Pub Date : 2021-08-29eCollection Date: 2021-01-01DOI: 10.1177/20363613211034710
Karlton Wong, Jomjit Chantharasamee, Scott Nelson, Mark A Eckardt, Kambiz Motamedi, Francis J Hornicek, Arun S Singh
Osteoblastomas and aneurysmal bone cysts (ABC) are rare benign bone tumors that make up about 1%-2% of primary bone malignancies, typically occurring in young patients with a median age of 20 years, most commonly effecting the axial skeleton. ABCs may develop independently as primary lesions, or secondary to other bony lesions including osteoblastomas, chondroblastomas, and giant cell tumors. Treatment of unresectable or extensive osteoblastomas can be challenging. In 2013, the Food and Drug Administration (FDA) approved denosumab for the treatment of giant cell tumors of the bone due to its efficacy in these morbid bony lesions. Various case reports have shown that osteoblastomas can respond to denosumab. Furthermore, numerous ABC case reports have described the efficacy of denosumab in these situations. We herein describe a unique case of a young patient with an aggressive osteoblastoma and secondary ABCs who was successfully treated with denosumab.
{"title":"Aggressive osteoblastoma with a secondary aneurysmal bone cyst treated with denosumab.","authors":"Karlton Wong, Jomjit Chantharasamee, Scott Nelson, Mark A Eckardt, Kambiz Motamedi, Francis J Hornicek, Arun S Singh","doi":"10.1177/20363613211034710","DOIUrl":"https://doi.org/10.1177/20363613211034710","url":null,"abstract":"<p><p>Osteoblastomas and aneurysmal bone cysts (ABC) are rare benign bone tumors that make up about 1%-2% of primary bone malignancies, typically occurring in young patients with a median age of 20 years, most commonly effecting the axial skeleton. ABCs may develop independently as primary lesions, or secondary to other bony lesions including osteoblastomas, chondroblastomas, and giant cell tumors. Treatment of unresectable or extensive osteoblastomas can be challenging. In 2013, the Food and Drug Administration (FDA) approved denosumab for the treatment of giant cell tumors of the bone due to its efficacy in these morbid bony lesions. Various case reports have shown that osteoblastomas can respond to denosumab. Furthermore, numerous ABC case reports have described the efficacy of denosumab in these situations. We herein describe a unique case of a young patient with an aggressive osteoblastoma and secondary ABCs who was successfully treated with denosumab.</p>","PeriodicalId":46078,"journal":{"name":"Rare Tumors","volume":null,"pages":null},"PeriodicalIF":0.9,"publicationDate":"2021-08-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/2c/5f/10.1177_20363613211034710.PMC8408893.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39388559","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2021-08-20eCollection Date: 2021-01-01DOI: 10.1177/20363613211039724
Mousa ElKhaldi, Rakan Radi, Maysa Al-Hussaini
Germ cell tumors (GCTs) are a histologically heterogeneous group of tumors that arise from the primitive germ cell of the embryonic gonad. Choriocarcinoma is a variant of GCTs that is prone to hematogenous metastasis to the liver, lung, and brain. Cutaneous metastasis in choriocarcinoma is rarely encountered with only a few cases reported in literature. We report the case of a 28-year-old male presenting with lower back pain that, upon further work-up, was diagnosed with pure choriocarcinoma of the testes. Around 9 months after his initial presentation, he developed a cutaneous back lesion. Microscopic examination confirmed the presence of choriocarcinoma composed of mononuclear cytotrophoblasts which interweave with multinucleated syncytiotrophoblasts. The patient passed away 3 weeks after the onset of cutaneous metastasis.
