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Dyke-Davidoff-Masson syndrome (DDMS) represents a severe disease that is caused by brain anomalies of different mechanisms early in life during fetal period or within first years after birth. This is a brief point of view of DDMS through an endocrine perspective. In DDMS, low TSH (thyroid stimulating hormone) hypothyroidism is part of a variable cocktail of endocrine anomalies like pituitary insufficiency that actually seem less relevant opposite to massive neurological damage which marks the clinical picture. Overall, the most spectacular anomaly and the most specific in DMMS is the imaging aspect of the brain hemiatrophy. The syndrome itself, even with a very low prevalence in general population (the level of statistical evidence is case report or series), has a heterogeneous presentation and a large area of clinical combo involving not only neurological field. On the other hand, cerebral hemiatrophy (also a rare neuroimaging finding) may have other causes, either acquired or congenital. Seizures induced by brain damage may be presented long before the imaging recognition of the syndrome is actually done. The endocrine disturbances vary and they may be subtle like central hypothyroidism or life threatening like adrenal crisis due to secondary adrenal insufficiency. Overall, DDMS represents a complex challenge from severe neurological deterioration to neuro-imagery features centered on the brain hemiatrophy to skin and bone anomalies as well as endocrine disorders which are either deficiency of pituitary hormones or diabetes mellitus and autoimmune thyroiditis. Early recognition is useful for long term prognosis, a multidisciplinary approach is essential. Underlying causes and specific clusters of classification are still running under a large shadow of unknown data.

This is a narrative review of literature introducing somatostatin receptors (SSTRs) as part of understanding the somatotroph cells since they are positive in normal cells but also in tumoral cells as seen in somatotropinoma, a growth hormone (GH)-producing neoplasia, which causes acromegaly. They are five subtypes of SSTRs (1 to 5), which are immunohistochemically positive in different proportions in somatotropinomas. SSTR types 2 and 5 are most frequent in GH-secreting adenomas and they are both targeted by medical therapy with somatostatin analogues (SSTAs) like first generation Octreotide and Lanreotide (mainly targeting SSTR2) and second generation Pasireotide (with highest affinity for SSTR5), thus heterogeneous SSTRs configuration into the tumor explains different pattern of response to treatment and it might predict it once the SSTRs immunostaining is performed. Monoclonal antibodies are used for immunohistochemical detection of SSTRs; currently, a lack of standardization is presented, and scoring systems, such as Volante, H-score or human epidermal growth factor receptor 2 (HER2)-score, are applied. Immunoreactive markers like SSTRs are the U-turn in clinical practice regarding somatotropinomas since the configuration of subtypes 2 and 5 explains the responsiveness to medical therapy like SSTA. Further achievement of disease control is imperiously necessary because acromegaly has an increased rate of morbidity and mortality.

Background Ovarian Leydig cell tumour is a very rare steroid hormones producing mass, causing clinical and biochemical hyperandrogenism. Even if the level of evidence is based on case studies, many authors (but not all) agree that raised androgens increase the cardio-metabolic risk thus early diagnosis and treatment are necessary On the other hand, the endocrine features pointing an ovarian tumour source of testosterone do not indicate the specific histological finding which needs a post-operative conformation. Case presentation We report a case of a 60-year-old woman with a 4-year history of progressive virilisation in association with hypertension, high number of red blood cells, impaired glucose tolerance and dyslipidemia. Total testosterone was 20 times above normal with suppressed gonadotropins, inadequate for menopause. Trans-vaginal ultrasound and pelvic and abdominal computerized axial tomography imaging revealed a right ovarian solid nodule, and no evidence of alteration in the adrenal glands. Total hysterectomy and bilateral salpingo-oophorectomy were performed. Histopathology and immunohistochemistry confirmed the diagnosis of Leydig cell tumour. After surgery, androgen levels returned to normal and the doses of anti-hypertensive drugs were reduced. Conclusions The hyperandrogenic state with elevated plasma testosterone and progressive signs of virilization raises suspicion of an ovarian androgen-secreting tumor. For a postmenopausal patient with hyperandrogenism the diagnosis of Leydig cell tumour should be considered. However, the exact diagnosis is provided by post-operative histological exam. Prolonged exposure to hyperandrogenism may generate cardiovascular abnormalities and metabolic syndrome which after tumor excision and removal of the source of androgen hormones are expected to significantly improve.

