Despite being one of the most frequent soft-tissue sarcomas, well-differentiated liposarcoma has never been reported near the spine. The authors present the case of a 67-year-old man with progressive history of back pain. Physical examination revealed a mass located within the right paravertebral muscles. MR and CT imaging showed a heavily ossified central mass surrounded by a peripheral fatty component. No connection with the underlying bone was detected on imagery and during surgery. After surgical resection, histopathological examination revealed a tumor harboring combined features of well-differentiated liposarcoma and low-grade osteosarcoma. Tumor cells displayed overexpression of MDM2, CDK4, and P16 by immunohistochemistry and CGH revealed amplification of 12q13-15 as the only genetic imbalance. MDM2 FISH analysis was performed but was inconclusive. The pathological, immunohistochemical, and genetic features, the differential diagnoses, and the therapeutic management of this unusual tumor are discussed. No complementary treatment was performed initially. Following first treatment, two recurrences occurred 6 and 9 years later, both displaying histological features similar to the first occurrence. Radiotherapy was started after the second recurrence. Follow-up shows no evidence of disease 11 years after initial diagnosis. This case was unusual due to the paravertebral location of the tumor and its divergent differentiation. Liposarcoma (LPS) is the most common type of adult soft-tissue sarcoma. The term LPS encompasses a heterogeneous group of neoplasms with different clinicopathological presentation, behavior, and genomic alterations although all share lipogenic differentiation. The WHO classification of soft-tissue tumors recognizes well-differentiated liposarcoma (WDL), dedifferentiated liposarcoma (DL), myxoid/round cell liposarcoma (M/RCL), and pleomorphic liposarcoma (PL). The most frequent sites of involvement of LPS are the retroperitoneum, the deep soft tissues of extremities, and the spermatic cord. Paravertebral involvement by LPS is rare  and M/RCWe report an interesting occurrence of a WDL/LGO in a 67-year-old man, unusual by its divergent differentiation and its location within the muscles of the paravertebral location.A 67-year-old man with a year-long history of chronic back pain presented with a large lump located at the thoracolumbar junction. CT scan showed an 8 × 4 × 4 cm tumorous mass lying in the paravertebral left muscles and composed of a central hyperdense core and a lower density rim . MR imag MDM2 was attempted several times on all occurrences, but in all case hybridization was not conclusive, probably owing to decalcification. Comparative Genomic Hybridization (CGH) revealed amplification of chromosome 12q13-15 as the only significant genomic imbalance, a region where MDM2 and CDK4 genes are located. Since there were no radiological nor macroscopic proofs of a connection with the underlying bone, the association of lipogenic and osteogenic differentiation favored the diagnosis of WDL/LGO.On gross examination, the mass was composed of a central ossified core surrounded by a yellowish adipose tissue. Microscopically, distinct histological patterns were present : a main No complementary treatment was initially performed. The patient experienced two local recurrences 6 years and 9 years after initial diagnosis; both were surgically resected and local radiotherapy was initiated following the first recurrence. CT and MR imaging of these two recurrences are shown in Figures 1(c)We have reported another case of WDL/LGO which was unusual owing to its location in the paraspinal soft tissues.Histologically, the differential diagnoses of a heavily ossified soft-tissue mass in this particular location may encompass different neoplasms: myositis ossificans, heterotopic ossification (soft-tissue osteomas), ossifying fibromyxoid tumor, or even extraskeletal osteosarcoma. Myositis ossificans typically shows a zonal phenomenon with a fasciitis-like cellular center surrounded by an outer layer of mature lamellar bone. Osteomas are rare well circumscribed lesions composed of mature trabeculae of bone with fatty or hematopoietic marrow. Ossifying fibromyxoid tumor presents as pseudo-encapsulated mass, lobulated by fibrous septa that may display mature bone formation in 80% of cases. Neoplastic cells are arranged in cordonal architecture within a fibromyxoid or hyaline matrix and usually display S100 expression. Albeit very rare, extraskeletal LGO has been reported near the spine. Extraskeletal LGO displays histologic features resembling those of a parosteal osteosarcoma but without adipose component nor connection with the underlying bone . In our  MDM2 argued for its neoplastic nature: both the osteogenic and lipogenic components of these tumors displayed amplification of MDM2. Low-grade bone formation has also been described in peculiar forms of ALN/WDL which arbor a “meningothelial-like” whorling architecture [Soft-tissue LPS are far more common than extraskeletal LGO. In LPS, low-grade mature bone formation is possible but is a rare phenomenon. Bone formation has been described with or without association with a high-grade dedifferentiated component. Until recently, mature bone formation in LPS was considered metaplastic, but the development of molecular and immunohistochemical techniques to assess the amplification ofitecture , 11: mositecture  in a ser MDM2 amplification, especially on surgical margin in doubtful cases. For tumors developing in the limbs, resection followed by radiotherapy is recommended to avoid recurrences [The prognostic significance of a LGO component in WDL may not be pejorative following the example of heterologous differentiation in DL . In the urrences . In our Before MDM2 testing was routinely available, other cases of WDL/LGO may have been overlooked: a case of an axillary extraskeletal LGO with a lipomatous mass closely associated to it has been reported in 1991. In this particular case, the fatty component interpreted as lipoma might have represented a low-grade liposarcoma which was underrecognized , as MDM2More recently, a parosteal osteoliposarcoma was reported , also deWe have reported a paravertebral WDL/LGO in which both components can be histologically mistaken for a benign process, particularly the fat-forming component. Immunohistochemical and molecular studies may be required for accurate diagnosis. The long-term prognosis seems good depending on adequate local treatment to avoid recurrences.


Adenomatoid tumor is an uncommon benign mesothelial neoplasm, usually localized in the epididymis. It is the most common paratesticular tumor of middle-aged patients . However, these tumors in pediatric and pubertal patients are extremely rare. Due to their rarity, we present a case of adenomatoid tumor of the tail of the epididymis in a 16-year-old patient. After systematic research of the current literature, we did not find another case report of epididymal adenomatoid tumor in a male patient aged 16 years old or less. This notice and our concern, as well, about the patient's surveillance protocol during the postoperative period were the motive for this case study. Adenomatoid tumor of the male genital tract is a nonhormone dependent tumor of mesothelial origin that is usually localized in the epididymis . ParatesAccording to its histological characteristics, adenomatoid tumor can be divided into 3 subtypes: tubular, angiomatoid, and plexiform. Amin and Parwani discriminate 4 kinds of adenomatoid tumors: adenoid or tubular glandular, angiomatoid, solid, and cystic or any transitional form of them . Major mAfter systematic research of the current literature, we did not find another published case report of epididymal adenomatoid tumor in a patient 16 years old or less. This notice and our concern, as well, about the appropriate patient's surveillance protocol during the postoperative period were the motive for this case study.A 16-year-old male with free medical history presented as an outpatient requesting for consultation regarding a small lump that he palpated two months ago in his right hemiscrotum during self-examination. He did not report any trauma or inflammation of the area neither at the referred period nor in the past. Except for mild discomfort in the hemiscrotum during exercise that subsided after its discontinuation, no other symptoms were reported. Importantly, the size of the lump did not change significantly during this two-month period.Physical examination revealed a small, round, hazelnut-sized, painless mass in the right hemiscrotum, localized in the tail of the epididymis. No other pathological signs were detected at the rest of the scrotum, testicles, or groin.U/S examination of the scrotum documented the presence of a solid hypoperfused, hyperechoic, well-demarcated, without invasive behavior mass, localized at the tail of the epididymis, 1.3 × 1.1 cm in size, and including 2 small hypoechoic lesions inside the mass arrows, .The typical preoperative laboratory examination (blood routine and coagulation profile) and the values of the specific tumor markers AFP, LDH, CEA, and b-HCG were normal. Surgical treatment was decided and a right scrotal exploration was performed. Macroscopic examination revealed a yellowish uncapsulated mass with maximum diameter of 13 mm located next to the tail of the epididymis .After meticulous dissection, the mass was totally dissociated from the epididymis and was excised en-block without any damage to adjacent structures.Histological examination of the mass revealed the presence of cuboidal epithelial cells in tubular clusters into a fibrous stroma . ImmunohPostoperative course was uneventful. After a 24-month follow-up period, the patient remains asymptomatic without signs of recurrence.Adenomatoid tumors are the most common tumors of middle-aged patients; average age of clinical presentation is 36 years , 2, 6–12Adenomatoid tumors are usually incidental findings. Sometimes patients report mild discomfort or pain, mainly during exercise. Rarely, adenomatoid tumors can present as posttraumatic acute scrotum. Usually, there is no correlation between the tumor and previous scrotal inflammation or trauma. Differential diagnosis includes all possible testicular and paratesticular masses as well as other scrotal abnormalities such as lipoma, sarcoma, metastatic tumor, granuloma, and hematoma of the spermatic cord .During the diagnostic approach, pediatric surgeon should emphasize on two major issues: the possibility of existence of subject malignancy and the feasibility of total and safe excision of the tumor. In older patients, when intraparenchymal involvement is noticed, differential diagnosis should necessarily include seminoma . UltrasoHowever, MRI should be performed when ultrasonographic findings are unclear concerning tumor's boundaries and its local invasive behavior or in those cases that the mass arises from the tunica albuginea. It should be noted that 14% of adenomatoid tumors arise from the tunica albuginea. In those cases, MRI can distinguish the separating margin of the mass from the testicular parenchyma . FNA is If there is a strong evidence of malignancy based on the preoperative findings, excision of an extratesticular tumor is conducted after inguinal approach. Otherwise, approach can be scrotal. Mass enucleation without any damage to the adjacent structures is the basis of the treatment. If dissection of the mass from the adjacent structures is difficult, intraoperative biopsy should be conducted, which—in case of an adenomatoid tumor—leaves no doubt about its benign nature. In case of a mass with invasive behavior or documented malignancy, the need of complete or partial epididymectomy should be evaluated .Microscopic features of adenomatoid tumor are characterized by three basic patterns: tubules, cords, and nests with cuboidal epithelium and amphophilic, eosinophilic, or vacuolated cytoplasm. Stroma is fibrous and occasionally hyalinized . In our Immunohistochemically, an adenomatoid tumor is positive for markers, such as CK (AE1/AE3) ΕMΑ, Cam5.2, CK 5/6, CK7, calretinin, vimentin, WT1, and HBME-1. Other tumor markers, such as AFP, LDH, CEA, and b-HCG, when measured, are negative, being substantial for the exclusion of malignancy , 16, 17.The mesothelial origin of adenomatoid tumor is confirmed by the identification of calretinin, which shows high sensitivity regarding identification of mesothelial cells. Calretinin is a calcium binding protein of the S-100 protein family that is expressed both in cytoplasm and in nucleus. Because of its expression in malignant tumors, absence of calretinin excludes the presence of malignancy . CalretiTotal tumor resection is considered to be curative as there are no reported cases of recurrence . Scope oFinally, we are concerned about the necessity of annual surveillance of the patient, which could consist of clinical examination, U/S examination of the upper and lower abdomen and the scrotum, chest X-ray, and specific tumor markers .