{"title":"Choriocarcinoma metastatic to the skin: A rare occurrence associated with dismal outcome.","authors":"Mousa ElKhaldi, Rakan Radi, Maysa Al-Hussaini","doi":"10.1177/20363613211039724","DOIUrl":"https://doi.org/10.1177/20363613211039724","url":null,"abstract":"<p><p>Germ cell tumors (GCTs) are a histologically heterogeneous group of tumors that arise from the primitive germ cell of the embryonic gonad. Choriocarcinoma is a variant of GCTs that is prone to hematogenous metastasis to the liver, lung, and brain. Cutaneous metastasis in choriocarcinoma is rarely encountered with only a few cases reported in literature. We report the case of a 28-year-old male presenting with lower back pain that, upon further work-up, was diagnosed with pure choriocarcinoma of the testes. Around 9 months after his initial presentation, he developed a cutaneous back lesion. Microscopic examination confirmed the presence of choriocarcinoma composed of mononuclear cytotrophoblasts which interweave with multinucleated syncytiotrophoblasts. The patient passed away 3 weeks after the onset of cutaneous metastasis.</p>","PeriodicalId":46078,"journal":{"name":"Rare Tumors","volume":null,"pages":null},"PeriodicalIF":0.9,"publicationDate":"2021-08-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/91/25/10.1177_20363613211039724.PMC8381407.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39344182","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2021-07-18eCollection Date: 2021-01-01DOI: 10.1177/20363613211034115
Maroun Bou Zerdan, Aram H Bidikian, Ibrahim Alameh, Clara El Nakib, Hazem I Assi
Soft tissue sarcomas remain one of the rarest malignancies with numerous subtypes that go undiagnosed. The PDGFRα antagonist Olaratumab (Lartruvo) was withdrawn from the market due to disappointing findings in the phase III studies. We share our experience with this medication in a tertiary care center in the Middle East and North Africa region. Monitor the effect of Olaratumab on sarcomas when it was used prior to its withdrawal, and compare our findings with the literature. We performed a retrospective analysis of adult patients with advanced-/metastatic soft tissue sarcomas treated with at least two cycles of Olaratumab at a tertiary care center in Lebanon during the period from January 1, 2017 to December 31, 2018. Fifteen patients were included in the study. The mean age was 49 with a range of 26-75 years. The median duration of the use of Olaratumab was 21.3 months with a range of 7.3-37 months. The average number of number of cycles received per patient was four. Five patients were deceased. Median PFS was 7.87 months (95% CI 5.28-10.45), and mean OS was 12.26 months (95% CI 8.47-16.05) Median OS was 9.8 months (95% CI 6.07-13.53). Olaratumab has been withdrawn from the market, and it is currently being investigated as part of the phase II ANNOUNCE 2 trial. Our experience from a tertiary care center shows results similar to those reported in the literature. The immunogenicity and heterogeneity of soft tissue sarcomas pose a challenge to the treatment of soft tissue sarcomas, but they also allow a wide array of possible management solutions.
软组织肉瘤仍然是最罕见的恶性肿瘤之一,有许多亚型未被诊断。PDGFRα拮抗剂Olaratumab (Lartruvo)因III期研究结果令人失望而退出市场。我们在中东和北非地区的一家三级保健中心分享我们使用这种药物的经验。监测停药前使用奥拉拉单抗对肉瘤的影响,并将我们的发现与文献进行比较。我们对2017年1月1日至2018年12月31日期间在黎巴嫩一家三级医疗中心接受至少两个周期奥拉拉单抗治疗的晚期/转移性软组织肉瘤成年患者进行了回顾性分析。15名患者参与了这项研究。平均年龄49岁,年龄范围26-75岁。使用Olaratumab的中位持续时间为21.3个月,范围为7.3-37个月。每位患者接受的平均周期数为4次。5名患者死亡。中位PFS为7.87个月(95% CI 5.28-10.45),平均OS为12.26个月(95% CI 8.47-16.05),中位OS为9.8个月(95% CI 6.07-13.53)。Olaratumab已退出市场,目前正在作为II期ANNOUNCE 2试验的一部分进行研究。我们在三级护理中心的经验显示了与文献报道相似的结果。软组织肉瘤的免疫原性和异质性对软组织肉瘤的治疗提出了挑战,但它们也允许广泛的可能的管理解决方案。
{"title":"Olaratumab's failure in soft tissue sarcoma.","authors":"Maroun Bou Zerdan, Aram H Bidikian, Ibrahim Alameh, Clara El Nakib, Hazem I Assi","doi":"10.1177/20363613211034115","DOIUrl":"https://doi.org/10.1177/20363613211034115","url":null,"abstract":"<p><p>Soft tissue sarcomas remain one of the rarest malignancies with numerous subtypes that go undiagnosed. The PDGFRα antagonist Olaratumab (Lartruvo) was withdrawn from the market due to disappointing findings in the phase III studies. We share our experience with this medication in a tertiary care center in the Middle East and North Africa region. Monitor the effect of Olaratumab on sarcomas when it was used prior to its withdrawal, and compare our findings with the literature. We performed a retrospective analysis of adult patients with advanced-/metastatic soft tissue sarcomas treated with at least two cycles of Olaratumab at a tertiary care center in Lebanon during the period from January 1, 2017 to December 31, 2018. Fifteen patients were included in the study. The mean age