report. A 27-year-old young woman presented with a slow growing mass at her anterior nasal septum for over eight years. She complained of persistent nasal obstruction, epistaxis, sometimes diffused facial pain, and chronic headache. 3 years ago, the tumor was partially resected for ventilation and a nasal septum perforation was left. Imaging findings indicated soft-tissue thickening of the anterior part of septum and adjacent lateral nasal walls. Pathological examination showed numerous inflammatory cells infiltrates containing eosinophils, fibroinflammatory lesion with a whorled appearance fibrosis which typically surrounded vessels. A diagnosis of eosinophilic angiocentric fibrosis was made. All laboratory tests were unremarkable. Skin prick test was positive. The tumor-like lesion was totally resected. Conclusions. EAF is a rare benign and progressive disorder causing destruction. Combined with radiological imaging of EAF historical findings contribute to the diagnosis. It is important to prevent tumor from recurrence by total resection of the lesion. Eosinophilic angiocentric fibrosis (EAF) is a rare, benign condition of unknown aetiology which may cause local tissue progressive destruction . It mainIt was first described by Holmes and Panje in 1983 who reported a case of so-called ‘‘intranasal granuloma faciale” . After tWe described a case of a young woman with primary nasal septum tumor-like lesion which was diagnosed as EAF based on historical findings and gave a literature review of EAF.A 27-year-old otherwise healthy young woman presented with a slow growing mass at her anterior nasal cavity for over eight years. Her symptoms included persistent nasal obstruction, recurrent nasal discharge and epistaxis, sometimes diffused facial pain, and chronic headache. The lesion completely obstructed both nostrils three years ago. She sought help for nasal ventilation in another ENT center. Submucous thickening tissue was locally resected and the involved anterior nasal septum cartilage was also partially removed. Histopathological examination of the biopsy indicated fibrous tissue with hyaline degeneration. Two years ago, bilateral alternative nasal obstruction reoccurred and got worse and worse. Physical examination showed that the patient had a broad nasal bridge. A large submucosal thickening of the anterior septum felt solid masses on palpation. There were no cutaneous lesions of nasal vestibule or her face. Endoscopic examination showed an anterior nasal septum perforation which might be due to the last surgery.Imaging findings showed soft-tissue thickening of the anterior part of septum and adjacent lateral nasal walls. On nonenhanced CT, the lesions appeared homogeneously isoattenuated to gray matter. Perforation of the anterior nasal septum was identified with its size approximately 1.3 × 1 cm. Bone window showed localized and discontinuous oppressive thinning of the lower edge of frontal process of maxilla (P1). The tumor lesion involved soft tissues of piriform aperture and is close to the anterior wall ethmoid bone. From the MRI images, the tumor lesion was located at the anterior 1/3 of the septum and the adjacent lateral nasal walls, bilateral anterior inferior turbinate. The inner side of upper lateral nasal cartilage was isointense on the T1-weighted image while being hypointense on the T2-weighted image (P2). Lacrimal sac and nasolacrimal duct were normal. Chest X-ray found no nodule or other abnormality.Pathological examination showed that numerous inflammatory cells were infiltrated with eosinophils, plasma cells, and lymphocytes. The predominant cells were eosinophils. Nasal biopsy revealed fibro-inflammatory lesions with a whorled appearance fibrosis which typically surrounded vessels. It also showed a concentric “onion-skin” fibrosis formed without any small-caliber vessels in its center. There were still some eosinophils infiltrations. Fibrinoid necrosis or true granuloma formation was absent in these biopsy tissues.Whole blood neutrophil count was slightly lower. Other blood routine examinations , blood biochemistry, erythrocyte sedimentation rate (ESR), and coagulation parameter were in normal ranges. Serum immunological examination indicators were in normal ranges except that IgA slightly increased to 613 mg/dL (82–453 mg/dL). Antinuclear antibody (ANA) and anti-double-stranded DNA (anti-ds-DNA) antibody were negative. Both anti-PR3 antibody (c-ANCA type) and anti-MPO antibody (p-ANCA type) were lower than 20 RU/ml. There was an excess of skin prick test with positivity to dandelion (++), dermatophagoides farine (++), and dermatophagoides pteronyssinus (++). Serum total IgE increased its level to 315 KU/L . Under general anesthesia, endoscopic surgery was performed with a 3 cm-long arc-shaped incision on the skin of the nasal vestibule from the lateral nasal cartilage to the septal cartilage. The masses profile was white and hypovascular. Perichondrium was stripped using periosteal elevator and the masses were totally fished out until the surrounding normal mucosa could be clearly identified. Endoscopic observation indicated the greater alar cartilage and septodorsal cartilage were partially involved and destroyed. Then the cartilage lesions were totally resected and the white formless tumor was fished out. The opposite nasal cavity was performed with the same procedures. The anterior nasal septum perforation, left without reconstruction. Two silicious tubes for dilation were placed from the anterior nares through the common meatus for three months. A light nasal pack was placed to safeguard against large clot accumulation. Antibiotics and glucocorticoid were used intravenously to prevent infection and alleviate edema. We recommended long-term intranasal topical corticosteroids and followup every three months. Two years after surgery, there was no sign of local recurrence and distant metastasis. EAF is a rare benign disorder which is classified as a chronic fibrosing vasculitis. The underlying aetiology and pathophysiology are unclear. It mainly affects the sinonasal tract of young to middle-age patients leading to upper airways obstruction. To date, fifty-one cases have been described and the nasal septum was the most common affected location. There was not an apparent sex predilection judging from the review of all the current English literature  .The EAF is a historical diagnosis with two stages which has no clear boundary. It is characterized by progressing from an early eosinophil-rich perivascular fibrosing inflammatory lesion to a late dense perivascular “onion-skin” fibrosis formation with decreased inflammatory infiltration , 30, 32.On non-enhanced CT, the lesion appeared to have homogeneous isodensity to gray matter and it may cause adjacent bone remodeling, thinning, and slight absorption with the lesion progress . In our Currently, the exact etiology of EAF remains unclear. Allergy, atopy, and trauma have been considered as predisposing factors , 18, 27.The differential diagnosis of EAF includes Wegener granulomatosis (WG) , 29, graUntil now, although consensuses did not reach the treatment strategy of EAF, surgery excision was performed in almost all reported case reports , 27, 30.EAF is a rare benign disorder which may be progressive and cause local tissue destruction. Radiological imaging and historical findings contribute to the diagnosis of EAF. Laboratory results may be nonspecific. The allergic and eosinophilic infiltration may represent an abnormal inflammatory response to nonspecific stimulus in predisposed individuals. It is important to prevent tumor from recurrence by totally resecting the involved tissues.


Adrenal myelolipoma is a rare, benign neoplasm that is usually asymptomatic, unilateral and nonsecreting. It develops within the adrenal gland and is composed of mature adipose tissue with elements of the hematopoietic series. We describe the case of what is, to the best of our knowledge, one of the largest secreting adrenal myelolipomas reported in the literature.A 52-year-old Caucasian man of medium build who had had moderate hypertension for three years presented to our hospital. He had no other significant symptoms. His hypertension was pharmacologically treated. He came to our hospital to undergo abdominal ultrasonography during a clinical checkup. The ultrasound scan showed the presence of a voluminous hyperechoic mass interposed between the spleen and the left kidney. It was reported as a myelolipoma of the left kidney on the basis of its structural characteristics and position. Computed tomography confirmed our diagnosis. All preoperative biochemical tests were normal, with the exception of high serum cortisol, which was being overproduced by the lesion and was probably responsible for the patient's hypertension. He underwent successful surgery, and his postoperative course was uneventful. The pathologic examination of the lesion confirmed the diagnosis of adrenal myelolipoma. The patient's blood pressure returned to within the normal range.The "incidental" discovery of an adrenal mass requires careful diagnostic study to plan adequate therapeutic management. Both of the primary investigations at our disposal, ultrasound and blood tests , helped in rendering the diagnosis and allowed us to move toward the most appropriate treatment, taking into account the size of the tumor and its probable hormonal production. The myelolipoma is a rare, benign neoplasm composed of mature adipocytes and hematopoietic tissue. It was first described by Gierke in 1905 and subsequently by Oberling in 1929, who used the term "myelolipoma" . In the These lesions are usually unilateral and asymptomatic. A certain number of bilateral tumors have been described in the literature. Myelolipomas are often smaller than 4 cm in diameter, although they can reach wider sizes . The larWe report the case of a 52-year-old Caucasian man of medium build who had had moderate hypertension for three years. He referred to no other noticeable symptom. His hypertension was pharmacologically treated. The patient came to our hospital to undergo abdominal ultrasonography during a clinical checkup. US showed the presence of a large hyperechoic mass Figure  with nonA surgical left adrenalectomy was performed. The surgically removed myelolipoma showed it to have an oval shape (about 25 cm × 20 cm × 20 cm), and it weighed 4.4 kg. Sectioning revealed areas representing well-differentiated fat. The histological examination revealed the presence of adipose tissue with hematopoietic elements without signs of cellular atypia, thus confirming the initial diagnosis. The patient's postoperative course was altogether uneventful.Adrenal myelolipoma is often an "incidentaloma," since its diagnosis is frequently based on autopsic findings or upon diagnostic imaging examinations performed for reasons unrelated to its presence. These tumors are rare, although they are increasingly being detected because of wider use of diagnostic imaging techniques. They are benign and nonfunctional tumors composed of mature adipocytes and active hematopoietic elements. Their histogenesis is uncertain. According to some authors, the tumor develops from residual embryonic cells of the bone marrow that, after embolization, reach the adrenal gland. According to a recent theory, myelolipoma originates from a metaplastic process of the reticuloendothelial cells within adrenal capillaries. Some authors even consider the development of adrenal myelolipomas as a response to various endocrine stimulations . This hyIn the clinical case we have described here, it is interesting to note the hyperincretion of cortisol by the myelolipoma. This hyperincretion was probably responsible for the patient's hypertension, since his blood pressure was normalized after surgical removal of the tumor and thereafter his blood pressure remained within normal values.Another rare feature to consider is the left-sided position of the myelolipoma in our patient, because they have been reported in the literature to be prevalent mostly on the right side. This location has probably facilitated the rise of a left spermatic varicocele even if the patient is asymptomatic.The "incidental" discovery of an adrenal mass requires careful diagnostic study to plan an appropriate treatment. Imaging techniques at our disposal today can help the clinician to render the diagnosis. Since myelolipomas consist mainly of adipose tissue, their sharp hyperechogenicity observed on US may orient the clinician toward the diagnosis, but US does not yield any certain detail about nature of lipomatous lesions. CT can clarify the nature of incidentalomas, as in our patient, and can indicate the best treatment, taking into consideration the tumor size and its possible hormone production. Biochemical studies are also useful and necessary in the typing of the mass.CT: computed tomography; MRI: magnetic resonance imaging; US: ultrasonography.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.The authors declare that they have no competing interests.AMB and GS collected the data and drafted the manuscript. AB and RF took care of the patient during his hospitalization. All authors read and approved the final manuscript.


