Overdiagnosis of bronchopulmonary carcinoid tumors together with overtreatment can cause serious postoperative consequences for the patient. We report of a patient with a typical bronchopulmonary carcinoid tumor, which was initially misdiagnosed and treated as an adenocarcinoma of the lung. GnrH receptors and the associated Raf-1/MEK/ERK-1/2-pathway are potential targets for analogs in cancer treatment. We suspected a correlation between the lack of tumor growth, application of leuprolide and the Raf-1/MEK/ERK-1/2-pathway. Therefore, we examined GnrH receptor status in the examined specimen.In 2010 a 77 year-old male patient was shown to have a tumor mass of about 1.7 cm diameter in the inferior lobe of the left lung. Since 2005, this tumor had hitherto been known and showed no progression in size. The patient suffered from prostate cancer 4 years ago and was treated with TUR-P, radiation therapy and the application of leuprolide. We conducted an explorative thoracotomy with atypical segment resection. The first histological diagnosis was a metastasis of prostate cancer with lymphangiosis carcinomatosa. After several immunohistochemical stainings, the diagnosis was changed to adenocarcinoma of the lung. We conducted a re-thoracotomy with lobectomy and systematic lymphadenectomy 12 days later. The tumor stage was pT1 N0 MX G2 L1 V0 R0. Further immunohistochemical studies were performed. We received the results 15 days after the last operation. The diagnosis was ultimately changed to typical carcinoid tumor without any signs of lymphatic vessel invasion. The patient recovered well from surgery, but still suffers from dyspnea and lack of physical performance. Lung function testing revealed no evidence of impairment.The use of several immunohistochemical markers, careful evaluation of hematoxylin-eosin sections and the Ki-67 labelling index are important tools in discriminating between carcinoids and other bronchopulmonary carcinomas. Although we could not detect GnrH-receptors in the examined specimen, there may be individual differences in expression. GnrH receptor profiles in typical and atypical carcinoids should be scrutinized. This could lead to new therapeutical options, since the GnrH receptor has already been described on atypical carcinoids. Clinically tested drugs such as leuprolide could come to use. Laennec was the first one to report on an intrabronchial tumor mass, published posthumously in 1881. This was the first description of a bronchial carcinoid tumor . The terIn March 2010 a 77 year-old male patient was shown to have a tumor mass of about 1.7 cm diameter in the inferior lobe of the left lung (see Figure It is generally assumed that carcinoids arise from Kulchitsky cells disseminated in the bronchopulmonary mucosa . TypicalTTF1 is a transcription factor with a molecular weight of 38 kDa, which plays an important role in lung morphogenesis and the expression of surfactant factor. It is considered as a dependable marker in distinguishing between primary and metastatic lung adenocarcinomas and can also be helpful in discerning pulmonary neuroendocrine tumors from gastrointestinal ones. The diagnostic value seems to depend on the used antibody clone -30. AccoGnrH receptors and the associated Raf-1/MEK/ERK-1/2-pathway are potential targets for analogs in cancer treatment. Activation of the Raf-1/MEK/ERK-1/2-pathway by leflunomide and teriflunomide can inhibit proliferation and growth of BON carcinoid cells in vitro and in vivo and also decrease expression of neuroendocrine markers in both BON and H727 carcinoid cells . Similar68Ga-DOTA-peptide PET/CT scans are currently the most accurate imaging procedures for the diagnosis of NETs such as bronchial carcinoids with a sensitivity of 97% and a specificity of 92%. Even the detection of tumors smaller than 1 cm is possible. According to a study published by Ambrosini et al., 68Ga-DOTANOC PET/CT either affected stage or caused a therapy modification in more than 50% of the NET patients, proving its superiority to conventional imaging [18F-FDG PET and 68Ga-DOTATOC PET/CT [68Ga-DOTANOC PET/CT is superior to 111In-pentetreotide in the detection of CUP-NETs, with a localization rate of the primary tumor of 59% vs. 39%, respectively. The authors concluded that this method can play a major role in the management of patients with CUP-NETs [18F-FDG PET, although solitary cases of carcinoids with high 18F-FDG uptake have been reported [131I-MIBG and 111In-pentetreotide achieved an overall sensitivity of 95% for localization of carcinoid tumors. However, this approach was not further pursued [18F-FDG PET/CT scan is known to be highly sensitive, yet has its limitations due to a lower specifity. Like the above mentioned FNAB, a negative PET/CT scan does not rule out the possiblity of malignancy of a solitary pulmonary nodule and cannot replace surgical resection [According to an analysis of eight studies by Gustafsson et al., approximately 58% of all patients show unspecific symptoms: cough in 32%, hemoptysis in 26%, pneumonia in 24% of all cases and, to a lesser extent, fever, dyspnoe and chest pain. A carcinoid syndrome occurs seldomly in approximately 1-3% . Long te imaging . In thisC PET/CT . Prior tCUP-NETs . Bronchireported -65. Inte pursued . Accordi pursued . Endo- a pursued . In this pursued ,70. Also pursued . 18F-FDGesection ,70, we pSurgical resection is still considered to be gold standard in treatment. Nowadays, tissue-sparing procedures become more and more popular, since they show no great difference in long-term survival and a better postoperative quality of life. For this purpose, early diagnosis is essential. The extents of surgical resection and lymph node dissection are still a matter of ongoing debate. Some authors concluded that parenchyma sparing procedures in ACs should be avoided because of a higher recurrence rate ,72 and aThe 10-year survival rate for typical carcinoids ranges from 82% to 93%, for atypical carcinoids from 56% to 64% ,72,75. MMisdiagnosis of bronchopulmonary carcinoid tumors with surgical overtreatment can cause serious consequences for the patient like intra- and postoperative complications with further possible serious limitations of lung function. The use of several immunohistochemical markers and careful evaluation of hematoxylin and eosin sections together with the Ki-67 labelling index are important tools in discriminating between carcinoids and other bronchopulmonary carcinomas. Simultaneously, the pre-operative progression of the disease should be considered before defining the extent of initial surgical resection. In case of metastatic disease, TCs may show a similar clinical course like ACs and a significant worsening of prognosis. Therefore, we think that even small TCs which are considered as "stable disease" like our reported case should be resected. Also, a 10-year follow up is recommended.GnrH receptors and the associated Raf-1/MEK/ERK-1/2-pathway are potential targets for analogs in cancer treatment. Activation of the above mentioned pathway can inhibit proliferation and growth of various carcinoid cell types. The typical carcinoid tumor described in our case report showed almost no progression in 5 years. The patient suffered from prostate cancer 4 years ago and was treated with TUR-P, radiation therapy and the application of leuprolide. We suspected a correlation between the lack of tumor growth, application of leuprolide and the Raf-1/MEK/ERK-1/2-pathway. Therefore, we examined GnrH receptor status in the examined specimen. Although we could not detect GnrH-receptors in the examined specimen, there may be individual differences in expression. In this regard, GnrH receptor profiles in TCs and ACs should be scrutinized. Further investigations could lead to new therapeutical options in the treatment of TCs and especially ACs, since the GnrH receptor has already been described on the latter. Clinically tested and approved drugs such as leuprolide could come to use.Written informed consent was obtained from the patient for publication of this Case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.TCs: typical carcinoid tumors; ACs: atypical carcinoid tumors; CgA: Chromogranin A; SCLC: small cell lung carcinoma; LCNECs: large cell neuroendocrine carcinomas.The authors declare that they have no competing interests.ID: preparation and drafting of the manuscript, literature research; SH: editing of the manuscript; AK: editing of the manuscript; BK: immunohistochemical staining and examination of the specimen, editing of the manuscript; MF: editing of the manuscript; HJ: editing of the manuscript. All authors read and approved the final manuscript.