This is a 36-year old menstruating female who accused abdominal pain of non-specific pattern during the last days. Abdominal ultrasound and computer tomography confirmed a left adrenal mass of 6 centimetres with cystic appearance without solid elements. No other causes of abdominal pain were identified. Adrenalectomy was performed. The pre- and post-operatory panel of endocrine assessments was normal. Pathological report confirmed an endothelial cyst at the level of a normal adrenal gland. The endothelial cells are introduced in hematoxylin eosin stain at histological report (Figure I: 4X, Figure II: 40X). The immunochemistry reaction is positive for factor VIII in cyst cells of adrenal origin. (Figure III: FVIII positive immunostain in fused and cubic cells, 20X, Figure IV: FVIII in fused cells, 20X). Further on the clinical evolution was good. Adrenal cysts may be endothelial related, epithelial derived and pseudocysts. They are found in both adults and children but the level of evidence varies from cases series to cases reports. (1,2,3) The risk of malignancy or rupture are the major complications. (1) Endothelial cysts seem more frequent in women. (2) The scenario of detection may be of a typical adrenal incidentaloma or starting from non-specific local as seen here.

Background Solitary fibrous tumours (SFTs) rarely occur in the orbit, especially in the lacrimal area. These tumours are mostly solid. Cystic changes have been documented, but they remain very rare. Only three cases of primary orbital solitary fibrous tumours with cystic changes have been reported in the literature, but no cases have been reported to occur in the lacrimal gland. Solitary fibrous tumours generally follow a benign course and are treated definitively with surgical excision. Data from the literature suggest that the cystic nature of SFT presents a risk of recurrence and could be a harbinger of malignancy. Case presentation A 42-year-old woman was admitted to the endocrinology department for right unilateral exophthalmia and epiphora in the last 8 months. An ophthalmological evaluation showed exophthalmia only in the right eye (22 mm) and normal visual acuity, visual field and extraocular movements. Investigations revealed normal thyroid function. Orbital magnetic resonance imaging detected a 4 × 2,2 × 2,7 cm septate pseudocystic mass in the right lacrimal gland. Given her lacrimal gland tumour diagnosis, the patient was submitted for neurosurgical intervention with total ablation of the tumoural mass and complete right dacryoadenectomy. Although the intraoperative extemporaneous examination results were suggestive of a haemangiopericytoma, histological and immunocytochemical examination showed an extrapleural SFT. The postoperative clinical evolution was favourable, with remission of the exophthalmia. Fifteen months after surgery, no signs of recurrence were noticed. Conclusions We report the first case of an SFT with cystic changes in the lacrimal gland. Although the presence of cavitary lesions alone does not necessarily indicate aggressive behaviour, cystic changes pose a risk of recurrence and may suggest malignant transformation over time. As a result, our case requires long-term follow-up due to recurrence and malignant potential.

Menopause comes with different changes including estrogen deficiency – related qualitative and quantitative progressive skeleton deterioration. The physiological process turns to pathology, respective osteopenia and osteoporosis at central DXA (Dual-Energy X-Ray Absorptiometry) or low lumbar – DXA derived TBS (Trabecular Bone Score) or increased 10-year probability of major osteoporotic/hip fractures provided by FRAX. We aim to briefly introduce the methods of menopausal fracture risk assessment if back pain is presented from an endocrine point of view. This is a vignette type of manuscript focusing on imagery tools. A 60-year old smoker woman with 10 years since menopause and prior corticotherapy accuses persistent back pain for a few weeks, with mild to medium intensity and responsive to small doses of pain medication. Profile plain X-Ray showed no vertebral fractures. FRAX estimated risk was low. Lumbar DXA (GE Lunar Prodigy) showed osteopenia (a T-score of -1.9 SD) while TBS showed a deterioration of lumbar micro-architecture based on a value of 1,199. Further recommendations of stop smoking, healthy diet, physical exercise, vitamin D and calcium supplementation are necessary. Retesting using DXA is not necessary sooner than 2 years or even longer. Related to back pain, rheumatologic and balneology support and intervention are required for spondylarthrosis. For daily practice, the approach of bone health and estimation of future fragility fractures in menopause combines gold standard DXA to most modern instrument TBS together with easy accessible online algorithms as FRAX. These do not display individual medical decision and adequate use of guidelines.