Granular cell tumours, first described by Abrikossoff in 1926, are known to occur in skin, connective tissue, breast, gastrointestinal and genital tracts. While they are rare, they are more common in people of African descent and show a slight female preponderance, usually presenting as solitary and painless masses. Less than 10% of occurrences are multiple, and fewer than 3% of tumours behave in a malignant fashion. The mean age, at presentation, is 40–60 years. We report a case of granular cell tumour in a young white male presenting with a painful soft tissue tumour in his buttock. The presentation is unusual because of the age, patient demographic, body site, and clinical presentation. The clinical and histological aspects are reviewed in the context of this clinical case and the associated literature. Granular cell tumours were first described by Abrikossoff in 1926 and are known to occur in skin, connective tissue, breast tissue, and gastrointestinal and genital tracts—with the head and neck being the most common regions. The tongue is the most common site in the head and neck . The tumThe case we are reporting is that of a young white male, aged 27, who presented with a two-month history of a 2 cm mass in his buttock, which was preventing him from sitting down, due to pain. Our initial clinical impression of this fibrofatty mass was of a well-circumscribed lipoma or neurofibroma, and the differential diagnosis included a cyst. The pain and tenderness to touch were attributed to pressure effects on his sciatic nerve. Given that this was a young male patient presenting with a painful dermal/subcutaneous mass, we did not consider granular cell tumour as part of our differential diagnosis until histopathological examination.A 27-year-old white male was referred to our centre, by his GP, with a lump in the patient's right buttock. The mass was 2 cm in diameter and was felt to be a lipoma clinically. The patient himself was not aware of the lump and had visited his GP only because every time he sat down he felt pain over his buttock region, which radiated down his leg. This symptom was easily reproducible and prevented the patient from sitting down on hard surfaces. The patient was otherwise well, with no other medical conditions. There was no family history of any malignancy or cutaneous masses or lipomata.On examination, we felt a well-localized, approximately 2 cm soft tissue mass, which was clinically located in the deep dermis or in the subcutaneous fat. There was no attachment to muscle and no overlying skin changes. Our differential diagnoses included lipoma, neurofibroma, or cyst. Given that the lesion was well localized, not greater than 2 cm and not adherent to muscle or deep fascia, we proceeded to excise the lesion under local anaesthesia, without imaging.During the operation, the lesion seemed well localized and intraoperatively resembled a sebaceous cyst or pilomatrixoma. Histological reports are detailed below. A compete skin and lymph node examination revealed no other abnormalities. After reviewing the histopathology, this patient was managed with a wide local excision with 1 cm margins.The tumour was well circumscribed, spanned the entire dermis, and showed broad interface with the underlying adipose tissue. The interface with the epidermis was quite irregular, with prominent epidermal pseudoepitheliomatous hyperplasia Figures  and 2.Histologically, the tumour cells were quite monomorphous, with small round nuclei and abundant granular eosinophilic cytoplasm . The tumGranular cell tumours are uncommon and when they occur, they are most common in the head and neck. They present as painless masses. Surgical excision is the treatment of choice. The recurrence rate for granular cell tumours has been reported at 2%, when local wide excision has been undertaken . Most grHistologically the tumour presented no diagnostic difficulty. However if the tumour was sampled superficially, the irregular interface with the overlying epidermis would create a well-known diagnostic pitfall. With superficial biopsy, one can see how easily a diagnosis of invasive, well-differentiated, squamous cell carcinoma can be made. This can lead to a potentially harmful, unnecessary surgery, especially when the lesion is present in the tongue. Our patient presentation was unusual, given that it was the symptomatic nature of the lesion that led to the diagnosis—with the patient being unable to sit due to buttock pain—which resulted in the initial referral; the impression, prior to surgery, of a neuroma; during surgery, a cyst or pilomatrixoma. A survey of buttock tumours had suggested that when pain is present it is usually due to cyst formation in an old haematoma, and pain along the course of the sciatic nerve, and its branches, was present in 40 percent of the cases .Malignant behaviour is rare and is seen in up to 2% of the cases. Most of the malignant tumours arise in the thigh, while malignant granular cell tumours of the head and neck are very rare . The malThe tumour we present was excised completely, as incomplete excision may produce recurrences in 21–50% of the cases . This case report suggests that granular cell tumours must be considered in the differential diagnosis of painful buttock tumours.


Purpose. To describe a patient with Bilateral Diffuse Uveal Proliferation who presented initially with a clinical picture consistent with choroidal melanoma. Methods. Presentation of a clinical case with fundus photos, fluorescein angiography, and optical coherence tomography. Results. A 70-year-old Caucasian male with history of esophageal cancer presented with an asymptomatic pigmented choroidal lesion in his left eye initially diagnosed as choroidal nevus. This lesion enlarged over the course of a year and developed orange pigment and increased thickness. A metastatic workup was negative, and a radioactive iodine plaque was placed on the left eye. Over the next six months, the visual acuity in his left eye decreased. His clinical picture was consistent with unilateral Diffuse Uveal Proliferation. A recurrence of his esophageal carcinoma with metastasis was discovered and palliative chemotherapy was initiated. Although his visual acuity improved in the left eye, similar pigmentary changes developed in the right fundus. His visual acuity in both eyes gradually decreased to 20/200 until his death a year later. Conclusion. BDUMP should always be considered in the differential diagnosis of patients with pigmented fundus lesions and a history of nonocular tumors. Bilateral Diffuse Uveal Melanocytic Proliferation (BDUMP) was first described by Machemer in 1966 , 3.We report an atypical intial presentation of BDUMP mimicking a small choroidal melanoma. A 70-year-old Caucasian male with a history of esophageal cancer status after surgery, chemotherapy, and radiation presented with a 2 × 2 mm flat pigmented choroidal lesion inferior to the optic nerve in his left eye (OS) on a routine eye examination. This lesion enlarged over the following 12 months to approximately 5 × 5 mm with 1 mm elevation and a significant amount of lipofuscin . After nBDUMP is a rare paraneoplastic condition with approximately 30 reported cases in the literature. Patients typically present with sudden bilateral vision loss which antedates the discovery of a primary nonocular tumor in approximately half of the cases. Survival after diagnosis is between 8 and 24 months [The initial presentation of our patient was unique. The patient had an asymptomatic unilateral small pigmented lesion which was initially diagnosed as a choroidal nevus. The lesion enlarged over the course of one year and demonstrated classic signs of a small choroidal melanoma . At that time, there were no signs of any fundus abnormalities in the fellow eye. Consultation with two ocular oncology experts was arranged who agreed with the diagnosis of melanoma and subsequent treatment with RAI plaque placement. Typical signs of BDUMP did not develop until months after the plaque treatment. It was then that a recurrence of his primary esophageal tumor was detected as well. Of note, the original elevated choroidal lesion remained hypofluorescent on fluorescein angiography whereas the subsequent lesions that developed were flat, smaller and underwent a transformation on fluorescein angiography from pinpoint hyperfluorescence to larger window defects with areas of staining later in the course.This unique case illustrates that BDUMP should always be considered in the differential diagnosis of patients with pigmented fundus lesions and a history of nonocular tumors even if a systemic workup reveals no signs of recurrence upon presentation.


Malignant triton tumors (MTTs) are a rare subtype of malignant peripheral nerve sheath tumor (MPNST) showing rhabdomyoblastic differentiation, which have no treatment consensus and a poor prognosis. This case report presented the case of a 42-year-old male patient with a large MTT located in the anterior mediastinum. The patient underwent palliative chemoradiotherapy and interstitial chemotherapy and received traditional Chinese medicine. Localization of an MTT in the anterior mediastinum is extremely rare. To the best of our knowledge, this is only the fourth study of an MTT localized in the anterior mediastinum that has been documented in the English literature. Malignant peripheral nerve sheath tumors (MPNSTs) are believed to arise from Schwann cells or nearby cells with perineural differentiation. The incidence of MPNSTs is one per 100,000 cases. In total, ~50% of MPNST cases arise in patients with neurofibromatosis type 1  and the five-year overall survival rate is 34% . To dateA 42-year-old male patient with a history of a dry cough for 2 months and chest distress for 1 month was treated at the clinic of Zhangjiagang Bo’ai Hospital  on April 23, 2013. The results of the chest radiograph  and enhaHistological analysis revealed a spindle cell tumor with interlacing fascicles of wavy spindle cells and prominent mitotic figures. Loose and impact arrangements were detected. In certain focal areas, the tumor was comprised of round rhabdomyoblasts, with abundant eosinophilic cytoplasm and eccentric nuclei in a loose matrix. Immunohistochemical analysis revealed that the spindle cells were positive for S-100, and the rhabdomyoblasts were positive for myogenin and desmin, respectively . SimultaConsidering the locally advanced staging of the anterior mediastinal MTT, surgical excision was ruled out. Chest computed tomography re-examination revealed that the anterior mediastinal MTT had enlarged rapidly to 17.0×15.0×12.0 cm on May 23, 2013 . Palliatet al introduced the term MTT in a review of seven cases . The median survival time of patients with MTT was 13 months, the overall local recurrence rate was 50% and the median time to progression was 6 months. The study suggested that complete surgical resection and adjuvant radiotherapy should be the gold standard treatment for MTT. The study also concluded that conventional chemotherapy does not appear to be of benefit (With regard to location, MTTs occur predominantly in the head, neck and trunk regions. In total, ~20% of MTT cases arise in the head and neck, with 32% in the trunk and 24% in the extremities ,16. To d