was 49 with a range of 26-75 years. The median duration of the use of Olaratumab was 21.3 months with a range of 7.3-37 months. The average number of number of cycles received per patient was four. Five patients were deceased. Median PFS was 7.87 months (95% CI 5.28-10.45), and mean OS was 12.26 months (95% CI 8.47-16.05) Median OS was 9.8 months (95% CI 6.07-13.53). Olaratumab has been withdrawn from the market, and it is currently being investigated as part of the phase II ANNOUNCE 2 trial. Our experience from a tertiary care center shows results similar to those reported in the literature. The immunogenicity and heterogeneity of soft tissue sarcomas pose a challenge to the treatment of soft tissue sarcomas, but they also allow a wide array of possible management solutions.</p>","PeriodicalId":46078,"journal":{"name":"Rare Tumors","volume":null,"pages":null},"PeriodicalIF":0.9,"publicationDate":"2021-07-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1177/20363613211034115","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39276934","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2021-07-02eCollection Date: 2021-01-01DOI: 10.1177/20363613211029493
Philip T Sobash, Krishna Vedala, Daniel Alfano, Heather Pinckard-Dover, Jason L Muesse, Raman Desikan
The notochord is the defining structure of all chordate embryos. It is a midline structure ventral to the ectoderm, neural plates, and neural arch. Remnants of the notochord ultimately give rise to the nucleus pulposus. The function of the notochord is to organize the surrounding structures. Chordoma is a rare malignant bone tumor arising from remnants of the notochord. These tumors are indolent and can present as incidental or locally advanced involving adjacent structures. These tumors typically present at the skull base and sacral spine but more rarely can be seen on the cervical and thoracic spine. Rare cases of chordoma invading the brachial plexus have been recorded. Surgical resection is the mainstay of treatment for chordomas. We would like to discuss a novel presentation of a chordoma as a Pancoast tumor, and aim to highlight the clinical importance of accurate diagnosis and planning therapy along with poor prognosis of incomplete surgical resection.
{"title":"A rare case of chordoma presenting as a Pancoast tumor.","authors":"Philip T Sobash, Krishna Vedala, Daniel Alfano, Heather Pinckard-Dover, Jason L Muesse, Raman Desikan","doi":"10.1177/20363613211029493","DOIUrl":"https://doi.org/10.1177/20363613211029493","url":null,"abstract":"<p><p>The notochord is the defining structure of all chordate embryos. It is a midline structure ventral to the ectoderm, neural plates, and neural arch. Remnants of the notochord ultimately give rise to the nucleus pulposus. The function of the notochord is to organize the surrounding structures. Chordoma is a rare malignant bone tumor arising from remnants of the notochord. These tumors are indolent and can present as incidental or locally advanced involving adjacent structures. These tumors typically present at the skull base and sacral spine but more rarely can be seen on the cervical and thoracic spine. Rare cases of chordoma invading the brachial plexus have been recorded. Surgical resection is the mainstay of treatment for chordomas. We would like to discuss a novel presentation of a chordoma as a Pancoast tumor, and aim to highlight the clinical importance of accurate diagnosis and planning therapy along with poor prognosis of incomplete surgical resection.</p>","PeriodicalId":46078,"journal":{"name":"Rare Tumors","volume":null,"pages":null},"PeriodicalIF":0.9,"publicationDate":"2021-07-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1177/20363613211029493","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39199384","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Germ cell tumors are a heterogeneous group of tumors that can present primarily as gonadal tumors in either a localized or metastatic pattern. Rarely these tumors can initially present at extra-gonadal locations, including the gastrointestinal tract. We report two young male patients who presented with nonspecific gastrointestinal symptoms caused by a mass lesion involving the duodenum. Pathologically, both were confirmed to be germ cell tumors; an unfamiliar initial presentation of germ cell tumors. In both cases, evidence of pre-existing gonadal tumor in the form of a testicular mass and a burned-out tumor with microlithiasis, in the first and second cases, respectively was detected following the confirmed diagnosis of extra-gonadal germ cell tumor. Each patient's clinical course and outcome emphasizes the importance of a high index of suspicion, timely diagnosis, and appropriate management.