The thyroid cancer had an increasing frequency during the last decades mostly related to better and accessible detection methods. The papillary type has the major epidemiologic impact. We present a case with mixed symptoms before surgery, a rare combination of both thyrotoxicosis and thyroid malignancy and an unusual pathologic report in a 56-year-old female known with total hysterectomy at age of 42, diagnosed with toxic goiter 2 years ago and under intermittent therapy with thiamazol. She presented for unspecific hot flashes and palpitation at gynaecology and she was referred for endocrine evaluation that revealed unspecific cervical compression complains, and a large goiter. The Thyroid-Stimulating Hormone was normal under thiamazol and so were the thyroid antibodies and calcitonin. The computed tomography pointed an isthmus and left lobe nodule of maximum 7.28 cm with mass effect on trachea. Total thyroidectomy was performed without any significant complications. The patient was hospitalised for 4 days (less than 48 hours postoperatively). The pathological report showed a papillary thyroid cancer (stage III) with oxyphil variant and an insular and solid pattern component. A high proliferative index is detected by vessels invasion and a ki-67 of 15 %. She received radioiodine therapy and then she started suppression levothyroxine treatment. The insular and oxyphilic pattern as well as the large tumour size of 6 cm suggest a severe prognosis. On the other hand, it is still a matter of debate whether non-autoimmune hyperthyroidism and solid pathologic features subscribe to the hypothesis of an aggressive cancer phenotype. Lifelong follow up is needed.

Endosalpingiosis is a rare benign gynaecologic condition characterised by the presence of epithelial inclusions resembling tubal epithelium outside the fallopian tubes. It is usually found incidentally in postmenopausal women. The ovary seems to be the most common site of the lesions; however it can develop almost in any pelvic or abdominal organ including the lymphatic structures. We present the case of a 49-year-old patient who was submitted to surgery for bilateral ovarian tumors strongly resembling to malignant tumors. A total hysterectomy with bilateral adnexectomy, pelvic lymph node dissection and omentectomy were performed. The histopathological studies revealed the presence of bilateral ovarian endosalpingiosis associated with lymph node involvement.

Rarity of Sertoli cell tumours contributes to a low index of suspicion and therefore a thorough knowledge of the clinicopathological and immunological characteristics of such tumours is essential to diagnosis and proper management of the treatment and follow-up. The current narrative review of literature was planned to focus on ovarian Sertoli cell tumours that arise from the sex cords cells, which are typically benign unilateral neoplasia incidentally detected, or associated with hormonal hyperactivity, in women of reproductive age. A priory unpublished case of a 35-year old female is also introduced as the base of discussion Abdominal massrelated syndrome and vaginal bleeding anomalies have been reported. Genetic background, if presented, is mostly related to Peutz-Jeghers syndrome caused by STK11/LKB1 mutation. The tumour displays a microscopic tubular pattern and rarely displays cords or trabecular, retiform, spindles, diffuse or areolar structures. Although immunohistochemistry can be helpful in establishing the diagnosis, the results are sometimes inconclusive and the current results require new research to establish a specific immunological panel.

