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Any instrumental examination may lead to unexpected diagnosis that in turn can radically change the clinical pathway of a patient.

The aim of the study was to evaluate survival in patients with advanced glottic laryngeal squamous cell carcinoma treated by bioradiotherapy (BioRT) with cetuximab and eventual salvage surgery (group A, n = 66) or upfront surgery (total laryngectomy or near-total laryngectomy) with or without postoperative radiotherapy (PORT) (group B, n = 66). The predictive role of HER1 expression in the bioselection of tumors was evaluated. Relapse-free (RFS), metastasis-free (MFS), overall (OS) survivals, salvageability, and rates of larynx preservation were analyzed. The two groups were balanced by propensity score method on their baseline characteristics. No significant differences in RFS and OS were found, while MFS results were significantly higher in group A (p = 0.04). Group A showed a 22% reduction in the probability of nodal metastasis (p = 0.0023), mostly in tumors with higher HER1 expression. The salvageability with TL at 3 years was 54% after prior BioRT and 18% after prior upfront NTL (p < 0.05). BioRT with cetuximab showed a reduction in the risk of lymph node relapse, particularly in the case of HER1 positive tumors, and it allowed to achieve a higher rate of functional larynx preservation and a higher salvageability compared with upfront surgery. HER1 analysis could be clinically useful in the bioselection of tumors that may benefit from BioRT with cetuximab, particularly in those with neck node metastatic propensity.

Microglia and macrophages appear to be the most common cells in the GBM microenvironment. In the present study we investigated the status of macrophages/microglia activation in surgical specimens from 41 patients diagnosed with grade IV GBM. For each patient we analyzed both the center of tumor and the parenchyma surrounding the tumor. The specimens were stained for: i) IBA1, a 17-kDa EF hand protein specifically expressed in microglia/macrophages ii) CD163, a cell surface antigen associated with M2 phenotype; iii) iNOS, taken as a functional marker of M1 phenotype, and iv) ARG-I, taken as a functional marker of M2 phenotype. Staining was scored in a double-blinded score on a scale from 0 to 5. Our results suggest that CD163 expression is higher within the tumor than in surrounding periphery in both male and female patients; while iNOS is higher within the tumor in males, no significant difference was found for ARG-1. In addition, analyzing the data in TGCA database, we found that CD163 expression was significantly and inversely correlated with mean survival times, with average survival times ranging from 448days in patients having low expression, to 319 in mid, and 353 in patients with high CD163 expressing tumors. In contrast, no significant association was found between survival time and ARG-1 or iNOS expression.

Background Cardiac synovial sarcoma (CSS) is an extremely rare malignant tumor with a severe prognosis, due to frequent relapses and metastases. To obtain useful information for treatment protocols, we analyzed survival and therapy data from the cases reported in the literature. Methods A search of MEDLINE was performed throughout December 2018. Using key words relating to primary CSS, we collected from the literature a total of 97 cases, mainly consisting of single case reports. To identify predictors of overall survival, statistical analyses were performed on a selected cohort of 55 patients for whom relevant clinicopathological data were available, including surgery and adjuvant therapy. Results The univariable analysis revealed that patients in their first three decades of life have better overall survival. The univariable analysis also showed that patients not receiving adjuvant chemotherapy are at increased risk of death. In the multivariable analysis, tumor resection and chemotherapy are factors significantly improving overall survival. Conclusion The survival of patients with CSS is positively influenced by a young patient's age and greatly improved by the administration of chemotherapy, even in the absence of tumor resection.

Neuromuscular choristoma (NMC), also called neuromuscular hamartoma or nerve rhabdomyoma, is a rare lesion of the spinal and cranial nerves composed of skeletal muscle intimately associated with nerve fibers. Its origin has not been precisely clarified and a malformative event, resulting from aberrant differentiation or a true neoplastic growth, have been proposed by authors. We hereby present a cerebellopontine angle NMC enlarging the eighth cranial nerve in a 3-year-old child, that histologically appeared composed of a large amount of striated muscle mixed with nerve fibers. We also provide a review of the intracranial NMC cases reported in the literature and an analysis of proposed hypotheses to explain the presence of muscle cells in nerve trunks.