Primary pleural squamous cell carcinoma is very rare, and there is a lack of experience in the diagnosis and treatment of this condition. An asymptomatic 75-year-old man was referred to us after a right pleural nodule was found on computed tomography during a routine health examination. He underwent surgery for his pleural tumor twice over the following 2 years. Histopathological examination eventually led to a diagnosis of primary pleural squamous cell carcinoma. Primary pleural squamous cell carcinoma (SCC) is very rare . A revieUnfortunately, pleural SCC and localized mesothelioma have different oncological characteristics. Localized mesothelioma usually has a good prognosis , but pleIn August 2009, a 75-year-old man  was referred to our clinic after a right pleural nodule was found on chest CT during a routine health examination. He was asymptomatic, and physical examination was unremarkable. Chest CT showed a soft tissue nodule with homogeneous enhancement arising from the right pleura, measuring 31 × 15 mm. The nodule had clearly demarcated margins and there was no evidence of invasion into the adjacent ribs. There was no enlargement of mediastinal lymph nodes  of the pleura. However, these two conditions have different progression and prognosis. In order to avoid misdiagnosis and inappropriate treatment, it is necessary to distinguish between the two carefully.SFT of the pleura is also uncommon, accounting for 4% of all pleural neoplasms . SFT is Early pleural SCC that has not invaded the surrounding structures is usually asymptomatic. As the disease progresses, some atypical symptoms may appear, such as chest pain, cough, sputum production, weakness and weight loss. Early pleural SFT is also asymptomatic ,9, but dThe lack of specific features makes it difficult to differentiate between early primary pleural SCC and early pleural SFT on preoperative imaging examinations such as chest radiography and computed tomography ,10. ThesPreoperative biopsy is important for the diagnosis of pleural tumors. The reported rate of accurate diagnosis on CT-guided fine needle aspiration is about 45% in some series ,11. HoweSeveral tumor markers may also be helpful for determining the type of pleural tumor. P63 is always negative in cases of mesothelioma and almost always positive in cases of SCC . CalretiSurgery is the preferred method of treatment for pleural nodules. Cardillo et al.  reportedVATS is the preferred procedure for excision of pleural nodules because it is minimally invasive. The surgical treatment of choice is local excision with intraoperative assessment of the surgical margins . If tumoSome reports have suggested that CT should be performed every 6 months for the first 2 years after excision of pleural tumors to monitor for recurrence. Most recurrences of malignant pleural tumors occur within 24 months of the initial resection. Recurrence rates after complete resection were reported to be 8% in patients with benign pleural SFT and 63% in patients with malignant pleural SFT . FurtherFor recurrent pleural SFT, repeat surgical resection may be the first choice of treatment, and is associated with good cure rates . If recuPrimary pleural SCC is very rare. Because of the lack of clinical experience, early primary pleural SCC is easily misdiagnosed as localized mesothelioma, leading to delayed or inappropriate treatment. However, SCC can be diagnosed by preoperative investigations such as FDG-PET, measurement of tumor markers, and transthoracic Tru-Cut needle biopsy. The prognosis of this disease is not clear at present.Written informed consent was obtained from the patient for publication of this Case report and the accompanying image. A copy of the written consent is available for review by the Editor-in-Chief of this journal.SCC: Squamous cell carcinoma; CT: Computed tomography; VATS: Video-assisted thoracic surgery; SCCA: Squamous cell carcinoma antigen; SFT: Solitary fibrous tumor.The authors declare that they have no competing interests.YY wrote the manuscript. CC, YL and XML performed the surgery. PL carried out the pathological examinations. XML and JC were involved in the final editing. All authors read and approved the final manuscript.


Malrotation of the midgut is generally regarded as paediatric pathology with the majority of patients presenting in childhood. The diagnosis is rare in adults, which sometimes leads to delay in diagnosis and treatment. A high index of suspicion is therefore required when dealing with patients of any age group with abdominal symptoms. We present a case of a 55-year old man who presented with an acute abdomen with preoperative computed tomography scan and operative findings confirming midgut rotation. The duodenum, small bowel, caecum and appendix were abnormally located, with the presence of classical Ladd's bands. There was no evidence of intestinal volvulus. The patient underwent an emergency laparotomy with an uneventful postoperative recovery.A review of the literature is presented to highlight the rarity of intestinal malrotation and the controversies surrounding its management in the adult population. Midgut malrotation is a congenital anomaly in the embryological development of the foetal intestinal rotation. It has been estimated that it affects approximately 1 in 500 live births . It is rWe report a case of an adult patient with an acute presentation of midgut malrotation which highlights the dilemmas of preoperative diagnosis, as supported by a review of the literature.A 55-year old gentleman was admitted to the Accident and Emergency department with a three day history of acute onset, cramp like abdominal pain. There was associated nausea but no vomiting. Bowels had been opened the day before admission but no flatus had been passed for 24 hours. There were no other associated red flag symptoms. The patient had never experienced similar symptoms and had no previous medical or surgical history.On examination, the patient was afebrile and haemodynamically stable. The abdomen was moderately distended with significant tenderness in the central, epigastric and left hypochondrial regions. There was no evidence of peritonitis.9 and a neutrophilia of 7.0 × 109.Routine admission blood tests including serum electrolytes, urea, amylase, lactate, liver function tests (LFTs), clotting profile, C-reactive protein (CRP) and an arterial blood gas (ABG) were normal. However, a full blood count (FBC) demonstrated a haemoglobin level of 14.2 g/dl with a slightly raised total white cell count (WCC) of 10.3 × 10A chest radiograph did not reveal air under the diaphragm. Abdominal radiograph showed non-dilated gas filled loops of bowel in the central and upper abdominal regions. The diagnosis remained elusive until an emergency computed tomography (CT) scan Figures , 2, 3, 4The patient was resuscitated with intravenous fluids, analgesia and prepared for an emergency exploratory laparotomy. The findings at operation included dilated small bowel in the upper abdomen, partial torsion and necrosis of the greater omentum, the caecum was on the left side of the abdomen tethered by torted omentum, and loops of small bowel occupying the right paracolic gutter and the right iliac fossa. There were fibrous bands over the distal part of the duodenum, on the right side of the abdomen, confirming midgut malrotation Figures  &6.The twisted, necrotic omentum was excised, the congenital bands were divided and an appendicectomy was carried out. The anatomical malrotation was left uncorrected.The patient had an uneventful postoperative recovery and was discharged home on the fifth day post- surgery. On follow up he was well and there had been no late complications. He had returned to his premorbid level of function and did not report any symptom recurrence.Initial presentation of symptomatic midgut malrotation is rare in adults. However, a significant number of cases remain quiescent during childhood. Incidental diagnosis may then occur in adulthood; when imaging investigations are carried out for other symptoms or, during surgery for unrelated pathology. It has been reported that the incidence of malrotation in adults is approximately 0.2%. However, it is probable that this rate will rise with future developments in diagnostic imaging. It is difficult to ascertain the true incidence, but evidence from post mortem studies suggest that gut malrotation may affect up to 1 in 6000 ,4.Midgut malrotation is broadly considered a deviation from the normal 270 degree counterclockwise rotation of the gut during embryonic development. During week 4 of foetal development, the embryonic gut, consisting of a straight endodermal tube, develops vascular pedicles to be divided into the foregut, midgut and hindgut based on the anatomical blood supply. The midgut is supplied by the superior mesenteric artery (SMA) and by the fifth week of embryonic life, it begins a process of rapid elongation and outgrows the capacity of the abdominal cavity. This leads to a temporary physiological herniation into the umbilical cord at about the sixth week of life with return to the abdominal cavity about 4 to 6 weeks later. During this period, the midgut undergoes a 270 degree counterclockwise rotation around the SMA axis. This process leads to the formation of the duodenal C-loop, placing it behind the SMA in a retroperitoneal position and emerging at the ligament of Treitz. The progressive reduction of the physiological midgut herniation commences at about week 10 of embryonic development. The duodeno-jejunal flexure (DJF) and jejunum to reduce first and lie to the left. The distal small bowel then follows and lies progressively to the right of the abdominal cavity. The descent of the caecum from its higher position in the right upper quadrant forms the latter part of this complex rotational development; it becomes positioned in the right lower abdomen. The ascending colon then assumes a retroperitoneal position, also on the right side. The base of the small bowel mesentery subsequently fuses with the posterior peritoneum in a diagonal fashion, from the ligament of Treitz at the DJF to the caecum, completing the whole process at about the eleventh week of foetal development [The failure of the normal physiological rotation of the midgut leads to various degrees of anomaly including the entire small bowel remaining on the right side of the abdomen, the caecum, appendix and colon on the left and an absent ligament of Treitz. In addition, the small bowel mesentery may develop a narrow vertical attachment and the peritoneal fibrous bands fixing the duodenum and caecum to the abdominal wall may persist. These congenital bands extend from the right lateral abdominal wall, across the duodenum and attach to the undescended caecum and are known as Ladd's bands ,4,6,7. LMidgut malrotation in adults presents in numerous ways and the symptoms are non-specific. The clinical diagnosis in adolescents and adults is difficult because it is rarely considered on clinical grounds. Many patients remain asymptomatic and the diagnosis is discovered incidentally during investigations or laparotomy for other unrelated problems in adult life. Wang and Welch  showed tThe other group of symptomatic adults typically present with symptoms of acute bowel obstruction and these patients may or may not report a previous history of abdominal symptoms, as with our patient. These patients may on occasion, have symptoms and signs of an impending abdominal catastrophe. Moldrem et al  reportedWe can expect an increase in the incidental diagnosis of gut malrotation with increasing and widespread use of radiological investigations. Diagnostic features of midgut malrotation can be identified using plain abdominal radiograph, ultrasound scan (USS), computed tomography (CT) scan, magnetic resonance imaging (MRI) scan and mesenteric arteriography ,11. ConvCT scan with or without UGI contrast study is increasingly used preferentially as it is now considered the investigation of choice; providing diagnostic accuracy of 80% ,9,11. CTMesenteric angiography was previously used but is now rarely indicated in the evaluation of malrotation. It has the capacity to demonstrate the abnormal relationship between, and detect the patency of, the mesenteric vasculature. Angiography was used to demonstrate the characteristic corkscrew appearance of a whirling SMA and its branches; the 'barber pole sign' as well as extensive collaterals caused by proximal SMA occlusion . HoweverSymptomatic midgut malrotation undoubtedly requires surgical intervention although the management of asymptomatic patients is more controversial. Choi et al  reviewedThe surgical management of intestinal malrotation was first described by William Ladd in 1936 -6,9. MosThere are recent reports of the use of the laparoscopic approach in the surgical treatment of intestinal malrotation. The technique appears to be safe and effective when performed by experienced laparoscopic surgeons, especially in the absence of volvulus ,8,18,19.Intestinal malrotation is a rare condition but is considered an important cause of bowel obstruction in adults. The diagnosis of malrotation after childhood is difficult and usually not readily considered as the cause of intra-abdominal symptoms. The presentation is usually nonspecific and this often leads to diagnostic and treatment delay with possible bowel ischaemia and necrosis. Evidence of which portends a poor prognosis and death. Therefore, a high index of suspicion needs to be maintained and prompt surgical intervention must be considered in order to prevent an abdominal catastrophe and fatality. There are no reliable means of identifying which group of patients with intestinal malrotation will develop subsequent complications. In the light of this, many authors are now advocating early surgical intervention in the form of a standard and modified Ladd's procedure. There is evidence in the literature that the use of Ladd's procedure or ordinary division of Ladd's bands and adhesiolysis relieves symptoms and in fact, prevents recurrence in the majority of patients.Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.ABGs: Arterial blood gases; CRP: C-reactive protein; CT: Computed tomography; DJF: Duodenal jejunal flexure; FBC: Full blood count; LFTs: Liver function tests; MRI: Magnetic resonance imaging; SMA: Superior mesenteric artery; USS:Ultrasound scan; WCC: White cell count.The authors declare that they have no competing interests.OFE was involved in postoperative care, conceived the write up, performed the literature search and manuscript preparation.AAA performed the operation with TWD, involved in the preoperative and postoperative care, conceived the write up, performed the literature search and manuscript preparation.TWD performed the operation with AAA, involved in the preoperative and postoperative care, conceived the write up, performed the literature search and manuscript preparation.All authors read and approved the manuscript for submission.