Introduction. Elephantiasis is a chronic manifestation of filariasis; it commonly affects limbs, scrotum, and trunk. Females have lower incidence of filarial infection. Vulval elephantiasis due to filariasis is still rarer. It is difficult to make the diagnosis on histopathology alone, more so in view of the fact that the parasite is usually not identified in tissue sections. Identification of microfilariae in night samples of peripheral blood or seropositivity for filarial antigen is requisite for the correct diagnosis. Case Presentation. A young female presented with progressively increasing vulval swelling over a period of two years. The swelling was soft and measured 5 × 6 cm. Other possible differential diagnoses were excluded, and ancillary tests were performed to reach a conclusive diagnosis of vulval elephantiasis on histopathology. Conclusion. Vulval elephantiasis due to filariasis is rare. Its diagnosis on histopathology is more often by exclusion. High index of suspicion on microscopic findings and corelation with relevant diagnostic tests are required to reach the correct diagnosis. Elephantiasis is a rare disorder involving lymphatic channels of the affected part of the body. It is characterised by gross enlargement of the particular body part. Most commonly, it is caused due to filarial parasites. The prototype of this disease was depicted by involvement of lower limbs. Other areas like trunk, breasts, upper limbs, and external genitalia can also be involved. Genital elephantiasis, also known as esthiomene, is a rare, dramatic end result of lymphatic obstruction. Although mainly associated with filariasis and sexually transmitted diseases such as lymphogranuloma venereum and donovanosis, it could also be an uncommon complication of tubercular lymphadenitis , 2. FilaAn 18-year-old, unmarried female presented with the complaint of low grade fever on and off and a perineal swelling. The swelling had been present for 2 years, slowly increasing to its present size. The patient was a migrant from Azamgarh, Uttar Pradesh, India.On general physical examination, the patient was afebrile and there was no lymphadenopathy. Local examination revealed a polypoidal growth measuring 5 × 6 cm, arising from the left labium minus. The growth was soft, and the overlying skin showed rugosities. A clinical diagnosis of fibrolipoma was suggested. Fine needle aspiration of the growth was performed. Microscopy showed hypocellular smears with an occasional lymphocyte in a proteinaceous background. Subsequently, the growth was excised and submitted for histopathological examination. Grossly, it was a single, polypoidal soft tissue mass measuring 5.2 × 3.4 × 2.2 cm. The cut section was grey-white to grey-yellow and firm. Microscopic examination showed a polypoidal lesion covered by acanthotic epidermis. The dermis and subcutis showed dense collagenization and contained numerous dilated lymphatics . In addiZiehl-Neelsen stain for acid-fast bacilli and Grocott's methenamine silver and periodic acid Schiff (PAS) stains for fungus were negative. Buffy coat preparation of the patient's peripheral blood was also examined, but no microfilariae could be seen. The histopathological diagnosis was suggested as vulval elephantiasis, and further workup was suggested.Subsequent to histopathologic report, filarial antigen serology was done, which was positive. The patient was put on ivermectin, and she responded well.Lymphatic filariasis affects 120 million people in more than 80 countries throughout the tropics and subtropics. One-third of those infected live in India, one third in Africa, and the remainder in South-Asia, the Pacific and the Americas . ElephanWuchereria bancrofti (98%) and the remaining to Brugia malayi (2%) [Culex quinquefasciatus for bancroftian filariasis, whereas the genus Mansonoides for brugian filariasis [The most widespread filarial infection is due to ayi (2%) . In Indilariasis . Filariasis can present as asymptomatic/subclinical microfilaremia, acute disease characterised by lymphadenitis or lymphangitis, and chronic manifestation characterised lymphedema or elephantiasis. The gender of the individual has an important influence on the incidence of chronic pathology in lymphatic filariasis; males are more commonly susceptible to chronic sequelae than women. The manifestations in descending order of occurrence are hydrocele, followed by elephantiasis of the entire lower limb, the scrotum, the entire arm, the vulva, and the breast. The rare condition of vulval elephantiasis has been reported to be caused by a variety of etiologies like lymphatic filariasis, lymphogranuloma venereum, donovanosis, and tuberculous lymphadenitis. Irrespective of the etiology, the basic process remains the same, that is, permanent obstruction of lymphatic channels → lymphatic stasis → stimulation of growth of fibroblasts → destruction of lymph nodes → lymphedema and elephantiasis .This case presented as a polypoidal mass involving the left labium minus. Typically, elephantiasis diffusely involves the body part affected; exceptionally, lymphedema involves circumscribed region of skin producing a solitary, large polyp, indurated (solid) plaque, papillomatous plaque, pendulous swelling, or large, sarcoma-like mass . The histopathological features in this case were suggestive of vulval elephantiasis. Ancillary tests are required to reach a definite diagnosis as in the present case, neither the adult parasite was seen in the tissue examined, nor were the microfilariae demonstrated in the peripheral blood. It is postulated that as long as the adult worms are alive, the lymphatic vessels, though damaged, still remain patent . Death oFibroepithelial polyp was considered as another possible differential diagnosis. However, absence of loose myxoid stroma, acanthosis of overlying epidermis, and presence of numerous dilated lymphatics ruled out this possibility. Due to finding of granulomas, giant cells, and cellular infiltrate comprising lymphocytes, plasma cells, and eosinophils, the other possibilities that enter into the differential diagnosis include tuberculosis and fungal infection. Presence of noncaseating granulomas, absence of acid-fast bacilli, and heterogenous admixture of cellular infiltrate ruled out tuberculosis. Absence of fungal profiles by special stains excluded possibility of fungal infection.In conclusion, thorough sampling of the excised tissue to search for the parasite and high index of clinical and histopathological suspicion led to the suggestion of vulval elephantiasis on histopathology. The diagnosis was further supported by positive filarial antigen serology.


Myelopathy at the level of the atlas is rarely encountered by the practicing spine surgeon. Due to the region's unique anatomy, compression of the cord at this level is either caused by a large compressing lesion or an abnormally stenotic canal. We describe a rare instance of a congenitally stenotic canal due to a hypoplastic intact posterior arch of atlas, coexisting with an extremely rare ossified transverse ligament of the atlas. The coexistence of these two lesions has only been documented thrice before. We describe the clinical presentation, imaging findings, and favorable response to surgery. Hypoplastic intact posterior arches of the atlas and symptomatic calcification of the transverse atlantoid ligament are very rarely seen at the craniovertebral junction, Having only been reported 17 and 15 times, respectively, in the literature .We present a case of cervical myelopathy attributable to atlas posterior arch hypoplasia and an ossified transverse ligament, whose coexistence led to the slow development of cord compression and myelopathy. We present the radiologic appearance of these lesions and surgical management, to which the patient responded favorably. We also conducted a literature review and an attempt to explain why these may have coincided in our case.This is the fourth such case in the literature, with two being identical to ours having both lesions concurrent , 4, whilA 68-year-old Saudi female presented with progressive deterioration of her walking and gait balance for 5 months. She has long history of cervicalgia for several years. There was no history of head or neck trauma or rheumatological diseases. General physical examination was normal, without cervical spine tenderness or limitation of range of motion. Neurological examination revealed normal cognitive function and cranial nerves. She had significant quadriparesis, where she was not able to raise her arm above her shoulders level and was unable to stand. Her weakness was more marked on the right side that was associated with hypoesthesia of the upper extremities, hyperreflexia in all limbs, and positive Babinski's sign. Routine laboratory tests, including CBC, electrolytes, renal, and hepatic profiles, were within normal. Her cervical X-rays revealed multiple degenerative disk diseases without evidence of instability on dynamic views. Magnetic resonance imaging (MRI) of the cervical spine revealed severe canal stenosis at the level of the atlas with significant cord compression Figures . The posCervical canal stenosis is rarely encountered at the level of the atlas. This is due to the region's anatomy protective against compression, with the canal diameter at the retrodental level being between 17 to 25 mm while the spinal cord diameter at that level is 10–12 mm in diameter [Although the majority of anomalies at the level of atlas reported in the literature are defects of either arches of atlas , these rHypoplasia of an intact posterior arch of the atlas as a cause of symptoms has only been reported 16 times in the literature . Anomali Presentation is usually in middle age, with the exception of two cases of juvenile onset, both of who were Caucasian . The reaThis stressor may be in the form of trauma, or the gradual degenerative changes of age-related spondyloarthropathy . AlthougThe usual presentation is with myelopathy , and diaResponse in reported cases has been favorable to simple posterior decompression with a C-1 laminectomy. This is to address the canal stenosis, but since the anomaly is rarely the sole cause of the canal stenosis, the other stressors have to be addressed and if needed, surgically corrected . Calcification and ossification of the transverse atlantoid ligament is a disease suggested to be part of the spectrum of vertebral ligament ossification . A recenCauses of paravertebral ligament calcification are numerous and not different from those of ligament calcification elsewhere, these include insulin resistance, calcium pyrophosphate dihydrate deposition diseases, rheumatoid arthritis, advancing age, obesity, and perhaps a genetic predisposition . AnotherDiagnosis of TAL calcification is by CT, showing a calcified retro-odontoid mass following the contour of the TAL. MRI would be useful for CVJP as it better displays the noncalcified portions in addition to neural element compression. Contrast is usually unnecessary; it may reveal an inflammatory element, especially when associated with arthritides .The management of transverse ligament calcification should then be both targeted at the underlying cause of calcification , the calcified ligament itself if symptomatic, and its effect  if present .The natural history of this disease is similar to that of cervical spondylosis; it has been displayed in several instances that, with the continuous degenerative forces causing ligament injury, hypertrophy and ossification, these masses can attain considerable size thereby causing myelopathy . We beliWe cannot come up with a reason as to why this is the first and only encounter our institution would have with these entities and why this is the first case to be reported in the whole of the Middle East and North Africa region. This could either be due to a unique characteristic of our demographic or simply due to unfamiliarity to this disease from the part of clinician, radiologist, or pathologist . It has A newly emerging concept is to regard TAL ossification, AAOA, and AAI all as, perhaps, part of the craniovertebral junction's peculiar spondylotic process , 16. A rWe regret the fact that we only realized the full scope of this anomaly in retrospect, with the patient lost to follow up. With the many associations in the reviewed literature, we would have been more vigilant in searching for them in the patient herself and her family. We present a rare cause of myelopathy in our population, with none of the reported cases being in natives of the MENA region, despite the ubiquity of its risk factors. Studies are showing that ossification of the transverse ligament of atlas, if incidentally found, is not to be considered of no significance, but as a marker for local degenerative changes or systemic syndromes. Its presence should entail a thorough search for the growing list of reported associations. A thorough understanding of its significance, pathophysiology, and natural history will only be possible with more cases being reported and studied; this is only possible by increasing awareness among the medical community.


Collision tumors of the sellar region are relatively uncommon and consist mainly of more than one type of pituitary adenoma or a cyst or cystic tumor. The association of a pituitary adenoma and a craniopharyngioma is particularly rare. This study describes a rare occurrence in which a pituitary adenoma and a craniopharyngioma coexisted in the sellar region. The case involves a 47-year-old woman who underwent transsphenoidal surgery with subtotal tumor resection and reoperation using an interhemispheric transcallosal approach for total microsurgical resection of the tumor because the visual acuity in her left eye had re-deteriorated. Histopathological and immunohistochemical examinations of the excised tissue revealed a pituitary adenoma in the first operation and a craniopharyngioma in the second operation. Retrospective analysis found the coexistence of a pituitary adenoma and a craniopharyngioma, known as a collision tumor. Instead of the transsphenoidal approach, a craniotomy should be performed, to explore the suprasellar region. Pituitary adenomas and craniopharyngiomas are two types of common tumors in the sellar or suprasellar areas. Pituitary tumors represent 10% to 15% of all intracranial tumors, with an annual incidence of 0.2 to 2.8 cases per 100,000 persons [A 47-year-old right-handed woman presented with intermittent blurred vision of the left eye and headaches, which she had had for 5 months. She had also suffered from sore roughening or splitting of the palms and arches for 6 months. The patient had been pregnant twice with normal deliveries, and she had not reached menopause at admission. Neurological examination revealed no obvious clinical signs. The patient complained of decreased vision in her left eye. An examination of her visual acuity revealed that her left eye had almost no distant vision. Visual field testing showed that her left eye’s mean sensitivity and mean defect were significantly decreased compared with the normal value. Ophthalmic fundus examination of both eyes did not show any obvious abnormality. The vision of the patient’s right eye was 5/4, and the mean sensitivity and mean defect were decreased but higher than in the left eye. An endocrine evaluation revealed increased levels of prolactin  and adrenocorticotropic hormone (ACTH) . Levels of other hormones, including luteinizing hormone, growth hormone, and follicle-stimulating hormone were normal. Other physical examinations revealed that the patient had no other clinical symptoms, neurologic deficits, or other hormonal dysfunction. Biochemical evaluations, including analysis of blood chemistry, electrolyte levels, and urine did not show any obvious abnormality. Computed tomography (CT) and magnetic resonance imaging (MRI) of the patient’s brain revealed an abnormal signal in the sellar and suprasellar areas, owing to the presence of a partial contrasting mass with clear edges , we would have avoided the second operation resulting from the misdiagnosis. For a patient with a pituitary adenoma and craniopharyngioma collision tumor, a craniotomy should be performed to explore the suprasellar region, rather than a transsphenoidal approach.Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the editor-in-chief of this journal.ACTH: Adrenocorticotropic hormone; CT: Computed tomography; H & E: Hematoxylin and eosin; MRI: Magnetic resonance imaging.The authors declare that they have no competing interests.GJ and SH reviewed the literature and drafted the manuscript. FL was clinically responsible for the patient’s care and revised the manuscript. JX and RM were responsible for the pathology and radiological images. All authors read and approved the final manuscript.