Background Minichromosome maintenance protein 7 (MCM7) is a downstream of human epidermal growth receptor (HER1) signaling. We examined MCM7, geminin, and HER1 expression in patients with laryngeal squamous cell carcinoma (SCC) treated with radiotherapy and cetuximab. Methods MCM7, geminin, and HER1 were evaluated by immunohistochemistry on 61 patients with laryngeal SCC. The follow-up (median, 32.1 months; range, 2–139 months) went from the beginning of therapy to tumor progression-free survival (PFS) and death (overall survival [OS]). Results MCM7, but not geminin, was associated only with HER1 expression, whereas no association was found with other clinicopathological characteristics. Patients with MCM7 high - geminin high and MCM7 high - geminin low tumor status had a risk of progression 3.1 times and 17.7 times greater, respectively, than patients with MCM7 low – geminin high tumor status. Tumor site, MCM7, and geminin were independent determinants of PFS, whereas MCM7 was an independent prognostic marker of OS. Conclusion MCM7-geminin tumor status may be prognostic for patients with laryngeal SCC treated with cetuximab and radiotherapy. © 2016 Wiley Periodicals, Inc. Head Neck, 2016

The occurrence of ganglion cells in the sella turcica, in association or not with a pituitary adenoma, has been rarely reported. Various names have been employed for this rare entity, gangliocytoma being frequently used and recommended by WHO classification. Expression of cytokeratin in these ganglion cells has been previously occasionally reported, a very intriguing observation raising questions on the possible nature and derivation of these cells. We describe the pathological findings in three cases of growth hormone-producing adenomas, all sparsely granulated, showing the presence of a ganglion cell population admixed with an adenomatous component. A review of the literature is also provided.

Under the definition “glomus tumors” are often erroneously enclosed neoplasms that are absolutely unlike for origin, location, and behavior. Glomus tumors (GTs) are small but extremely painful skin tumors of mesenchymal origin. GTs derive from the neuromyoarterial glomus in adults of middle-age and are generally benign. Due to their small size, diagnosis is often difficult and patients harboring these tumors usually consult many physicians, including sometimes neurosurgeons. More familiar to neurosurgeons are neoplasms as glomus jugular, glomus vagale, and glomus tympanicum that instead all belong to the family of paragangliomas (PGs) and for this reason should not be confused with the aforementioned skin tumors. Here we present a brief review of these two different classes of tumors and also the clarification of any misunderstanding that may derive from an improper use of the terminology. In order to illustrate why skin tumors may interest neurosurgeons, we have reviewed our institutional series of outpatient surgical procedures. Differential diagnosis with other tumors that appear as cutaneous nodules is also discussed. From January 2012 to May 2015, seven patients harboring a GT (six male and one female) were treated. The age ranged from 34 to 71 years (mean, 54.1). The clinical suspect of GT, was validated by ultrasound (US) and, if necessary, by magnetic resonance imaging (MRI). All patients underwent surgery for total tumor removal. Immediate pain relief was obtained in all the patients, and no recurrences were observed during follow-up. Histology confirmed the diagnosis of GT. Subcutaneous painful nodules, originating from the glomus body, are properly called GTs. Unlikely from other tumors, as schwannomas or neurofibromas, GTs are the cause of pain that is disproportionate to their tiny size and that is not associated to neurological disturbances. Surgical treatment allows a complete regression of pain with significant patient satisfaction. Neoplasms originating from neuroepithelial cells, on the contrary, should not be defined as GTs.

Adrenocortical carcinomas (ACCs) with sarcomatous areas represent an extremely rare type of highly aggressive malignancy of unknown molecular pathogenesis. The current study was planned to gain insight into its molecular genetics using a targeted next-generation sequencing approach and to explore the status of epithelial-mesenchymal transition (EMT)-associated markers (E-/P-/N-cadherins, MMP-2/-9 and caveolin-1), downstream transcriptional regulators of EMT-related signaling pathways (ZEB-1/-2, Slug), stem cell factors (Oct3/4, LIN28, SOX2, SO17, NANOG, CD133, nestin), and markers of adrenocortical origin/tumorigenesis (SF-1, β-catenin, p53) in phenotypically diverse tumor components of 6 cases. Thirteen pathogenic variants of ACC-associated TP53 and CTNNB1 genes were detected in epithelial and/or nonepithelial components in 4 out of 6 tumors. Three cases had identical mutations in distinct components, 1 of which contained TP53/CTNNB1 in 3 out of 5 components, whereas 1 harbored a single TP53 mutation only in the nonepithelial component. By immunohistochemistry, SF-1 and E-/P-/N-cadherins were found positive only in the epithelial component of all cases, whereas the nonepithelial components were mainly enriched for nestin, ZEB-1, and MMP-2/-9. β-Catenin demonstrated an aberrant nuclear localization in the sarcomatoid component of 5 cases, whereas p53 was strongly positive in nonepithelial constituent in 4 of 6 cases. In summary, we have shown that Wnt/β-catenin signaling pathway dysregulation and mutational inactivation of TP53 are common genetic events in sarcomatoid ACCs, a subset of which being monoclonal in origin. These tumors are enriched for EMT-related markers and stem cell factors, potentially conferring a poor prognosis, which might be exploited as novel therapeutic targets.