Premature ovarian failure is associated with many other autoimmune diseases. We report 3 cases reports in this paper associating thyroid autoimmune disease, and at least 1 more autoimmune pathology. A 45-year female is diagnosed at age of 42 with acute adrenal insufficiency, Hashimoto’s thyroiditis induced hypothyroidism, secondary amenorrhea (follicle-stimulating hormone (FSH) of 18 mUI/ml), and osteoporosis. Adrenal and thyroid substitution was started, and intravenously zoledronic acid. Within 6 months, the thyroid stimulating hormone normalized, and the menses re-started. A 64-year female has premature ovarian failure since age of 40, autoimmune hypothyroidism since age of 54, psoriasis vulgaris, and osteoporosis since the age of 53. Raloxifene was used for 3 years then Dual-energy X-ray Absortiometry pointed osteopenia that was conserved up to the present. A 53-year female with menopause since age of 40 is treated for autoimmune hypothyroidism, while scleroderma was diagnosed. Later esofagitis developed, as well as osteopenia. FSH was not as high as expected for ovarian insufficiency in case 1, suggesting a second mechanism of hypothalamic origin based on consumptive syndrome in severe glucocorticoids deficiency. Transitory secondary amenorrhea is an argument that re-balancing the general biochemical and endocrine parameters, might improve the ovarian function. The cases 2 and 3 had untreated premature ovarian failure but the therapeutic opportunity window was lost at the moment of admission. The skin lesions as psoriasis or sclerodermia are a marker of severity. We highlight the importance of autoimmune clusters including the thyroid pathologies in females underlying premature ovarian failure. We also encourage the routine bone mineral density check since menopausal osteoporosis/osteopenia might be already presented.

Background and aimThe cysts may potentially affect any organ; adrenals cysts are rare. This is a review of the literature regarding adrenal cysts, focusing on children and young adults.General dataThree major types have been described: pure cysts (endothelial, epithelial, and hemorrhagic or pseudocyst), parasitic (as hydatid) cysts and cystic part of a tumour (most frequent are neuroblastoma, ganglioneuroma, pheocromocytoma, and teratoma). The complications are: bleeding, local pressure effects; infection; rupture (including post-traumatic); arterial hypertension due to renal vessels compression. Adrenal hemorrhage represents a particular condition associating precipitating factors such as: coagulation defects as Factor IX or X deficiency, von Willebrand disease, thrombocytopenia; antiphospholipid syndrome; previous therapy with clopidogrel or corticosteroids; the rupture of a prior tumour. At birth, the most suggestive features are abdominal palpable mass, anemia, and persistent jaundice. Adrenal insufficiency may be found especially in premature delivery. The hemorrhage is mostly self-limiting. Antenatal ultrasound diagnosis of a cyst does not always predict the exact pathology result. The most important differential diagnosis of adrenal hemorrhage/hemorrhagic cyst is cystic neuroblastoma which is highly suggestive in the presence of distant metastases and abnormal catecholamine profile. The major clue to differentiate the two conditions is the fact that the tumor is stable or increases over time while the adrenal hemorrhage is expected to remit within one to two weeks.ConclusionPediatric adrenal cysts vary from simple cysts with a benign behavior to neoplasia- related lesions displaying severe prognosis as seen in cystic neuroblastoma. A multidisciplinary team is required for their management which is conservative as close follow-up or it makes necessary different surgical procedures in cases with large masses or if a malignancy suspicion is presented. Recently, laparoscopic approach is regarded as a safe procedure by some authors but generally, open surgery is more frequent used compare to adults; in most cases the preservation of normal gland is advisable.

Ovarian teratoma has various presentations. We present a 28-year female diagnosed with a tumour after a fainting episode. Medical history was non-contributory except for mild intermittent pelvic pain. Cardiologic and neurological evaluation found no cause of syncope. Abdominal ultrasound revealed a right ovarian tumour that was laparoscopically removed. The menses continued to be normal. The pathological exam showed an encapsulated tumour of 8 cm with hair and small bone parts (mature teratoma with a cystadenoma). Immunohistochemistry was positive for CK34beta E12 in stratified squamous epithelium of skin glands; positive CK7 in kidney tubular parts; positive actin in smooth muscle. The endocrine profile was normal and the patient remained asymptomatic. The challenging in the pathological report was to differentiate a cystic part of a solid tumour to a teratoma associated cystadenoma. No apparent cause of syncope was found so a possible tumour related local pain and inflammation mechanism might be involved.