Myxoid liposarcoma occurs in middle age, and is characterized by extrapulmonary metastasis, including bone metastasis. Bone scans and [18F]-2-fluoro-2-deoxy-D-glucose-positron emission tomography (FDG-PET) are widely used for assessment of tumor extension, including vertebral metastasis. However, both methods have a low positive rate with regard to vertebral metastasis arising from myxoid liposarcoma. This is particularly true for bone scans for intramedullary lesions that have no cortical involvement.We present the case of a 53-year-old male with myxoid liposarcoma in the leg. He had been treated for multiple metastases over a ten-year period, and was experiencing back pain due to a pathological fracture in the second lumbar vertebra (L2). Magnetic resonance imaging of all the vertebrae showed abnormal signal intensity suggestive of metastasis in eight vertebrae, and revealed extraskeletal extension in three vertebrae. Bone scans and FDG-PET were negative except for the L2 fracture which was indicated on a bone scan.Both bone scans and FDG-PET can be negative in cases of vertebral metastasis that arise from myxoid liposarcoma, even when extraskeletal extensions are present. Similarly, even a fractured vertebra may not always be visible on FDG-PET. Myxoid liposarcoma is one of the subtypes of liposarcoma. This subtype typically peaks in the fourth and fifth decades of life. Myxoid Bone scans and 18F]-2-fluoro-2-deoxy-D-glucose-positron emission tomography (FDG-PET) are useful for detecting distant metastases from various tumors. Bone scans are sensitive to unusual bone remodeling activity because radioactive material is taken up by osteoblasts. On the other hand, FDG-PET can be evaluated using the standardized uptake value (SUV). An increased SUV reflects increased glucose metabolism as a result of various factors such as increased glucose transporters, high levels of hexokinase and a reduction in glucose-6-phosphatase,8. FDG-PF-2-fluorBone scans and FDG-PET have been reported to lack sufficient sensitivity for the detection of vertebral metastases arising from myxoid liposarcoma. Among vertebral metastases detected by magnetic resonance imaging (MRI), positive rates with bone scans and FDG-PET have been reported to be 16% and 14%, respectively, in a number of vertebrae. In the A 43-year-old man with a tumor in his left leg was referred to our institute. The diagnosis was myxoid liposarcoma based upon biopsy findings. Radiation and chemotherapy were administered. Consequent wide resection with the surrounding normal tissue was performed. Two years after the initial surgery, when the patient was 45 years of age, metastasis appeared in the right neck region and the retroperitoneum, and resection of these lesions was performed. Seven years after the initial surgery, when the patient was 50 years of age, lung metastasis was found and resection was performed. Eight years after the initial surgery, when the patient was 51 years of age, recurrence was found in the thigh and involved the major vessels. Amputation at the thigh was carried out. Histological diagnosis of myxoid liposarcoma was confirmed in each of the resected materials. Chemotherapy was performed after every resection of metastatic and recurrent lesions. Ten years after the initial operation, at 53 years of age, the patient experienced back pain. Plain radiographs showed a compression type fracture in the second lumbar vertebra. MRI of all the vertebrae showed abnormally high signal intensity on T2-imaging with fat suppression in eight vertebrae: the fifth cervical vertebra (C5), the seventh, tenth, eleventh and twelfth thoracic vertebrae , the second and fourth lumbar vertebrae (L2 and L4), and the second sacral vertebra (S2). High signal intensity on the MRI was seen throughout the entire vertebral body in L2 accompanied by deformity, suggestive of a pathological fracture. Extraskeletal extension of the lesion was seen in the T12, L2 and S2 vertebrae (Figure Myxoid liposarcoma is characteristic in giving rise to extrapulmonary metastasis, such as to the retroperitoneum, the axilla and the mesentery, while other soft-tissue sarcomas demonstrate a tendency to metastasize to the lung,11,12. TBone scans are known to be more sensitive than plain radiographs for detecting bone metastasis. A low pAn increased uptake of FDG-PET in cells reflects increased glucose metabolism,8, and hIn summary, a case of myxoid liposarcoma with multiple vertebral metastases is reported. Bone scans and FDG-PET were negative, even for lesions with extraskeletal extension, except for the L2 fracture which was visible on a bone scan. Bone scans and FDG-PET can thus be negative in cases of multiple vertebral metastases of myxoid liposarcoma, and even a fractured vertebra may not be visible on FDG-PET.Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.FDG-PET: [18F]-2-fluoro-2-deoxy-D-glucose-positron emission tomography; MRI: magnetic resonance imaging; SUV: standardized uptake value; C: cervical vertebra; T: thoracic vertebra; L: lumbar vertebra; S: sacral vertebra.The authors declare that they have no competing interests.AS drafted the manuscript. AS, YF, YM, KH and YI administered the treatment. OY and YI conceived the study, participated in its design and coordination, and helped to draft the manuscript. All authors read and approved the final manuscript.


The purpose of this study was to assess the magnetic resonance imaging findings of alveolar soft part sarcoma.Magnetic resonance images of pathologically proven alveolar soft part sarcoma in 14 patients were retrospectively reviewed, including lesion location, size and shape, border definition, signals on T1-weighted and T2-weighted images, presence or absence of peritumoral and intratumoral flow voids, and enhancement pattern.n = 2), ovoid (n = 8) or irregular (n = 4) shape with ill-defined margins. The lesions mainly demonstrated isointense or mildly hyperintense compared to muscle on T1-weighted images, and heterogeneous high signal intensity on T2-weighted images. Peritumoral edemas were observed in six patients. Ten lesions showed intense inhomogeneous enhancement after contrast. Intra- and peritumoral tubular flow voids representing tortuous dilated vessels with rapid blood flow were present in all cases.Patients included five women and nine men, ranging in age from 27 to 54 years, with a mean age of 36 years. A slow-growing mass without pain was the chief complaint. Eight patients had pulmonary metastases at presentation. Ten lesions arose from the extremities, two were located in the gluteal regions, one affected the presacral space and one occurred in the back. The mean maximal size of the lesions was 9.8 cm, ranging from 6.2 to 16 cm. All lesions appeared as a round (Alveolar soft part sarcoma has some distinctive magnetic resonance imaging features including a slow-growing, large mass in the soft tissue of the extremities in young adults, with numerous signal voids on T1-weighted and T2-weighted images, and strong enhancement after contrast. The 2002 World Health Organization classification of soft-tissue tumors included alveolar soft part sarcoma (ASPS) in the category 'Tumors of Uncertain Differentiation’ . ASPS isDiagnostic imaging plays a crucial role in not only lesion identification but also preliminary lesion characterization. Magnetic resonance imaging (MRI) is the favored imaging modality for evaluation of this lesion because of its excellent soft tissue contrast, multiplanar imaging capability and lack of radiation exposure. Except for case reports, there have been few studies on MRI findings of ASPS -6. The pThe present study was approved by the institutional review board of Tianjin Medical University Cancer Institute and Hospital. The requirement for informed consent was waived for this retrospective study. Fourteen patients with ASPS proved by pathology in our hospital between July 2007 and June 2013 were enrolled in this study.Of the 14 patients undergoing MRI, 10 patients underwent post-contrast T1-weighted imaging (T1WI). All MRI examinations were performed on a 1.5 T MRI system . Fast spin-echo pulse sequences were used in these patients. Un-enhanced MRI consisted of axial and coronal T1WI, axial and coronal T2-weighted imaging (T2WI), and axial and coronal fat-suppressed T2WI. Post-contrast T1WI with or without fat-suppressed technique were acquired in the axial, coronal and sagittal planes. The imaging parameters were: for T1WI, repetition time 440 to 800 ms, echo time 8 to 12 ms; for T2WI, repetition time 2,900 to 3,020 ms, echo time 80 to 90 ms; excitations, two to three; matrix, 256 × 192 or 320 × 192; field of view, 16 × 16 cm or 34 × 34 cm; section thickness, 4 mm; and interval gap, 1.0 mm.Clinical histories were reviewed, including patient age, sex and clinical presentation. Two experienced musculoskeletal radiologists reviewed the MR images in consensus. The analysis of MR images included the location, shape , margin , size in centimeters (largest diameter), signal intensity, presence or absence of peritumoral and intratumoral flow voids, and presence and pattern of contrast material enhancement. Skeletal muscle was used as the reference tissue for signal intensities on T1WI and T2WI. The pattern of enhancement was graded subjectively but in consensus as homogeneous enhancement, heterogeneous enhancement and peripheral enhancement.These patients consisted of five women and nine men, ranging in age from 27 to 54 years, with a mean age of 36 years. All patients presented with a chief complaint of a slow-growing mass without pain. The duration of symptoms before referral to our hospital ranged from one week to two years. Eight patients had pulmonary metastases at presentation.n = 10), followed by gluteal region (n = 2), presacral space (n = 1) and the back (n = 1). The mean maximal size of the lesions was 9.8 cm, ranging from 6.2 to 16 cm. All lesions appeared as a round (n = 2), ovoid (n = 8) or irregular (n = 4) shape with partly or wholly ill-defined borders. MRI revealed that the lesions mainly demonstrated isointense or mildly hyperintense compared to muscle on T1WI, and heterogeneous higher signal intensity compared to muscle on T2WI. Peritumoral edema on fat-suppressed T2-weighted sequence was seen in six patients. Intra- and peritumoral tubular areas of flow void on both T1WI and T2WI, representing rapid blood flow in distended vessels, were present in all cases , representing a relatively indolent mass, and averaged 9.8 cm  in largest diameter, indicating late recognition. Moreover, in our series, 10 patients had metastases at the time of initial diagnosis, all with pulmonary locations. These features are consistent with the previously reported literature, except for a slight male preponderance (n = 9).ASPS is an extremely rare malignant tumor that accounts for less than 1% of all soft tissue sarcomas . It is c30 years . The mosASPS is a markedly hypervascular lesion, a feature reflected on imaging studies. Regarding the radiological findings, the tumors in our series mainly demonstrated equal or slight hyperintensity compared to muscle on T1WI and heterogeneous high signal intensity on T2WI. The exact cause of the high signal intensity of ASPS on T1WI has not yet been elucidated. Some investigators think it can be attributed to slow-flowing blood in or around the tumor ,9.As previously reported, ASPS is usually a well-circumscribed mass ,10. HoweMultiple intra and extratumoral signal voids on MRI are frequently observed in ASPS ,5. In thOn the basis of MRI findings, the lesion might mimic arteriovenous malformations due to the tortuous vessels, especially on magnetic resonance angiography. In ASPS, there are significant soft tissue components surrounded by vascular tissues. By contrast, arteriovenous malformations comprise pure vascular tissue without any accompanying tissues. Another differential diagnosis is hypervascular metastasis, in which case the previous malignancy history of the patient should be sought .This study was limited by its retrospective analytic nature and bias in that observers were not blinded to the diagnosis. Another limitation was the small number of cases owing to the rarity of the tumor. Moreover, angiography was not performed in any of the cases to reflect the hypervascular features of the tumor. Despite these limitations, we believe our results add to a marked understanding of the MRI appearance of ASPS.ASPS should be considered a possible diagnosis when a slow-growing, large mass with high signal intensity on both T1WI and T2WI, multiple peritumoral and intratumoral tortuous signal voids, and intense enhancement on contrast-enhanced MRI are seen in the soft tissue of the extremities, particularly in a young adult patient. Recognition of these characteristic MRI findings may lead to the early diagnosis of ASPS, which is important in the treatment of a small primary tumor.Written informed consent was obtained from the patients for publication of this paper and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.ASPS: alveolar soft part sarcoma; MRI: magnetic resonance imaging; T1WI: T1-weighted imaging; T2WI: T2-weighted imaging.The authors declare that they have no competing interests.XL contributed mainly to the literature review and the writing of the manuscript. ZY revised the initial manuscript. Both authors read and approved the final manuscript.