Adenosquamous carcinoma is defined as a tumor in which both glandular and squamous elements are histologically malignant. Although some published studies have analyzed and discussed adenosquamous carcinomas, hybrid malignancy of the ampulla of Vater has rarely been discussed thus far in the literature. In this study, we report the case of a 64-year-old man who presented with jaundice and intermittent abdominal dull pain that persisted for several weeks. The patient was diagnosed with adenosquamous carcinoma of the ampulla of Vater and underwent pancreaticoduodenectomy. The final diagnosis was adenosquamous carcinoma of the ampulla of Vater, T3N1M0, stage IIB. Although R0 resection was performed, he had multiple liver metastases 2 months after the operation; he died 4 months later. Upon reviewing the medical records of our institute, we identified 4 patients who were diagnosed with adenosquamous carcinoma of the ampulla of Vater in the past 2 decades. We also identified only five reported cases of this lesion in the English literature. Adenosquamous carcinoma of the ampulla of Vater is a rare disease with a dismal prognosis. Surgical intervention does not appear to prolong patient survival. Early recurrence and distal metastasis may be encountered after surgery. Adenosquamous carcinoma (ASC) is defined as a tumor in which both glandular and squamous elements are histologically malignant. The mixed tumor was first reported in 1907. Its occurrence is more common in areas where adenocarcinomas arise frequently, such as the stomach, intestine, and uterus. ASC has also been identified in the esophagus, anus, and vagina, where squamous cell carcinomas predominate. Although some published studies have analyzed and discussed this condition, ASC of the ampulla of Vater (AmV) has rarely been discussed in the literature. In this report, we present a case of ASC of the AmV and review the experience of this rare disease in our institute.A 64-year-old man presented with jaundice and intermittent abdominal dull pain that persisted for several weeks. The only findings on physical examination were icteric sclera and yellowish skin. No tenderness or palpable mass was detected in his abdomen. On admission, the laboratory examination revealed a serum total bilirubin level of 6.4 mg/dL, aspartate aminotransferase level of 19 IU/L, alanine aminotransferase level of 21 IU/L, and alkaline phosphate level of 229 IU/L. All tumor marker levels were normal. Contrast-enhanced computed tomography (CT) revealed a protruding tumor in the AmV without invasion into adjacent organs. There were several enlarged regional lymph nodes. There was no distal metastatic lesion in the lungs, bone, or liver. The major vessels were free of this tumor. This patient underwent endoscopic retrograde cholangiopancreatography and a concurrent biopsy, and the histopathological diagnosis was of a poorly differentiated adenocarcinoma of the AmV, tumor, node, metastis (TNM) classification T2, N1, M0, stage IIA. The patient underwent standard pancreaticoduodenectomy (PD) with lymph node dissection.2 per month combined with local radiation was arranged for this patient 6 weeks after the operation. Follow-up CT was scheduled for 3 months after surgery, but the patient presented with progressive jaundice 2 months after surgery. Abdominal CT revealed multiple metastatic nodules in the liver. This patient received palliative chemotherapy as Gemcitabine and Cisplatin once after the tumor progression. Nevertheless, his condition deteriorated, and the tumor progressed rapidly. He died 6 months after surgery.On gross examination, a polypoid tumor, measuring 3.4 cm × 2.2 cm, with invasion to the pancreas was detected in the AmV Figure A. MicrosThe histogenesis of ASC remains uncertain. Four hypotheses regarding its histogenesis are summarized as follows: (1) pluripotent epithelial stem cells capable of inducing the malignant transformation of both cell types; (2) squamous metaplasia in the intestinal mucosa; (3) adenocarcinoma transforming into squamous cell carcinoma; and (4) collision of both malignant tumors. Although we do not understand the histogenesis of ASC, it exhibits a more aggressive biologic behavior and is associated with a worse prognosis than conventional adenocarcinoma according to previous reports.th tumor stage classification of these patients are listed in Table Four cases of ASC of the AmV have been reported in the malignancy registry in our institute in the past two decades. Compared to adenocarcinoma, ASC of the AmV is an uncommon malignancy. The most common presentation of the patients in this series was jaundice with intermittent right upper quadrant pain. All of the patients received surgical interventions. Three patients underwent PD, and one underwent ampullectomy. The demographic characteristics and the American Joint Committee on Cancer (AJCC) 7et al. [A review of the English literature revealed only five reported cases of ASC of the AmV. We have summarized these reports, including the clinical course and final prognosis of ASC of the AmV, in Table et al. , completet al. ,2. BecauSome authors have suggested conservative management of patients with ASC of the AmV diagnosed before surgery, to reduce postoperative morbidity and achieve comparable survival as observed in patients undergoing surgery . AlthougIn conclusion, ASC of the AmV is a rare disease with a dismal prognosis. Its symptoms are similar to those of adenocarcinoma of the AmV, and accurate preoperative diagnosis is difficult. Surgical interventions do not appear to prolong patient survival, and early recurrence and distal metastasis may be encountered after surgery. However, the rarity of this malignancy makes it difficult to draw firm conclusions regarding its clinical course and overall prognosis.Written informed consent was obtained from the patients or the next of kin of the patients for publication of this report and any accompanying images. Copies of the written consent forms are available for review by the Editor-in-Chief of this journal.None of the material has been previously published.AJCC: American Joint Committee on Cancer; AmV: Ampulla of Vater; ASC: Adenosquamous carcinoma; CT: Computed tomography; PD: Pancreaticoduodenectomy; PPPD: Pylorus-preserving pancreaticoduodenectomy.The authors declare that they have no competing interests.S-J Y, S-Y W, and Y-Y L performed the surgical treatment and drafted the manuscript. C-S O and I-M K revised the registry record and performed the pathological studies. T-J W revised this manuscript. C-H L critically reviewed the manuscript and gave final approval for publication. All authors have read and approved the final manuscript.


A 32-year-old right hand dominant female presented with a 2-year history of a slowly growing firm, nodular, and nontender right thumb mass. Sensation and strength in the involved digit were intact. Range of motion was mildly restricted. Surgical dissection of the lesion is shown in Figures.What is the differential diagnosis?What are the clinical and morphological characteristics of this condition?What are the treatment options?What are current recommendations for preventing recurrences?The differential diagnosis of this patient's hand lesion included giant cell tumor of the tendon sheath (GCTTS), ganglion, synovial cyst, fibrous histiocytoma, and nodular fasciitis.,2,5,7-2,3First described by Chassaignac in 1852,3,2,8Treatment of localized GCTTS consists of complete open or arthroscopic resection of the tumor as well as all residual satellite nodules or intraosseous lesions. The open approach with wide surgical exposure and meticulous dissection with an operating microscope or magnifying loupes is preferred to prevent contamination and reduce recurrence rates.8,3,7,2Following surgical excision, GCTTS has a reported recurrence rate of 4% to 44%. Recurrence is most strongly associated with incomplete excision and is more variably associated with the presence of satellite, diffuse-type, or multicentric lesions; intraosseous invasion; and direct extension of the tumor to tendons, the joint capsule, or the distal or thumb interphalangeal joints.2This patient's GCTTS was more consistent with a type I lesion. During gentle dissection of the patient's GCTTS lesion, the golden to yellow tumor  was foun