Autoimmune thyroiditis (AI) and differentiated thyroid cancer as papillary type (PTC) are sometimes associated and several common pathogenic mechanisms have been described: BRAF mutations, hOGG1 loss of heterozygosity, interleukin-10 activation, selenoproteomas disturbances. Controversies are related to a more aggressive profile of PTC if AI is presented by interferences with oxidative stress and secondary carcinogenesis. This is a case report of a 37-year old female diagnosed a decade ago with multi-nodular goiter and hypothyroidism. She was treated in different endocrine centers. She started to accuse intermittent breathing difficulties which were not related to her previous diagnosis of asthma. On admission, the thyroid function was normal under levothyroxine (LT4) therapy with high anti-thyreoperoxidase antibodies levels of 1000 UI/mL (Normal

Papillary thyroid carcinoma (PTC) is well known as a differentiated thyroid carcinoma with an established treatment protocol and high survival rates. Nevertheless, its tall cell variant (TCV) is more frequent diagnosed with larger tumor size, advanced age and metastasis comparing to other PTC cases, resembling an increased level of aggressiveness attributable to the histological subtype according to recent studies. We present the case of a 60-year-old woman who came for a recently increase of the thyroid and a left laterocervical mass associated with pain and alteration of general status. Thyroidectomy revealed a TCV of PTC with infiltration of the extrathyroid tissue. The laterocervical mass posed difficulties to surgical excision but in a second attempt proved to be an undifferentiated carcinoma. The findings were associated to a rapid deterioration of the patient's general condition finalized with death within less than three months from the initial presentation.

The adrenal tumours represent a large area of pathological diagnosis. The radiological and endocrine features are the best tools in patients’ management. The medical history of a cancer increases the metastasis suspicion. We present a 64-year-old hypertensive female with a previous diagnosis (20 years ago) of uterine cervix cancer that was remitted after surgery and local cobalt therapy. Four years before she was found with a non-secretor right adrenal tumor of 2 cm during a routine ultrasound for a kidney stone crisis. Currently she is admitted for an endocrine control: a mildly increased serum chromogranin A and neuron specific enolase with basal plasma metanephrines 4 times above the upper normal limit and normal plasma normetanephrines are found. The morning plasma cortisol after 2 days of dexamethasone 2 milligrams is 2.3 micrograms/decilitre. The contrast computed tomography of the pelvis, abdomen and thorax found no anomalies but the right adrenal mass with enlarged diameters of 2.55 cm × 4 cm × 4.54 cm. Laparoscopic right adrenalectomy was performed without adrenal insufficiency after surgery, neither remarkable changes in arterial blood pressure profile. The pathological report confirmed an adrenocortical adenoma. The macroscopic aspects showed a yellow tumor of 4 cm. The immunohistochemistry revealed weak positive reaction of melan A+, INHIBIN and SYN (in some tumor areas), negative CROMO (into the tumor cells and positive in normal adrenal medulla), with an index of proliferation (Ki67) of 2%. 6 months after surgery the patient was clinically stationary and endocrine follow-up is recommended. The radiologic and endocrine anomalies related to an adrenal mass in a patient with oncologic medical history may conclude to the idea of tumor removal for a certain diagnosis provided by pathological exam.

Adrenocortical carcinomas are rare tumors in adults. They can be hormonally active and detected by a hormonal excess, or be non-secretory tumors. In the latter case, they become symptomatic after a long period of growth, usually after they have already invaded the surrounding tissues. In these cases, multiple visceral resections are sometimes required in order to obtain a complete R0 resection. We present the case of a 65-year-old patient who was addressed to our service for a giant abdominal tumor with compression phenomena in whom we performed a complete resection en bloc with left nephrectomy and adrenalectomy, distal pancreatectomy, splenectomy, left colectomy and para-aortic lymph node dissection. The early postoperative course was uneventful, the patient was discharged eight days after surgery. The histopathological findings revealed an adrenocortical carcinoma with no lymph node metastases.

The masses of the mediastinum may present as cystic lesions and the underling pathological report varies as well as their malignancy profile. The diagnosis may be established during adulthood years despite the fact that many of them are embryological remnants. Surgical approach is the best option because of compressive effects especially in large tumours.