Intravenous leiomyomatosis (IVL) is characterized by histologically benign tumors that exhibit aggressive clinical behavior. On rare occasions, the tumors may extend into the regional and systemic veins, thus reaching the heart. This may subsequently cause intracardiac leiomyomatosis (ICL), which may lead to congestive heart failure and occasionally, sudden fatalities. Due to its rarity and diffuse symptoms, the misdiagnosis of ICL is common and as a result, the condition may be under-reported. The present study reports a 33-year-old female who was admitted to Shandong Provincial Hospital Affiliated to Shandong University for myomectomy due to a rapidly growing myoma of the uterus. In routine pre-operative abdominal ultrasonography, a moderately sized echoic mass in the right internal iliac vein was observed, which extended to the common iliac vein, the inferior vena cava and the orifice of the right atrium. A presumptive diagnosis of ICL was made. The patient underwent a well-prepared one-stage thoraco-abdominal surgical procedure and the pathological report confirmed ICL. This case illustrates that the early detection of ICL may prevent a potential emergency situation and abdominal ultrasonography may be considered a useful tool in the diagnosis of ICL. Intravenous leiomyomatosis (IVL) is a rare clinical entity characterized by the intraluminal extension of leiomyoma pedicles into the regional and systemic veins. Typically, the tumor enters the uterine veins and may progressively extend to the iliac vein, the inferior vena cava and occasionally to the right atrium . LesionsA 33-year-old, gravida 2 para 1 patient was admitted to Shandong Provincial Hospital Affiliated to Shandong University  for myomectomy due to a rapidly growing myoma of the uterus. The patient’s medical history included hypertension and Hashimoto’s thyroiditis. The patient reported pelvic pain one month prior to the hospital admission and a clinical examination revealed a mobile, enlarged uterus with myoma. The patient reported no weight loss or cardiopulmonary symptoms. Four years earlier, and in association with a pregnancy, the myoma had measured 5 mm in size. The myoma was approximately the same size when observed during an abortion two years later. Vaginal US revealed a mass of 86×74×45 mm in size on the right side of the uterus. The results of a number of laboratory examinations, including those for tumor marker levels, were normal. Electrocardiography (ECG) demonstrated a sinus pattern with ST depression. X-rays of the thorax indicated a normal cardiac silhouette and a normal appearance to the lungs.Routine pre-operative abdominal US revealed a moderately sized echoic mass in the right internal iliac vein. The mass extended to the common iliac vein, the inferior vena cava and the orifice of the right atrium . The comIVL with intracardiac extension is an extremely rare type of benign tumor associated with a high rate of mortality . The etiThe clinical course of IVL is variable and dependent on the burden of the disease. It is most commonly observed in middle-aged females  and in cA diagnosis of ICL is usually made at the time of surgery. Abdominal US, computed tomography, MRI and echocardiography possess different advantages in identifying IVL and ICL. In the present case, IVL was suspected following abdominal US and confirmed by MRI. Detailed pre-operative information with regard to the localization and extension of the tumor is essential for a successful outcome .Surgical treatment with extensive resection has been demonstrated to provide the optimal mid- and long-term prognosis . The proHistologically, IVL resembles a typical leiomyoma and the rate of mitosis is low. Perinodular hydropic degeneration in a myoma (which was identified in our patient) may be a precursor of IVL , thus phFor middle-aged females with rapidly growing myomas and a history of pelvic surgery, IVL and ICL should be considered. Abdominal US is essential for the pre-operative assessment of ICL.


Squamous-cell carcinoma arising in a pilonidal sinus is a rare complication of a common disease. More than 60 cases are reported in literature. In all patients, squamous carcinoma arises in a long-term pilonidal disease. The gold-standard for treatment is radical excision of the neoplasm, with tumour-free margins. Some Authors consider the effectiveness of postoperative chemo- or radiotherapy, to reduce local recurrence.Authors report the case of a 65-years old male, with a 15-year history of recurrent pilonidal disease. He was admitted to the General Surgery Unit because of the development of a bleeding ulcerated lesion in the pilonidal area. The patient underwent an incisional biopsy (showing squamous-cell carcinoma), preoperative staging with total body CT-scan, and finally radical surgery. The large wound healed by secondary intention. Definitive histology confirmed diagnosis and revealed tumour-free margins. After being discharged, the patient was followed up on as an outpatient.Hospital stay took 10 days, due to the complexity of the first medications. A posterior rectocele was observed, but no functional diseases were identified. The complete formation of the scar took three months. After six months, no complication or signs of recurrence were observed.Squamous-cell carcinoma arising in a pilonidal sinus is a rare occurrence that develops only in long-standing pilonidal diseases. The tumour grows very slowly, but has a high local invasivity. Inguinal lymphadenopaties at the time of diagnosis is related to a poor prognosis. Local recurrence is usual and early. Enlargement of excision in case of local recurrence, and abdomino-perineal resection if sphincters are involved, may improve survival rates. There are not enough trials to assess the usefulness of preoperative or postoperative chemo- and radio- therapy.


Subcutaneous sacrococcygeal myxopapillary ependymoma is extremely rare tumor that has a tendency to develop in children and adolescents. There have been several case reports and sporadic reports in the literature. However, no case has been reported in an Asian patient, to the best of our knowledge. We describe a 25-year-old Asian female patient with a subcutaneous sacrococcygeal myxopapillary ependymoma that had been clinically diagnosed as a pilonidal cyst. The tumor was treated successfully by surgical excision and the patient is doing well without evidence of local recurrence or distant metastasis at 2 years after surgery. Myxopapillary ependymoma; Subcutaneous; Sacrococcygeal Ependymomas are slowly growing glial cancers of the central nervous system that account for over 60% of spinal tumors of glial origin . HoweverA previously healthy 25-year-old-Asian woman was referred for evaluation of an enlarging, painless, subcutaneous mass located intergluteal fold. It was clinically diagnosed as a pilonidal cyst by previous examiner. She could remember that a mass had been present in this region for 2 years. During the 2-year period the mass had slowly increased in size from less than 0.5 cm to over 2.5 cm in diameter. During the past few months, the mass had become tender and sometimes making it uncomfortable to sit down. There was no history of urinary or faecal problems. Physical examination revealed a 2 × 3 cm sized, solid, mildly tender, mobile mass over the coccyx. It was well circumscribed proximally but not in distally on palpation. Neurological examination was normal.Magnetic resonance imaging (MRI) revealed a 6 ×3 cm well-circumscribed subcutaneous mass located over the coccyx. This tumor was comprised of two ovoid mass and there was no direct invasion to the coccyx . UltrasoThe mass was completely excised. Intraoperatively, the lesion was seen near the tip of the coccyx and well-circumscribed. It was composed of two ovoid but contiguous masses, the larger 3.0 × 2.5 × 1.5 cm, and the smaller 1.2 ×1.0 ×0.8 cm . The hisDuring 2 years of follow-up no local recurrence or metastasis were detected, with chest radiography and abdomino-pelvic MRI .Because ependymal cells may be found within the coccygeal ligament as well as in heterotopic position and thus ependymomas may rarely occur in extraspinal locations. Extraspinal ependymomas may be found in four general situations: metastatic extension from a primary central nervous system neoplasm; direct extension into the soft tissue of the sacrococcygeal area from primary ependymoma of the spinal cord, filium terminale, or cauda equina; primary tumor of the skin and subcutaneous tissue without any demonstrable connection with the spinal cord; and primary presacral, pelvic, or abdominal tumor ,9. Our cSince the original description of an extraspinal ependymoma by Mallory in 1902 , there hThe most important treatment for cure lies with complete surgical excision at the initial operation if possible. Coccygectomy and adjuvant radiotherapy may be needed if the tumor is attached to coccyx, in case of incomplete excision, or metastatic cases ,7. Long-We report a case of a subcutaneous sacrococcygeal myxopapillary ependymoma in an Asian female. To our knowledge, there has been no previous report of a case in an Asian patient. Although very rare, extraspinal myxopapillary ependymomas must be considered in the differential diagnosis of postsacral mass lesion. And postoperatively, long-term follow-up should be needed for evaluation of metastasis.