Introduction: Primary lymphomas of peripheral nerves are extremely rare, and only a few cases have been reported. Methods: We describe the clinical, neurophysiological, radiological, and pathological findings in a 61-year-old woman affected by primary multifocal lymphoma of the peripheral nervous system without systemic involvement. Results: Fascicular left femoral nerve biopsy was decisive for the diagnosis of diffuse large B-cell non-Hodgkin lymphoma. Magnetic resonance imaging, fluorine-18 fluorodeoxyglucose positron emission tomography computed tomography, and nerve ultrasound contributed to the diagnosis. Conclusions: Primary lymphoma of peripheral nerves (PLPNs) is a rare but potentially treatable condition, which is frequently misdiagnosed. In the literature, there are very few descriptions of PLPNs, most of which are mononeuropathies. The possibility of a neuropathy associated with lymphoma should be considered in patients with poor response to treatment and severe pain symptoms. Muscle Nerve 50: 1016–1022, 2014

Myeloid sarcoma (MS) is a localized extra-medullary tumor mass of immature myeloid cells, arising de novo or related to acute myeloid leukemia, of which it can be a forerunner, a coinciding or late event. Less commonly, MS represents an acute blastic transformation of myelodysplastic syndromes or myeloproliferative neoplasms. This rare condition commonly consists of a proliferation of more or less immature cells with a myeloid immunophenotype, very exceptional cases showing a megakaryoblastic or erythroid differentiation. The most common localization of MS is the skin, lymph node, soft tissues and bones, but CNS involvement is exceedingly rare, with no cases reported in the sellar region. We report a 54-year-old man, affected by myeloproliferative neoplasm, JAK2 V617F-positive of 13 years duration, who acutely presented with a third cranial nerve palsy; neuroradiology documented a space-occupying lesion at the level of the sellar, upper clival and right parasellar regions, that was sub-totally removed with a trans-sphenoidal approach. The histological examination documented a proliferation of large, blastic cells, frequently multinucleated; a diagnosis of MS with megakaryoblastic differentiation, arising in a background of chronic idiopathic myelofibrosis, was suggested by immunohistochemistry, owing to CD42b, CD45, CD61 and LAT (linker for activation of T cells) positivity. In addition, homozygous JAK2 V617F mutation was detected from the myeloid sarcoma specimen. A few weeks after surgery, an acute blastic leukemic transformation occurred and, despite chemotherapy, the patient died 2 months after surgery. To the best of our knowledge, this is the first MS case with megakaryoblastic differentiation arising within the CNS.

Minichromosome maintenance protein 7 (MCM7) is a downstream of human epidermal growth receptor (HER1) signaling. We examined MCM7, geminin, and HER1 expression in patients with laryngeal squamous cell carcinoma (SCC) treated with radiotherapy and cetuximab.MCM7, geminin, and HER1 were evaluated by immunohistochemistry on 61 patients with laryngeal SCC. The follow-up (median, 32.1 months; range, 2-139 months) went from the beginning of therapy to tumor progression-free survival (PFS) and death (overall survival [OS]).MCM7, but not geminin, was associated only with HER1 expression, whereas no association was found with other clinicopathological characteristics. Patients with MCM7 high - geminin high and MCM7 high - geminin low tumor status had a risk of progression 3.1 times and 17.7 times greater, respectively, than patients with MCM7 low - geminin high tumor status. Tumor site, MCM7, and geminin were independent determinants of PFS, whereas MCM7 was an independent prognostic marker of OS.MCM7-geminin tumor status may be prognostic for patients with laryngeal SCC treated with cetuximab and radiotherapy. © 2016 Wiley Periodicals, Inc. Head Neck 39: 684-693, 2017.

Chordomas arise in the skull base and spine and usually occur in adults and are rare in the pediatric population. Cases of chordoma in pediatric age are often poorly differentiated, showing cytologic atypia, increased cellularity, and mitosis, and their aggressive behavior is associated with a high incidence of metastatic spread and a short patient survival. Recent studies have described loss of SMARCB1/INI1 protein in poorly differentiated chordomas associated not with point mutations but with SMARCB1/INI1 gene deletions instead. In this study, we considered immunohistochemistry and SMARCB1/INI1 mutational status to examine SMARCB1 status in a series of pediatric chordomas (7 classic and 1 poorly differentiated). We performed immunohistochemical tests for INI1, brachyury, S100, and cytokeratins and conducted a genetic analysis on the SMARCB1 coding sequence (NM_003073) using the Sanger method and multiplex ligation-dependent probe amplification to detect abnormal copy numbers of the gene locus. All 8 cases were positive for brachyury, whereas there was no nuclear SMARCB1/INI1 expression in 4 of the 8 cases, including the poorly differentiated chordoma. Genetic analysis identified a missense mutation in 2 cases and a nonsense mutation associated with loss of SMARCB1/INI1 protein and features of poorly differentiated tumor in 1. These mutations were novel variants occurring in heterozygosity, and they were judged to be pathogenic by 3 different bioinformatic tools. In 7 of 8 cases we performed multiplex ligation-dependent probe amplification, and 3 cases showed deletions at the SMARCB1 locus. Our results confirm the pathogenic involvement of SMARCB1/INI1 in childhood chordoma. We also describe 3 novel pathogenic mutations.