{"title":"Extragonadal germ cell tumor, a report of two cases presenting in the gastrointestinal tract.","authors":"Mousa Elkhaldi, Ahamd Moayad Naser, Yazan AlHalaseh, Maysa Al-Hussaini","doi":"10.1177/20363613211029487","DOIUrl":"https://doi.org/10.1177/20363613211029487","url":null,"abstract":"<p><p>Germ cell tumors are a heterogeneous group of tumors that can present primarily as gonadal tumors in either a localized or metastatic pattern. Rarely these tumors can initially present at extra-gonadal locations, including the gastrointestinal tract. We report two young male patients who presented with nonspecific gastrointestinal symptoms caused by a mass lesion involving the duodenum. Pathologically, both were confirmed to be germ cell tumors; an unfamiliar initial presentation of germ cell tumors. In both cases, evidence of pre-existing gonadal tumor in the form of a testicular mass and a burned-out tumor with microlithiasis, in the first and second cases, respectively was detected following the confirmed diagnosis of extra-gonadal germ cell tumor. Each patient's clinical course and outcome emphasizes the importance of a high index of suspicion, timely diagnosis, and appropriate management.</p>","PeriodicalId":46078,"journal":{"name":"Rare Tumors","volume":null,"pages":null},"PeriodicalIF":0.9,"publicationDate":"2021-06-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1177/20363613211029487","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39199383","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2021-06-30eCollection Date: 2021-01-01DOI: 10.1177/20363613211026483
Andrea Dekanić, Marko Velepič, Margita Belušić Gobić, Ita Hadžisejdić, Nives Jonjić
Malignant mesenchymal tumors of oropharyngeal mucosa are rare. Those with fibroblastic and histiocytic differentiation in the skin are called atypical fibroxanthoma (AFX) and in the soft tissue undifferentiated pleomorphic sarcoma (UPS). Here we present a case of an older patient with a history of multiple basal cell carcinomas and recently with a rapidly growing polypoid lesion in the mucosa of posterior oropharyngeal wall with AFX/UPS morphology. The differential diagnosis, histological pitfalls of this poorly characterized mesenchymal lesions, and the challenges associated with treatment are discussed.
{"title":"Undifferentiated pleomorphic sarcoma in oropharyngeal mucosa of patients with multiple basal cell carcinomas.","authors":"Andrea Dekanić, Marko Velepič, Margita Belušić Gobić, Ita Hadžisejdić, Nives Jonjić","doi":"10.1177/20363613211026483","DOIUrl":"10.1177/20363613211026483","url":null,"abstract":"<p><p>Malignant mesenchymal tumors of oropharyngeal mucosa are rare. Those with fibroblastic and histiocytic differentiation in the skin are called atypical fibroxanthoma (AFX) and in the soft tissue undifferentiated pleomorphic sarcoma (UPS). Here we present a case of an older patient with a history of multiple basal cell carcinomas and recently with a rapidly growing polypoid lesion in the mucosa of posterior oropharyngeal wall with AFX/UPS morphology. The differential diagnosis, histological pitfalls of this poorly characterized mesenchymal lesions, and the challenges associated with treatment are discussed.</p>","PeriodicalId":46078,"journal":{"name":"Rare Tumors","volume":null,"pages":null},"PeriodicalIF":0.9,"publicationDate":"2021-06-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/1e/84/10.1177_20363613211026483.PMC8256239.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39199381","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2021-06-30eCollection Date: 2021-01-01DOI: 10.1177/20363613211028498
Noellie Ducastel, Ioana-Mariana Cimpean, Ivan Theate, Olivier Vanhooteghem
Anaplastic large cell lymphoma (BIA-ALCL) associated with rough textured breast implants was first reported in 1997. It is a non-Hodgkin's lymphoma originating from a T lymphocyte which occurs on average 10.9 years after placement of the breast implant. BIA-ALCL mainly manifests as a periprosthetic seroma or a mass adjacent to the implant. To our knowledge, we describe the first case of BIA-ALCL with initial presentation by indurate erythematous plates located in both breasts and the progressive appearance of several asymptomatic metastatic nodular lesions that have been appearing on the right arm some weeks later.
