Interdigitating dendritic cell sarcoma is a very rare entity in the spectrum of histiocytic and dendritic cell neoplasms that mostly occurs in lymph nodes, generally presenting as solitary lymphadenopathy, but may affect every organ. Among extra nodal sites, cutaneous interdigitating dendritic cell sarcoma is exceedingly rare; to date, only 9 cases have been described in English literature. The mean age at diagnosis was 60 years, with a male-female ratio of 1,5 to 1; clinically, two different modalities of skin presentation have been reported: solitary, represented by a single red-brownish nodular lesion, or diffuse, characterized by multiple nodular lesions in one or more body districts. The extreme rarity of this sarcoma and its morphological similarity to other poorly differentiated tumors may lead to a delay in diagnosis; in particular, cutaneous localization may be difficult to differentiate from follicular dendritic cell sarcoma, Langerhans cell sarcoma, poorly differentiated squamous cell carcinoma and more generally sarcomatoid carcinoma, atypical fibroxanthoma, malignant melanoma and several sarcomas. Immunohistochemistry plays an important role in identifying this rare entity and formulating a correct histological diagnosis, fundamental requirement for choosing the best therapeutic approach. We report herein a further case of an 81-year-old Caucasian woman who presented to the Dermatology Department to remove an asymptomatic skin papule in the left temporal region, clinically diagnosed as dermatofibroma. The overall pathological and immunohistochemical features supported the diagnosis of a malignant dendritic cell tumor, consistent of interdigitating dendritic cell sarcoma.
Journal of the European Academy of Dermatology and VenereologyVolume 35, Issue 5 p. e311-e312 Letter to the Editor COVID-19 and HHV8 first spotted together: an affair under electron microscopy E. Leoni, Corresponding Author E. Leoni [email protected] orcid.org/0000-0003-4297-9432 Unit of Pathology, Department of Medicine and Surgery, University of Insubria, Varese, Italy Correspondence: E. Leoni. E-mail: [email protected]Search for more papers by this authorM. Cerati, M. Cerati Department of Pathology, ASST Sette Laghi, Varese, ItalySearch for more papers by this authorG. Finzi, G. Finzi Department of Pathology, ASST Sette Laghi, Varese, ItalySearch for more papers by this authorM. Lombardo, M. Lombardo Department of Dermatology, ASST Sette Laghi, Varese, ItalySearch for more papers by this authorF. Sessa, F. Sessa Unit of Pathology, Department of Medicine and Surgery, University of Insubria, Varese, Italy Department of Pathology, ASST Sette Laghi, Varese, ItalySearch for more papers by this author E. Leoni, Corresponding Author E. Leoni [email protected] orcid.org/0000-0003-4297-9432 Unit of Pathology, Department of Medicine and Surgery, University of Insubria, Varese, Italy Correspondence: E. Leoni. E-mail: [email protected]Search for more papers by this authorM. Cerati, M. Cerati Department of Pathology, ASST Sette Laghi, Varese, ItalySearch for more papers by this authorG. Finzi, G. Finzi Department of Pathology, ASST Sette Laghi, Varese, ItalySearch for more papers by this authorM. Lombardo, M. Lombardo Department of Dermatology, ASST Sette Laghi, Varese, ItalySearch for more papers by this authorF. Sessa, F. Sessa Unit of Pathology, Department of Medicine and Surgery, University of Insubria, Varese, Italy Department of Pathology, ASST Sette Laghi, Varese, ItalySearch for more papers by this author First published: 18 January 2021 https://doi.org/10.1111/jdv.17123Citations: 8Read the full textAboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onEmailFacebookTwitterLinkedInRedditWechat