Malacoplakia is a rare inflammatory condition characterized by the accumulation of benign macrophages associated with pathognomonic Michaelis-Gutmann bodies (MGBs). It is usually found in the genito-urinary tract, and has been associated with immunocompromised states. In this short report, we present 5 patients with pulmonary nodules clinically suspicious for primary or metastatic lung cancer. The histologic examination of the surgical specimens revealed a nonspecific granulomatous chronic disease, and despite the paucity of classical MGBs, a pulmonary malacoplakia was suspected. In all cases the opportunistic pathogen Rhodococcus equi (R. equi) was identified by 16S rRNA gene sequence analysis, leading to the final pathological diagnosis of malacoplakia. We conclude that pulmonary malacoplakia associated with R. equi is a rare disease affecting also immunocompetent patients. The pathogenesis and the diagnostic problems are discussed. Since infection by R. equi is treatable, the importance of its early recognition should be emphasized.

Primary, adult-type bone fibrosarcoma is an uncommon, malignant spindle-cell tumor of fibroblastic origin, rarely affecting children. Most frequently diagnosed among bone malignancies in the past, improved diagnostic techniques and further restrictive classification criteria have currently made the diagnosis of fibrosarcoma very unusual. We hereby report the case of a 7-year-old child with a right frontal swelling mass. A computed tomography scan showed an osteolytic lesion of the right frontal bone, involving the diploe and the outer table of the skull. An en bloc surgical excision, followed by a thorough immunohistological evaluation, led to the diagnosis of fibroblastic proliferation, with low cellularity and minimal atypias. The patient had four recurrences during the 4-year follow-up. With an increasing histological grade at recurrences, a diagnosis of adult-type fibrosarcoma was made. To the best of the authors’ knowledge, this is the first reported case of an adult-type fibrosarcoma arising in the frontal bone of a child.

BackgroundKi-67 labeling index (LI) is currently regarded as a useful prognostic marker of pituitary adenoma (PA) clinical behavior, although its relevance as a reliable clinical indicator is far from being universally accepted, since both validations and criticisms are found in the literature. Minichromosome maintenance 7 (MCM7), a cell-cycle regulator protein, has been recently proposed as a marker of tumor aggressiveness in tumors from many sites, including the CNS. Therefore, we evaluated MCM7, in comparison to Ki-67, as a potential marker of clinical outcome in PA.Design and methodsIn this single-institution retrospective study, 97 patients with PA (23 ACTH, 12 GH, 29 PRL, 10 FSH/LH, and 23 non-secreting adenomas) were recruited and the prognostic value of both MCM7 and Ki-67 was evaluated by immunohistochemical techniques. In addition, p53 nuclear expression and mitotic index were also evaluated.ResultsTwenty-six of the 97 PA patients recurred during the follow-up period. Cox's regression analysis showed that high nuclear expression of MCM7 LI, unlike Ki-67 LI, was directly associated with a higher (7.7-fold) risk of recurrence/progression. Kaplan–Meier analysis of recurrence/progression-free survival curves revealed that patients with high MCM7 LI (≥15%) had a shorter recurrence/progression-free survival than those with low MCM7 LI (ConclusionsData from this study suggest that MCM7 is a prognostic marker of clinical outcome in PA patients, more reliable and informative than Ki-67.

BACKGROUND Primary intramedullary neurocytoma is extremely rare. Due to its rarity, it is difficult to collect a wide series in a single institution to perform a survival analysis and give indications about prognosis and treatment. CASE DESCRIPTION Here, we report on a case of a 37-year-old woman with primary intramedullary neurocytoma and perform a systematic statistical analysis of cases reported in the literature. Of 21 articles found, 15 studies and the present case (22 patients) were eligible for the analysis. We studied the impact of age, sex, number of involved levels (≤2 vs. >2), entity of resection, postoperative radiotherapy (RT), proliferation index (Ki67) on the neurological outcome of patients, and on the recurrence of the tumor. Comparison of categorical variables was performed by the χ (2) statistic. The Kaplan-Meier curves were plotted to calculate the progression-free survival (PFS) of these patients. P < 0.05 was considered as statistically significant. The 1-year PFS was 95.45% and the 2-year PFS was 86.36%. A gross total resection was associated to a worsening of the neurological function with no impact on the tumor recurrence. Adjuvant RT significantly improved the neurological function. A lower Ki67 was strongly associated with a lower tumor recurrence. CONCLUSIONS We think that the goal of the surgery should be to preserve a good neurological function even if a residual tumor has to be left. Ki67 should be always evaluated due to its impact on the prognosis. Although adjuvant RT significantly improved the neurological function, its role in preventing the tumor recurrence is not clearly defined.