{"title":"Breast erythema and nodular skin metastasis as the first manifestation of breast implant-associated anaplastic large cell lymphoma.","authors":"Noellie Ducastel, Ioana-Mariana Cimpean, Ivan Theate, Olivier Vanhooteghem","doi":"10.1177/20363613211028498","DOIUrl":"https://doi.org/10.1177/20363613211028498","url":null,"abstract":"<p><p>Anaplastic large cell lymphoma (BIA-ALCL) associated with rough textured breast implants was first reported in 1997. It is a non-Hodgkin's lymphoma originating from a T lymphocyte which occurs on average 10.9 years after placement of the breast implant. BIA-ALCL mainly manifests as a periprosthetic seroma or a mass adjacent to the implant. To our knowledge, we describe the first case of BIA-ALCL with initial presentation by indurate erythematous plates located in both breasts and the progressive appearance of several asymptomatic metastatic nodular lesions that have been appearing on the right arm some weeks later.</p>","PeriodicalId":46078,"journal":{"name":"Rare Tumors","volume":null,"pages":null},"PeriodicalIF":0.9,"publicationDate":"2021-06-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1177/20363613211028498","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39199382","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2021-06-27eCollection Date: 2021-01-01DOI: 10.1177/20363613211026480
Neda Kardouni Khoozestani, Maziar Motiee-Langroudi, Ali Salehi, Paniz Ranji
Schwannoma, otherwise known as neurilemmoma, is a benign tumor that originates from Schwann cells. Ancient Schwannoma is a long-standing Schwannoma with degenerative and xanthomatous changes. The Head and neck region is one of the most common sites for nerve sheath tumors but Involvement of the jaw is a considerably infrequent event. We report a rare case of intraosseous ancient Schwannoma of the mandible in a 24-year-old male patient which was initially misdiagnosed with carcinoma. The clinical features, radiographic findings, and treatment plan are discussed. Furthermore, a thorough literature review demonstrated nine published cases of intra-osseous ancient Schwannoma with their summarized features. Recognition of various histopathologic features of ancient Schwannoma is essential to prevent over-treatment.
{"title":"Intraosseous ancient Schwannoma: A rare case in the mandible and a literature review.","authors":"Neda Kardouni Khoozestani, Maziar Motiee-Langroudi, Ali Salehi, Paniz Ranji","doi":"10.1177/20363613211026480","DOIUrl":"https://doi.org/10.1177/20363613211026480","url":null,"abstract":"<p><p>Schwannoma, otherwise known as neurilemmoma, is a benign tumor that originates from Schwann cells. Ancient Schwannoma is a long-standing Schwannoma with degenerative and xanthomatous changes. The Head and neck region is one of the most common sites for nerve sheath tumors but Involvement of the jaw is a considerably infrequent event. We report a rare case of intraosseous ancient Schwannoma of the mandible in a 24-year-old male patient which was initially misdiagnosed with carcinoma. The clinical features, radiographic findings, and treatment plan are discussed. Furthermore, a thorough literature review demonstrated nine published cases of intra-osseous ancient Schwannoma with their summarized features. Recognition of various histopathologic features of ancient Schwannoma is essential to prevent over-treatment.</p>","PeriodicalId":46078,"journal":{"name":"Rare Tumors","volume":null,"pages":null},"PeriodicalIF":0.9,"publicationDate":"2021-06-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1177/20363613211026480","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39185381","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2021-06-27eCollection Date: 2021-01-01DOI: 10.1177/20363613211026494