No abstract is available for this article. References 1Gisondi P, PIaserico S, Bordin C, Alaibac M, Girolomoni G, Naldi L. Cutaneous manifestations of SARS-CoV-2 infection: a clinical update. J Eur Acad Dermatology Venereol 2020; 34: 2499–2504. https://doi.org/10.1111/jdv.16774 10.1111/jdv.16774 CASPubMedWeb of Science®Google Scholar 2Lopes TRR, Gonçales JP, Silva Júnior JVJ, de Lorena VMB, Toscano ALCC, Akamatsu SM et al. Association of IL-6, IL-10 and CXCL10 serum concentrations with visceral Kaposi's sarcoma in people living with HIV/AIDS. Hum Immunol 2020; 81: 26–31. 10.1016/j.humimm.2019.11.007 CASPubMedWeb of Science®Google Scholar 3Colmenero I, Santonja C, Alonso-Riaño M, Noguera-Morel L, Hernández-Martín A, Andina D et al. SARS-CoV-2 endothelial infection causes COVID-19 chilblains: histopathological, immunohistochemical and ultrastructural study of seven paediatric cases. Br J Dermatol 2020; 183: 729-737. 10.1111/bjd.19327 CASPubMedWeb of Science®Google Scholar 4Nasonov E, Samsonov M. The role of interleukin 6 inhibitors in therapy of severe COVID-19. Biomed Pharmacother 2020; 131: 110698. 10.1016/j.biopha.2020.110698 CASPubMedWeb of Science®Google Scholar Citing Literature Volume35, Issue5May 2021Pages e311-e312 This article also appears in:JEADV COVID-19 articles ReferencesRelatedInformation
Pituitary apoplexy is a rare endocrine emergency that occurs in a small number of patients with a pituitary tumor. It is a clinical syndrome characterized by the sudden onset of headache, nausea, vomiting, visual impairment, and decreased consciousness, caused by hemorrhage and/or infarction of the pituitary gland. Pituitary apoplexy has very rarely been described during pregnancy, when it is potentially life-threatening to both the mother and the fetus, if unrecognized. Only a few cases have been published to date. The review of the existing literature underlines that pituitary apoplexy, although rare, should be borne in mind when a pregnant woman presents with severe headache and visual defects of sudden onset. After initial management, which includes intravenous glucocorticoid therapy, fluid and electrolyte replacement, the final selection of medical or surgical treatment should result from a multidisciplinary approach involving expert specialists, keeping into account both severity of clinical presentation and gestational week.
Well-established histopathological prognostic factors are lacking in primary central nervous system (CNS) lymphomas (PCNSL). The present study investigated the presence and prognostic role of tumour necrosis (TN) and reactive perivascular T-cell infiltrate (RPVI), defined as a rim of small reactive T-lymphocytes occurring alone or located between the vascular wall and large neoplastic cells, in tumour samples from 100 immunocompetent patients with PCNSL. World Health Organization histotypes of the patients were: 96 diffuse large B-cell lymphomas, two Burkitt-like lymphomas, one anaplastic large T-cell lymphoma and one unclassified B-cell lymphoma. TN was observed in 24 (24%) cases and RPVI in 26 (36%) of 73 assessable cases. Patients with RPVI-positive lesions exhibited a significantly better overall survival (OS) than patients with RPVI-negative lymphoma, particularly among patients treated with high-dose methotrexate-based chemotherapy (3-year OS: 59 +/- 14% vs. 42 +/- 9%, P = 0.02). By contrast, the presence of TN did not demonstrate prognostic significance. Multivariate analysis confirmed an independent association between RPVI and survival. In conclusion, the presence of RPVI is independently associated with survival in PCNSL. This parameter can be easily and routinely assessed at diagnosis on histopathological specimens.