A rare case of synchronous bilateral testicular germ cell tumor of different histological type is described. We report the case of a 39-year-old man with this unusual condition and discuss the value of the histopathological diagnosis in therapy and prognosis. A thorough review of the literature is also presented, and for the first time the precise histological subtype of each reported tumor, as diagnosed by the authors of the original paper, is illustrated.

Sir, We read with great interest, the recent review about capillary hemangiomas of cauda equina published by Liu et al.[2] Reviewing this article and the pertinent literature we noted that no intraoperative picture of a capillary hemangioma of cauda equina has ever been published until now. A 45-year-old woman with 2 years history of low-back pain was recently admitted to our department. On admission, neurological exam was negative. Spinal magnetic resonance imaging showed a space occupying lesion at L2 level displacing the cauda equina roots [Figure 1]. She was then submitted, under neurophysiological monitoring, to surgery for tumor removal. After an L1–L2 laminotomy and opening of dura mater, the lesion was identified [Figure ​[Figure2a2a and ​andb].b]. The lesion appeared easily detachable from the healthy nerve roots. Thus, after dissection, the pathological nerve root was cut proximally and distally allowing the total removal of the lesion [Figure ​[Figure2c2c and ​andd].d]. Microscopically, the lesion was composed of many thin-walled vascular channels, lined by endothelial cells with interposed nerve fibers [Figure 3]. Thus, a diagnosis of nervous capillary hemangioma was made. Postoperatively the patient complained of mild hyposthenia in left leg, totally recovered at 3 months follow-up. Figure 1 Sagittal (a) and axial (b) postcontrast T1-weighted spinal magnetic resonance imaging showing a well-circumscribed lesion at L2 level with intense enhancement after gadolinium administration Figure 2 Intraoperative view of lesion. The lesion appeared as a white reddish elliptical-nodular lesion intimately involved with the nerve root displacing the cauda equina (a) Enlarged vessels were also evident along the nerve root (b, white arrow). After dissecting ... Figure 3 Histological picture (a) of the surgical specimen shows many, variably sized, thin-walled vascular channels lined by endothelial cells with interposed nerve fibers (black arrows) (H and E, original magnification ×20). Immunohistochemical staining ... To our knowledge, we report the first intraoperative picture of a capillary hemangioma of cauda equina. Only a few cases of capillary hemangiomas have been reported at cauda equina.[1,2] Histopathological examination is needed for diagnosis. Typical features are the presence of a myriad of small capillary sized vessels, reticularly arranged with normal nerve fascicles dispersed within the nodules of clustered capillaries.[3,4,5] Our picture confirms that, according with the microscopically examination, this tumor arises and develops intrinsically to the nerve root. Surgery is the therapy of choice, but a total removal is possible only cutting (cranially and caudally to the tumor) the pathological nerve root.

Aims We describe the fourth reported case of granulomatous gastritis associated with gastric adenocarcinoma, with a review of the literature and considerations about the prognostic implications of this association. Results A 48-year-old woman who had been suffering from gastritis for ten years was admitted to our institute for increasing left epigastric pain associated with vomiting. After an endoscopic biopsy had revealed an ulcerated signet ring cell carcinoma, the patient was submitted to subtotal gastrectomy with regional lymph node dissection. Pathological examination of the resected specimen revealed a superficial signet ring cell carcinoma (early cancer) associated with multiple granulomas. The granulomas, which were observed within the mucosa and the submucosa at the periphery of the carcinoma, were composed of CD68-positive, CD15-negative epithelioid and giant cells of the Langhans type, confirming their true histiocytic nature, and were also extensively found within the dissected lymph nodes. Since no ocular, skin, pulmonary or other gastrointestinal lesions were found and the granulomas were negative for acid-fast and fungal stain, a diagnosis of granulomatous gastritis was made. Conclusions To the best of our knowledge this is the fourth example of gastric adenocarcinoma and granulomatous gastritis. These cases suggest an association between granulomatous gastritis and early gastric cancer.