Rui Caetano Oliveira, Maria João Martins, Carolina Moreno, Rui Almeida, João Carvalho, Paulo Teixeira, Miguel Teixeira, Edgar Tavares Silva, Isabel Paiva, Arnaldo Figueiredo, Maria Augusta Cipriano
Adrenocortical carcinomas (ACC) are aggressive tumors with a poor prognosis. Histological scores are advised for the diagnosis, however, there are borderline cases that may be misjudged as adrenocortical adenomas (ACA). The three main scores used are: Weiss Modified System (WMS), Reticulin Algorithm (RA), and Helsinki Score (HS). We intend to compare the accuracy of the three scores in ACC diagnosis and to identify predictive factors of overall survival (OS). Retrospective study (2004-2016) at Centro Hospitalar e Universitário de Coimbra of the adrenal tumors, classified as ACC or ACA, with a history of posterior tumor relapse/metastases, without lesions in the contralateral adrenal gland: 13F and 6M, with a median age of 51 ± 12.41 years. Nodules' median size was 9.20 ± 6.2 cm. Patients had a median OS of 52 ± 18.6 months, with 57.9% and 46.3%, at 3 and 5 years. Seven patients had local recurrence and nine had metastases. Thirteen cases were in stage II. The WMS and the HS allowed a diagnosis of ACC in 15 cases and the RA defined ACC in 17 cases. All cases had, at least, focal disruption of the reticulin framework. More than 5 mitosis/50 HPF was associated with worse OS: 49.67 ± 21.43 versus 108.86 ± 14.02 months (p = 0.026). In patients with stage II, tumor size ⩾10 cm was associated with worse OS: 19.25 ± 7.15 versus 96.11 ± 16.7 months (p = 0.007), confirmed by multivariate analysis (p = 0.031). The correct diagnosis of ACC is a pathologist responsibility. The RA seems the most accurate. Any loss of the reticulin framework should raise awareness for malignancy. In patients on stage II, a size ⩾10 cm is a predictor of worse prognosis.
肾上腺皮质癌(ACC)是侵袭性肿瘤,预后差。组织学评分是诊断的建议,然而,也有可能被误诊为肾上腺皮质腺瘤(ACA)的边缘性病例。使用的三种主要评分是:Weiss Modified System (WMS)、Reticulin Algorithm (RA)和Helsinki Score (HS)。我们打算比较三种评分在ACC诊断中的准确性,并确定总生存期(OS)的预测因素。回顾性研究(2004-2016年)在Universitário de Coimbra中心医院进行的,分类为ACC或ACA,有肿瘤后侧复发/转移史,对侧肾上腺无病变的肾上腺肿瘤:13F和6M,中位年龄51±12.41岁。结节的中位大小为9.20±6.2 cm。患者在3年和5年的中位OS为52±18.6个月,分别为57.9%和46.3%。7例局部复发,9例转移。13例为II期。WMS和HS诊断ACC 15例,RA诊断ACC 17例。所有病例至少有局灶性网状结构破坏。超过5次有丝分裂/50 HPF与较差的OS相关:49.67±21.43 vs 108.86±14.02月(p = 0.026)。在II期患者中,肿瘤大小小于10 cm与更差的OS相关:19.25±7.15与96.11±16.7个月(p = 0.007),经多变量分析证实(p = 0.031)。正确诊断ACC是病理学家的责任。RA似乎是最准确的。网状蛋白框架的任何缺失都应提高对恶性肿瘤的认识。在II期患者中,小于10厘米是预后较差的预测因子。
{"title":"Histological scores and tumor size on stage II in adrenocortical carcinomas.","authors":"Rui Caetano Oliveira, Maria João Martins, Carolina Moreno, Rui Almeida, João Carvalho, Paulo Teixeira, Miguel Teixeira, Edgar Tavares Silva, Isabel Paiva, Arnaldo Figueiredo, Maria Augusta Cipriano","doi":"10.1177/20363613211026494","DOIUrl":"https://doi.org/10.1177/20363613211026494","url":null,"abstract":"<p><p>Adrenocortical carcinomas (ACC) are aggressive tumors with a poor prognosis. Histological scores are advised for the diagnosis, however, there are borderline cases that may be misjudged as adrenocortical adenomas (ACA). The three main scores used are: Weiss Modified System (WMS), Reticulin Algorithm (RA), and Helsinki Score (HS). We intend to compare the accuracy of the three scores in ACC diagnosis and to identify predictive factors of overall survival (OS). Retrospective study (2004-2016) at Centro Hospitalar e Universitário de Coimbra of the adrenal tumors, classified as ACC or ACA, with a history of posterior tumor relapse/metastases, without lesions in the contralateral adrenal gland: 13F and 6M, with a median