Ovarian fibroma is the commonest benign tumor of the ovarian stroma. The cellular subtype accounts for around 10% of ovarian fibromatous tumors. The cellular fibroma is a tumor of uncertain malignant potential that may recur or be associated with peritoneal implants. Usually these are solid tumors, sometimes with small areas of cystic degeneration. This case is reported to highlight an unusual feature for an ovarian fibroma: the tumor was predominantly cystic with a small solid part; the multiple cavities contents consisted of viscous liquid that solidified under room temperature. The multiloculated cysts, the mucinous contents, and the solid areas simulated a borderline mucinous ovarian tumor on both CT scan and gross pathologic examination. Ovarian fibroma is the commonest benign tumor of the ovarian stroma , and it can occur at any age . The celα-fetoprotein, and β-HCG were negative. Exploratory laparotomy revealed a right-side adnexal cystic and a moderate quantity of ascitic fluid. The uterine annexal cystic tumor was sent to Department of Pathology to be analyzed by frozen biopsy. The lesion was considered consistent with cystic tumor of borderline malignancy, mucinous probably, or association between two tumors. The patient then underwent expanded total abdominal hysterectomy. The ascitic fluid, right and left parietocolic gutter, right and left pelvic lymph nodes, infracolic omentum, hepatic capsule, uterus, and contralateral ovary were free from neoplasia. A 53-year-old woman (G4N4A0) suffered right-side abdominal pain for eight months, associated with abdominal distension, increased abdominal size, nausea and vomiting. Ultrasonography (6 months before surgery): cystic image with smooth surfaced wall and coarse septa in the center; hypovascularization to Doppler study, measuring 19.9 × 12.9 × 19.2 cm; volume: 2562 cm³; located in the middle region, occupying the upper and lower abdomen. Computed tomography (CT)—5 months before surgery: a complex large-volume lesion extending from the pelvis to the upper abdomen , without signs of ureteral compression, ascites or enlarged lymph nodes Figures . Tumor mGross pathologic findings: the ovarian tumor measured 27 × 27 × 15 cm and weighed 6630 g, with smooth grayish external surface and absence of macroscopic rupture. Upon opening the cystic tumor, a multiloculated appearance with cavities was observed . The tumMicroscopically, the tumor showed alternating solid and cystic areas. However, there was no epithelium lining the cavities at any location; the cavities were limited by granulation tissue that, in turn, was surrounded by myxoid alteration. Part of the solid areas were slightly hypercellular with nonatypical spindle cells arranged in storiform pattern, compatible with fibroma . FrequenAfter surgery patient progressed well and is currently living in good health and without signs of tumor recurrence (follow up: 33 months). This case is interesting in several respects. The patient's complaints were gastrointestinal disorders, characterized by colic pain, bloating, nausea, and vomiting that started and had been progressing for about 8 months. The first place she sought medical care was at the clinic of digestive diseases. After voluminous abdominal mass was detected by imaging studies, she was referred for gynecologic evaluation. This fact is not new in relation to ovarian neoplasms. Early ovarian cancer-associated symptoms constitute a constellation of mostly nongynecological complaints, suggesting a visceral disturbance, which do not point immediately to a pelvic origin. Abdominal bloating and pain predominate with recent onset and multiple symptomatic episodes . GastroiAlthough the patient had ascites, commonly found in women with ovarian fibroma, both serum CA 125 and chest X-ray did not show the presence of pleural effusion. These findings departed the diagnosis of Meigs' syndrome. “This syndrome may be suspected when faced with an important pleural effusion, a very elevated CA-125 serum level, a negative cytologic examination of the ascitic effusion and no peritoneal implant on CT-scan” [ The surgical time was based on the diagnosis of cystic tumor of borderline malignancy by frozen biopsy, patient's age, CT, and intraoperative findings which showed ascites. Cellular fibromas are predominantly solid. Cystic areas are usually small and without multiloculation. But “some fibromas undergo proeminent cystic degeneration and may be misconstrued as surface epithelial stromal tumours. However, the cyst (pseudocyst) do not have an epithelial lining.”. We founIrving et al.  studied  Other authors also considered MIB-1 to be a useful additional parameter for distinguishing between ovarian cellular fibroma and fibrosarcoma . Huang eIn conclusion, ovarian cellular fibromas are usually solid and when cysts are present they are often scant and small. In the present case, the multiloculated cysts, the mucinous contents and the solid areas simulated a borderline mucinous ovarian tumor on both CT and gross pathological examination.


Pulmonary amyloidosis is rare and is often misdiagnosed due a lack of general awareness.In this case report we describe a 69-year-old Chinese woman who presented with a right lower lobe pulmonary nodule. After video-assisted thoracoscopic lobectomy, a histopathologic diagnosis of pulmonary nodular amyloidosis was rendered. She has done well postoperatively, showing no local recurrence or distal disease in an 8-month follow-up period.Distinguishing parenchymal nodular amyloidosis from a malignant lung tumor is often quite difficult. In the differential diagnosis of pulmonary nodules, nodular amyloidosis should be considered to avoid unnecessary lobectomy. Amyloidosis is an uncommon disease whereby insoluble proteinaceous amyloid fibrils are deposited in bodily tissues. Only eight patients per million per year are affected. Localized pulmonary amyloidosis is an even less likely occurrence, incorporating vague clinical features and limited treatment options. Here we report the case a 69-year old woman with a pulmonary nodule of her right lower lobe that was suspicious of lung carcinoma. Ultimately, localized pulmonary amyloidosis was confirmed following video-assisted thoracoscopic (VAT) lobectomy.A 69-year-old Chinese woman was admitted for cough and expectoration (yellow phlegm) of about 2 weeks’ duration. These symptoms developed without apparent cause and in the absence of other problems, such as chest pain and/or distress, breathlessness, fever, hemoptysis, nausea, or vomiting. A computed tomography (CT) scan of her chest showed a rough-edged shadow nearly 24×18×30mm in the right lower lobe of her lung  lower lobectomy and systematic mediastinal lymphadenectomy were performed. A tumor of her right lower lobe, roughly 3cm in diameter, was evident at surgery, with puckering of visceral pleura. Histopathologic examination later confirmed a diagnosis of nodular amyloidosis, supported by Congo red- and methyl violet-positive staining Figure A-C. BronHer postoperative course was uneventful. She was discharged 4 days after surgery and showed no signs of local recurrence or distal disease at an 8-month follow-up visit.Amyloidosis, first described by Virchow in 1854, encompasses a spectrum of clinical conditions with differing manifestations where abnormal extracellular deposition of amyloid is common . A totalAmyloidosis of the respiratory tract is subsequently divided into laryngeal, tracheobronchial, and parenchymal types, as noted by Gillmore and Hawkins . ParenchAccording to the literature, parenchymal nodular amyloidosis is not linked with systemic disease but may persist for years, running a benign course (with reticulonodular pattern) . On CT sMany of the above factors were in keeping with our patient. Although hospitalized for respiratory symptoms , chronic bronchitis, rather than the demonstrable amyloid nodule, may explain these complaints. It is difficult to diagnose these lesions, owing to their diverse clinical presentations and lack of definitive imaging properties. The imaging similarities with malignant lung tumors make differentiation especially difficult. A positron emission tomography (PET)-CT scan may help in distinguishing the two, given that amyloidosis for the most part is not fluorodeoxyglucose-avid. However, a negative result would not necessarily secure a diagnosis of amyloidosis, and PET-CT scans are costly. Routine fiberoptic bronchoscopy has a low diagnostic accuracy, but CT-guided percutaneous lung biopsy or transbronchial biopsy may afford better success in establishing a diagnosis . CurrentIn this instance, bronchoscopic washings and biopsy disclosed only chronic inflammation, and a CT-guided biopsy was refused by the patient due to the high risk of complications. The diagnosis of pulmonary nodular amyloidosis was ultimately achieved after VAT lobectomy, performed without intraoperative frozen section for two primary reasons: 1) puckering of visceral pleura was interpreted as a manifestation of malignancy, and 2) wide local excision would have proved challenging, given the location of the nodule (deep and close to hilar region).Because pulmonary nodular amyloidosis is so uncommon, no randomized controlled trials are available for therapeutic insight. By consensus, however, asymptomatic patients may not require surgical intervention. Periodic follow-up with careful monitoring may instead suffice. For symptomatic patients, carbon dioxide laser ablation or surgery (bronchoscopic or open resection) may be used as warranted , althougDistinguishing parenchymal nodular amyloidosis from malignant lung tumors is difficult due to imaging similarities. Although rare, pulmonary amyloidosis must be kept in mind, opting for intraoperative diagnosis if feasible to avoid unnecessary lobectomy.The patient granted written informed consent for publication of this manuscript and the accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.The authors have no competing interests to declare.ML collected all data and authored the manuscript. SW and XL were responsible for patient care and analysis of follow-up data. HL provided histopathologic confirmation. QZ participated in data analysis and manuscript revisions. JC performed the surgical procedure, also contributing to data analysis and shaping of the manuscript. All authors have read and approved the final manuscript.