Galectin-3, a marker of well-differentiated thyroid carcinoma, is expressed in thyroid nodules with cytological atypia Aims: The distribution of galectin-3, a widely recognized marker of well-differentiated thyroid carcinoma, was investigated in 95 thyroid lesions including nodules with foci of cytoarchitectural atypia. Methods and results: Twenty-eight papillary carcinomas, five follicular carcinomas, one Hurthle cell carcinoma, three poorly differentiated carcinomas, one anaplastic carcinoma, 25 nodular hyperplasias and 27 follicular adenomas, including nodules with atypical features, three neoplasms of undetermined malignant potential and two thyroiditis cases were examined. By immunohistochemistry, galectin-3 was consistently found in carcinomas; otherwise benign nodules exhibited galectin-3-positive clusters of cells with poorly developed features of differentiated carcinoma (mainly of papillary type) such as nuclear chromatin clearing, nuclear clefting, pseudoinclusions, which, in each case, were not histologically sufficient to warrant a definitive diagnosis of malignancy. In other nodules galectin-3 staining was negative. The latter were either clearly benign or showed constantly a minor degree of chromatin clearing and of other atypical features when compared with galectin-3-positive cases. Conclusions: Galectin-3, a reliable marker of differentiated thyroid carcinoma as confirmed in our series of malignant neoplasms, appears expressed in nodules with an overall benign appearance but with focal areas suspicious for malignancy. The significance of such findings needs to be further investigated.

We describe a rare case of angiomyofibroblastoma (AMF) of the vulva and one case of aggressive angiomyxoma (AAM) of the pelvic region and, with the help of an extensive revision of the literature, we attempt to define their histogenesis and peculiar biological behaviour by an immunohistological evaluation. Our results indicate that AAM, which is characterized by the presence of a high content of glycosaminoglycans in the stroma, expresses uniformly vimentin and hyaluronate receptor CD44, and heterogeneously muscle specific actin (MSA) and desmin, while AMF displays a positive reaction for vimentin, desmin and laminin, and only a weak and heterogeneous positivity for CD44. Both AMF and AAM showed no immunohistochemical reactivity for alpha-smooth muscle actin (ASMA), myoglobin, cytokeratin, collagen type IV, CD68 and S-100. The stromal cells of AAM were negative for laminin. These findings support the suggestion of an origin of the two entities by a common myofibroblastic progenitor, which normally occurs in the lower female genital tract and subsequently undergoes a neoplastic transformation. The expression of CD44 by AAM, which has never been reported before, could be responsible for its more aggressive behaviour, because this receptor is able to mediate migration of neoplastic cells on a hyaluronate rich extracellular matrix. It is speculated that the neoplastic cell of the AAM and AMF of the vulva is a specific myofibroblast which probably arises from undifferentiated mesenchymal cells normally occurring in the lower female genital tract.

The presence of cartilage in gliomas is a very unusual finding and has been mainly reported in ependymomas and in astrocytomas. A derivation of cartilage from neuroepithelial cells through a neuroepithelial-mesenchymal transition or directly from blood vessel-associated multipotent stromal elements has been proposed. We herein describe a further case of ependymoma with the presence of cartilage in a child affected by a tumor in the posterior fossa. In this case, only the last recurrence, characterized by focal areas of anaplasia, contained a nodule of cartilage. The immunohistochemical expression of fibronectin, tenascin-C, and CD44 was investigated, and the possible role of these molecules in the process of cartilage formation is discussed. Moreover, the literature on the subject is reviewed.

A 40-year-old man was admitted to our hospital for surgical treatment of aortic insufficiency and coronary ostial stenosis. Histopathology and serological tests revealed a syphilitic aortitis. 18F-FDG PET/CTwas performed to assess the extent of aortitis, showing increased radiopharmaceutical uptake along the ascending aortic wall. A repeated FDG PET/CTafter antibiotic therapy showed a markedly reduced uptake in the aortic wall, suggesting resolution of the infection according to clinical and serological data. This case highlights the usefulness of FDG PET/CT for the assessment of disease extent and treatment response in patients with syphilitic aortitis.

Ectopic immature renal tissue has rarely been reported in literature, associated or not with teratoma. Its finding could be matter of concern, owing to the occasional possibility that Wilms tumor may develop in this setting. We report a 1-year-old male patient who underwent surgery for a sacral subcutaneous small teratoma with a prevalent component of immature renal tissue. The lesion appeared completely excised and, in absence of features of malignancy, only follow-up was suggested. The patient was alive and well 15 months postoperatively. Whenever ectopic immature renal tissue is detected, a proper histological interpretation is mandatory, in order to plan a suitable treatment of the patient. From an extensive analysis of cases reported in literature we draw some practical suggestions for the diagnosis and treatment of this rare condition.