age of 51 ± 12.41 years. Nodules' median size was 9.20 ± 6.2 cm. Patients had a median OS of 52 ± 18.6 months, with 57.9% and 46.3%, at 3 and 5 years. Seven patients had local recurrence and nine had metastases. Thirteen cases were in stage II. The WMS and the HS allowed a diagnosis of ACC in 15 cases and the RA defined ACC in 17 cases. All cases had, at least, focal disruption of the reticulin framework. More than 5 mitosis/50 HPF was associated with worse OS: 49.67 ± 21.43 versus 108.86 ± 14.02 months (<i>p</i> = 0.026). In patients with stage II, tumor size ⩾10 cm was associated with worse OS: 19.25 ± 7.15 versus 96.11 ± 16.7 months (<i>p</i> = 0.007), confirmed by multivariate analysis (<i>p</i> = 0.031). The correct diagnosis of ACC is a pathologist responsibility. The RA seems the most accurate. Any loss of the reticulin framework should raise awareness for malignancy. In patients on stage II, a size ⩾10 cm is a predictor of worse prognosis.</p>","PeriodicalId":46078,"journal":{"name":"Rare Tumors","volume":null,"pages":null},"PeriodicalIF":0.9,"publicationDate":"2021-06-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1177/20363613211026494","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39185382","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2021-06-24eCollection Date: 2021-01-01DOI: 10.1177/2036361320975748
Anthony L Nguyen, Esther G Chong, Joanne Lee, Saied Mirshahidi, Hamid Mirshahidi
Cutaneous metastasis of breast cancer carries a poor prognosis, invokes a poor quality of life, and increases mortality by raising one's risk of bleeding and infection. Currently, options for treatment are systemic chemotherapy, surgical resection and radiation. These treatments are invasive and can have toxic side effects. A 50-year-old African-American woman with stage IV breast cancer with cutaneous metastasis to the left anterior chest and left supraclavicular area was successfully treated with topical imiquimod. She experienced improvement in appearance and symptoms within several months of starting treatment, resulting in near resolution of her cutaneous metastasis. Imiquimod is currently approved for several cutaneous conditions and has the potential to treat cutaneous metastasis of breast cancer.
{"title":"A case report of imiquimod topical therapy as treatment for cutaneous metastasis of breast cancer.","authors":"Anthony L Nguyen, Esther G Chong, Joanne Lee, Saied Mirshahidi, Hamid Mirshahidi","doi":"10.1177/2036361320975748","DOIUrl":"https://doi.org/10.1177/2036361320975748","url":null,"abstract":"<p><p>Cutaneous metastasis of breast cancer carries a poor prognosis, invokes a poor quality of life, and increases mortality by raising one's risk of bleeding and infection. Currently, options for treatment are systemic chemotherapy, surgical resection and radiation. These treatments are invasive and can have toxic side effects. A 50-year-old African-American woman with stage IV breast cancer with cutaneous metastasis to the left anterior chest and left supraclavicular area was successfully treated with topical imiquimod. She experienced improvement in appearance and symptoms within several months of starting treatment, resulting in near resolution of her cutaneous metastasis. Imiquimod is currently approved for several cutaneous conditions and has the potential to treat cutaneous metastasis of breast cancer.</p>","PeriodicalId":46078,"journal":{"name":"Rare Tumors","volume":null,"pages":null},"PeriodicalIF":0.9,"publicationDate":"2021-06-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1177/2036361320975748","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39173951","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}