Of all female genital tract tumors, 1-3% are stromal malignancies. In 8-10% of cases, these are represented by Müllerian adenosarcoma an extremely rare tumor characterized by a stromal component of usually low-grade malignancy and by a benign glandular epithelial component. Variant that arises in the pouch of Douglas is scarcely mentioned in the medical literature.A 49-year-old para-0 woman, was seen at our OB/GYN-UNIT because she complained vaguely of pelvic pain. She had a mass of undefined nature in the pouch of Douglas. A simple excision of the mass showed low-grade Müllerian adenosarcoma with areas of stromal overgrowth. One and a half year after surgery, at another hospital, a mass was detected in the patient's posterior vaginal fornix and removed surgically. Six months later she came back to our observation with vaginal bleeding and mass in the vaginal fornix. We performed radical surgery. The pathological examination showed recurrent adenosarcoma. Surgical treatment was supplemented by radiation therapy.The case of Müllerian adenosarcoma reported here is the third known so far in the literature that was located in the pouch of Douglas. To date, only two other such cases have been reported, including one resulting from neoplastic degeneration of an endometriotic cyst. Malignant stromal tumors account for 1-3% of all female genital tract tumors; 8-10% of these are Müllerian adenosarcomas. Adenosarcomas are rare tumors scarcely reported in the medical literature that associate benign glandular epithelium with a malignant endometrial stromal component of usually low histological grade. Occasionally, a high-grade malignant stromal component may raise a differential diagnosis issue with leiomyosarcomas and carcinosarcomas .Adenosarcomas arise more frequently in the uterus , but casOf great interest is the association between adenosarcoma and endometriosis, especially in extrauterine forms, because the disease-free survival rate in endometriosis-associated tumors is far better than in unassociated mixed Müllerian tumors ,14.The standardization of treatment for extrauterine adenosarcoma is difficult because of the limited clinical experience and the variability of its presenting features.Here we report the third known case of Müllerian adenosarcoma located in the pouch of Douglas together with a review of the literature.B.V., a 49-year-old para-0 woman, was seen at our Hospital's Operating Unit because she complained vaguely of pelvic pain, without any history of endometriosis. In her past medical history, the patient reported appendectomy and bilateral inguinal hernia surgery. The screening tests  that she underwent periodically were normal. During her hospital stay, routine blood tests, a chest X-ray, and vaginal and cervical smears were performed. The gynecological examination was normal, except for some mild pain on digital exploration at the posterior vaginal fornix. There was no vaginal bleeding. Transvaginal sonography revealed a 10 × 6 cm mass of undefined nature in the pouch of Douglas. Tumor marker tests indicated slightly elevated levels of CA19-9 and CA125. The magnetic resonance imaging (MRI) scan and the computed tomography (CT) scan excluded any distant metastatic spread. Based on the patient's clinical picture, we advised radical surgery. She accepted the surgical removal of the mass, but did not agree to the recommended total hysterectomy, bilateral adnexectomy, and pelvic lymphadenectomy. Therefore a simple excision of the mass was planned. During surgery, intraoperative frozen sections were performed that diagnosed a low-grade mesenchymal tumor to define better after fixation and embedding.The final slides showed a biphasic tumor associating epithelial and stromal components. The epithelial component was characterized by endometrial-type cells without atypia showing a variety of metaplastic changes, including ciliated and eosinophilic, that lined polypoid vegetations, and glandular and phyllodes-like structures Figure . The mesThe post-operative course was uneventful and after a few days the patient was discharged from hospital.One and a half year after surgery, a mass was detected in the patient's posterior vaginal fornix. It was removed surgically at another hospital unit and submitted for histology, which confirmed a recurrence of low-grade Müllerian adenosarcoma showing features similar to those of the primary lesion. No significant changes in the proliferative activity of the tumor were observed as assessed by Ki-67 staining. The patient chooses not to receive any adjuvant therapy. Six months later she came back to our observation with vaginal bleeding and a 4 cm polypoid mass in the posterior vaginal fornix. The MRI scan and the CT scan excluded any metastatic disease. Piver's type-III radical hysterectomy with bilateral adnexectomy, selective pelvic lymphadenectomy, and upper colpectomy were performed. Postoperative course regular, without fever. The pathological examination showed recurrent adenosarcoma infiltrating the uterus and the posterior wall of the cervix . The parametria were not involved. The recurrent tumor showed morphological and antigenic features similar to the primary lesion. The lymph node examination was negative for metastasis. There was no evidence of endometriotic foci. Surgical treatment was supplemented by radiation therapy in the pelvic cavity with a total dose of 50 Gy.Müllerian adenosarcoma is an uncommon mixed epithelial-mesenchymal tumor of low-grade malignancy that affects the female genital tract. In most cases, it occurs in women aged 14 to 89 years , is located in the uterus and is accompanied by non-specific symptoms, i.e. vaginal bleeding, uterine enlargement, and vague pelvic pain .Since the first literature report by Clement and Scully  in 1974,First of all, it is essential to provide a histological diagnosis of the lesion. Adenosarcoma should be distinguished from its uncommon benign counterpart, adenofibroma, but the differential diagnosis cannot be established with certainty from curettage or biopsy specimens .Histological criteria for malignancy include at least mild atypia with a mitotic count of 2 per 10 HPF, distinctive periglandular cuffs of cellular stroma with or without intraglandular protrusions of stromal element, and invasion .Heterologous differentiation is also found in 10-15% of cases and helps in the differential diagnosis. Indeed, a recent paper challenges the existence of adenofibroma, as aggressive tumor behavior has also been reported in cases without sarcomatous overgrowth and with only mild nuclear atypia and a negligible mitotic rate .Once the neoplastic transformation has been established, the major factor affecting the outcome is tumor location. In patients with genital tumors, disease recurrence occurs in about 25% of cases, usually in vagina or in pelvis, with a mortality rate of about 10%. By contrast, extragenital tumors are reported to recur in over 50% of cases and have a mortality rate of about 35% .Extragenital tumors are distinctively less common and are primarily located in the pelvic peritoneum, retroperitoneum, broad and round ligaments, vesicouterine pouch, and rectouterine pouch. Only two cases of Müllerian adenosarcoma located in the pouch of Douglas, described by Ostor et al.  have beeClements and Scully  in 1990,In this study, too, we were able to confirm a significant correlation between extrauterine location or myometrial invasion and recurrence rates. On the other hand, the degree of myometrial invasion and the recurrence rate are strongly correlated to the differentiation grade and the mitotic index of the sarcomatous stromal component ,11.Sarcomatous overgrowth - as defined by a confluent growth of the sarcomatous component occupying at least one quarter of the tumor - and a deep muscular invasion are considered adverse prognostic factors ,13.However, sarcomatous overgrowth is seen more frequently in ovarian than in uterine tumors.Ultimately, the most important negative prognostic factors are lesion location, the degree of myometrial invasion, and sarcomatous overgrowth. A further strongly unfavorable prognostic factor is disease recurrence following primary treatment .By contrast, a favorable prognostic factor in adenosarcoma patients is the presence of endometriosis. The possibility of a neoplastic transformation of endometriotic foci has been suggested by several studies. In their study, Stern et al.  (2001) iOn immunohistochemical testing of endometriosis-associated adenosarcoma, it is frequent to find positivity for estrogen and progesterone hormone receptors. Soslow et al.  (2008) aNotwithstanding the great progress made in the diagnosis and treatment of Müllerian adenosarcoma, its rarity and its heterogeneous behavior make difficult to set up well-defined therapeutic protocols. Given the risk of recurrence, metastasis and progression to high-grade sarcomas, even low-grade tumors should be carefully considered especially when associated with unfavorable risk factors.As Müllerian adenosarcoma can evolve into the most aggressive form of all mixed Müllerian tumors , i.e. adRadical surgery remains a therapeutic cornerstone. Shi et al.  2008) r08 r20] (Thus, while awaiting to acquire a wider clinical experience on this rare form of genital tumor, we recommend a "customized" treatment that uses surgery and either neoadjuvant or adjuvant chemo/radiation therapy, which, in the event of early diagnosis, can be expected to achieve a disease-free result.The authors declare that they have no competing interests.TSP and SG have made substantial contribution to conception and design. LF and RB have been involved in drafting the manuscript. EMS and ET performed the pathological examination. AL revising it critically for important intellectual content. GBN and ABM have given final approval of the version to be published. All authors read and approved the final manuscript.Written informed consent was obtained from the patient for publication of this case report. A copy of the written consent is available for review by the Editor-in-Chief of this journal.The pre-publication history for this paper can be accessed here:http://www.biomedcentral.com/1471-2407/11/171/prepub


Torsion of ovarian cyst is a common cause of acute abdomen especially in women of reproductive age-group. It commonly presents with colicky abdominal pain associated with nausea and vomiting. It could however mimic acute intestinal obstruction.The patient was a 32-year-old multipara with no previous history of pelvic or abdominal surgery. She was admitted with colicky lower abdominal pain associated with repeated episodes of vomiting and nausea. Laboratory investigations were essentially normal. Abdominopelvic USS showed a hypoechoic mass lesion in the left adnexium measuring 7.1 × 5.5 cm; surrounding bowel loops were hypoactive, dilated, and fluid filled. Diagnosis of acute abdomen secondary to suspected torsion of ovarian cyst was made. Management began for acute abdomen with intravenous hydration, prophylactic antibiotics, and analgesics. An emergency laparotomy revealed about 6 cm defect in the left broad ligament in which a 20 cm segment of terminal ileum was encased. Liberation of the ileal segment was done and the broad ligament defect closed. Bowel obstruction requires high index of suspicion in a patient with acute abdomen due to suspected torsion ovarian cyst most especially in the absence of previous pelvic or abdominal surgery. Intestinal obstruction occurring from internal hernia is very rare, with a reported incidence between 0.2% and 0.8% , 2. An iAn adnexal torsion accounts for roughly 3% of gynaecologic emergencies . AdnexalAdnexal torsion often can be a challenging diagnosis to make, because the classic symptoms of severe, sharp unilateral abdominal pain and nausea may not be present  and occa0 (3A), who presented complaining of acute lower abdomen colicky pain of 12-hour duration with nausea and repeated episodes of vomiting for 10 hours before presentation. Vomitus contained essentially recently ingested food initially but later bilious. She had no prior relevant medical history. There is no prior history of pelvic or abdominal surgery. On physical examination her left lower quadrant was tender with a palpable mass of about 6 cm by 4 cm, with mild voluntary guarding and increasing bowel sounds. Rectal examination did not show any abnormalities. Laboratory studies showed no definite abnormalities.This case concerns a 32-year-old P3+An urgent abdominopelvic ultrasound revealed a hypoechoic mass lesion in the left adnexia measuring 7.1 by 5.5 cm, with surrounding hypoactive, dilated and fluid filled. Supportive therapy was commenced with analgesics and prophylactic antibiotics which did not improve patient clinical condition. An urgent exploratory laparotomy was carried out which revealed a defect of about 6 cm in the left broad ligament between the left round ligament and the left fallopian tube through which about 20 cm length of the ileum had herniated . No evidInternal hernia is responsible for about 0.9% of intestinal obstruction. Hernia of the broad ligament is extremely rare and accounted for less than 7% of all internal hernias.The earliest reported case of an incarcerated hernia through a defect of the broad ligament of uterus was in 1861 by Quain, found at autopsy : the fenPreoperative diagnosis of hernia of the broad ligament is quite difficult. The precise pathogenesis of a defect of the broad ligament remains unknown; its causes are considered to include surgery, pelvic inflammatory disease, delivery trauma, and congenital anomaly  10]. Ou. Ou10]. We emphasize that intestinal hernia through a defect of the broad ligament should be added to the list of differential diagnosis for female patients presenting with features suggestive of torsion ovarian cyst or acute abdomen without any prior history of laparotomy. Early diagnosis and surgical repair reduce morbidity and mortality from strangulation.