Myoepithelioma is a rare salivary gland neoplasm. They most commonly affect the major and minor salivary glands with the parotid gland being the most common, approximately 40%. Only 1% of all salivary gland neoplasms are myoepitheliomas. Myoepithelioma is usually a benign tumor arising from neoplastic myoepithelial or basket cells which are found between the basement membrane and the basal plasma membrane of acinar cells. They also contain multiple cellular elements. We present a case of a 73-year-old female with myoepithelioma of the parotid gland, an extremely rare neoplasm. There have been approximately 42 cases reported through 1985 and fewer than 100 cases through 1993. We will discuss the clinical presentation, pathophysiology, diagnosis, and treatment of such neoplasms. Of all benign major and minor salivary gland neoplasms, myoepitheliomas account for 2.2% and 5.7%, respectively. The parotid gland is affected in approximately 40% of cases. In total, myoepitheliomas only account for 1% of all salivary gland neoplasms. The overwhelming majority of myoepitheliomas are benign but malignant transformation can take place in recurrent cases and cases left untreated . We presA 73-year-old female with a past medical history of hypertension, peripheral arterial disease, asthma, gastroesophageal reflux disease, and diabetes mellitus presented to our otolaryngology clinic with the complaint of a “nodule” behind her left ear. She stated that the mass had been slowly increasing in size over the last 5 years. It was described as nonpainful and she had never experienced any discharge from the area. Her only other complaints were decreased hearing, xerostomia, and hoarseness; no dysphagia or weight loss was reported. Past surgical history was significant for angioplasty and stent placement in her legs. Social history was significant for a 20-pack-year tobacco history. No significant family history was reported. On physical examination pertinent findings consisted of a left posterior auricular mass approximately 2 centimeters (cm) that was fixed and nontender. All cranial nerves, most notably the facial nerve, were intact. No cervical adenopathy was palpated. Nasopharyngeal laryngoscopy demonstrated arytenoid edema consistent with reflux but the true vocal folds were mobile and no other lesions were visualized. Treatment plan at that time consisted of smoking cessation and a computed tomography (CT) scan with contrast of the neck.The CT scan revealed a multilobulated and cystic mass in the left parotid gland with an infiltrating appearance consistent with neoplasm. There was no pathologic cervical lymphadenopathy reported. The differential diagnosis at this time included benign mixed tumor, Warthin's tumor, epidermoid tumor, and adenoid cystic carcinoma. A fine needle aspiration was performed at this time and the results were nondiagnostic. The decision was made to proceed with a left parotidectomy.Left superficial parotidectomy with facial nerve monitoring was performed approximately one month after presentation. A 2 × 2 cm lesion was removed from the tail of the left parotid gland. The gross description was an encapsulated, lobulated mass with a brown-yellowish coloration. The specimen was sent for analysis by the pathology department. The tumor was composed of neoplastic myoepithelial cells arranged in a fascicular nested pattern separated by collagen stroma . There wThe patient recovered from the surgery without complications and her facial nerve was functioning well. The patient had no sign of recurrence at 10 months and is currently being seen regularly for routine monitoring.The description of a salivary neoplasm resembling a myoepithelioma was first attempted in 1943 . MyoepitMyoepitheliomas only account for approximately 1% of all salivary neoplasms . PrimariOf all benign major and minor salivary gland neoplasms, myoepitheliomas account for 2.2 and 5.7%, respectively. On gross inspection, they have a solid, tan or yellow-tan, glistening cut surface, similar to what was seen in our gross examination. Subtypes of myoepitheliomas are classified by cell morphology: spindle (interlacing fascicles with a stroma-like appearance), plasmacytoid/hyaline , epithelioid , and clear  . Of thosMost of these tumors occur in adults, with some cases reported in the pediatric population. Both genders are affected equally. The average age of a patient with myoepithelioma is 44 years, with a range of 9–85 years. The most common clinical presentation is a slow growing, painless mass, as seen in the clinical presentation of our patient . TypicalThe recurrence rate for myoepitheliomas has been reported to be 15–18%, with possible malignant transformation in long-standing tumors or recurring disease . HoweverAfter complete surgical excision, routine follow-up is needed in the treatment algorithm of myoepitheliomas secondary to the recurrence rate. Currently in our patient there has not been any sign of recurrence. In conclusion, although myoepithelioma of the parotid gland is the most common site for a myoepithelioma to appear they are still a rare salivary gland neoplasm. It is challenging to obtain an exact number of cases that have been reported in the literature. However, it has been elucidated that there were 42 cases reported through 1985 and fewer than 100 cases through 1993 . Additio


Sebaceous lymphadenomas are rare and account for less than 1% of primary salivary gland tumors. These rare tumors are mostly found in men older than 50 years. The clinicopathological features of these tumors are poorly understood and no definite causative factor has been reported for them till now. They are not often diagnosed prior to surgery, which could be due to their rarity and lack of enough preop radiological and cytological findings. Few case reports have been published in literature about their pathogenesis and accompanying malignant lesions. The cytological and imaging findings have been dealt with in some articles. Here we describe the histologic, cytologic, and radiologic findings of sebaceous lymphadenomas of parotid gland, all together, and discuss their differential diagnoses in various diagnostic methods. Sebaceous type cells are often found within normal parotid gland. Although they are found in a variety of lesions, primary neoplastic lesions with predominant sebaceous component, such as sebaceous adenoma and sebaceous lymphadenoma, are very rare and account for less than 1% of salivary gland tumors [A 39-year-old man was referred to ENT service with 4-month duration of left-sided, painless swelling in the face. Clinical examination revealed a semimobile, nontender, round mass in left parotid gland.Sonographic findings revealed a hypoechoic mass measuring about 20 × 18 mm in left parotid gland suggestive of tumoral lesion. Multiple lymph nodes with benign appearance were also identified in left side of the neck.Spiral CT scan of head and neck with contrast showed a hyperdense soft tissue lesion measuring 17 × 15 mm in posteromedial part of left parotid gland suggestive of lymph node .The patient was scheduled for sonography guided-fine needle aspiration (FNA) from the mass. Prepared slides showed cellular smears, composed of numerous lymphocytes with variable size. Some clusters of acini and ductal epithelial cells were also present. There were no oncocytes or fat cells in prepared smears. Therefore the diagnosis was made as benign process, suggestive of intrasalivary gland lymph node with reactive changes .M: 3.5∗3∗2 cm with attached normal parotid gland tissue. No necrosis or hemorrhage was seen (Excision of the mass was planned in a complete parotidectomy and the specimen was sent for pathology. Grossly there was a well-defined shiny light brown mass was seen .Microscopically, the lesion was completely encapsulated and showed ductal epithelial components with squamous and sebaceous differentiation. The lymphoid background was composed of multiple lymphoid follicles with germinal centers . There wThe last follow-up of the patient was after 6 months and showed no sign of recurrence. The accompanying enlarged neck lymph nodes were also vanished.Sebaceous lymphadenoma is a rare, benign tumor with nests and islands of bland epithelium composed of sebaceous and squamous elements, in a prominent lymphoid stroma . Over 90The pathogenesis of the lesion and its relation to viruses has been discussed previously by Seethala et al. , and no In imaging studies the contrast mediated CT scan was restricted by multiple metallic artefacts due to amalgam dental fillings. However it demonstrated a well-defined enhancing solid lesion in the deep portion of left parotid gland with Hounsfield unit 65. These imaging findings are not specific. Well-defined margin is characteristic in benign lesions but degree of contrast enhancement of tumor cannot distinct between benign and malignant . In contIn FNAC, the lesion is mostly diagnosed as intraparotid lymph node or Warthin tumor due to presence of numerous lymphocytes, bland looking ductal epithelial cells, and few oncocytes . The diaFNAC can also be helpful as a preop diagnostic method, because it establishes that the lesion is either benign or nonneoplastic, or at least low grade.In histology, by adequate sectioning, sebaceous component can be easily diagnosed. Lymphoid follicles with germinal centers help to differentiate this tumor from lymphoid neoplasms. Also the fact that this is a well circumscribed lesion helps us distinguish it from lymphoepithelial sialadenitis. Lack of mitosis or cellular atypia in the epithelial component distinguishes them from malignant counterparts.In summary, in order not to miss it, one should keep in mind the diagnosis of sebaceous lymphadenoma in list of differential diagnoses when facing a lymphoepithelial lesion.