The sellar and parasellar (SPS) region is a complex area rich in vital neurovascular structures and as such may be the location of first manifestation of a systemic malignancy. Metastases to this region are rare; breast cancer is the most common source among those that metastasize to the SPS region. Ophthalmoplegia, headache, retroorbital or facial pain, diabetes insipidus, and visual field defects are the most commonly reported symptoms. Lack of specific clinical and radiological features renders SPS metastases difficult to differentiate from the other frequently encountered lesions in this area, especially when there is no known history of a primary disease. Currently accepted management is multimodality therapy that includes biopsy and/or palliative surgical resection, radiation, and chemotherapy. Although no significant survival benefits have been shown by the surgical series, surgical resection may improve quality of life. Here we review the relevant literature and present six illustrative cases from our own institution. Metastatic lesions comprise approximately 1% of the tumors in the sellar/parasellar (SPS) area for which patients undergo transsphenoidal surgery (TSS) ; howeverDespite the advancement in the imaging modalities, tumors that have metastasized to the SPS areas may still be difficult to differentiate from pituitary adenoma on radiographic studies , 14, 15.Management options are multimodal and vary depending on whether a primary source is known or on the likely differential diagnoses based on the clinical and radiological findings. Multimodal options include radiation therapy, chemotherapy, and/or surgery , 17, altIn this paper, we review the clinical, endocrine, and radiological features of the metastatic SPS tumors with currently accepted therapeutic options based on the pertinent literature. In addition, we report six cases from our institution and discuss their management with long-term clinical outcome.A systematic review of the literature was performed using PubMed and the bibliographies of reviewed articles. The medical records of six patients admitted to the University of Utah Health Sciences Center between 2001 and 2011 were reviewed retrospectively. Clinical presentation, radiographic studies, treatment, histopathological confirmation, outcome, and prognosis were recorded . The insA 77-year-old man with known prostate cancer presented with a four-month history of left retro-orbital pain followed by left eye ptosis. At presentation, he had complete left third nerve palsy. His visual acuity was intact in both eyes, with full visual fields to confrontation. Brain magnetic resonance (MR) imaging  showed aAn 82-year-old woman with known history of breast cancer presented with several weeks' complaint of progressive left-sided hearing loss as well as facial pain and numbness in the first and the second divisions of the trigeminal nerve, respectively. On neurological examination, left-sided hearing loss and facial numbness along the V1 and V2 distributions were confirmed. MR imaging showed evidence of a heterogeneously enhancing mass in the left petrous apex that extended to involve Meckel's cave, the lateral cavernous sinus, and the internal auditory canal . A left A 79-year-old man with known history of prostate cancer presented with several weeks' history of progressively worsening double vision and eventual right eye ptosis. Neurological examination revealed complete third and sixth nerve palsies on the right side. The visual fields were full to confrontation in both eyes with intact visual acuity. MR imaging disclosed an enhancing soft tissue mass involving the clivus, pituitary fossa, cavernous sinus, and posterior nasal cavity  that surA 21-year-old man with a remote history of osteosarcoma and newly diagnosed metastatic renal cell carcinoma had complaints of worsening vision and facial pain. Because there was a discrepancy in the pupillary size between his eyes, he underwent a computed tomography (CT) scan of the head, followed by craniofacial MR imaging, which revealed an enhancing mass in the right sphenoid sinus with adjacent extension. On neurological examination, he was noted to have complete hemifacial numbness and Horner's syndrome on the right side. MR imaging of the face demonstrated a homogeneously contrast-enhancing lesion centered within the right sphenoid sinus measuring 33 × 20 × 27 mm . The lesA 42-year-old woman with several weeks of frontal headaches initially presumed to be secondary to a sinus infection underwent MR imaging after antibiotic medications failed to alleviate her symptoms. Her neurological examination was nonrevealing except for mild gait instability. Brain MR imaging demonstrated a lobulated, 25 × 20-mm sellar and suprasellar lesion with extension into the right cavernous sinus and encasement of the right internal carotid artery. This lesion was isointense on T1- and T2-weighted images and heterogeneously enhancing with gadolinium administration. A CT scan was consistent with a lytic lesion involving the central skull base extending laterally and posteriorly to involve the bilateral medial sphenoid wings and the clivus, respectively. A transnasal approach was undertaken to obtain a biopsy of the lesion. Histopathologic evaluation was consistent with a diagnosis of diffuse large B-cell lymphoma .A 53-year-old woman presented with a known diagnosis of breast cancer and a one-year history of worsening left eye vision with a more precipitous decline in the last month. MR imaging of the brain demonstrated a skull base lesion involving the left sphenoid bone, the anterior clinoidal process, and the cavernous sinus. On neurological assessment, the patient was noted to have an acutely diminished visual acuity in her left eye to a level of finger counting with left temporal visual field cut. MR imaging (not shown) showed a homogenously contrast-enhancing lesion of the skull base involving the greater sphenoid wing and the anterior clinoidal process with encasement of the optic nerve on the left. A left frontotemporal craniotomy was performed for biopsy of the lesion and to decompress the optic nerve and the cavernous sinus with a subtotal resection. Histopathological analysis confirmed the diagnosis of metastatic breast carcinoma. The patient did not experience improvement in her vision postoperatively. She underwent fractionated radiotherapy to the involved area. One year after surgery, her neurological findings were unchanged, and her systemic disease was under control. Neoplasms originating from a multitude of sites have been reported to metastasize to the SPS region. Breast and lung cancer account for approximately two-thirds of SPS metastases, being the most common sources in women and men, respectively, , 18–21. The possible metastatic pathways to the pituitary and parasellar region include direct blood-borne metastasis to the posterior pituitary lobe, pituitary stalk, clivus, dorsum sellae, or cavernous sinus or leptomeningeal spread with involvement of the pituitary capsule , 26, 27.Clinical symptomatology varies depending on the location of metastatic involvement. Cranial nerve palsies are the most frequent symptoms in cases of cavernous sinus metastases. These may be isolated, such as diplopia or ptosis, with the third (oculomotor) and the sixth (abducens) nerves being the most commonly involved, followed by the fourth (trochlear) nerve , 29–31, Because of their invasiveness, pituitary metastatic lesions have a high potential to cause visual deficits from suprasellar extension, with an incidence as high as 50% reported by Branch Jr. and Laws Jr.  and otheSymptoms strongly suggesting metastasis in the parasellar or sellar space include painful ophthalmoplegia in association with the sudden onset of DI , 34, 42.Because pituitary adenomas also present with invasion of the sellar floor, cavernous sinus, or clivus, no specific neuroimaging criteria to define metastatic lesions in SPS region have been reported. The diagnostic imaging tools for SPS metastasis mainly include high-resolution CT and MR imaging. Although CT is superior to MR imaging in detecting the bone involvement, the latter is preferable to determine the relationship of the lesion to the surrounding neurovascular structures . AlthougThe management of SPS metastases is multimodal, including surgical resection, radiation therapy, and chemotherapy . TreatmeBesides its role as an adjunct after surgery , 42, 56,Chemotherapy is commonly used alone or along with radiation therapy mostly for palliation in the treatment of metastatic disease in SPS region . Its valThe prognosis of patients with metastases to the SPS region is grim in a majority of cases because of the aggressive character of the primary disease . Even inSellar and parasellar metastatic lesions are relatively rare. Breast and lung have been reported to be the most common sources in both sexes. Suggestive symptoms include rapid onset of progressive ophthalmoplegia with retro-orbital or facial pain, visual impairment, and/or DI. Management varies depending on whether a primary source is identified, the symptomatology, the location and extent of the lesion, the stage of the primary disease, and the medical comorbidities. Subtotal or partial surgical resection is aimed mainly for symptom relief. A multimodal approach involving subtotal resection of the lesion followed by radiation and/or chemotherapy is widely accepted, especially in symptomatic patients whose primary disease is under control. Radiation with or without chemotherapy is generally recommended as first-line treatment in patients with advanced primary disease or in those with high-risk medical comorbidities. A biopsy usually precedes radiation therapy if the primary source of the metastasis is unknown. The prognosis for patients is generally poor, independent of the therapeutic modality, and the overall survival is less than two years.