A left paraduodenal hernia is a rare congenital malrotational anomaly of the midgut that occurs in the paraduodenal fossa of Landzert to the left of the fourth duodenum. It is responsible for approximately 1% of small bowel obstructions.We report a case of left paraduodenal hernia combined with small bowel obstruction in a 47-year-old Mediterranean woman who had a history of recurrent abdominal pain. An abdominal computed tomography scan showed a saclike mass clustered in the left upper quadrant but failed to yield a clear diagnosis. We describe the surgical anatomy of this disease and the emergency surgical management together with a short review of the literature.Even though a left paraduodenal hernia is rare, it must be suspected in any upper intestinal occlusion. The high morbidity and mortality rate of complicated cases should motivate preventive treatment in case of incidental operative discovery. Paraduodenal hernias are the most common form of internal hernias and are responsible of approximately 1% of small bowel obstructions [They were first described in the nineteenth century under various names: left paraduodenal hernia (LPDH), Treitz retroperitoneal hernia, hernia of the fossa of Landzert, mesentericoparietal hernia of Longacre, and hernia into the descending mesocolon of Callander .We describe a case of LPDH revealed by acute intestinal obstruction and explain its surgical management together with a brief review of the literature.A 47-year-old Mediterranean woman presented to our emergency department with abdominal pain and vomiting suggesting an acute small bowel obstruction. Her past medical history was marked by recurrent diffuse abdominal pain several years ago that resolved spontaneously or after taking phloroglucinol. An abdominal examination found abdominal distention with vaulting at the left hypochondrium. The umbilical, inguinal and crural area examination was normal. A computed tomography (CT) scan showed an aggregation of jejunal loops forming a retrocolic ‘pocket’ with a mass effect on the posterior stomach wall, a torsion zone at the root of the mesentery, and stretched and engorged mesenteric vessels Figure . IntestiParaduodenal hernias are the most common form of internal hernias and are responsible for approximately 1% of small bowel obstructions , the LPDThis anatomical abnormality could be explained by two hypotheses :•The first one is the mechanical theory: increased abdominal pressure pushes the bowel in areas of low peritoneal adhesion;•The second one seems more acceptable. It suggested that LPDH is secondary to errors in midgut rotation during the fifth to eleventh weeks of gestation, with the loops of bowel becoming interposed between the attachment of the mesentery and the posterior abdominal wall; the rotation of the midgut dorsally to the colic branches of the inferior mesenteric artery instead of ventrally allowing invagination into the mesocolon.Even if it is a congenital disease, most of the cases are diagnosed between the fourth and sixth decades. The most common clinical presentation is intestinal obstruction . PatientA spiral CT scan appears to be the most helpful imaging for diagnosis; distended small bowel loops appear as encapsulated in a pouch that pushes the stomach forward; the mesenteric vessels look stretched and engorged -7.In the case of occlusion, urgent surgery must be performed, the bowel must be reduced from the paraduodenal space and the hernia sac opened. Although the inferior mesenteric vein and the upper left colic vessels can be divided without compromising the blood supply of the colon, thanks to the shunt with the upper mesenteric system, they should be preserved whenever possible . IntestiA LPDH is rare but must be suspected in any upper intestinal occlusion. The high morbidity and mortality rate of complicated cases (for example strangulated hernia) should motivate preventive treatment in case of incidental operative discovery.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 scan: Computed tomography scan; (L)PDH: (Left) Paraduodenal herniaThe authors declare that they have no competing interests.SMB and FR wrote the manuscript. MJ prepared the iconography. All authors have read and approved the manuscript.


Introduction. The nonsquamous carcinomas of the larynx are considered rare with the majority of malignant tumors in this area, reaching the rate of 95%, to be squamous cell neoplasms. Case Report. The case refers to a 53-year-old man that presented with symptomatology of motor nerve disease. During the evaluation of the neurologic disease, a subglottic mass of the larynx was revealed accidentally in the imaging examination. Under general anesthesia, we performed direct laryngoscopy and biopsy of the mass. The histopathologic examination revealed a hybrid carcinoma coexistence of two different carcinomas, an adenoid cystic carcinoma and an adenocarcinoma, not otherwise specified with poor differentiation. Regarding the therapeutic plan, the mass was considered inoperable due to its expansion to trachea and the patient received radiotherapy. Conclusions. Both the adenocarcinoma and adenoid cystic carcinoma are extremely rare types of malignant tumors in the larynx. The special interest of the present case is the coexistence of these two rare tumors in the same region of the larynx, being a hybrid tumor of the salivary glands in the larynx, which is the second reported case, based on our systematic literature review.  The cancer of larynx represents an important portion of clinical oncology, accounting for 30%–40% of the head and neck carcinomas and the 1%–2,5% of the whole human malignancies [The adenoid cystic carcinomas are usually primary tumors of salivary glands, and their location in larynx is extremely rare, representing 0,1%–0,7% of the laryngeal carcinomas. The adenoid cystic tumors proceed from small ectopic submucosal salivary glands, presenting a slow development. An adenoid cystic carcinoma of the larynx is diagnosed in advanced stage as it causes no special symptoms and usually locates in supraglottic region , 4. LaryThe aim of our study is the presentation of a case of hybrid carcinoma in the larynx, diagnosed and treated in our department and composed of adenoid cystic carcinoma and adenocarcinoma with poor differentiation and without special characters (not otherwise specified: NOS). The special interest of our patient is based on the infrequency of hybrid carcinomas of salivary glands in the region of larynx, with the systematic review of the literature to reveal that our patient would be the second reported case .Our patient, a 53-year-old man, presented to the neurology clinic of our hospital with symptomatology of motor nerve disease, reporting progressive weakness of both legs and arms during the last 4 months, especially in left side, difficulty to walk, muscle fasciculation, and cramps. During the neurologic diagnostic assessment, a subglottic mass of the larynx was found accidentally in the imaging examination of the head and neck. Particularly, the computed tomography (CT) and magnetic resonance imaging (MRI) of the neck revealed a mass with subglottic extension, starting from the region of the left vocal cord, invading the cricoid cartilage, and expanding in the trachea until the level of 4th thoracic vertebra Figures  and 2. Aα-SMA, and S-100 protein in contrast with the cells of adenocarcinoma that were negative . Histopathologically, three types are distinguished into cribriform (the most frequent), tubular (with best prognosis), and solid (with the worst prognosis). Regarding the prognosis, the natural course of such tumors seems unforeseeable as they remain stable or grow slowly for many years, and, suddenly, they may become extremely aggressive –11. The The salivary duct adenocarcinoma is a rare tumor, with its incidence to vary between 0.35% and 0.5% . At the Seifert and Donath used the term hybrid tumor for salivary glands firstly in 1996 to report the coexistence of two different types of carcinomas, growing as a sole entity, microscopically and macroscopically . The etiDue to the infrequency of hybrid tumors and the lack of published case series, our knowledge about the characteristics, the natural course, and the prognosis of such lesions remains limited. Most authors support that the biological behavior and the prognosis of a hybrid tumor are determined by the most aggressive of the coexisted carcinomas. For the same reason, the treatment is based on the therapeutic strategy for the most malignant of the coexisted tumors , 19. The minor salivary gland tumors of the larynx are rare clinical entities, with the simultaneous coexistence of them in a hybrid carcinoma to be considered an extremely infrequent case. The systematic review of the literature revealed that our case is the second reported one of hybrid carcinoma (adenocarcinoma and adenoid cystic) of the larynx. The understanding of the clinical and histopathological characteristics and natural course of such tumors would lead to more effective therapeutic strategies.


