Atypical cellular neurothekeoma (ACN) of the elderly: case report and brief review of the literature

Atypical cellular neurothekeoma (ACN) is an aggressive and rare variant of cellular neurothekeoma. Only few cases have been reported in the literature and the biological behavior seems to be uncertain. We describe the case of an ACN presenting on the scalp of an elderly man, emphasizing the cytologic features of malignancy. In addition, we provide a brief overview of the literature and discuss the differential diagnosis with other entities, and the possible diagnostic pitfalls

The contribution of the 1H-MRS lipid signal to cervical cancer prognosis: a preliminary study

Background The aim of this study was to investigate the role of the lipid peak derived from H-1 magnetic resonance (MR) spectroscopy in assessing cervical cancer prognosis, particularly in assessing response to neoadjuvant chemotherapy (NACT) of locally advanced cervical cancer (LACC). Methods We enrolled 17 patients with histologically proven cervical cancer who underwent 3-T MR imaging at baseline. In addition to conventional imaging sequences for pelvic assessment, the protocol included a single-voxel point-resolved spectroscopy (PRESS) sequence, with repetition time of 1,500 ms and echo times of 28 and 144 ms. Spectra were analysed using the LCModel fitting routine, thus extracting multiple metabolites, including lipids (Lip) and total choline (tCho). Patients with LACC were treated with NACT and reassessed by MRI at term. Based on tumour volume reduction, patients were classified as good responder (GR; tumour volume reduction > 50%) and poor responder or nonresponder (PR-or-NR; tumour volume reduction <= 50%). Results Of 17 patients, 11 were LACC. Of these 11, only 6 had both completed NACT and had good-quality H-1-MR spectra; 3 GR and 3 PR-or-NR. A significant difference in lipid values was observed in the two groups of patients, particularly with higher Lip values and higher Lip/tCho ratio in PR-NR patients (p =0.040). A significant difference was also observed in choline distribution (tCho), with higher values in GR patients (p = 0.040). Conclusions Assessment of lipid peak at H-1-MR spectroscopy could be an additional quantitative parameter in predicting the response to NACT in patients with LACC

High-grade serous carcinoma of unknown primary origin associated with STIC clinically presented as isolated inguinal lymphadenopathy: a case report

Serous tubal intraepithelial carcinoma (STIC) is a precancerous lesion of high-grade serous ovarian carcinoma (HGSOC). Usually, it arises from the fimbrial end of the tube, and it is associated with metastatic potential. On average, the time to progress from STIC to HGSOC is 6.5 years. Therefore, whenever a STIC lesion is found, surgical staging and prophylactic salpingectomy are recommended in order to prevent ovarian cancer. We report a rare case of a 45-year-old female patient who clinically presented an isolated right inguinal lymphadenopathy. The remaining clinical examination was normal. Therefore, an excisional biopsy of the lymph node was performed. Pathological analysis revealed a high-grade serous carcinoma, most likely of gynecological origin. Due to histological evidence, a computed tomography (CT) scan was carried out. There was no CT evidence of ovarian disease, pelvic involvement, intra-abdominal lymphadenopathies, metastatic disease, or ascites. All tumor markers were negative. The patient underwent laparoscopic hysterectomy and bilateral salpingo-oophorectomy followed by surgical staging. Surprisingly, pathological examination showed a STIC lesion in the fimbria of the left fallopian tube. We aim to report the potential capability of STIC to spread particularly through lymphatic pathways rather than peritoneal dissemination

RET/PTC3 translocation in a rare hemorrhagic brain metastasis of papillary thyroid cancer post Chernobyl radiation affects vessels ultrastructure

Abstract Background Slow progression and good prognosis are the usual characteristics of papillary thyroid carcinoma (PTC). The presence of brain metastases (0.4–1.2%) is suggestive of a worse prognosis. RET/PTC rearrangements were particularly prevalent in PTCs developed after Chernobyl nuclear accident. Case description A 50-year-old woman born in Slovakia, exposed to radiation resulting from the accident at the Chernobyl nuclear power plant, affected since 2017 by papillary thyroid cancer and in therapy at our hospital, experimented cerebral hemorrhagic metastasis. Biopsy analyses revealed a RET/PTC3 rearrangement, so our aim was to find possible morphological relation between hemorrhagic metastasis and RET/PTC3 translocation. Results Immunohistochemical analysis showed diffuse and intense positivity for VEGF in endothelial cells of the neoplasm’ vascular network. Transmission electron microscopy images showed vessels with unorganized pattern and uneven diameters. In particular, metastasis endothelial cells (MECs) showed irregular shape and size, thickened cytoplasm and swelling of endoplasmic reticulum. MECs organized in irregular monolayers or multiple layers, surrounded by a thickened but unstructured extracellular matrix. Absence of strong junctional complexes among MECs resulted in a further weakened vessels wall. Conclusion RET/PTC3 translocation causes VEGF overexpression via STAT3 signaling cascade and the increased amount of VEGF adds to the greater amount of VEGFRs expressed by MECs. Our ultrastructural investigation show that this condition creates a massive growth of altered vessels prone to bleeding. The clinical significance of our study consists in alert oncologist and surgeons on possible arising of hemorrhagic brain metastases in patients with PTC and RET/PTC3 translocation exposed to ionizing radiation as people living in areas caught up in Chernobyl or Fukushima disasters

