Rare TumorsPub Date : 2021-09-23eCollection Date: 2021-01-01DOI: 10.1177/20363613211044566
Sobiya Ansari, Yixiang Liao, Summer Dewdney, Dian Wang, Parul Barry
{"title":"Vaginal oligometastatic disease of colorectal primary: Report of a novel therapeutic approach.","authors":"Sobiya Ansari, Yixiang Liao, Summer Dewdney, Dian Wang, Parul Barry","doi":"10.1177/20363613211044566","DOIUrl":"https://doi.org/10.1177/20363613211044566","url":null,"abstract":"<p><p>Vaginal oligometastatic disease of colorectal primary is a rare malignancy with few reported cases in the literature and no standardized treatment paradigm. We report on the definitive management of an unusual case of an elderly woman with the aforementioned disease. A 78-year-old African-American woman presented with vaginal spotting and was found to have a vaginal lesion. Final pathology was consistent with moderately differentiated adenocarcinoma of colorectal primary. Extensive work up, which included endoscopies, pathologic analyzes, and imaging workup, did not reveal a primary gastrointestinal malignancy. The patient underwent partial vaginectomy and final pathology once again confirmed moderately differentiated adenocarcinoma of colorectal primary (CDX 2 and CEA positive, ER/PR, and CK 7 negative) with negative margins. She went on to receive adjuvant concurrent chemoradiation with 5-FU based chemotherapy. She received 45 Gy in 25 fractions to the whole pelvis followed by an HDR brachytherapy boost to 12 Gy in two fractions. Unfortunately, 10 months after completing radiation, she was found to have adenocarcinoma arising from a hepatic flexure colon polyp on colonoscopy. She required definitive surgical resection and was staged as mpT3N0M1. She received 12 cycles of 5-FU and at 2-year follow-up was found to be disease free with no evidence of locoregional recurrence or distant metastatic disease. Continued long-term follow up is warranted.</p>","PeriodicalId":46078,"journal":{"name":"Rare Tumors","volume":" ","pages":"20363613211044566"},"PeriodicalIF":0.9,"publicationDate":"2021-09-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/cd/6f/10.1177_20363613211044566.PMC8474297.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39494352","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}
Rare TumorsPub Date : 2021-08-29eCollection Date: 2021-01-01DOI: 10.1177/20363613211043662
Akwasi Ofori Abayie, Kofi Mensah Nyarko, Markus Bährle, Alfred Brütting
{"title":"The first case report of primary thyroid teratocarcinosarcoma: An analog to sinonasal teratocarcinosarcoma.","authors":"Akwasi Ofori Abayie, Kofi Mensah Nyarko, Markus Bährle, Alfred Brütting","doi":"10.1177/20363613211043662","DOIUrl":"https://doi.org/10.1177/20363613211043662","url":null,"abstract":"<p><p>Teratocarcinosarcoma is a rare and aggressive tumor usually affecting the sinonasal tract. It arises primarily from the nasal cavity, paranasal sinuses with some reported cases arising from the nasopharynx and oral cavity and commonly referred to as Sinonasal Teratocarcinosarcoma (SNTC). We present the first case of teratocarcinosarcoma as a primary thyroid cancer in a 17-year-old male patient who presented with a rapidly growing anterior neck mass with no symptoms. Physical examination revealed circa 4 cm × 5 cm slightly right sided, non-tender, firm anterior neck swelling. A thyroid ultrasound revealed an enlarged thyroid gland with multiple thyroid nodes. Magnetic Resonance Imaging (MRI) of the head and neck showed no sinonasal tract tumor. Thyroidectomy and surgical resection of the tumor was performed. Histological examination revealed teratocarcinosarcoma of the thyroid gland, an analog to SNTC with no primary sinonasal tissue involvement. This implies that, teratocarcinosarcoma can occur in primary tissues other than sinonasal origin contrary to conventional knowledge.</p>","PeriodicalId":46078,"journal":{"name":"Rare Tumors","volume":" ","pages":"20363613211043662"},"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/ea/1b/10.1177_20363613211043662.PMC8408892.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39386457","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}
Rare TumorsPub 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
{"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":" ","pages":"20363613211034710"},"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}
Rare TumorsPub Date : 2021-08-20eCollection Date: 2021-01-01DOI: 10.1177/20363613211039724
Mousa ElKhaldi, Rakan Radi, Maysa Al-Hussaini
{"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":" ","pages":"20363613211039724"},"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}
Rare TumorsPub 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
{"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":" ","pages":"20363613211034115"},"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}
Rare TumorsPub 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
{"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":" ","pages":"20363613211029493"},"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}
{"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":" ","pages":"20363613211029487"},"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}
Rare TumorsPub Date : 2021-06-30eCollection Date: 2021-01-01DOI: 10.1177/20363613211026483
Andrea Dekanić, Marko Velepič, Margita Belušić Gobić, Ita Hadžisejdić, Nives Jonjić
{"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":" ","pages":"20363613211026483"},"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}
Rare TumorsPub Date : 2021-06-30eCollection Date: 2021-01-01DOI: 10.1177/20363613211028498
Noellie Ducastel, Ioana-Mariana Cimpean, Ivan Theate, Olivier Vanhooteghem
{"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":" ","pages":"20363613211028498"},"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}
Rare TumorsPub Date : 2021-06-27eCollection Date: 2021-01-01DOI: 10.1177/20363613211026480
Neda Kardouni Khoozestani, Maziar Motiee-Langroudi, Ali Salehi, Paniz Ranji
{"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":" ","pages":"20363613211026480"},"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}