Primary localized amyloidosis presenting as an isolated mediastinal mass is extremely rare, especially in the thymus. Sclerosing thymoma is also an extremely rare anterior mediastinal tumor, pathologically characterized by extensive sclerotic lesions with hyalinization and calcification. Only 14 cases of sclerosing thymoma and five cases of thymic amyloidosis have been reported to date.A 78-year-old Japanese woman was diagnosed as having sclerosing thymoma -like thymic amyloidoma. She was diagnosed as having either lung cancer or mediastinal tumor with pericardial dissemination, and received palliative treatment. Three years later, she was readmitted with a complaint of general malaise. Since minimal change nephrotic syndrome was suspected based on the disease onset and selectivity index of urinary protein, steroid pulse therapy was started. Subsequently, because a marked reduction in tumor size was observed during maintenance treatment with prednisolone, a thoracoscopic needle biopsy was performed for a definitive diagnosis. According to the pathological findings and clinical investigations, a final diagnosis of sclerosing thymoma -like thymic amyloidoma was made.This is a case report of sclerosing thymoma-like thymic amyloidoma. Both sclerosing thymoma and thymic amyloidoma are extremely rare diseases: only 14 cases of sclerosing thymoma and five cases of thymic amyloidosis have been reported to date. In either diagnosis, our case is the first case in which marked reduction in tumor size was observed with steroid therapy. All reported cases of sclerosing thymomas underwent surgical resection, but steroid therapy to sclerosing thymoma has not been reported. It is still unknown whether steroid therapy is effective or not. The hyalinized components of sclerosing thymoma possibly contain amyloid deposits. The marked reduction in tumor size with steroid therapy may result in amyloid deposits. The association between sclerosing thymoma and thymic amyloidoma remains uncertain. Sclerosing thymoma should be stained with Congo red. Primary localized amyloidosis presenting as an isolated mediastinal mass is extremely rare, especially in the thymus. Sclerosing thymoma is also an extremely rare anterior mediastinal tumor, pathologically characterized by extensive sclerotic lesions with hyalinization and calcification. Only 14 cases of sclerosing thymoma and five cases of thymic amyloidosis have been reported to date.Amyloidosis can be classified as a systemic disease (80 to 90%) or as a localized disease 10 to 20%) [0 to 20% Sclerosing thymoma is an anterior mediastinal tumor, pathologically characterized by extensive sclerotic lesions with hyalinization and calcification. It is an extremely rare thymoma subtype, first reported in 1994. The clinical characteristics and causes remain unclear, and it is not well known. Tumor cell nests are not evident in the small specimens from needle biopsies. Since sclerosing thymoma consists of extensive hyalinization of fibrous tissue, it is difficult to obtain a definitive diagnosis. When hyalinized fibrous tissue is collected from a mass biopsy specimen in the anterior mediastinum, sclerosing thymoma and amyloidoma should be considered possibilities in the differential diagnosis. Special attention must be given to tumors found in the thymus gland that are composed primarily of fibrous tissue by, for example, preparing serial sections of the entire tumor, to ensure that minute thymomas are not overlooked. This case report describes a patient with sclerosing thymoma-like thymic amyloidoma that showed marked reduction in tumor size when steroids were administered for paraneoplastic minimal change nephrotic syndrome (MCNS).A 78-year-old Japanese woman presented with a chief complaint of general malaise. Her past medical history included hypertension, dyslipidemia, and rectal prolapse. She was on medication to treat hypertension and dyslipidemia, and their control was good. She did not have any history of autoimmune diseases, multiple myeloma, or dialysis treatment. She had never smoked tobacco. Her family history showed that her eldest son had colon cancer. Nobody in her family had a history of amyloidosis.In 2013, she first presented with dyspnea. Chest computed tomography (CT) showed a mass in her anterior mediastinum and cardiac tamponade. Following the removal of approximately 1200 ml of pericardial effusion by pericardial drainage, pericardial adhesion therapy was conducted. Class III  cells were identified from the pericardial effusion, leading to a diagnosis of either lung cancer or mediastinal tumor with pericardial dissemination. She received 1.0 mg of dexamethasone alone for palliative treatment because resection was not possible due to her age. While the size of the tumor increased gradually, she continued to receive out-patient care because she did not have any subjective symptoms. In June 2016, she was readmitted with a complaint of general malaise.2) 98% (room air), and body temperature (BT) 37.1 °C. Her heart sounds were normal with no murmurs, decreased breath sounds in both lung fields, and no rales. Her abdomen was flat and soft, with no abdominal tenderness and normal bowel sounds. Clear pitting edema was observed in both forearms and legs.On admission, her height was 147.5 cm and weight 44.0 kg. She was lucid, with heart rate (HR) 88/minute, blood pressure (BP) 134/94 mmHg, peripheral oxygen saturation  was started. Edema of her legs reduced gradually and urinary protein excretion decreased. Subsequently, since a marked reduction in tumor size was observed and her general condition improved during maintenance treatment with 30 mg prednisolone, a thoracoscopic needle biopsy was performed in September 2016 for a definitive diagnosis. The pathological findings did not show malignancy, and prednisolone was tapered and maintained at 10 mg. Enlargement of the tumor and relapse of nephrotic syndrome has not been observed, and it remains under observation  was negative -negative, and calretinin-negative. The surrounding small lymphocytes were terminal deoxynucleotidyl transferase (TdT)-positive. Therefore, the expressed epithelial cells were considered to be thymus-derived cells. The expressed epithelial cells showed weak atypia, and there was no atypia of lymphocytes. Epstein–Barr virus ive Fig. . The hyaive Fig. . HoweverBased on these findings, a final diagnosis of sclerosing thymoma -like thymic amyloidoma was made.This case report presented a patient with sclerosing thymoma-like thymic amyloidoma that showed marked reduction in tumor size when steroids were administered for paraneoplastic nephrotic syndrome. Our patient was receiving palliative treatment because she could not undergo resection due to her age and pericardial dissemination.The most common types of amyloidosis are light chain amyloidosis (AL amyloidosis) and AA amyloidosis. AL amyloidosis is associated with an underlying monoclonal plasma cell disorder. AA amyloidosis is associated with chronic inflammatory conditions, such as autoimmune diseases. In our case, our patient did not have any history of autoimmune diseases, multiple myeloma, or dialysis treatment. Nobody in her family had a history of amyloidosis. Immunohistochemical studies were negative both for kappa and lambda light chains and for AA amyloidosis. M protein was negative, and there was no evidence of systemic amyloidosis in clinical investigations.Amyloidomas in the mediastinum are extremely rare, especially in the thymus. Only five cases of thymic amyloidosis have been reported to date –6 Table. The aveSclerosing thymoma is a rare thymoma subtype, which was first reported in 1994 by Kuo ; 14 caseWhile thymomas are the most common anterior mediastinal tumors, they are relatively rare, with an incidence of 1.5 cases per million people , 13. TheThere are many reported cases of nephrotic syndrome as a secondary complication of malignant tumors and connective tissue disease; however, nephrotic syndrome as a complication in patients with thymoma is rare. In nephrotic syndrome associated with thymoma, many patients develop: (1) membranous nephropathy when the onset of nephrotic syndrome occurs concurrently with the thymoma; and (2) MCNS when the onset occurs after thymus resection . In the This is a case report of a patient with sclerosing thymoma-like thymic amyloidoma that showed marked reduction in tumor size when steroids were administered for paraneoplastic nephrotic syndrome. Our patient was receiving palliative treatment because resection was not possible due to her age. When hyalinized fibrous tissue is collected from a biopsy of an anterior mediastinal mass, sclerosing thymoma and amyloidoma should be considered possibilities in the differential diagnosis. Special attention must be given to tumors found in the thymus gland that are composed primarily of fibrous tissue by, for example, preparing serial sections of the entire tumor to ensure that minute thymomas are not overlooked. All reported cases of sclerosing thymomas underwent surgical resection, but steroid therapy to sclerosing thymoma has not been reported. It is still unknown whether steroid therapy is effective or not. The reports of these 14 cases of sclerosing thymomas did not indicate whether the hyalinized components were stained with Congo red. The hyalinized components of sclerosing thymoma possibly contain amyloid deposits. The marked reduction in tumor size with steroid therapy may result in amyloid deposits. The association between sclerosing thymoma and thymic amyloidoma remains uncertain. Sclerosing thymoma should be stained with Congo red. Further investigations are needed.