PURPOSE:To identify survival predictors and to design a prognostic score useful for distinguishing risk groups in immunocompetent patients with primary CNS lymphomas (PCNSL). PATIENTS AND METHODS:The prognostic role of patient-, lymphoma-, and treatment-related variables was analyzed in a multicenter series of 378 PCNSL patients treated at 23 cancer centers from five different countries. RESULTS:Age more than 60 years, performance status (PS) more than 1, elevated lactate dehydrogenase (LDH) serum level, high CSF protein concentration, and involvement of deep regions of the brain (periventricular regions, basal ganglia, brainstem, and/or cerebellum) were significantly and independently associated with a worse survival. These five variables were used to design a prognostic score. Each variable was assigned a value of either 0, if favorable, or 1, if unfavorable. The values were then added together to arrive at a final score, which was tested in 105 assessable patients for which complete data of all five variables were available. The 2-year overall survival (OS) +/- SD was 80% +/- 8%, 48% +/- 7%, and 15% +/- 7% (P =.00001) for patients with zero to one, two to three, and four to five unfavorable features, respectively. The prognostic role of this score was confirmed by limiting analysis to assessable patients treated with high-dose methotrexate-based chemotherapy (2-year OS +/- SD: 85% +/- 8%, 57% +/- 8%, and 24% +/- 11%; P =.0004). CONCLUSION:Age, PS, LDH serum level, CSF protein concentration, and involvement of deep structures of the brain were independent predictors of survival. A prognostic score including these five parameters seems advisable in distinguishing different risk groups in PCNSL patients. The proposed score and its relevance in therapeutic decision deserve to be validated in further studies.
Spinal cord subependymoma is a rare tumour with only 39 reported cases in the literature. The authors report a further case of this neoplasm in a 53 year old man with a progressive paraparesis, paraesthesias of the lower limbs and sphincter disturbance. The tumour was partly removed, without progression 5 years after surgery. After a careful review of the literature, the optimal treatment of this spinal tumour is debated.
INTRODUCTION:The malignant optic glioma in adulthood is a rare tumour of middle-age which causes an early loss of vision and always leads to death within a year.CASE REPORT:The authors report a case of this disease in a 68-year-old woman with a history of rapidly deteriorating vision and death 6 months after surgery.CONCLUSION:A review of the previous cases showed the accordance of these with the syndrome defined first by Hoyt et al. in 1973; a statistical analysis reveals that the radiotherapy improves the survival whereas the role of chemotherapy is still not definite.
Six cases of meningioma showing oncocytic changes are described. The lesions were composed mostly of sheets, nests, and cords of large polygonal cells with finely granular eosinophilic cytoplasm rich in mitochondria. Neoplastic cells showed nuclear pleomorphism with prominent nucleoli. Necrosis and high mitotic rate were present in the majority of cases. Oncocytic differentiation was demonstrated by conventional histology, immunocytochemistry, electron microscopy, and Western-blot analysis. Oncocytic meningiomas showed an aggressive behavior; recurrences were observed in three cases, and invasion of brain cortex was evident in other two cases.
A case of a 56-year-old woman that developed subarachnoid haemorrhage from iatrogenic post-traumatic aneurysm of the cortical middle cerebral artery due to a ventricular tap for hydrocephalus is presented. Previous reported cases of postsurgical aneurysms of the cerebral vessels are discussed and the pathogenesis, the clinical course, the outcome and the advisable treatment of this condition are debated.
The authors describe the case of a 71-year-old man without signs of phacomatosis with a history of progressive psychomotory lowering and two simultaneous tumors of the olfactory groove and of the cerebellopontine angle, as demonstrated by CT scans. Histologic examinations showed the lesions to be a meningioma and a neurinoma. The rarity and the possible pathogenesis of this association are discussed.
The authors describe the case of a 58-year-old man with an history of seizures because of right parietal parasagittal meningioma. Twenty-two months after complete removal of this neoplasm, the patient showed progressive mental deterioration and at CT-scans revealed a right temporal anaplastic astrocytoma which was operated. In literature the association between intracranial meningioma and astrocytoma is reported in 41 cases. After a brief review of the previous cases, the authors debate the clinical presentation, the diagnostic problems, the aetiopathogenetic hypotheses and the best surgical treatment of this association.
The case of a 63-year-old patient with spontaneous cerebrospinal fluid rhinorrhea from a intraethmoidal encephalomeningocele is presented. The patient was asymptomatic until the old age. The preoperative coronal CT-scan and the preoperative and postoperative MRI are shown. The rarity of this onset in an elderly man and the surgical indications of this disease are debated.