Background: The tyrosine kinase inhibitor, a new treatment option in hepatic carcinoma, may associate thyroid dysfunction like spontaneously remitting thyrotoxicosis followed by hypothyroidism. Case report: A 66-year Caucasian female was first diagnosed and operated for a moderately differentiated rectal adenocarcinoma of 5 centimeters. Immunohistochemistry showed positive VEGF receptor 2 (Flk-1, KDR), VEGF receptor 1(Flt-1), and a Ki67 of 30%. 4 years later a hepatic adenocarcinoma (clear cells variant) was diagnosed. After surgery, daily 400 mg of sorafenib was introduced. Three months later mild symptomatic thyrotoxicosis was seen: palpitations, fatigue, and mild bilateral pedal clonus. Thyroid-stimulating hormone (TSH) was suppressed (0.044 μIU/mL, normal levels between 0.4 and 4.5 μIU/mL), and free levothyroxine (fT4) elevated. The TSH receptor antibody, the antithyreoglobulin and antithyreoperoxidase antibodies were negative. Thyroid ultrasound pointed hypoechogenic, inhomogeneous aspects. She received beta-blocker and within two months thyrotoxicosis remitted but TSH progressively increased suggesting hypothyroidism with level less 5 μIU/mL so no replacement levothyroxine therapy was added yet. Discussions: The exact mechanism of the tyrosine kinase inhibitors-related thyroid malfunction is not fully understood. Non-autoimmune destructive thyroiditis of unknown trigger causes thyrotoxicosis and later hypothyroidism as seen in our case. The clinical features vary from one person to another. The hormone replacement is rarely necessary. The baseline cancer seems irrelevant for thyroid toxicity. In our unusual case the patient had a history of two metachronous cancers. The thyroid follow up is essential during each patient therapy yet a specific pattern of follow-up is not precisely designed. Conclusion: The tyrosine kinase inhibitor-induced thyroid dysfunction includes both thyrotoxicosis and hypothyroidism. We emphasize the idea of periodic endocrine evaluation in oncologic patient treated with this class of drugs.

Tuberculosis continues to be a universal health problem and recent studies showed an increased incidence of all forms of this disease. We report the case of a 81 year old female patient with a large left cervical tumour and cutaneous lesions that proved to be an unusual form of tuberculosis. The patient had a 2 years history of slowly increasing left cervical mass with no significant clinical manifestation. Medical assistance was requested mostly by the family, concerned about the large mass visible on the left side of the patient’s neck. Clinical and laboratory investigation revealed: a large left laterocervical tumour and the presence of erythematous, non pruriginous skin lesions. No significant changes were shown by routine blood tests. Thyroid function was in the normal range. Neck ultrasound revealed the presence of a 5/6 cm, well defined hypoechoic mass and the thyroid scan showed no accumulation of the radiotracer in the cervical tumour. Routine tuberculosis test was negative and the patient had no sign of pulmonary involvement. Histopathological examination after ablation of this tumour showed the presence of granulomatous lesions suggesting tuberculosis or sarcoidosis. The same aspect was seen after histopathological examination of the specimens from cutaneous biopsy. The diagnosis was certified by the PCR amplification of the DNA extracted from the skin lesions, which led to the identification of Mycobacterium tuberculosis. The cutaneous lesions were clearly improved after tuberculostatic treatment, supporting the hypothesis of a rare form of cutaneous tuberculosis.

The aim of this study was to make immunohistochemical analyses with Inhibin alpha-subunit, Melan A and MNF116 (pan-Cytokeratin antibody) in pheochromocytomas, because immunohistochemistry is useful for the distinction between adrenal tumors.We used 20 patients with pheochromocytomas submitted to laparoscopic (n=19) or classical (n=1) surgery and we have explored immuno-staining with Inhibin alpha-subunit, Melan A and MNF116 in these tumors. This can be helpful when we cannot make the distinction between adrenal tumors.Pheochromocytomas did not stain with Inhibin alpha-subunit, Melan A and MNF116.In our study, Inhibin alpha-subunit, Melan A and MNF116 were not sensitive for pheochromocytomas.