Background: Pancreatic neuroendocrine tumors (PNETs) are rare, and metastases when present are most commonly found in the liver or the peripancreatic lymph nodes. In this study, we present a patient who developed a metastatic PNET in the liver in the setting of multiple concomitant autoimmune disorders, including pernicious anemia and atrophic gastritis with hypergastrinemia.Case presentation: The patient is a 70-year-old woman with a history of Hashimoto's thyroiditis, thymoma, gastric carcinoid tumors, and autoimmune atrophic gastritis with pernicious anemia. She was found to have a 2 cm mass in the pancreaticoduodenal groove originating from the pancreas. A preoperative endoscopic ultrasound with fine-needle aspiration showed a well-differentiated PNET. During surgery, she was found to have multiple subcentimeter liver lesions, which on frozen section were shown to be a metastatic neuroendocrine tumor. After surgical resection, final pathology revealed a PNET with metastases to the liver. The metastatic lesions stained positive for gastrin.Conclusion: We were only able to find one other example in the literature of a PNET occurring in association with pernicious anemia. Our patient developed a metastatic PNET in the setting of multiple autoimmune disorders, including pernicious anemia. She had a history of several other concomitant and rare autoimmune disorders, including autoimmune atrophic gastritis and pernicious anemia. It is known that gastric carcinoid tumors are associated with pernicious anemia, likely stimulated by the associated hypergastrinemia.2 Our patient additionally had a history of gastric carcinoid tumors.Pancreatic neuroendocrine tumors (PNETs) are rare, accounting for <1% to 2% of all pancreatic masses.A young-appearing 70-year-old woman presented with a mass in the head of her pancreas in the setting of multiple known autoimmune diseases. She had a long-standing history of Hashimoto's thyroiditis and a prior thymoma resection. She also had a history of hypergastrinemia and atrophic gastritis, both of which were manifestations of pernicious anemia, as evidenced by anemia associated with a low serum B12 level of 117 pg/mL , an equivocal intrinsic factor blocking Ab assay, and an elevated gastric parietal cell antibody titer of 39.5 (positive >25). Because of this, and her lack of any history of peptic ulcer disease, the patient was not thought to have had hypergastrinemia because of the Zollinger–Ellison syndrome. Her pernicious anemia required monthly vitamin B12 injections. She also had several small, gastric fundal polyps followed yearly with esophagogastroduodenoscopy, and she had a history of endoscopic resection of these gastric carcinoid tumors arising in the polyps.Owing to some vague abdominal pain, she underwent a computed tomography scan that showed a 2 cm mass extending extrinsically from the pancreaticoduodenal groove . An endoExploration of the abdomen revealed a marble-sized mass just inferior to D1 in the superior head of the pancreas. The remainder of the pancreas was firm and the duodenum was not involved. Assessment of the liver demonstrated myriad pale blue-colored, subcentimeter, cystic-appearing lesions. A frozen section from these lesions in the liver showed metastatic neuroendocrine tumor. The lesion in the superior aspect of the pancreatic head was then dissected and found to be fairly deep in the gland. With distant disease and being wary of the proximity of the mass to the main pancreatic duct, a partial enucleation of the primary pancreatic mass was performed. She did well postoperatively and was discharged home on postoperative day 4. Her further recovery was uneventful.Final pathology confirmed metastatic neuroendocrine tumor in the liver and a primary PNET. The pancreatic tumor stained positive for synaptophysin and CD56, with a Ki67 proliferation index of 10%. Owing to the degree of necrosis of the primary pancreatic tumor, immunohistochemistry staining for gastrin was nonspecific. The liver biopsy showed features similar to the PNET and stained positive by immunohistochemistry for gastrin .4 Gastric carcinoid tumors are well-differentiated neuroendocrine tumors that arise from the enterochromaffin-like (ECL) cells of the digestive tract. Gastrin, released by antral G cells of the stomach, stimulates the ECL cells to secrete histamine, which acts on parietal cells to secrete hydrogen ions (acid) to lower the pH of the gastric secretions.5 Gastrin also directly stimulates acid secretion by parietal cells. Repeated overstimulation of gastric ECL cells by a hypergastrinemic state is the presumed pathogenesis of gastric carcinoid tumor formation.2 Pernicious anemia and the resulting achlorhydria and hypergastrinemia are one such cause for the development of G cell hyperplasia and elevated gastrin levels. At the time of presentation, our patient had a serum gastrin level of 3484 pg/mL (ref. <100 pg/mL), in retrospect perhaps because of both her achlorhydria and her metastatic PNET, and gastrin positive in the liver. Such patients may also have other associated endocrinopathies such as hypothyroidism, diabetes, and hypoadrenocoricoidism. Our patient had a history of thymoma and hypothyroidism from Hashimoto's thyroiditis.Carcinoid tumors are relatively rare, with reports describing an annual incidence of 2.0–4.7 out of 100,000 patients.6 It must be differentiated whether the hypergastrinemia is associated with a hypo- or achlorhydic state, such as pernicious anemia, atrophic gastritis, gastric cancer, postvagotomy, or antisecretory drug induced, as compared with a hyperchlorhydria, as in Helicobacter pylori infection, peptic ulcer disease with obstruction, antral G-cell hyperplasia, renal failure, short bowel syndrome, or Zollinger–Ellison syndrome.6 In addition, a gastrin provocation test with secretin has a high sensitivity and specificity for diagnosing patients with gastrinomas.6 Our patient had a high gastrin level and did not undergo a gastrin provocation test. She unfortunately likely has both a metastatic gastrinoma and achlorhydic hypergastrinemia because of pernicious anemia.There are many conditions that may cause a patient to have an increase in fasting gastrin level and it can be difficult to distinguish benign causes from the gastrinomas. Two-thirds of patients with gastrinomas had <10-fold increase in gastrin, which overlaps with more common conditions, and <10% have a very high (>100-fold increase) in gastrin.7 Although they tend to have a more favorable prognosis than exocrine pancreas cancer, hepatic metastases are observed in more than 50% of patients with PNETs. The 5-year overall survival for patients with PNETs is reported to be 92–100% for stage I and 57% for stage IV disease, depending on the staging system utilized.8 Without a strong index of suspicion, it would be easy for a physician to contribute the sole cause of a patient's hypergastrinemia to his or her underlying autoimmune and endocrinopathies. In doing so, they could miss a potentially more aggressive pancreatic exocrine cancer.PNETs arise from islet cells. Up to 50–70% of these tumors are nonfunctioning and do not secrete the hormones of their cells or origin. They have a low annual incidence, <1 out of 100,000, and comprise only about 1% to 2% of all pancreatic tumors.9 In this study, we present a patient with a similar history who was found to have a primary PNET with hepatic metastatic disease, in the setting of pernicious anemia, autoimmune atrophic gastritis, and gastric carcinoid tumors. She has been referred to medical oncology and is receiving treatment with small molecule therapy and depo-octreotide, now 3 months postresection.Upon our review of the literature, we found only a single case report of a woman with gastric carcinoid tumors in the setting of atrophic gastritis, who subsequently developed a pancreatic endocrine neoplasm.


Hydatid disease is an endemic disease especially in underdeveloped and developing countries affecting mostly the liver and lungs. The hydatid cysts located in other sites are mostly due to rupture of primary liver or splenic cysts. We present a primary small intestine hydatid cyst resected laparoscopically with the affected intestinal segment. As far as we know, this is the first report of a primary small intestine hydatid disease in the literature. Hydatid cysts may be found in almost any site of the body, either from primary inoculation or via secondary spread. The liver (66%) and lung 25%) are the most commonly affected sites, but other organs  can also be affected -2. Mesen5% are thPatients who have an extrahepatic hydatid cyst present mostly with abdominal pain and discomfort. Diagnosis can be challenging -4. We prA 31-year-old woman presented with a palpable mass on the left upper quadrant of the abdomen. She had a 14-month history of intermittent abdominal pain attacks, discomfort, and early satiety. The patient's medical history and family history were unrevealing. Abdominal examination revealed a palpable, partially mobile mass at the left hypochondriac region.Laboratory findings were unremarkable. Hydatid serology (IHA) was negative. Abdominal computed tomography (CT) scans revealed a heterogeneous mass originated from the stomach wall, measuring 4.1 x 4.3 cm Figures . No pathA preliminary diagnosis of a gastrointestinal stromal tumor (GIST) originating from the stomach was made. During diagnostic laparoscopy, the stomach was intact, but we found a 5 cm mass located at the jejunum, 30 cm distally to the Treitz ligament. The affected jejunum segment was resected 5 cm proximally and 5 cm distally with a stapling device. The mass was taken out of the abdomen inside a specimen retrieval bag. The two jejunum ends were anastomosed. The postoperative period was uneventful and the patient was externalized on day three.The pathology report revealed a 5.5 x 5 x 4 cm mass on the serosal aspect of the small intestine. The mass was rounded, had regular borders, and a white color. There was no association between the mucosa of the intestine and the mass. The inside cavity of the mass was filled with cheese-like necrotic tissue. There was no immune expression on CD117, DOG1, SMA, S100, and Ki-67 dying of the fibrotic capsule of the cystic lesion. Around the cystic lesion, periodic acid-Schiff (PAS) staining positive for cuticular membrane was detected. In the fibrotic tissue surrounding the cystic lesion, collagenation was detected with Masson's trichromatic dye. These histological and morphological findings supported a diagnosis of a hydatid cyst. After the pathological report of this rare clinical entity became absolute, informed consent was gained from the patient to publish these findings.A three-cycle albendazole treatment was ordered to the patient. In each cycle, the patient took 400 mg albendazole twice a day for 28 days and stopped for 14 days. No recurrence was noted during the 12-month follow-up period.Echinococcus granulosus is an endemic disease especially in regions like Eurasia and South American countries [Hydatid disease, caused by the ountries . The smaountries . The mecountries .Extrahepatic hydatid disease usually remains asymptomatic for years. Patients mostly present after the cyst becomes large enough to palpate or to cause non-specific symptoms as abdominal discomfort. The combination of clinical, laboratory, and radiological findings help for a preliminary diagnosis. Among all imaging modalities, ultrasonography is superior to both MRI and CT in visualization and evaluation of the morphology of liver cysts and hydatid disease . In the Commonly used serological methods in the diagnosis of hydatid disease are the enzyme-linked immunosorbent assay (ELISA), the indirect hemagglutination test (IHA), the latex agglutination test and immunoblots . NeverthThe gold standard treatment for hydatid disease is complete surgical excision though according to the site of origin, partial or subtotal cystectomy can be performed to avoid adjacent organ injuries . In our As far as our knowledge, this is the first case report of a primary small intestine hydatid cyst resected laparoscopically. Hydatid disease is a significant public health problem in underdeveloped and developing countries affecting mostly the liver and lungs. Primary small intestine hydatid disease is a very rare clinical entity, which should be kept in mind for patients with an intra-abdominal mass.