Anaplastic large cell lymphoma (ALCL) is characterized by large anaplastic cells of T-cell or null-cell phenotype expressing CD30 (Ki-1 antigen). In most cases this neoplasm expresses the anaplastic lymphoma kinase (ALK), a chimeric protein resulting from the t(2;5)(p23;q35) translocation. ALK-positive anaplastic large cell lymphoma is most frequent in the first three decades of life and shows a male predominance, involving both nodal and extranodal sites, but rarely the CNS. We report a 21-year-old patient with a previous history of nodal ALK-positive ALCL, lymphohistiocytic subtype, who was admitted for recent occurrence of left-sided anesthesia with pain and progressive motor weakness of both legs. An MRI of the spine documented an intradural extramedullary mass dislocating the thoracic cord, suggesting a meningioma and the patient underwent surgical decompression. Histological examination revealed a lymphoproliferative neoplasm with morphology and immunophenotype of ALK-positive anaplastic large cell lymphoma. After surgery, all preoperative symptoms disappeared. To our knowledge, no cases of ALCL presenting as secondary localization with an intradural extramedullary spinal mass have been reported in the literature.

The occurrence of a palisaded myofibroblastoma with amianthoid fibres in the left inguinal lymph node of a 51 year old man prompted an investigation of the factors underlying its exclusive location. The antigen profile was characterised which confirmed the homogeneous expression of vimentin and smooth muscle actin as well as the lack of desmin. Use of monoclonal antibodies to check for a differential distribution of myofibroblasts and the putative cell of origin of palisaded myofibroblastoma showed that inguinal lymph nodes have abundant vimentin and actin positive cells and desmin negative cells. This suggests that the selective occurrence of myofibroblastoma is related to the nodal microenvironment, providing a source of available and potentially proliferating myofibroblasts. Mast cells abounded in this lesion, particularly around amianthoid fibres, as well as in pelvic and inguinal lymph nodes. In view of the known role of mast cells in interstitial matrix degradation it is postulated that the core of amianthoid fibres represents degraded interstitial matrix, analogous to the sclerotic areas commonly found in the above mentioned lymph node groups, while the peripheral spokes, so peculiar to this entity, are the result of vimentin and smooth muscle actin, directly shed by proliferating myofibroblasts.

Immunohistochemical (IHC) assays using the monoclonal antibodies (MoAbs) B72.3 and B6.2, recognizing two distinct and independently expressed breast tumor-associated antigens (BTAAs), recently have been shown to significantly improve the accuracy of cytodiagnosis of breast nodules by fine-needle aspiration (FNA). To evaluate whether the same method may be useful diagnostically in distinguishing gynecomastia from breast cancer in men, a retrospective avidin-biotin immunoperoxidase assay study was performed on 50 cases of gynecomastia and 30 cases of breast carcinoma in men, using a panel of five MoAbs known to recognize different BTAAs. The results of this study demonstrated that MoAbs B1.1, HMFG2, and MBr1 displayed a strong reactivity with gynecomastia and carcinoma, but MoAbs B72.3 and B6.2 separated benign and malignant lesions in a high percentage of cases. When used in combination, the latter two reagents reacted with 96% of the carcinomas that were analyzed but labeled only 67% of gynecomastia cases. Thus, the conjoint use of these two reagents may enhance the use of FNA biopsy as a valuable tool in the presurgical diagnosis of breast nodules in men.

Reports about adrenocortical carcinomas (AC) mixed with sarcomatous areas are very rare. The terminology and pathogenesis of such biphasic tumors remain controversial. Herein, we report a case of sarcomatoid carcinoma of the adrenal gland in a 75-year-old woman who presented with left abdominal pain of one month's standing. The results of abdominal ultrasonography and computed tomography (CT) showed the presence of a large heterogeneous adrenal mass. A left adrenalectomy and complete splenectomy were performed. Histologically, the neoplasm showed areas of adrenocortical carcinoma and areas of sarcomatoid spindle cell proliferation. When examined immunohistochemically, the carcinomatous cells stained positively for S-100 protein, Melan-A protein, and neuron-specific enolase (NSE), and focally for vimentin and the cytokeratin marker MNF 116. Also, the carcinomatous cells were immunoreactive to the monoclonal antibody HMB-45. The sarcomatous component expressed vimentin, as well as other smooth and skeletal muscle markers. Liver metastases appeared 3 months postoperatively. Twelve months after removal of the primary tumor, the patient died of her disease. To the best of our knowledge, only seven cases of adrenal sarcomatoid carcinoma have been reported in the medical literature. We review the reported cases according to the 2004 classification of the World Health Organization (WHO) of lung tumors, and highlight the histogenesis, diagnosis, and clinical course of this very aggressive tumor.