PD-L1 testing in metastatic triple negative breast cancer: Results of an Italian survey

Background: Immunotherapy has revolutionized the approach to metastatic triple-negative breast cancers. Atezolizumab was approved for patients with metastatic triple-negative breast cancers whose tumors express PD-L1, determined by SP 142 assay. To assess the availability and practice of SP142 test we administered a survey to all the 15 pathology departments of the Lazio Region during a six-month period. Methods: The survey comprised 12 questions regarding the availability of SP142 in the pathology departments, the percentage of positive tests, the difficulties of pathologists in cases close to cut-off value and the tested samples. Results: The SP142 assay was available in only eight centers. In case of positive result, most centers (5/8, 62.5%) reported values of PD-L1 expression ranging from > 1 to ⩽ 5%, with values close to the cut-off point (⩾ 1% or < 1%) being the greatest challenge.Most of the centers (6/8, 75%) tested material from both their own and other hospitals. In most centers, the evaluations were performed either on primary tumors or metastasis, in particular lymph nodes (5/8, 62.5%), followed by lung (3/8, 37.5%) and liver (1/8, 12.5%) metastasis. Conclusion: Our results raise some important issues concerning the evaluation of PD-L1 in the "real-life" setting, providing strategies for its implementation

Extremely late-onset pulmonary metastasis from uterine PEComa

A 79-year-old woman underwent surgical resection of a peripheral, solitary, pulmonary lesion that was diagnosed as malignant PEComa. Her clinical history was positive for uterine leiomyosarcoma, excised 20 years before. Re-evaluation of the primary uterine lesion led to the final diagnosis of lung metastasis from uterine PEComa. While long latency between primary tumour and metastasis is a known and characteristic feature of PEComas, a 20-year interval is unprecedented in the literature

Synovial chondromatosis and calcium pyrophosphate deposition of the temporomandibular joint: challenging diagnosis

BACKGROUND: The association between the synovial chondromatosis (SC) and the calcium pyrophosphate deposition (pseudogotta) in temporomandibular joint (TMJ) is very rare and has been described just 1 patient in the literature. CLINICAL PRESENTATION: A 64-year-old woman was referred to Dipartimento di Scienze Odontostomatologiche e Maxillo-Facciali, Sapienza Università di Roma after complaining about right temporomandibular pain, limitation in mandibular movements, and tumefaction in the right preauricular region. The patient was hospitalized for the surgery. The microscopic examination of the excised material revealed calcium pyrophosphate dihydrate (CPPD) deposits crystals associated with cartilaginous proliferation. CONCLUSION: The association between the SC and the calcium pyrophosphate deposition (pseudogotta) is a challenging diagnosis among TMJ neoplasms

PD-L1 expression in TNBC: A predictive biomarker of response to neoadjuvant chemotherapy?

Triple negative breast cancer (TNBC) has an aggressive clinical behaviour, with a poorer prognosis compared to other subtypes. Recently, tumor-infiltrating lymphocytes (TILs) have been proposed as a predictive biomarker for a better clinical outcome and pathological response (pR) after neoadjuvant chemotherapy (NACT) in TNBC. These data confirm the role of the immune system in the neoplastic progression and in the response to therapy. We performed a retrospective analysis of 54 pre-NACT biopsies of TNBC and compared both the percentage of stromal TILs and the degree of PD-L1 expression with the extent of pR to standard NACT. A pathological complete response (pCR) was achieved in 35% of cases. Univariate analysis showed (i) a significant association between PD-L1 expression in ≥25% of neoplastic cells and the achievement of a pCR (p=0.024); (ii) a significantly higher frequency of pCR in cases showing ≥50% stromal TILs (p<0.001). However in the multivariate analysis only PD-L1 expression on tumor cells remained significantly associated with pCR (OR = 1,13; 95% CI 1,01–1,27), suggesting that the expression of this biomarker could be associated with a subpopulation of TNBC more likely to respond to chemotherapy. These data need to be confirmed by larger studies

“Cheerios in the lung” as first metastases from endometrial endometrioid adenocarcinoma with adequate response to immunotherapy

Abstract Background Metastases from endometrial carcinoma to the lungs are uncommon and cavitating metastases are even rarer. In some cases, lung cavitating metastases may resemble diffuse “Cheerios in the lung.” Case presentation A 58-year-old woman, smoker, with a history of hysteroannessiectomy and pelvic lymphadenectomy for a low stage endometrial endometrioid adenocarcinoma, came under our attention for dry cough without fever that persisted for over three months, and computed tomography (CT) revealed diffuse cavitating lung metastases resembling "Cheerios in the lung". After standard chemotherapy proved ineffective, Lenvatinib plus Pembrolizumab (immunotherapy) was administrated, leading to the disappearance of all lung nodules, leaving behind a diffuse emphysematous-like pattern. Conclusions This case is a rare example of metastatic endometrioid adenocarcinoma, manifested with chronic dry cough, with “Cheerios in the lung” appearence. The metastases responded well to immunotherapy, resulting in the regression of nodules into areas of diffuse emphysematous-like changes