Esthesioneuroblatoma (Olfactory neuroblastoma) is a rare malignant neoplasm arising from the olfactory epithelium with bimodal age distribution between with first peak in second decades and second peak in sixth decade. Proptosis due to esthesioneuroblastoma is one of the rare causes. They have a long natural history characterized by frequent local or regional recurrence. Computed tomography and magnetic resonance imaging are the imaging modalities for diagnosing these tumors. A multidisciplinary approach with surgery and radiation therapy is an excellent treatment options for these tumors with chemotherapy being used to treat advanced or recurrent disease. Esthesioneuroblastoma (olfactory neuroblastoma) is a rare neuroectodermal malignant neoplasm that originates from the olfactory sensory epithelium -3. It acA 60 years old male presented to with complaints of nasal obstruction, nasal bleeding and bulging of left eye with decreased vision for 1.5 years. He also noticed a rapidly increasing swelling over nasal bridge for 4 months and associated headache and neck pain. There was insignificant past history and family history. On systemic examination, there was loss of smell sensation. Local examination revealed a diffuse, firm, nontender swelling with ill-defined margins over the nasal bridge, glabella and over left maxillary region. There was no rise in local temperature. Pinkish mass was visible in both nostrils with purulent nasal discharge. There was proptosis of left eye and mild lateral deviation with limited eyeball movement; however visual acuity was normal. There is no evidence of lymphadenopathy. Blood profile demonstrates normal limits of complete blood count, renal function test and liver function test.Computed tomography (CT) scan of paranasal sinuses including orbit was performed in axial section with coronal and sagittal reconstructions. CT revealed large, ill-defined, heterogeneously enhancing soft tissue density mass in both nasal cavities and ethmoid sinuses with destroyed nasal septum Figure A, 1B. ThHistopathology revealed monomorphic malignant round cell tumour with rossette formation, consistent with esthesioneuroblastoma. Patient was advised for chemotherapy/radiotherapy and was referred to oncology institute.Esthesioneuroblastoma has been reported to originate from sphenopalatine ganglion, vomeronasal organ of Jacobson, neuroepithelial cells of the olfactory membrane, the ectopic olfactory epithelium in the nasal mucosa and the amine precursor uptake and decarboxylation cells ,2. It is. Fine-cut CT scan (3 mm slice thickness) with direct coronal imaging is the initial radiological study of choice [CT and magnetic resonance imaging (MRI) are helpful to identify the margin and spread approaches of the tumorf choice . On CT sf choice ,6. AngioThere are several staging systems described for olfactory neuroblastoma; Kadish system  has beenDifferential diagnosis of esthesioneuroblastoma includes inverting papilloma, squamous cell carcinoma, adenocarcinoma, sinonasal undifferentiated carcinoma, hemangioma and metastasis. In addition to imaging, histopathological correlation is must in making diagnosis . EsthesiOpen craniofacial resection or endonasal endoscopic resection is done for stage A and B tumors. Chemotherapy is usually implemented in patients with locally advanced, metastatic or recurrent disease -14. The In conclusion, clinicians should be aware of this malignant disease and proptosis should be considered as one of the differential diagnosis of esthesioneuroblastoma. Computed tomography and magnetic resonance imaging are the modalities of choice in diagnosing, looking for extent and staging of tumor in esthesioneuroblastoma. A multidisciplinary approach with surgery and radiation therapy is the treatment options for these tumors with chemotherapy being used to treat advanced or recurrent 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.CT: Computed tomography; MRI: Magnetic resonance imaging.Authors declare no conflict of interest.SA has done substantial contributions to conception and design, acquisition of data, Analysis and interpretation of data, drafting the article, revising it critically for important intellectual content and final approval of the version to be published. KA has done analysis and interpretation of data, revising it critically for important intellectual content, final approval of the version to be published. KD has done substantial contributions to conception and design, revising it critically for important intellectual content and final approval of the version to be published. MKG has done substantial contributions to conception and design, revising it critically for important intellectual content and final approval of the version to be published. MFA has done drafting the article, revising it critically for important intellectual content and final approval of the version to be published. All authors read and approved the final manuscript.


Ewing sarcoma and primitive peripheral neuroectodermal tumor (PNET) are high-grade malignant tumors typically found in children and adolescents. These tumors belong to the family of small round cell tumors and are of neuroectodermal origin. Primary Ewing sarcoma of the kidney is rare and because of that is an infrequent differential diagnosis in urologic malignancies. Renal PNET mostly presents with nonspecific symptoms such as hematuria and abdominal pain. The imaging findings are uncharacteristic. The diagnosis is based on the histology, immunohistochemistry, and molecular pathologic findings. Once PNET has been diagnosed, multimodal treatment is indicated. Despite all treatment options, the prognosis of those with metastatic disease is poor. Ewing sarcoma and primitive peripheral neuroectodermal tumor (PNET) were originally described as two distinct pathologic entities, although both share common stem-cell precursor and unique chromosomal abnormality. Because of their similar histologic and cytogenetic characteristics, these tumors are now considered part of a spectrum of neoplastic diseases now known as Ewing's sarcoma family tumors (ESFT), which also includes other malignancies –3. The ERenal primary sarcomas are a rare group of renal tumors. Ewing sarcoma/PNET of the kidney is distinctly rare, with more than 100 cases reported globally. Among these, leiomyosarcoma is the most common (40–60%) followed by liposarcoma (10–15%) , 3.A 19-year-old man presented with sudden sharp right flank pain, accompanied by low-grade fevers and vomiting. He had no other significant medical history and denied any episodes of gross hematuria. Due to worsening of the symptoms, an abdominal ultrasound was performed, which showed an infiltrative mass in the upper pole of the right kidney, invading the right liver, concerning for malignancy . A stagiRenal cell carcinoma (RCC) is the most common malignant neoplasm of the kidney that constitutes more than 90% of kidney tumors , 4. RenaSarcomas of the kidney usually remain asymptomatic until they are large enough to produce symptoms; the average size at the time of diagnosis varies from 5.5 to 23 cm . The cliNo specific signs of PNET have been described in ultrasonography, computed tomography (CT), or magnetic resonance imaging (MRI). The imaging characteristics of most renal sarcomas are indistinguishable from those of RCC . These tThe differential diagnoses include Wilms tumor, neuroblastoma, renal cell carcinoma, malignant lymphoma, metastatic renal involvement from sarcoma elsewhere in the body, and renal involvement by a primary retroperitoneal sarcoma , 3.The diagnosis of PNET is based on the pathologic findings, assisted by immunocytochemistry and/or molecular analysis. The pathologic findings show multiple areas of necrosis and hemorrhage. In some cases, the tumor exhibits calcifications and can be surrounded by a fibrous capsule. Overexpression of the surface membrane protein CD99 is a universal feature of EFTs but not pathognomonic because it is also found in synovial sarcomas and gastrointestinal stromal tumors , 9.In the case of a large renal tumor with necrotic and hemorrhagic areas, fine needle aspiration is a valuable diagnostic tool . Image-gEwing sarcoma and PNET exhibit aggressive course characterized by early metastatic disease (25–50% at the time of presentation) and is considered to be a systemic disease. Patients undergoing only local therapy have a recurrence rate of 80% to 90%, presumably because of subclinical metastasis at the time of initial diagnosis . In addiSuccessful treatment requires multimodality approach that consists of surgery, chemotherapy, and radiotherapy . PostopePNET is an infrequent differential diagnosis in urologic malignancies. Renal PNET occurs predominantly in young adults and has a tendency to be extremely aggressive. Patients often have nonspecific symptoms such as hematuria and abdominal pain. The CT and MRI findings are nonspecific, but they are useful methods for local assessment of resectability and detection of metastases. The diagnosis is based on the histologic, immunohistochemistry, and molecular pathologic findings. Image-guided renal biopsy may play an important role in determining preoperative diagnosis. Once PNET has been diagnosed, multimodal treatment is indicated. Despite all treatment options, the prognosis is poor.