We report here the case of an epithelioid haemangioendothelioma (EHE) arising in the nasal cavity which is, to the best of our knowledge, the first ever described example in the world literature in that particular site.The patient is a 23-year-old male who presented with repeated episodes of epistaxis from the nasal cavity and with a 1.5 cm reddish, polypoid, smooth, spontaneously bleeding nodule in the rightmiddle meatus. This lesion was histologically diagnosed as epithelioid haemangioendothelioma. Immunohistochemically the neoplasm displayed striking positivity for CD31, CD34 and vimentin. A surgical approach was performed by ’facial degloving’, removing the right inferior turbinate, the anterior two-thirds of the middle turbinate and the medial wall of the ethmoid bone. After 12 months follow-up the patient is disease-free, without any local or distant recurrence.

Background Evaluation of palpable neck masses may be a diagnostic problem in pediatric patients, with differential diagnosis including congenital, inflammatory, tumoral and traumatic lesions. Ultrasonography is usually a satisfactory method to make a correct pre-operative evaluation of neck masses, although diagnosis is often challenging for the surgeon and the radiologist and sometimes only possible after a histopathological examination of the resected lesion. Case presentation We report an 8-month-old patient with a cervical, anterior midline mass. Ultrasonographic images showed features suggesting a partly cystic lesion, with a preoperative suspect of thyroglossal duct cyst. Histological examination, performed after surgical removal of the mass, led to a diagnosis of lymph node angiomyomatous hamartoma (AH). Conclusions AH, a rarely occurring benign lymph node lesion, has been reported in the neck lateral region only twice. This case, presenting as a palpable neck midline mass, is the first reported case occurring in infancy. Although rare, AH should be included in the differential diagnosis of head and neck masses.

We have investigated Langerhans cell (LC) distribution in 38 prostatic carcinomas, of various degrees of differentiation, by immunohistochemistry with a polyclonal anti-S-100 serum, furthermore evaluating the expression of HLA class II-DR by neoplastic cells using a monoclonal antibody (MoAb) that reacts with a monomorphic determinant in formalin-fixed paraffin-embedded tissue. Antiserum to S-100 protein identified LCs mostly in carcinomas ranging from grade 1 to grade 2, while LCs were inconspicuous in grade 4 and virtually absent in grade 5 cancers. Moreover, sections stained with the anti -HLA-DR MoAb displayed an immunoreactivity, both cytoplasmic and apical, especially confined to neoplastic glands of low grade (1–2) carcinomas. Although we did not find a direct correlation between the two parameters under investigation and lymphoid infiltrate, we were able to document an increased number of HLA class II-positive interstitial cells in low-grade carcinomas, corresponding mostly to macrophages. Our results indicate that LC number is inversely correlated to the histopathological grade and directly to the expression of HLA class II-DR molecules by tumor cells; we believe that this might be important in understanding the more favorable biological behavior of low-grade prostate carcinomas as opposed to the higher grades, since LCs and HLA class II molecules may provide a means of eliciting the immune response, both LCs and epithelial cells expressing HLA class II molecules being capable of direct antigen presentation to immune cells. In this context macrophages might play a primary role in controlling tumor progression. To the best of our knowledge this is the first time that an attempt is made to correlate LCs and HLA class II expression to histopathological grading of prostatic carcinomas. We would also suggest that the presence of LCs and HLA class II molecules, either singly or in combination, in carcinoma of the prostate represents a good prognostic indicator, being constantly associated with the clinically less aggressive low-grade tumors. The evaluation of these two parameters might prove useful in the assessment of intermediate grades where no valid histologic criteria have been found to predict the clinical course of the disease.

This report describes the antigenic profile of the proliferating cells of pulmonary histiocytosis X (HX) in a patient treated with chemotherapy for Hodgkin's lymphoma; the association of pulmonary HX and Hodgkin's disease has rarely been described in the literature. The histopathological diagnosis of HX was confirmed with the aid of monoclonal antibodies (mAbs) to CD4, CD1a, and polyclonal serum anti S-100 protein. The phenotype of HX cells has been analysed using a panel of mAbs against HLA class I A, B, C monomorphic determinants, locus A and B, beta 2-microglobulin, HLA class II distinct monomorphic determinants, DP, DQ, DR, intercellular adhesion molecule-1 (ICAM-1) and vitronectin receptors. Our results indicate that HX cells express HLA class I and II, including locus A, locus B and DP, DQ, DR, like their normal counterpart (represented by Langerhans cells) and detectable levels of ICAM-1 but not vitronectin receptors. We would like to stress the possibility of the association of HX and Hodgkin's lymphoma extending the immunophenotypic profile of HX cells.