The neuroendocrine tumors (NETs) have an increased incidence related to the age. Secondary osteoporosis might be found in patients with bone metastases and in those with NETs associated Cushing's disease or primary hyperparathyroidism. Primary osteoporosis might be found in postmenopausal women, but in case with non-metastatic NET as G1 NET it is difficult to establish the NET contribution to the bone loss. We present the case of a 53-year-old female accidentally diagnosed with G1 lung NET after surgery of the tumor. The immunohistochemistry pointed positive reaction for CHROMO, SYN and negative for CK7 and TTF1, and a Ki67 of 1-2% (well-differentiated neuroendocrine tumor). The central Dual X-Ray Absorptiometry (DXA) showed osteoporosis based on a T-score of -3. The patient had normal neuroendocrine markers and she was asymptomatic. She remained so for one year and the only therapy provided was weekly alendronate with adequate vitamin D and calcium supplements. Based on the pathological and immunohistochemistry profile, the low risk NET was diagnosed. We encourage the skeletal status assessment as central DXA in postmenopausal women with NETs, regardless clinical evidence of bone loss. The future will provide more epidemiological and pathogenic connections between the two dynamic fields of medicine as neuroendocrine tumors and osteoporosis.

The G2 neuroendocrine tumors (NET) or well-differentiated neuroendocrine carcinomas (2010 WHO Classification of Tumours of the Digestive System) embrace different types of evolution despite the fact that they actually are included in the same group of prognosis based on mitotic count and the Ki-67 proliferation index.We studied the pathological and clinical aspects in patients with G2 NET.This is a retrospective pilot observational study in patients admitted between January 2008 and January 2013 in "Constantin I. Parhon" National Institute of Endocrinology, Bucharest, Romania. They were evaluated based on the pathological report, imagistic scan, and neuroendocrine markers.Nine patients (female/male ratio: 5/4) with G2 NET were included (mean age at diagnosis 54.11 years). Surgery was performed in 66.66% of cases. 44.44% of tumors had unknown origin. 22.22% of patients had negative immunostain for chromogranin A. Synaptophysin was positive in all cases. Neuronal specific enolase (NSE) was performed in 44.44% of cases and it was positive in all these situations. 88.88% of patients had high neuroendocrine markers. Multiple tumors were found in two cases (follicular thyroid adenoma, and a carcinoma of the port vein, respective bilaterally pheochromocytomas). The youngest patient (39-year-old) had atypical onset with bilateral adrenal tumors (positive for CHROMO, EMA, CK-19, CK-20, negative for SOMATO, CK-7, S-100, glucagon, CD57, and a Ki-67 of 15%). Death was registered in two cases, both with bone metastases.Some poor prognosis factors may be taken into account as lack of CHROMO immunostain, young age at diagnosis, genetic background, and lack of therapy options as surgery. Larger databases will provide more information.It is possible that the G2 NET group of tumors actually includes some different subtypes or in fact, a late diagnosis of the tumor might be associated with a poor diagnosis.

Dedifferentiation is a process that may be found in metastasis from the neuroendocrine tumors. We present the case of a female within the seventh decade of life incidentally diagnosed with a mature teratoma with element of cartilages, bronchia, mucinous glands, and a poorly differentiated neuroendocrine carcinoma (Ki67 of 30%). After six months of chemotherapy and another six months of disease free interval, a metastasis of the great omentum was removed. The dedifferentiation was diagnosed based on much higher Ki67 (of 70%). The loss of estrogen receptor of 40% from the initial site to 3% into metastasis indicates an exclusive neuroendocrine aggressive pattern. The ovarian carcinoid is a rare situation, and metastasis to the great omentum with dedifferentiation is even seldom.