Background. Uterine fibroids are common benign tumors in women. Clinical manifestations are well known. Acute complications necessitating emergent surgical intervention are rare. Case. We report a case of a 53-year-old woman with a history of uterine fibroids presenting with acute-onset severe abdominal pain. Imaging indicated massive free fluid and a large partially solid uterine mass. Vitals were consistent with hypovolemic shock. Examination revealed a surgical abdomen. She underwent an emergent laparotomy and total hysterectomy. Surgery revealed 4.5 L of hemoperitoneum and a 15 cm degenerated uterine fibroid with active bleeding. Pathology was consistent with intraoperative findings. She required transfusion of numerous blood products perioperatively. Her postoperative course was uncomplicated. Conclusion. It is rare for a uterine fibroid to spontaneously rupture. However, prompt recognition of this severe complication is critical for expeditious, life-saving surgical management. Uterine fibroids are common benign tumors in women. Most women with fibroids are asymptomatic. However, when clinically apparent, women usually experience heavy or prolonged menstrual bleeding or pelvic pressure . Acute cA 53-year-old  perimenopausal African-American woman presented to the emergency department with worsening severe, acute-onset abdominal pain. The patient also reported dizziness and nausea. Her past medical history was significant for hypertension and known uterine fibroids. Vitals were notable for blood pressure 78/50 mm Hg and pulse rate 116 bpm. Examination revealed a 14-week-sized fibroid uterus, a distended abdomen, and diffuse abdominal tenderness with positive rebound and guarding. Hemoglobin concentration was initially 10.6 g/dL. A bedside abdominal sonogram was performed which revealed a large amount of free fluid, extending into Morison's pouch. Computed tomography, obtained by the emergency department prior to consultation, revealed an enlarged uterus with multiple fibroids as well as an 8.8 × 7.3 × 8.3 cm multicystic, partially solid, enhancing structure and moderate ascites . Repeat Given the acute nature of the patient's intra-abdominal bleeding and deteriorating hemodynamic status, she was taken emergently to the operating room for exploratory laparotomy. Prior to induction of anesthesia, the patient's blood pressure nadired at 60/53 and pulse was in the 140's. Vasopressors were started and additional transfusion products were given. Upon entry into the abdomen, approximately 4.5 liters of hemoperitoneum was observed. Intraoperative findings were significant for a ruptured degenerated fundal subserosal fibroid, which was actively bleeding posteriorly . The patAlthough uterine fibroids are very common, they infrequently cause acute complications. The spontaneous rupture of a degenerated fibroid is extremely rare with only around 10 cases reported in the last half decade , 4. SignIntraperitoneal hemorrhage from rupture of a fibroid is usually venous and secondary to an increase in abdominal pressure, which causes rupture of superficial veins. Less often, bleeding can be arterial and associated with hypertension. Trauma causing avulsion of a fibroid, torsion of a pedunculated fibroid, and pregnancy causing venous congestion resulting in vessel rupture are other possible etiologies for intra-abdominal bleeding from a fibroid , 4, 6–8.Although the pathogenesis and degeneration of fibroids are incompletely understood, steroid hormones are known to play a role . FibroidImaging modalities can aid in diagnosis of hemoperitoneum. However, computed tomography and ultrasound may not be able to delineate the origin of the bleeding . Timely


Acute abdomen is a common presentation in the emergency department and radiologic imaging plays a pivotal role in the evaluation of such patients. Multi-detector computed tomography (MDCT) is the most commonly utilized radiologic investigation in such patients as it can be performed fairly rapidly and has excellent accuracy for diagnosing various causes of an acute abdomen. Additionally, MDCT may also reveal clues towards an alternative diagnosis that was not even suspected on the basis of a history and physical examination. Consequently, it is indispensable for radiologists to be able to accurately and efficiently recognize imaging features of disorders that may present as an acute abdomen. While gastrointestinal, hepatobiliary and genitourinary causes account for most cases of acute abdomen, vascular etiologies may also be implicated in a small—but significant—proportion of cases. Therefore, in this pictorial review, we describe the typical MDCT imaging features of various vascular etiologies that may present as an acute abdomen. Non-traumatic acute abdominal pain is one of the most common presentations in the emergency department (ED) and an estimated 8 million patients visit the ED annually for evaluation of this complaint . ClinicaEmergency abdominal CT scans are often interpreted preliminarily by trainee radiologists, especially during holidays and after usual working hours . In emer1. Mesenteric ischemiaAcute mesenteric ischemia is a serious life-threatening condition. Early detection and treatment is of utmost importance as any delay in diagnosis can lead to bowel necrosis, perforation and septic shock . Acute m2. GI hemorrhageAcute GI bleeding is another abdominal emergency with a high morbidity and mortality rate. The most common cause of acute upper GI bleed is peptic ulcer disease, while the most common cause of acute lower GI bleed, especially in the Western world, is diverticular disease . In many3. Abdominal aortic dissectionAbdominal aortic dissection is another cause of acute abdomen which may have a catastrophic outcome if not diagnosed and managed promptly. Aortic dissection can be secondary to trauma, or it may be a consequence of various non-traumatic conditions, such as atherosclerotic disease, collagen vascular disease, or connective tissue disorders . CT dete4. Ruptured abdominal aortic aneurysmAnother life-threatening disorder is rupture of an abdominal aortic aneurysm, which typically presents with severe abdominal pain radiating to the back along with possible hypotension. Aneurysms greater than 5.5 cm pose significant risk for rupture and severe abdominal pain in a patient with a known aortic aneurysm should raise suspicion for aneurysmal rupture . The CT 5. Visceral artery aneurysms and pseudo-aneurysmsVisceral artery aneurysms are usually discovered incidentally and are often secondary to atherosclerosis and collagen vascular diseases . Pseudo-6. Tumor-related hemorrhageHyper-vascular tumors, such as hepatoma and angiomyolipoma, may rupture spontaneously, especially if they are exophytic . Such pa7. Inferior vena cava thrombosisInferior vena cava (IVC) thrombosis is usually secondary to extension of lower extremity deep venous thrombosis, although it may occur in patients with malignancy or other hypercoagulable states . In someThe most common manifestation of IVC thrombosis is bilateral leg edema—typically, in the absence of upper limb edema—and pain. The most feared complication is pulmonary embolism, which may cause ventilation-perfusion mismatch and/or pulmonary infarction . Such pa8. Renal infarctionRenal infarcts and thrombi can be secondary to numerous diseases, such as atherosclerosis, neoplasm, thromboembolism and vasculitis. Small renal infarcts may be asymptomatic, but, large infarcts usually present with acute flank pain and hematuria . CT is t9. Iatrogenic causesi. Aortic Stent Graft ThrombosisAortic stent grafts are used as part of endovascular procedures to repair ruptured abdominal aortic aneurysms or dissections and have less morbidity as compared to open surgical procedures . Thromboii. Aorto-enteric FistulaAorto-enteric fistula can occur as a result of aortic reconstructive surgery with or without graft placement. However, rare instances of sporadic aorto-enteric fistula formation secondary to aortitis have been reported . In caseiii. EndoleaksEndoleak is one of the most common complications occurring after endovascular aneurysm repair . Differeiv. Hemorrhage from Misplaced Stents and CathetersStents and catheters are often inserted in the GI, hepatobiliary and genitourinary tracts to bypass obstructions and provide a means for drainage. However, malposition of such stents and catheters can result in a number of complications including hemorrhage.Misplaced drainage catheters: Drainage catheters are often placed within the peritoneal cavity to drain ascites or collections. As such catheters are usually placed under radiologic guidance, injury to neighboring structures and resultant complications are infrequent. However, in cases where a collection closely abuts a bowel loop, the drainage catheter may be inadvertently placed into a bowel loop . This coMalpositioned percutaneous nephrostomy: Percutaneous nephrostomy is usually performed to divert the flow of urine outside the body in an obstructed renal system to avoid renal injury . This prMalpositioned biliary stent: Placement of a common bile duct (CBD) stent is most commonly performed to bypass a biliary stricture . AlthougAcute abdomen is one of the most frequently encountered scenarios in the emergency department. Abdominal CT is the modality of choice in such cases as it can be rapidly performed and has excellent diagnostic accuracy. Vascular etiologies of acute abdomen are infrequently encountered but are often overlooked by trainee radiologists, who are usually the reviewing radiologists during after hours and on weekends. This pictorial review provided a succinct review of the most commonly encountered vascular etiologies with prototype examples of each. Recognition of vascular etiologies of acute abdomen will help radiologists and clinicians to improve patient outcomes by avoiding misdiagnosis and instituting appropriate, timely management.


A cavenous hemangioma of the appendix (CHA) is rare. The clinical pathophysiology and adequate management of a CHA have not been sufficiently explained since reports on CHA are scarce.A 56-year-old woman presented with chronic right lower quadrant pain. Abdominal contrast-enhanced computed tomography revealed a thickened appendix (1.5 cm in diameter) and some focal calcifications in the appendiceal wall. No acute inflammatory signs were visible around the appendix. For diagnosis and treatment, we performed a laparoscopic surgery. Intraoperative findings included purple granular lesions that were spread diffusely along the surface of the appendix. Since these lesions were spread to the terminal ileum, laparoscopic ileocecal resection was performed. Upon macroscopic inspection, purple-colored, raspberry-like lesions were found diffusely on the serosal surface of the appendix. No lesions were found on the mucosal surface. Hematoxylin and eosin staining indicated the presence of blood-filled sinus-like spaces largely in the subserosal layer. Immunohistochemistry analysis indicated that CD34-positive cells lined these spaces. Given these findings, we diagnosed the patient with a diffuse cavernous vascular malformation of the appendix.CHA is difficult to diagnose. A laparoscopic approach may be useful for both the diagnosis and treatment of the disease. Hemangiomas of the gastrointestinal tract are infrequently encountered. They are also referred to as vascular malformations since their pathology consists of a collection of malformed blood vessels . AlthougCavernous hemangioma of the appendix (CHA) is rare, and reports of CHA are limited and have been written in English, Russian, Japanese, German, and French –7. Thus,In this article, we report a case of diffuse CHA, which was resected using a laparoscopic ileocecal procedure.A 56-year-old woman presented to our hospital with right lower quadrant pain. She experienced pain once every few months beginning a few years previously. Her abdomen was soft, with no peritoneal irritation signs or palpable masses. An abdominal contrast-enhanced computed tomography (CT) revealed a thickened appendix (1.5 cm in diameter) and some focal calcifications in the appendiceal wall. Abnormal findings were not seen in the terminal ileum. There was no evidence of tumor and no acute inflammatory signs (Fig. Intraoperative findings included purple-colored, granular lesions that were raspberry-like in appearance and were spread diffusely along the surface of the appendix Fig. a. These Macroscopic inspection of the resected specimen revealed purple-colored, raspberry-like lesions, which were spread diffusely on the serosal surface of the appendix. No lesions were found on the mucosal surface Fig. a. HistolAs shown in this case, unexplained chronic right lower quadrant pain and appendiceal wall thickening with focal calcification, as evidenced by CT, may possibly be a case of CHA, which is otherwise difficult to diagnose preoperatively. Owing to limited available reports, clinical manifestations and findings of various diagnostic images of CHA are not well-known. Previously, CHA was reported to cause no symptoms. For this reason, it was only incidentally discovered during a surgery to treat other lesions , 5. AnotThis case also demonstrates that laparoscopic surgery can be a useful tool for both diagnosis and treatment. Diagnostic laparoscopy for suspected appendicitis has been shown to be useful for its diagnosis and treatment because it is usually a simple and safe procedure that is helpful in obtaining other diagnoses . CHA hasSimilarly, cavernous vascular malformation of the mesoappendix has been reported , 12, 13.CHA is a rare disease that is difficult to diagnose by history taking, physical examinations, and various diagnostic imaging modalities because of its rarity. However, unexplained chronic right lower quadrant pain and appendiceal wall thickening with focal calcification, as indicated by CT imaging, may provide a basis for suspecting a diagnosis of CHA. A laparoscopic approach may be useful for both the diagnosis and treatment of CHA.