Retroperitoneal schwannomas are quite rare tumors. Isolated renal hydatid cyst is also rare, and it forms 2–4% of hydatid disease. Because of their infrequent occurrence, nonspecific signs and symptoms, and lack of distinguishing radiologic features, we report herein a case of right retroperitoneal mass in a 26-year-old woman which was found to be benign schwannoma following a percutaneous core needle biopsy and a large cortical cyst in the lower pole of the left kidney which was diagnosed as isolated renal hydatid cyst following exploration. Schwannomas originate from Schwann cells of peripheral nerve sheaths, and while they are commonly seen in head, neck, and flexor surfaces of the extremities, they are quite rare in the retroperitoneal region. Schwannomas are most often diagnosed by histologic examination and immunohistochemical staining of the excised mass.Echinococcus granulosus, in which humans are an intermediate host. Renal hydatid cysts are also rare and correspond to 2–4% of all cases of hydatid disease.Hydatid disease is caused by the larval stage of Here we report an incidentally diagnosed bilateral retroperitoneal masses in a young woman which were found to be right benign schwannoma following a percutaneous core needle biopsy and left renal hydatid cyst following exploration. The therapeutic approach is also presented.The present case is a 26-year-old woman. One year prior to the current admission, in an ultrasonography study, a well-defined lobular mass (78 × 28 × 33 mm in size) with a hypoechoic content in the posteroinferior of right kidney and a univesicular cyst of 105 × 98 × 73 mm in the lower pole of left kidney causing mild hydronephrosis had been reported . The intIn noncontrast CT-scan, the right mass had been hypodense and it was heterogeneously enhanced following contrast injection and a large cortical cyst in the lower pole of the left kidney with thin peripheral contrast enhancement without any other organ involvement .There had been no urinary symptom, gross hematuria, headache, sweating, flushing, or tachycardia. Physical exam had been normal, and abdominal masses had not been palpable.Her medical history was also unremarkable, and no family history of hydatid disease was identified.Medical laboratory tests including CBC, urinalysis, urine culture, and blood chemistries had been all in normal range. The patient had not been referred due to the lack of any symptom until feeling occasional mild and vague pain in her right flank from 2 to 3 months prior to the current admission.In the second admission, no significant change in the masses was observed and all medical laboratory tests were normal. Histologic study following ultrasound-guided percutaneous core needle biopsy of the right retroperitoneal mass revealed benign schwannoma . Open suRadiologic findings led us to drain the cyst percutaneously with the diagnosis of left simple renal cyst.After three month, using local anesthesia, the cyst was punctured with an 18-gauge Chiba needle under ultrasound guidance; 120 mL clear fluid was aspirated. Then 50 mL of 95% ethanol (40% of the cyst volume) were injected into the cavity. The ethanol was left for 20 min following which all ethanol was aspirated and the needle was withdrawn. Biochemical and cytological analysis of the cyst was unremarkable and culture was negative.No evidence of retroperitoneal tumor recurrence and no significant change in the left renal cyst were observed in the ultrasound followup performed three months later.Because the left mass was diagnosed as a large simple renal cyst, an operation with flank position was planned. At exploration, the large cystic mass in the lower pole of the left kidney presented with a regular but thick wall. Aspiration of the cystic fluid was down. It was like clear water. When the cystic wall was opened, sloughed and wrinkled daughter cysts were seen. After taking precaution to prevent hydatid seeding, by means of sponges saturated with povidone iodine, the daughter cysts were extracted. The internal surface of the cyst wall was washed with povidone iodine, and cystectomy was performed, leaving a portion of the cyst wall at the renal side.Followup at 6 months and 1 year with ultrasonography revealed no evidence of tumor recurrence or hydatid disease in the perinephric space from spillage of the hydatid fluid.Schwannomas (neurilemmomas) are neurogenic benign tumors arising from schwann cells of peripheral nerve sheaths. While schwannomas commonly appear in head, neck, and extremities, they are quite rare in the retroperitoneal region, so that just 0.7% of benign and 1.7% of malignant types are observed in this region . SchwannImmunohistochemical (I.H.C) staining is typically positive for S-100 , vimentiPreoperative diagnosis is often difficult. In facing retroperitoneal tumors, schwannomas should be in mind. Schwannoma manifests as a well-defined homogenous mass in CT-scan, and following intravenous contrast injection its fibrous capsule has rim enhancement .Nowadays mass needle biopsy has been reconsidered and paid more attention particularly in young patients and those who are potential candidates of different therapeutic approaches from observation to surgical excision.Core needle biopsy is simple and safe and has the accuracy rate of 98% in differentiating soft-tissue benign tumors from malignant ones .Management options are ranged from radiologic followup in asymptomatic patients to surgical resection in symptomatic patients. Subtotal resection in order to minimize the risk of surgery and preserve adjacent vital tissues in benign types is recommended. benign schwannomas have good prognosis, and recurrence is very rare .Echinococcus spp. . Human infection is most common in sheep-herding areas, such as Australia, Argentina, Spain, Greece, and the Middle East. Dog is the principal definitive host, and sheep is the most common intermediate host. Human may become intermediate host through contact with a definitive host or ingestion of contaminated water or vegetables. Virtually all parts of the human anatomy have been reported to have hydatid cysts [Hydatid disease is a worldwide zoonosis caused by the larval stage of the cestode id cysts . The cysid cysts –12.There are no specific symptoms or signs that will reliably confirm the diagnosis of renal hydatid disease. The combination of clinical history, radiologic findings, and serological and urinary studies yields a reliable diagnosis in only 50% and a presumptive diagnosis in 71% of cases . Renal hThe cyst may rupture into the collecting system, and the patient experiences severe renal colic and passage of debris resembling grape skins in the urine (hydatiduria), which is pathognomonic and seen in 5–25% of all renal hydatidosis .Ameur and coworkers reported 34 cases with renal hydatid cysts. Clinical presentations were pain (63%), hematuria (31.4%), mass (26%), hydatiduria (11.4%), prolonged fever (23%), and hypertension (3%), respectively . Also, t Radiological studies have an important role in the diagnosis of renal hydatid disease. Intravenous pyelography (IVP) typically shows a thick-walled cystic mass, occasionally calcified.Ultrasonography usually shows a multiloculated or multicystic mass.On CT scan, several patterns of renal hydatidosis may be presented. The most specific pattern is a cystic mass with discrete and round daughter cysts and a well-defined enhancing membrane. The less specific is that of a thick-walled multiloculated cystic mass. The presence of daughter cysts is strongly suggestive of hydatid disease and differentiates the lesion from a simple renal cyst, renal abscess, infected cysts, and necrotic neoplasm. Nevertheless, radiologic findings are usually suggestive but inconclusive in diagnosis of renal hydatid cyst . ParenchRetroperitoneal mass needle biopsy is simple, safe, and accurate in differentiating soft-tissue benign tumors from malignant ones and has been paid more attention.Hydatid cystic masses may not always present with characteristic radiological findings, and extreme caution should be practiced by the surgeon and radiologist in order to prevent iatrogenic echinococcal dissemination.


The concomitant presence of three histopathologically different entities in the pituitary gland is a rare occurrence. Most publications identify at least two distinct pathologies, mainly, a pituitary adenoma coexisting with a second intrasellar lesion. We present a case of a 71-year-old female referred for evaluation and treatment of acromegaly. Questioning revealed she was experiencing facial palsy, visual disturbances, and syncopal spells for several weeks. When laboratory evaluation showed elevated somatomedin (IGF-I) levels and an oral glucose tolerance test failed to demonstrate any suppression of her growth hormone (GH) values, an MRI of the pituitary revealed a sellar mass. A presumptive diagnosis of pituitary adenoma was established. The patient underwent transsphenoidal resection of the sellar mass, which proved to be a large B-cell lymphoma (Stage I-E) associated with areas of adenoma and lymphocytic hypophysitis. Acromegaly is a relatively rare endocrine disease characterized by sustained hypersecretion of growth hormone (GH) from a pituitary adenoma in the majority of cases . Typicalμg/L with sensitive assays and IGF-I levels reduced to the age-gender-adjusted normal range is recommended for acromegaly to be excluded or to be considered nonactive. A mean integrated 24 h level of GH less than 2.5 μg/L also excludes the diagnosis [Currently, GH suppression after an oral glucose tolerance test (OGTT) to less than 1 iagnosis . Howeveriagnosis . But siniagnosis , 8. Howeiagnosis . The fact that most patients with acromegaly have a GH-secreting pituitary adenoma has emphasized the coexistence between the two and to some extent it has overlooked the possibility of another type of sellar mass as the etiology for acromegaly. Here we describe a patient with coexistent intrasellar adenoma, lymphocytic hypophysitis, and primary pituitary lymphoma (PPL) in association with clinical acromegaly. Hypophysitis is a chronic inflammatory process of the pituitary gland presumably of autoimmune etiology. Hypophysitis is classified according to pituitary anatomical site involvement, cause, or histopathological tissue diagnosis. Adenohypophysitis, infundibulohypophysitis, or panhypophysitis have been described depending if the clinical and radiological signs affect the anterior, posterior, the stalk, or both. The cause of hypophysitis has been associated to primary or secondary forms. Primary hypophysitis is the most common and it can manifest as an idiopathic occurrence or as part of a multiorgan disease (polyglandular autoimmune syndrome and IgG related systemic disease). Secondary hypophysitis includes drug-induced agents like interferon alpha administration or cases where hypophysitis is part of a multiorgan systemic involvement  or inflammation of the pituitary gland due to the presence of another sellar lesion . From the histopathological point of view two distinct lesions are found, lymphocytic and granulomatous hypophysitis, and four variants, mainly, xanthomatous, necrotizing, plasma cell rich, and IgG4-related hypophysitis. Lymphocytic hypophysitis is the most common, occurring uniquely in relation to pregnancy or the postpartum period in about 40% of the women , 11. PPLμg/dL , thyroid-stimulating hormone of 5.4 μU/mL , ACTH of 24 pg/mL, and a morning serum cortisol of 11.8 μg/dL . Signs or symptoms of posterior pituitary involvement were absent. Notable findings were raised somatomedin (IGF-I) levels and an oral glucose tolerance test that failed to demonstrate any suppression of her GH levels , total thyroxine of 6.8 H levels . RadiogrNeurological examination was compatible with a bilateral lesion of the optic nerve , further confirmed by visual field examination and evoked visual potentials. MRI of the pituitary revealed a 2.3 × 2.2 × 1.7 cm well-circumscribed mass extending into the suprasellar cistern and compressing the optic chiasm. There was also penetration into the right cavernous sinus with encasement of the right cavernous carotid artery. After gadolinium administration there was nonhomogenous enhancement of the tumor, with no intradural invasion noted. No other brain lesions were seen Figures . A presuThe patient underwent transsphenoidal resection of the pituitary mass  without any intraoperative or postsurgical complications. At light microscopy, the mass contained loosely cohesive cells with numerous mitotic figures. A group of cells suggestive of lymphocytic hypophysitis were also identified adjacent to the tumor . MoreoveAfter surgery, the patient received a 5040cGy course of intensity-modulated radiation delivered to the pituitary, sphenoid sinus, posterior nasal cavity, nasopharynx, and oropharynx over 6 weeks in 180-cGy fractions. Radiation therapy was complicated by oropharyngeal thrush which required interruption of treatment for one week. Since the lymphoma was very well localized (Stage I-E) and whole body 18F-FDG PET/CT and bone marrow biopsy excluded disseminated disease, no systemic chemotherapy was suggested . One yeaOur patient was found to have three histopathologically different entities coexisting in the sellar region; a non-functional adenoma, lymphocytic hypophysitis, and a primary pituitary lymphoma. A preoperative presumptive diagnosis of only a pituitary adenoma was made on the basis of the patient's presentation, imaging findings, and the extensive research data pointing, that is, the most common cause of a mass in the sella (up to 10%–15%) . Hence, Primary CNS lymphoma (PCNSL) is now thought to constitute 3% of all intracranial neoplasms , 15. HowThe origin of PPL still remains unknown. Some etiologic theories suggest that the tumor may arise from resident lymphoid tissue in the CNS, while others consider the possibility of normal lymphocytes migrating to the sellar region due to inflammatory processes and subsequently undergoing malignant transformation , 13. ThiBased on the obvious clinical features, the elevated serum IGF-I levels and nonsuppressible GH levels after OGTT, it is evident that our patient has acromegaly. But from where is the GH actually coming from? Our patient had no evidence of systemic diseases. Hence, a secondary GH-releasing tumor is unlikely. Since in our patient immunostains of the adenomatous component was negative for all anterior pituitary hormones, one plausible explanation is the role of the tumor itself in the basal production of GH-dependent factors, thus contributing to the neoplastic transformation of cells and the clinical manifestation of acromegaly. Immunoreactivity has established the production of GH and IGF-I by lymphoblasts and lymphocytes , 22. In The concomitant presence of a pituitary adenoma with other morphologically different lesions is an uncommon phenomenon . So in oAlthough the case we described here has definite clinical, radiological, and biochemical evidence to support the diagnosis of acromegaly, the source of GH is not completely understood. However, possible theories might be raised including the slow, continuous hypersecretion of GH from the adenoma or even from the lymphocytes of the lymphoma as previously explained. Future lines of research should be directed to the understanding of the pathological and immunological mechanisms that lead to a better characterization of such processes. We also propose that although acromegaly and pituitary adenoma are highly intertwined across the medical literature, careful exclusion of primary neoplasm and other inflammatory lesions of the pituitary gland should be warranted.