Hamartoma is a rare splenic benign tumor usually accidentally detected as a radiologic finding. Preoperative diagnosis poses a challenge and thus surgery becomes necessary to confirm the clinical suspicion. Laparoscopic splenectomy has gained consensus as a standard surgical procedure particularly for autoimmune hematological diseases. This former experience has allowed this technique to be extended to other splenic pathologies. Here we report a case of total laparoscopic splenectomy for a bulky splenic hamartoma in a young male patient. Mass forming splenic tumors are rare. Malignancies include lymphoma and other less common lesions as primary splenic sarcoma, plasmacytoma, or metastatic disease from non-gastrointestinal tumor  –7. BenigA 28-year-old Italian male presented at the ER in August 2011 with diarrhea and an antibiotics refractory fever. Laboratory findings did not show any significant problem, apart from a mild hypertransaminasemia: (ALT 65 u/L AST 46 u/L).The patient was discharged with the indication for further investigations. Abdominal ultrasonography was performed two weeks later, showing normal liver, biliary ducts, and pancreas morphology. Nevertheless a 15 cm increased spleen was found, containing in its cephalic portion a hypoechoic and asymptomatic 70 × 77 mm solid mass. Color Doppler showed blood flow along the edge of the round mass, without relevant signals within the neoformation.CT scan was also executed, to better evaluate the splenic lesion: images before contrast material administration displayed a round mass, hypodense compared to the normal splenic parenchyma. After injection of intravenous contrast, it showed a mild contrast enhancement during the arterial phase, followed by a complete washout during the late venous phase, without relevant vascular components inside .After these preoperative findings suggestive but not definitive for a well-defined histological lesion, considering the high risk for splenic bleeding in the case of biopsy and the risk for spontaneous rupture, the patient was candidate for totally laparoscopic splenectomy two months after first clinical evaluation. Routine preoperative examination, chest X-ray, blood test, and anaesthesiologic evaluation did not set any contraindication to the operation.The patient was placed in a 30-degree right lateral decubitus, with the help of a cushion positioned along his back.Right arm was arranged for intraoperative intravenous fluid infusion, whereas left arm was suspended with bendages above patient's head to allow full access to the left hemiabdomen. A 12 mm disposable Hasson's trocar was placed using an open technique in supraumbilical region for the first induction of pneumoperitoneum and for optical access. A second 10–12 mm disposable trocar was positioned in left hypochondrium, at the intersection between midclavicular line and infracostal line and two more 5 mm accesses, the first in left flank, along anterior axillary line and the second in right hypochondrium, along midclavicular line . LaparosVessels were finally dissected using two vascular 30 mm endostapler refills  obtaining a perfect hemostasis. Spleen was then mobilized toward the patient's right side, to allow dissection of the posterior peritoneal reflection or splenorenal ligament. After placing the totally freed organ inside an EndoBag  a Pfannestiel suprapubic incision was performed to remove the specimen. The peritoneal-sac access was carried on with a partial surgical section of the rectus muscle in order to allow an easier removal of the unbroken specimen. After clearing the spleen, laparotomic incision was sealed in multiple layers. An accurate hemostasis control and a generous abdominal cavity washing were performed after the recreation of pneumoperitoneum; a single drainage was positioned in the left subphrenic space and major accesses were finally closed.A short-term perioperative antibiotic prophylaxis based on cefazolin (1 gr every 8 hours) was administered for the first 24 hours. Pain control was always optimal during the entire hospitalization: during the first 24 hours after surgery pain control was managed with opiates and paracetamol; on the following day opiates were replaced with anti-inflammatory nonsteroidal drugs removed from the 3rd day on; during the same day the drainage was removed. The patient was allowed to eat from the 2nd postoperative day. The complete functionality of the gastrointestinal tract was regained between the 3rd and 4th postoperative day. The patient was dismissed on the 5th day after surgery and stitches were removed 6 days later during an outpatient clinical control. Vaccination against Streptococcus pneumoniae, Haemophilus influenza type B, and Neisseria meningitidis infections was administered 20 days after surgery. Perioperative anticoagulant prophylaxis with subcutaneous heparin (4000 I.U. per day) was applied and continued for 4 weeks after surgery.The anatomopathological analysis of the spleen showed a 14 × 7 × 8 cm organ, weighting 430 gr, containing a 7,5 cm reddish brown nodule of slightly augmented stiffness, compared to the surrounding splenic tissue . The hisSolid lesions of the spleen are quite rare and generally asymptomatic. About half of these are accidentally detected during imaging studies carried out for unrelated causes . DiffereSplenic hamartoma is a rare benign tumor which occurs in any age group and has the same male and female occurrence. Its size tends to be longer in females, probably due to hormonal influence on tumor growth –26. The It is difficult to make a definitive preoperative diagnosis basing on conventional imaging including ultrasound and CT scan. Generally splenic hamartomas present as homogeneous, hypoechoic masses with multiple radial blood-flow signals at Color Doppler sonograms . ContrasFine needle aspiration biopsy may be useful to establish a pathological diagnosis. However this technique is associated with some dreadful complications including bleeding and abdominal seeding. These are the reasons why this procedure has been rarely reported , 33, 34.Histologically hamartoma presents as an overgrowth of the normal spleen components . MicroscLaparoscopic splenectomy (LS) is the standard procedure for most of benign and malignant hematologic diseases . The lapIn conclusion the rapid advancement of imaging modalities leads to the identification of more asymptomatic lesions of the spleen. In most of these cases malignancy cannot be ruled out by preoperative clinical and radiological evaluation and surgery becomes necessary. In the absence of massive splenomegaly total laparoscopic splenectomy may be a safe and feasible procedure for the diagnosis and treatment of both benign and malignant solid tumors of the spleen as demonstrated by this particular case of splenic hamartoma.


A 23 year old man presented with intestinal evisceration from stab injury to the left side of the abdomen with a hand-held cow horn at a local night party. He complained of severe abdominal pain and bleeding at the site of injury. He was hemodynamically stable. At emergency exploration, the eviscerated bowel was viable with no adjacent mesenteric tear. Other intra abdominal organs were normal. The eviscerated bowel was lavaged and reduced into the abdomen through the 7cm anterior abdominal wall laceration. The laceration was repaired and abdomen closed in layers. Post operative recovery was uneventful. The hand-held cow horn can easily be concealed and may pass through security checks undetected. It should be added to the ever increasing list of weapons of small scale terror. In this world of ever increasing terrorist's activities, this surely is a potential weapon of small scale terror. This is a report of serious morbidity caused by this ‘new weapon’.Detached cow horns are used widely in some cultures for internal decorations . UnfortuA 23 year old young Fulani man presented to the Accident & Emergency room in the early hours of the morning with history of stab in the abdomen with a hand-held cow horn. An hour earlier he was at a local party with his peers when a scuffle ensued and he was stabbed with a cow horn on the left side of the abdomen. He had severe abdominal pain and noticed that his intestine was protruding through the wound. He was helped to the hospital by his friends. There was no loss of consciousness. He bled from the site of injury. No vomiting or abdominal distension. There was no hematuria. Examination revealed an anxious young male who was not pale. He did not have any alcohol smell or evidence of any parenteral drug abuse, but no objective alcohol test was done. There was tachycardia of 92/min and a blood pressure of 100/60mmHg. Chest examination was normal. There was an obvious eviscerated hyperemic loop of bowel in the left paraumbilical region. The abdomen was moderately tender but there was no evidence of significant free peritoneal fluid collection. Rectal exam was normal. He was resuscitated with intravenous fluids and antibiotics, and tetanus prophylaxis was given. Eviscerated bowel was covered with sterile warm moist gauze soaked in saline. Complete blood counts, electrolytes and urea laboratory test results were within normal limits. Abdominal ultrasonography suggested minimal fluid collection in the peritoneal cavity. At surgery, the eviscerated bowel was found to be viable. It was thoroughly lavaged with saline. Via a midline incision, the abdomen was then opened. The peritoneal cavity was found to be relatively clean with just about 30 mls of hemoperitoneum in the pelvis. There was a 7 cm transverse rent involving all layers of the anterior abdominal wall to the left of the umbilicus, through which about 35 cm loop of jejunum eviscerated. There was no mesenteric injury. All other abdominal organs were normal. The eviscerated bowel was reduced with ease. The laceration was debrided and repaired in layers. The abdomen was lavaged with normal saline and the wound closed in layers. Post operative recovery was uneventful and he was discharged home on the 8th post operative day. The patient has remained well and stable at 12-month follow-up.This report aims at highlighting how a seemingly innocuous hand-held cow horn could cause serious injury and instill fear and scare amongst innocent people.3A majorities of these injuries occurs amongst cow rearing populations, although a few incidences are recorded during bull plays. The cow’s main defense is the use of its horn to deter potential threat or chase away invading enemy. However, when the organ (horn) is detached, it is of not much use to man except for in-house decorations as practiced by certain communities. Unfortunately it may have some other harmful use as in this report. The detached organ is cut and trimmed to size towards its pointed end, and is easily pocketed or hidden under the garment. It is brought out when the need arises and the situation permits. An otherwise enjoyable party came to an abrupt end as the outcry from the stabbed victim and the evisceration of bowel caused scare and pandemonium.Abundant literature exists on cow gore causing variable degrees of injury. The abdomen is the commonest region so affected.The site of stab could potentially injure vital organs- bowel and mesentery; (L) kidney and ureter; tail of pancreas and any of the great abdominal vessels on the left. Injury to any of these would have been more catastrophic. Luckily in our patient the injury was restricted to the anterior abdominal wall muscles. We therefore had to deal with the problem of eviscerated bowel and the potential inoculums of microbes associated with the horn. As the patient was brought to hospital early, contamination and desiccation of the bowel was minimal and features of obstruction and strangulation had not supervened. In addition, broad spectrum intravenous antibiotics including metronidazole were administered prophylactically. Thus, prompt reporting and resuscitation, absence of significant intra abdominal injury, as well as early surgical intervention are thought to have contributed to the successful outcome in our patient.This type and cause of injury adds a new dimension of violence and small scale terror. The magnitude of terror could be amplified if the assault is directed at the pilot of an aircraft, captain of a ship, or driver of a vehicle, as it is enough to change the direction and destination of a large number of people! Furthermore, this modified weapon consists of keratin and some structured proteins like tubulin and elastin. It does not contain any metal and may therefore not ignite the alarm of metal detector. Although we have not been able to see how it would look like on airport scanner, we believe that the pointed end could easily be hidden by interposition with similar non pointed piece of horn. The detached cow horn, is handy, may be easily concealed and could be a weapon of small scale terror. It could also be well decorated as a camouflage to divert attention from its potential terrorist’s unsuspecting weapon. It should be added to the ever increasing list of weapons of violence and terror.