Type 1 neurofibromatosis associates various abdominal tumors as gastrointestinal stromal tumors, duodenal or pancreatic carcinoid, and adrenal tumors like pheochromocytoma. We present the immunohistochemistry report in two cases with different profile regarding the evolution. One case is a 7th decade women diagnosed with unilateral pheochromocytoma and GISTs, with a good prognosis after surgery. The other case is a 41-year-old male diagnosed with duodenal metastatic somatostatinoma after an intestinal occlusive syndrome and later the hormonal profile leaded to the diagnosis of pheochromocytoma. The patient had a fulminate evolution within six months from diagnosis.

Thyroid metastasis is atypical. We present a 70-year-old female case that was first diagnosed as gastric cancer after surgical approach. Two years later a thyroidectomy was performed and the immunohistochemistry (IHC) profile revealed a neuroendocrine tumor (NET): poorly differentiated neuroendocrine carcinoma (with small cells), with positive reaction for SYN, CROMO, negative for calcitonin, TTF1 and thyreoglobulin. The Ki-67 index was 25%. Considering the unusual metastasis, the IHC exam of the stomach tumor was performed pointing the same features as the thyroid findings. This proved that the thyroid tumor was a metastasis from a primary gastric neoplasia. This is an unusual case of NET because of the thyroid involvement. Nevertheless, the IHC exam played the major role in elucidating the diagnosis and the prognosis of the case.

One of the rarest situations regarding an adrenal incidentaloma is an adrenal cyst. We present the case of a 61Z–year old male patient diagnosed with peritonitis. During surgery, a right adrenal tumor of 2 cm is discovered. The patient was referred to endocrinology. 6 months later the diameter of the tumor is 7 times bigger than the initial stage. It has no secretory phenotype, except for the small increase of serum aldosterone and the 24–h 17–ketosteroids. Open right adrenalectomy is performed and a cyst of 15 cm is removed. The evolution after surgery is good. The pathological exam reveals an adrenal cyst with calcifications and osteoid metaplasia. The immunohistochemistry showed a positive reaction for CD34 and ACT in the vessels and VIM in the stroma. The adrenal cysts are not frequent and represent a challenge regarding the preoperative diagnostic and surgical procedure of resection. The pathological exam highlights the major aspects.

Diffuse sclerosing variant (DSV) is a rare variant of papillary thyroid carcinoma (PTC) and its features have not yet been fully characterized. The aim of this case report is to analyze the immunohistochemistry profile of this disease and to highlight this rare entity. We analyzed the histology and immunohistochemistry of a female patient admitted in the Surgery Department of the "Sf. Ioan" Emergency Hospital, Bucharest, in April 2008. We especially used a wide panel of antibodies (Thyroglobulin mono, Thyroglobulin poly, Ki-67, TTF 1, Cytokeratin 19, Cytokeratin 34betaE12, and p63) in order to point out the follicular origin of the cells and to investigate the extensive squamous metaplasia lesions. The immunohistochemistry (IHC) was performed on 3 mum thick sections from 10% formalin-fixed paraffin-embedded tissues, according to the indirect tristadial Avidin-Biotin-Complex method. Our case report reveals that the DSV of PTC has distinctive pathologic features and its diagnosis importance is suggested by the high incidence of recurrence after operation.

A small percentage of ovarian neoplasms are transitional cell tumors, which proves to be a distinct group with various histological and immunohistochemical patterns. In this study, 13 archived formalin-fixed paraffin-embedded samples of transitional cell tumors of the ovary have been assessed using standard HE stain and the indirect tristadial ABC peroxidase IHC method for 11 antibodies (CA125, CK7, CEA, EMA, MNF116, CK20, Vim, ER, PgR, PCNA, Ki-67). More than 50% were malignant Brenner tumors. CA125 was positive in all malignant tumors (of Brenner type and transitional cell carcinomas), but not in benign and borderline tumors, while CK7 was positive in approximately 70% of all cases. These two antibodies have shown a high sensitivity and low specificity, but do not correlate to each other. PCNA was positive in the study batch with a mean value of 40% and Ki-67 with a mean value under 25%. A direct correlation statistically significant has been noted between the aforementioned proliferation factors and the tumor grade (r = 0.4, p = 0.05). The other markers were unspecific, with low sensitivity and independently of the histopathological type.