Osseous dysplasia (OD) is the most common fibro-osseous lesion of the jaw affecting the periapical region. Early stages of OD can resemble periapical radiolucencies, thus mimicking the radiological aspects of an endodontic pathology. Such radiolucent lesions affecting previously decayed or treated teeth are even more complex to interpret.The aim of this paper is to report a case-series of representative clinical situations describing the radiological features and illustrating the diagnostic workup of patients with florid osseous dysplasia (FOD). Emphasis is given to the endodontic implications of such periapical bone disease and the complexity of accurate diagnosis in the context of endodontic retreatment. We then propose a practical radiological-based diagnostic algorithm to assist the clinician in the diagnostic of OD periapical lesions.Periapical lesions may be confused with bone diseases such as osseous dysplasia, especially in the radiolucent initial stage. Knowledge of clinical features associated with a careful reading of cone beam CT images, such as fine opacities within the hypodense periapical lesion, may help determine the right diagnostic. Osseous Dysplasia (OD) is the most common fibro-osseous lesion of the jaws affecting all parts of the maxilla and mandible, including the periapical region. OD lesions are classified as benign odontogenic neoplasms of the jaws with other fibro-osseous lesions in the 2005 World Health Organization classification, updated in 2017 .Different clinico-radiographic presentations are described, depending on whether the lesions are isolated or affect multiple anatomical areas and their specific location: focal, periapical, florid and a specific rare variant, the familial gigantiform cementoma , 5.Stage 1 – osteolytic stage: radiolucent lesions,Stage 2 – mixed stage: radiolucent and radiopaque lesions,Stage 3 – osteogenic stage: radiopaque lesions.Florid osseous dysplasia (FOD) is the most extensive form with bony lesions involving multiple quadrants of the jaws. The etiopathogenesis of FOD is unknown but could derive from the periodontal ligament, the medullar bone or both . Within As most FOD lesions are asymptomatic, diagnosis of such disease is based on incidental radiographic findings, usually during a routine dental exam, especially in middle-aged black female patients.Diagnosis of FOD is based on the typical radiographic appearance assessed with standard two-dimensional projection radiographs . Biopsy of such lesions is not recommended in typical presentations because of the risk of significant post-operative infection, probably secondary to the reduced vascular supply in these lesions .In atypical cases involving the periapical region, three-dimensional radiographic assessment via cone-beam CT (CBCT) may be mandatory, especially in the early stages of disease, to facilitate differential diagnosis with other radiolucent periapical pathoses. Indeed, in the early radiolucent stage, such FOD lesions can mimic apical periodontitis, often leading to unnecessary endodontic treatment of healthy teeth and secondary infection of the underlying hypovascular bone. Diagnosis becomes even more challenging when the periapical radiolucent FOD lesion is associated with an endodontically-treated tooth.The aim of this paper is to present a clinical case-series illustrating the radiological features of FOD involving the periapical region. Knowledge of such radiological features is essential for the differential diagnosis of apical periodontitis, especially on previously-treated teeth. Based on this illustrative case series and the authors’ experience of FOD, a practical radiological-based diagnostic algorithm is proposed to aid in the diagnosis of such perplexing radiographic presentations, a common clinical conundrum in routine endodontic practice.We report three representative clinical cases of incidental discovery of florid osseous dysplasia, illustrating the different radiographic stages of the disease, the associated pathological consequences and finally the complexity of accurate diagnosis of periapical FOD in previously endodontically-treated teeth.A 64 year-old African male patient, without any significant medical history, consulted the dental emergency department of the Bretonneau Hospital in Paris, with a mobile and painful upper left first molar resulting from a periodontal abscess. Aside from moderate to severe generalized alveolar bone loss, preoperative dental panoramic radiography revealed multiple periapical radiolucencies especially within the mandibular body Fig. . ConsideExtraction of the upper left first molar was performed under antibiotic prophylaxis (amoxicillin) and the patient was referred for a full periodontal treatment. At the one-week follow-up, the healing was uneventful.Upon further medical interview, the patient did not report any history of trauma or swelling and all the teeth were vital except the upper left first molar. Thus, endodontic treatment in such clinical context is certainly not justified.A 50 year-old African female patient, without any significant medical history, consulted the dental emergency department of the Bretonneau Hospital in Paris, with chronic pain and tenderness on the lower left second molar. Clinical and radiographic examinations  revealeHigh-resolution CBCT imaging of the lower left second molar also allowed to highlight the previous endodontic treatment failure of the lower left second molar, namely an untreated mesio-lingual canal. Treatment therefore consisted of an endodontic retreatment of the tooth, using mechanical Protaper® for root canal preparation followed by a vertical condensation obturation technique with heated gutta-percha. At the six-months follow-up, the patient was pain free and a ceramo-metallic crown was installed.Although the patient could not recall the history of the lower left second molar and more specifically the reasons for the initial endodontic treatment, it is plausible that the initial consultation was made when the radiographic aspects of the FOD (stage 1 - radiolucent) could mimic periapical pathology, thus potentially leading to an endodontic overtreatment, especially on a tooth that was probably previously decayed . This clinical case thus illustrates the difficulties of accurate diagnostic workup of periapical radiolucencies in previously treated and decayed teeth.A 72 year-old Cameroonian female patient, with a medical history of joint pain, gastric ulcer and hypertension, consulted the dental emergency department of the Bretonneau Hospital in Paris, with multiple tooth pain, more severe in the upper left maxillary region. After clinical and radiographic examinations, a diagnosis of symptomatic apical periodontitis on the upper left second molar was made and immediate root canal disinfection was undertaken. Orthopantomogram Fig.  and CBCTFollowing initial diagnostic workup, the endodontic treatment of the upper left second molar was performed using a conventional technique without need of any specific premedication. The healing was uneventful.In patients with advanced FOD lesions, diagnosis of such a condition is quite straightforward. Nevertheless, a doubt can still remain regarding teeth that present radiological aspects of OD but that also show significant tooth decay. Rigorous clinical exams including pulp sensitivity testing and periodontal probing as well as radiographic exams (2D and 3D imaging studies) are thus mandatory to allow precise diagnostic and suitable treatment of the periapical pathology. For instance, thickening of the Schneiderian membrane adjacent to periapical osteolytic image with micro-perforations of the sinus floor support the endodontic origin of the periapical radiolucency in the upper left second molar compared to the right side Fig. .Diagnostic workup of unexplained radiolucent lesions must follow a strict methodology to evoke or rule out a possible FOD lesion. This methodology is summarized in Fig. Lesions occurring in middle-aged woman, especially those of sub-Saharan origin,Unique or multiple radiolucent, mixed or radiopaque lesions in the tooth bearing area, especially if occurring on vital teeth,Absence of symptomatology (in most of the cases).First, clinical interview should focus on past history of dental pain, trauma, tooth decay or cracks. When faced with a previously endodontically-treated tooth, understanding the indication of such treatment is essential to determine if a possible misdiagnosis could have occurred. Indeed, spontaneous pulp necrosis in the absence of tooth decay, trauma or other local aggressive factors seems quite unlikely, especially in totally asymptomatic teeth. On the other hand, when faced with a periapical radiolucent lesion in an otherwise healthy tooth with a vital pulp, a non-endodontic origin of such lesion must be envisaged. Periapical osseous dysplasia should be considered in the following circumstances:If such arguments are present, a provisional diagnosis of osseous dysplasia can be provided. Management will consist in a close follow-up until these lesions develop the typical radiopaque appearance, confirming the diagnosis. To that extent, CBCT sections are of use in assessing the internal pattern of the lesion, with attention to minute microcalcifications, but also to rule out lesion expansion onto surrounding structures. In doubt, another origin must be evocated; typically odontogenic and non-odontogenic cysts or tumors of the jaws. A biopsy of such lesions is then required and treatment adapted to the underlying etiology.Osseous dysplasia (OD), a rare and benign fibro-osseous lesion of the jaws, can mimic a radiolucent endodontic periapical lesion in the early stage of its maturation. Whereas differentiating such conditions in a vital tooth can be relatively straightforward, the situation becomes more complex when faced with endodontically-treated teeth, especially in cases of presumed inadequate treatments. Correct differential diagnosis is thus of paramount importance for the clinician, in order to avoid unnecessary iatrogenic dental treatments.Florid osseous dysplasia (FOD) is the widespread form of osseous dysplasia; periapical OD is confined to the anterior mandible; focal OD occurs in a single sextant and familial gigantiform cementoma is an uncommon variant encountered preferentially in young patients that can lead to considerable bone expansion , 9.In FOD, the molar and premolar teeth are the most frequently involved. FOD is usually asymptomatic and thus an incidental finding during a routine dental radiographic examination. The rare clinical symptoms observed are pain, swelling and local drainage but these are only encountered in cases of secondary infection, when the calcified masses are exposed in the oral cavity.FOD follows an ethnic distribution, as shown in the systematic review of MacDonald-Jankowski in 2003: 59% of cases arise in African patients, 37% in Asians and 3% in Caucasians. In our patient series, collected within a French Parisian hospital, 92% of cases arose in African patients. Women are more affected than men and the disease occurring mostly around the fourth decade . SeveralBecause of its typical clinico-radiographic presentation, diagnosis of FOD is usually straightforward and does not require any additional investigations. For instance, the discovery of multiple periapical radiolucencies on vital teeth in different quadrants is a sufficient argument to evoke the diagnosis of Florid Osseous Dysplasia. There is no need for a biopsy in typical cases, especially considering the risk of secondary infection of these hypovascularized lesions. Nevertheless, when in doubt, a biopsy can be performed to rule out other fibro-osseous lesions or bone diseases .Differential diagnosis of early stage FOD Fig.  should iAs FOD is a benign usually self-limiting disease, treatment of such condition mostly consists of simple monitoring of the lesions, with an annual long-term clinical and radiographic follow-up , 14, 15.FOD is a condition of significant importance for the endodontist and general dental practitioner. Endodontic lesions and FOD may co-exist Figs.  and 3 anA classic clinical conundrum for the dental clinician is trying to accurately diagnose the radiolucent periapical lesion in an endodontically-treated tooth in a patient presenting with FOD. Several questions arise: Is the periapical lesion a FOD lesion, an apical periodontitis or a surinfection of a FOD lesion? Is the endodontic treatment a consequence of a misdiagnosed FOD lesion or an adequate treatment of a superinfected FOD lesion? Indeed, reports of lesions mimicking periapical endodontic lesions leading to inappropriate endodontic treatments are frequent in the literature .In this paper, we presented several clinical cases of patients presenting typical FOD lesions, in healthy teeth, endodontically-treated teeth or decayed teeth Figs. , 2 and 3Florid Osseous Dysplasia is a rare disease composed of slowly growing, quiescent fibro-osseous lesions presenting as radiolucent and/or radiopaque masses in the jaws and periapical region, which usually do not require any treatment except in case of complications.Whereas typical radiopaque lesions are highly suggestive of the condition, early stage radiolucent periapical lesions can easily be confused with apical periodontitis especially in endodontically-treated teeth. Diagnosis and management of such confusing presentations must thus follow a rigorous methodology to avoid unnecessary iatrogenic dental treatments and possible disease-related complications.