Adenomyomas are benign tumors composed of smooth muscle and endometrial tissue. These tumors usually arise from the myometrium. Extrauterine adenomyomas are rare with only a few case reports available in the literature. Here, we report an unusual case of multiple adenomyomas in a 39-year-old woman six years after hysterectomy for multiple leiomyomata. To the best of our knowledge, this is the first case of extrauterine adenomyoma presenting as an upper abdominal mass. Uterine adenomyomas are benign tumors composed of smooth muscles, endometrial glands, and endometrial stroma. They are distinguished from adenomyosis by their sharp demarcation from the surrounding normal tissues, and from leiomyomas by the presence of intrinsic endometrial glandular and stromal elements –3.An extrauterine location is extremely rare and to the best of our knowledge, only four cases of extrauterine adenomyoma have been reported in the English literature. We are reporting a fifth case, and only the first case in the upper abdomen. The differential diagnosis includes uterine-like mass lesions, parasitic leiomyoma, disseminated peritoneal leiomyomatosis, endomyometriosis, and leiomyoma with entrapped benign endometrial tissue.In this case study, we will focus on this rare entity with a brief review of the literature, discussion about differential diagnosis, possible pathophysiology, and treatment options.The patient was a 39-year-old woman who presented with complaints of abdominal pain and rectal bleeding. A computed tomography (CT) scan of the abdomen and pelvis was performed that showed one mass in her left paracolic gutter  and one Her medical history was significant for a supracervical morcellated hysterectomy in 2006 for multiple leiomyomata, removal of a cervical leiomyoma a few months ago, and history of endometriosis.At laparotomy, the patient had an approximately 6.0 centimeter mass in the cul-de-sac. It was free from either ovary or tube and was not attached to the cervix. It was attached to the upper part of the rectum and was below the vagina. There was also a secondary mass in the left upper quadrant of the abdomen which was 7.5 cm. On gross examination, both masses appeared to be encapsulated with a tan-white firm homogenous cut surface and multiple areas of cystic changes with dark brown viscous fluid.A frozen section was performed and interpreted as leiomyoma with foci of endometriosis most consistent with adenomyoma.A microscopic evaluation showed benign anastomosing fascicles of smooth muscle cells. No nuclear atypia or necrosis was noted. Foci of cystic changes lined with endometrial glands and stroma were randomly distributed throughout both masses. No distinct central cavity was identified Figures  and 3.The microscopic slides from the patient's previous hysterectomy from 2006 were also reviewed and showed multiple leiomyomata. No evidence of adenomyosis or intrauterine adenomyoma was noted.All of the previously described cases of extrauterine adenomyomas have been confined to the pelvis  .In this paper we document a fifth case in this series and only the first one in the upper abdomen.The mean age in these cases was 51 years (range 39–65 years). Most reported cases presented with a single mass and have ranged in size from 0.8 cm to 6.3 cm.The differential diagnosis includes uterine-like mass lesions, parasitic leiomyoma, disseminated peritoneal leiomyomatosis, endomyometriosis, and leiomyoma with entrapped benign endometrial tissue.The uterine-like mass lesions typically show an organoid arrangement consisting of a single central cavity lined by endometrial-type epithelium with surrounding myometrial-like tissue . The lesSeveral cases of “extrauterine adenomyoma with uterine-like mass features or uterine-like mass with features of extrauterine adenomyoma” have been documented in the literature. Here, we emphasize the differences between the two entities.It is also important to mention that some of the reported cases of uterine-like mass lesions have been associated with renal or urinary tract anomalies. It is possible that some of the uterine-like mass lesions arise as a result of congenital abnormalities such as müllerian duct fusion defects , 3.Parasitic leiomyoma develops when a uterine leiomyoma or fragments of a leiomyoma detach and implant on the abdominopelvic cavity and gradually grow to become a clinically significant lesion , 5. ThisDisseminated peritoneal leiomyomatosis is excluded because of the presence of cycling endometrial tissue, large size of the lesions, and lack of recent or current pregnancy.The lesions described in this paper were well-circumscribed masses composed of intersecting short fascicles of smooth muscle bundles reminiscent of uterine leiomyoma or adenomyoma, and there was no adhesion or obvious continuity with surrounding normal stromal tissue. This excludes the possibility of endomyometriosis.Another possible differential diagnosis of benign leiomyoma with entrapped, not intrinsic, endometrial tissue has been suggested. Similar lesions have been described within the uterus , 3. LackSo far two theories are proposed to explain the etiology of the extrauterine adenomyoma: (a) the uterine/müllerian duct fusion defect theory and (b) the subcoelomic mesenchyme transformation theory. The first theory explains the abnormality in the development of the female genital tract. Each male or female fetus has two pairs of genital ducts: wolffian (mesonephric) and müllerian (paramesonephric). The müllerian duct, as the main female genital duct, begins as a longitudinal folding of the coelomic epithelium on the anterolateral surface of the urogenital ridge. Lack of fusion of the müllerian duct system may explain various duplications or atresias of the uterus . The samThe subcoelomic mesenchyme is a layer of tissue that lies underneath the mesothelial surface of the peritoneum. In fetus, this layer of tissue gives rise to the mesenchyme of the urogenital ridge that surrounds the early müllerian and wolffian ducts. In adults, the subcoelomic mesenchyme presents as an inconspicuous layer of flattened cells that lie immediately underneath the subserosal stroma of the uterus, ovaries, tubes, and uterine ligaments. The cells of this layer, which is also called secondary müllerian system, are thought to be multipotential and can proliferate in response to hormonal stimulation .The extrauterine adenomyomas in our patient are best explained by the second theory of subcoelomic mesenchyme transformation. The patient in our case did not have any evidence of urogenital abnormality, so it is less likely that she had a müllerian duct fusion defect.Long-term followup of patients with extrauterine adenomyomas has been documented in a study by Carinelli et al. for the first time. They reported two cases of multiple extrauterine adenomyomas and uterine-like masses. They observed a relapse in one of their patients after six months. In both cases, surgery followed by long-term therapy with monthly GnRH agonist to stop the estrogen-stimulated proliferation of the neoplastic tissue prevented recurrence for long periods of observation (4 and 10 years in their experience) . This su


In most patients, adrenocortical carcinoma is diagnosed at an advanced stage of the disease. A sudden retroperitoneal hemorrhage may sometimes be the first symptom of the adrenal cancer. We describe four patients with adrenocortical carcinoma diagnosed during a retroperitoneal hemorrhage. A clinical analysis suggests that such a complication is a poor prognostic factor. The detectability of adrenal cancer has significantly improved in recent years due to the development of new imaging techniques. Unfortunately, most of these tumors are identified at the locally invasive or metastatic stage . Rare caA 12-year-old girl, after physical exercise at school, had a sudden pain in the right lumbar area accompanied by a brief lack of consciousness and tachycardia, followed by progressive weakness. Emergency surgery was indicated by a gradual fall in the hematocrit. A tumor of the right adrenal, 10 cm in diameter, and a retroperitoneal hemorrhage from the right adrenal vein were the main intraoperative findings. The histopathological diagnosis was adrenocortical carcinoma. After the operation the patient felt well. However, four months later signs of Cushing’s syndrome appeared . At admission to the Department of Endocrinology the girl was 160 cm in height and weighed 66 kg. Computed tomography (CT) revealed an abnormal mass (6 cm in size) at the upper pole of the right kidney; multiple metastatic tumors were identified in the lungs by a routine x-ray examination. An increase in the daily urinary excretion of steroids was also noted: 17-hydroxycorticosteroids (17-OHCS) - 30.4 mg , 17-ketosteroids (17-KS) - 44.4 mg . During a second operation (7 months after the first) the recurrent adrenal tumor and a metastatic mass in the omentum were removed. Therapy with aminoglutethimide  was introduced - 1.0 g daily for three months - together with mitotane (LYSODREN - Bristol) in a long-term program. The daily doses of mitotane ranged from 3.0 to 8.0 g. Clinical remission, associated with a return of steroid excretion to normal values  and a reduction in the size and number of pulmonary metastatic tumors, was reached within about five months. After two years, androgenization reappeared and pulmonary as well as abdominal carcinomatous recurrences progressed. The patient died about 2.5 years after the first surgical intervention. This patient has been registered in the IPACTR  was the initial surgical finding. After evacuation of the blood, a right-sided adrenal tumor (6 cm in diameter) and the right kidney  were removed. Histopathologically the tumor was identified as adrenocortical carcinoma with hemorrhagic and necrotic areas. Shortly before the surgery, diabetes mellitus and hypertension were diagnosed. Normal 17-OHCS and 17-KS values were recorded after the operation. Mitotane was administered in doses ranging from 3.0 to 8.0 g daily. After the first year of therapy, characterized by partial clinical remission, the abdominal mass progressed and left-sided pulmonary metastatic tumors appeared. Chemotherapy (Cisplatin) was introduced, simultaneously with the administration of Mitotane, but without success. The patient died after two years of follow-up.A young woman, aged 21 years, was admitted to our department because of a right adrenal tumor, found incidentally two weeks before, during routine ultrasonography after an emergency appendectomy for acute appendicitis. CT revealed a non-homogenous tumor of the right adrenal gland, 7 cm in size, and enlarged paracaval lymph nodes. No features of adrenal hyperfunction were observed. In hormonal examinations, slightly increased serum cortisol levels of 28 μg/dL , and androstendione elevated to 475 ng/dL  were recorded. On the third day of observation the patient experienced sudden pain in the right lumbar area. A hemorrhage into the adrenal tumor was suspected and this was confirmed by three-dimensional sonography and CT Figs. . SurgeryA 65-year-old woman was observed as an outpatient, admitted to our clinic (Department of Endocrinology) four weeks after emergency surgery for a retroperitoneal hemorrhage, with evacuation of 3.5 L of blood and removal of a tumor of the left adrenal gland. Her past history was a sudden lumbar pain in the left side with progressive weakness followed within six hours by shock. CT revealed a retroperitoneal, non homogenous tumor. Histopathology identified an 11 × 8 × 4.5 cm sized adrenocortical carcinoma with large necrosis of the tumor. Hormonal measurements performed after the surgery were within normal limits. Mitotane was administered, 4.5 g daily. Over the next six months, metastatic tumors were found in the left lung, as well as in the second lumbar vertebral body, with progressive paresis of the legs. The patient died two weeks after an orthopedic operation.Adrenocortical carcinoma is a rare tumor, representing approximately 0.02% of all cancers , 5. MostIn none of our four patients were typical somatic signs of hypercorisolism or hyperandrogenism apparent prior to the hemorrhagic incident. The development of Cushing’s syndrome during the phase of carcinomatous dissemination in the patient No. 1 was a rare observation. Diabetes mellitus and hypertension preceding the hemorrhage in the patient No. 2 were probably the first symptoms of metabolic and vascular disorders due to adrenal carcinoma.In all four patients the adrenal cancer followed an aggressive course, with rapid development of local recurrences and distant metastases. In case No. 3 a fulminant course of the adrenal cancer caused death within about six months following the hemorrhage, despite immediate mitotane administration and chemotherapy. Massive dissemination of neoplastic cells during such a severe vascular incident was probably responsible for the extensive local invasion and metastatic process in all of these patients.In our experience  and in tIn conclusion, a retroperitoneal hemorrhage due to an adrenocortical cancer should be considered as a bad prognostic factor.