Histoplasma capsulatum var. capsulatum infection was made. Without signs or symptoms of active, systemic disease, no further treatment was recommended. The patient was provided risk factor counseling for symptoms or signs of active histoplasmosis and outpatient follow-up. Histologically, most masses will return as benign and mesenchymal in origin. However, soft-tissue masses may arise from uncommon etiologies and a broad differential is needed to ensure appropriate management and recommendations.Patients are commonly referred to general surgery clinics for evaluation of soft-tissue masses of the trunk and extremities. The primary goal of surgical referral is to confirm the presence of a mass, to assess the need for additional imaging and/or testing, and to gauge amenability to surgical biopsy, whether incisional or excisional. This is a case of a 67-year-old woman who was referred to surgery clinic for a small soft-tissue mass near her left elbow that had increased in size and pain over the past year. The mass had been present for several years. After MRI imaging revealed a nonspecific process, an excisional biopsy was performed. Following a careful review of the patient's history, risk factors, and histological results, a diagnosis of localized subcutaneous Patients are commonly referred to general surgery clinics for evaluation of soft-tissue masses of the trunk and extremities. These lesions can range from common, benign masses of multiple etiologies to rare, malignant tumors that can be life-threatening. The primary goal of surgical referral is to confirm the presence of a mass, to assess the need for additional imaging and/or testing, and to gauge amenability to surgical biopsy, whether incisional or excisional.Histologically, most masses will return as benign and mesenchymal in origin, with lipomas being the most common histologic type. However, soft-tissue masses may arise from uncommon etiologies and a broad differential is needed to ensure appropriate management and recommendations. We present a case of 67-year-old woman who presented to a tertiary care teaching hospital with a small soft-tissue mass. She had multiple extrinsic risk factors for a deep-tissue fungal infection and was ultimately diagnosed with localized subcutaneous histoplasmosis by history, physical examination, and histological results following excisional biopsy.A 67-year-old woman was referred by her primary care provider to surgery clinic for a small soft-tissue mass near her left elbow. The mass had been present for multiple years. Over the past few months it had become increasingly painful, and it had increased in size over the past year. She said that she had experienced no overlying skin changes, drainage, or known trauma to the extremity. She had no other known lesions, masses, or adenopathy.The initial physical examination demonstrated a mobile, 2.5 cm × 2 cm soft-tissue mass over the dorsal aspect of the left forearm, just distal to the olecranon process. The mass was not tender to palpation, and there were no overlying skin changes or regional adenopathy. Results of an examination of her head, neck, chest, abdomen, and other extremities were normal. A magnetic resonance imaging (MRI) scan of the patient's elbow was obtained, which revealed a nonspecific, poorly defined infiltrative process within the subcutaneous fat, with a broad differential diagnosis including lipoma, sarcoma, nodular fasciitis, or benign versus malignant fibrous histiocytoma.µm to 4 µm in diameter with single, narrow-based buds was consistent with Histoplasma capsulatum (H capsulatum)   1]. In . In µm tIn addition to the patient's residence along a Mississippi River tributary, further epidemiologic history was obtained revealing multiple high-risk fungal exposures, including a history of rose gardening (too remote for sporotrichosis), frequent travels to mountainous Mexico , and living in Southwestern Wisconsin (outside Wisconsin's hyperendemic area of blastomycosis). However, additional inquiry into her social history was notable for the long duration of bat colony in the attic of her home that had caused guano and urine contamination of the underlying living space. Histoplasma capsulatum var. capsulatum (H capsulatum) infection was made. Without signs or symptoms of active, systemic disease, no further treatment was recommended. The patient was provided risk factor counseling for symptoms or signs of active histoplasmosis and monitored in the Infectious Disease clinic, and remained healthy over the subsequent 18 months.Following a careful review of the patient's history, risk factors, and histological results, a diagnosis of subcutaneousThe clinical differential diagnosis for this patient's soft-tissue lesion included benign and malignant soft-tissue tumors, lymphoma, noninfectious inflammatory changes, and infection. Magnetic resonance imaging, although helpful in defining the anatomic location of the mass and excluding vital structure involvement, was unable to further refine the differential diagnosis, and excisional biopsy was completed. The patient's histologic studies and epidemiologic history, including prolonged exposure to bat guano from the bat colony in her attic and her geographic locale within the Mississippi River Valley system, supported the diagnosis of histoplasmosis. Given that the patient's chest radiographic, antibody, and urinary antigen studies were negative, this was an unusual case and likely represented a localized, subcutaneous histoplasmosis infection due to traumatic inoculation. This rare occurrence has been reported in the past .Fungal infections can cause a wide range of diseases in humans. In the United States, deep-tissue mycoses are common but usually asymptomatic, and recent studies suggest an increased incidence of these infections in the Midwest, especially in elderly populations . As ubiqEndemic mycoses, such as histoplasmosis, blastomycosis, and coccidioidomycosis, are known to affect both healthy and immunocompromised individuals and tend to arise in specific geographic locations. Usually developing after a primary pulmonary infection, these fungi have the capacity to involve both the skin and deep organs. Thus, the endemic fungi can range from localized skin and soft-tissue infections to disseminated disease involving multiple organ systems. For disseminated disease, the point of entry is typically through the respiratory tract, from which the infection can spread to other sites . In rare H capsulatum is the lung. Localized cutaneous and subcutaneous infections with H capsulatum are rare, with only a handful of cases described, the majority of which were in immunosuppressed patient groups [When histoplasmosis first came to medical attention in the early 1900s, it was characterized as a rare but severe systemic infection that was inevitably fatal in most patient groups . More ret groups –10. H capsulatum is worldwide, it is hyperendemic to eastern North America, notably the mid-to-lower Ohio and Mississippi River valleys. Bird and bat excrement enhance the growth of the organism in soil by accelerating sporulation. As in our case, environmental sites are typically visibly contaminated by heavy accumulations of bat or bird guano.Infection may be due to direct inoculation or reactivation of preexisting, quiescent subcutaneous disease in patients with previous subacute pulmonary histoplasmosis. Although distribution of Histoplasma antibody and antigen detection of fungal cell elements in urine, serum, and cerebrospinal fluid by enzyme-linked immunoassay were proven to be important in the diagnosis, treatment monitoring, and prognosis of acute disseminated histoplasmosis, but they are not sensitive tests for a localized, subcutaneous infection. Fungal cultures can also be useful in patients in whom there is concern for either local or systemic disease [Most cases will be subclinical in nature, but a prompt and accurate diagnosis of any potential fungal infection is important to improving morbidity and mortality in some infected patients. A comprehensive history, including a detailed travel history, and a complete physical examination are needed at the time of initial evaluation. Chest radiographs and/or computed tomography scans are useful in finding the characteristic features of pulmonary disease. Isolation of the organism with histopathology and/or culture is the gold standard to confirm diagnosis and speciation, although this can be time consuming and insensitive. Various laboratory tests have been developed that may provide early clues to the diagnosis. disease . In case


Primary hepatic malignancies are less common than metastatic diseases, but a recognition of these lesions is important for diagnosis and treatment planning. Magnetic resonance imaging (MRI) provides the most imaging information to diagnose lesions noninvasively and to narrow differential diagnoses. This paper reviews the imaging findings of chronic liver disease and primary hepatic malignancies, including hepatocellular carcinoma (HCC), intrahepatic cholangiocarcinoma (CCA), epithelioid hemangioendothelioma, hepatic angiosarcoma, and primary hepatic lymphoma. Clinical and MRI features are reviewed to improve the readers’ recognition of these tumors, allowing for a narrower differential diagnosis when liver masses are encountered on abdominal imaging. With the increased use of medical imaging, liver lesions are found in both symptomatic and asymptomatic patients. Primary hepatic lesions are less common than metastatic diseases; however, their recognition is important for diagnosis and treatment planning. While ultrasound and computed tomography (CT) scans can identify liver lesions, magnetic resonance imaging (MRI) provides the most imaging information to diagnose lesions noninvasively and to narrow the differential diagnosis.Primary hepatic malignancy is the second leading cause of death from cancer worldwide and is the fifth most common type of cancer in men and the ninth in women . Within MRI TechniqueA single MRI protocol, including magnetic resonance cholangiopancreatography (MRCP), can adequately evaluate pathology involving the liver, the biliary tree, and other solid organs of the abdomen. The routine abdominal MRI protocol should include precontrast axial and coronal T1-weighted (T1W) three-dimensional (3D) gradient echo (GRE) images with fat saturation; axial and coronal T2-weighted (T2W) single-shot fast spin echo (ssT2) images without fat saturation; axial ssT2 images with fat saturation; axial dual echo gradient echo images (in- and opposed-phases); axial steady state free precession images; and heavily T2-weighted fast spin echo MRCP , intrahepatic , or posthepatic  level. In the setting of chronic liver disease, portal hypertension is most commonly due to the underlying cirrhotic change causing increased resistance to portal venous flow . ImagingHepatocellular CarcinomaThe most common malignant liver lesion in the setting of a chronic liver disease is hepatocellular carcinoma (HCC); however, these patients are also at increased risk for cholangiocarcinoma (CCA) and combined HCC–CCA. The hepatocarcinogenesis sequence accounts for the development of HCC. As the hepatocytes regenerate to replace cells lost to necrosis and apoptosis, deoxyribonucleic acid (DNA) mutations cause nodules to become dysplastic and eventually malignant. As these nodules undergo malignant transformation, tumoral neovascularity transforms the nodule blood supply from primarily portal venous supply to a predominantly hepatic arterial supply, and these arterial vessels are often abnormal ,9. This Unlike many malignancies, HCC can be diagnosed by its imaging features without the need for histologic confirmation if an appropriate imaging technique is utilized. The administration of intravenous contrast is critical to diagnosis, and multiple phases of contrast timing are required for the complete characterization of the liver mass enhancement pattern. Regenerative nodules tend to have a T1 signal intensity similar to or brighter than the adjacent parenchyma and the T2 signal same as the liver. The nodules are usually not well identified after contrast because they enhance the same as the surrounding liver unless there are surrounding fibrotic bands with delayed enhancement Figure .Dysplastic nodules are considered a premalignant lesion and are differentiated by increased enhancement during the arterial phase, but there are no T2 signal changes, washout, or pseudocapsules on later phases Figure . A shortThree enhancement features are used to diagnosis HCC: arterial hyperenhancement, portal venous and/or delayed washout, and late enhancing rim or pseudocapsule Figure . The mosOther imaging features can help identify HCC but are not part of the imaging definition. Lesions tend to have mildly increased (bright) T2 signal intensity but this signal change can be difficult to identify in cases with diffuse hepatic parenchymal heterogeneity. Some lesions contain areas of intracellular lipid, easily confirmed on the dual gradient echo sequence Figure .On diffusion imaging, HCC tends to have a bright signal in high b value series with a corresponding dark signal on apparent diffusion coefficient maps, which is consistent with restricted diffusion. These sequences can be prone to artifacts from ascites or patient motion and have reduced yield in patients with a significant iron deposition in the liver. Current AASLD guidelines do not take into account the role of hepatobiliary contrast agents that have both extracellular properties and uptake and excretion by hepatocytes, allowing for delayed hepatobiliary phase imaging ,18.In addition to identifying and characterizing the lesion as HCC, the hepatic vasculature should be carefully inspected for thrombus. Microvascular invasion is a finding of advanced HCC in pathologic specimens that are not readily apparent in current imaging studies; however, a progression to macrovascular invasion with malignant thrombus can be identified. The tumor can invade both the portal and hepatic vein branches in the region of the tumor and grow along the vessels towards the liver hilum or the inferior vena cava (IVC). This tumor should not be confused for a bland thrombus in the setting of portal hypertension. A tumor thrombus is usually contiguous with the malignant lesion and has irregular arterial phase enhancement. A bland thrombus should never enhance, and the presence of any enhancing thrombus in a vessel should raise concern about a tumor thrombus. Subtraction sequences can be helpful to bring out subtle enhancement .While HCC is often mass forming with discrete lesions visible on imaging, the liver must be carefully assessed for infiltrative HCC Figure . This paAn MRI scan provides staging information in addition to diagnosis. Multiple staging systems have been proposed for HCC. The Barcelona Clinic Liver Center (BCLC) staging system is recommended by the AASLD because it considers the imaging features for staging as well as the clinical parameters of liver function and performance status. These combined features are used to guide treatment. Five different stages are described by the system, four of which include radiologic findings. The clinical factors are beyond the scope of this paper, but briefly, these components assess liver function with the Child-Turcotte-Pugh score and patient performance status with the Eastern Cooperative Oncology Group (ECOG) scale recommendations ,21-23. Radiologic BCLC stage 0 (very early stage) consists of a single HCC less than or equal to 2 cm in size. These lesions have extremely low chance of vascular invasion or satellite lesions. These patients can be managed with targeted resection or liver transplant, depending on bilirubin and portal pressures. Radiologic BCLC stage A (early stage) consists of a single HCC greater than two cm or up to three lesions, each less than three cm in size Figure . If the Radiologic BCLC stage C disease (advanced stage) has HCC with a vascular invasion of any type and/or extrahepatic spread Figure . These pHCC can occur in patients without an underlying chronic liver disease, particularly in patients with chronic hepatitis B infection, metabolic syndrome, and aflatoxin B exposure. The distribution is bimodal in the second and seventh decades with less male predominance than in cases with chronic liver disease. The MRI features are the same as classic HCC, but tumors tend to be larger and the disease more advanced, in part because these patients may not be included in surveillance protocols -27. BecaFibrolamellar Variant of HCCThe fibrolamellar variant of HCC also arises in patients without cirrhosis, and these patients are usually less than 40 years' of age (average age: 23-25 years) without any risk factors for a chronic liver disease. The pathway for the development of these tumors is not well known, but they are pathologically different from HCC with prominent fibrous tissue and a dense central scar. Alpha-fetoprotein (AFP) levels are usually normal -29. At iCholangiocarcinomaMalignancies arising from the biliary tree are the second-most common type of hepatic malignancy after HCC and develop from the epithelial lining of the bile ducts . CCA accThe tumors are classified by intrahepatic or extrahepatic location, with extrahepatic tumors further subdivided into hilar and distal categories. The majority of tumors are extrahepatic, slightly more in the hilar location than the distal extrahepatic bile duct (50% vs 40%). Tumors at or beyond the second order biliary branches are classified as intrahepatic and make up the remaining 10%. Intrahepatic CCA (ICC) are predominantly adenocarcinoma with mucin production and a variable degree of differentiation .Three growth patterns of ICC include mass-forming, periductal-infiltrating, and intraductal intrahepatic. Mass forming is the most common subtype and has a variable appearance at MRI scan depending on the degree of fibrosis and necrosis Figures -12. UsuaPeriductal-infiltrating and intraductal ICC growth patterns are associated with biliary tree findings rather than a discrete mass. The periductal-infiltrating ICC pattern results from tumoral growth along the bile ducts, resulting in diffuse periductal thickening and enhancement. Ducts may be dilated or narrowed, and this pattern is difficult to distinguish from benign strictures. Intraductal ICC has variable patterns of ductal dilation and stricture with or without papillary and/or polypoid intraluminal masses . Combined Type HCC-CCACombined type HCC-CCA is a rare primary liver malignancy that has the histological features of both HCC and ICC in patients both with and without a chronic liver disease. An initial description by Allen & Lisa (1949) reports three subtypes of tumors: 1) completely separate masses of HCC and ICC; 2) contiguous nodules of HCC and ICC that intermingle; and 3) a complete mixed mass of HCC and ICC. Given the mixed and variable distribution of the two neoplastic cell types, the imaging appearance can vary Figure . LesionsRare Primary Hepatic MalignanciesAlthough liver malignancies arising from tissue other than hepatocytes and bile ducts are extremely rare, these tumors may have features that overlap with more common malignancies or have unique appearances that allow for precise diagnosis. Malignant vascular tumors include epithelioid hemangioendothelioma and hepatic angiosarcoma.Epithelioid hemangioendotheliomaEpithelioid hemangioendothelioma is a low- to intermediate-grade malignant neoplasm that arises from the vascular endothelium with peripheral growth along the sinusoids and a variable dense central fibrosis. The tumor is composed of dendritic spindle cells and epithelioid round cells with an abundant mixoid matrix and fibrous stroma. Up to 30% may progress to some degree of sclerosis  Figure . On MRI Primary hepatic angiosarcomaPrimary hepatic angiosarcoma is an aggressive vascular malignancy with very poor one-year survival. The tumor is composed of poorly organized vessels and cavities lined by malignant endothelial cells. Solid nodular areas composed of spindle cells may also form . While tThe lesions tended to have large hypovascular spaces without enhancement over the typical post-contrast duration. While some lesions have an enhancement pattern of delayed peripheral to central enhancement similar to hemangioma, these lesions had much more bizarre and disorganized enhancement and exhibited rapid growth over serial studies. Other lesions had the reverse pattern, enhancing from the center to the periphery over time. Masses compressed or displaced but did not invade the hepatic vasculature. Metastatic lesions were found in 45.7%, most commonly to the spleen, followed by the peritoneum, lungs, and pericardium.Primary hepatic lymphomaThe primary hepatic lymphoma accounts for less than 1% of all cases of lymphoma and occurs when lymphoma is confined to the liver, without disease in the lymph nodes, spleen, bone marrow, or other lymphomatous structures -43. The By understanding the MRI features of primary hepatic malignancies, these lesions can be appropriately included in the differential diagnosis of lesions on abdominal imaging studies. Recognizing a background chronic liver disease helps narrow the differential toward HCC and CCA. With good imaging techniques, biopsies can be avoided in patients whose lesions meet the imaging definition for HCC. An MRI scan can help determine which patients may be candidates for liver transplantation. More rare malignancies may not have definitive imaging features but should be considered in the correct setting, and awareness of these tumors allows for a more complete differential diagnosis.


Acute appendicitis in a neonate and premature baby is still considered a rare entity as diagnosis is always made after surgical exploration for acute abdominal findings mimicking necrotizing enterocolitis.Our reported case is a premature baby who had a perforated appendix with no evidence of peritonitis.We describe the case of a premature Bahraini girl born at 29 weeks of gestation by spontaneous vaginal delivery to a 39-year-old G6P5 mother. She was kept on a ventilator for the first 6 days of life, and had an uneventful Neonatal Intensive care stay until her 47th day of life when she developed sepsis that required ventilator support for 3 days. At day 51 she developed abdominal distension and was referred to a pediatric surgeon by day 54 with pneumoperitoneum. Her abdomen was soft with minimal tenderness and no evidence of erythema or edema. In view of pneumoperitoneum and previously reported sepsis, she was taken for exploratory laparotomy. The findings were consistent with a perforated appendix with no evidence of peritonitis or necrotizing enterocolitis. An appendectomy was performed. She had a smooth postoperative recovery.Neonatal appendicitis continues to be a diagnostic challenge. Only with a high index of clinical suspicion and teamwork can these cases be managed successfully and mortality and morbidity rates may reduce. Although no age is free from the risk of appendicitis, it is extremely uncommon in newborns .The rarity of neonatal appendicitis (NA) together with lack of specific signs and low index of suspicion has led to delay in diagnosis and surgical intervention . Most ofIt was proposed that NA is actually a limited form of necrotizing enterocolitis (NEC) . The obsAlthough it has been reported for over 100 years, the total collective cases reported are around 100 of which most presented with peritonitis .We describe the case of a premature Bahraini girl born at 29 weeks of gestation by spontaneous vaginal delivery, to a 39-year-old gravida 6 para 5 mother. She was born “flat” with an APGAR score of 4 and 9 at 1 and 5 minutes respectively and a birth weight of 910 g. Ventilatory support was required during her first 6 days of life, with a dose of surfactant. She had an uneventful Neonatal Intensive care stay, tolerating breast milk and was gaining weight at a steady pace. On her 47th day of life she developed sepsis and required repeated ventilator support for 3 days. At day 51 she weighed 1400 g, but developed abdominal distension and feed intolerance. She was referred to the pediatric surgeon on day 54 when pneumoperitoneum was detected by a shoot-through lateral plain abdominal film  is classically attributed to the broad orifice of the appendix , liquid diet, near-constant supine positioning, the lack of fecaliths in neonates, and the presumed relative infrequency of lymphatic hyperplasia in the periappendiceal region caused by lack of infectious stimuli . The infGeneralized peritonitis and intestinal congestion of perforated appendicitis is difficult to distinguish from that of NEC . ExistinThe most common presenting clinical feature in the literature was abdominal distension, which was present in 89% of patients  and it wPneumoperitoneum is the single most useful sign which was seen in 23 of the 44 (52%) patients with perforation. Even in the absence of correct clinical diagnosis, perforation – as indicated by pneumoperitoneum – appears to have prompted surgical exploration; this was the reason for the referral of our patient. NA does not appear to have any causal relationship with Hirschsprung’s disease  and our Although rare, NA can be expected to occur with increasing frequency as perinatal conditions associated with appendicitis, such as prematurity and survival after severe hypoxia or ischemia, are much more common in this era of improved neonatal care .NA continues to be a diagnostic challenge. Only with a high index of clinical suspicion and teamwork can these cases be managed successfully and the mortality rate may reduce.