{"title":"Dysgerminoma with Estrogen-Producing Functioning Stroma Presenting Precocious Puberty.","authors":"Shunsuke Nagase, Kanako Ogura, Karin Ashizawa, Nana Nakazawa-Tanaka, Masahiko Urao, Masaharu Fukunaga, Yuto Yamazaki, Hironobu Sasano, Toshiharu Matsumoto","doi":"10.1155/2021/5545645","DOIUrl":"https://doi.org/10.1155/2021/5545645","url":null,"abstract":"<p><p>Dysgerminoma is a malignant ovarian germ cell tumor, and unlike sex-cord stromal tumors, endocrine manifestation is considered rare. Here, we report the first case of dysgerminoma presenting precocious puberty. The patient is a 7-year-old girl who presented with a breast development in Tanner stage 3. Serum estradiol (E<sub>2</sub>) was markedly elevated while luteinizing hormone (LH) and follicle-stimulating hormone (FSH) were suppressed below the detection limit. Microscopically, the right ovarian mass displayed nests of large polygonal cells and fibrous septa which were focally concentrated by theca-like plump spindle cells. Immunohistochemistry revealed that the spindle cells expressed various steroidogenic enzymes involved in estrogen biosynthesis including P450 aromatase. The tumor was diagnosed with pure dysgerminoma with estrogen-producing functioning stroma. After the operation, serum E<sub>2</sub> declined below the detection limit; LH and FSH returned within the normal range. This case demonstrates that even a conventional dysgerminoma can present endocrine manifestation through functioning stroma.</p>","PeriodicalId":45638,"journal":{"name":"Case Reports in Pathology","volume":null,"pages":null},"PeriodicalIF":0.6,"publicationDate":"2021-04-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8112997/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39049436","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}
M Rodríguez-Zubieta, K Albarenque, C Lagues, A San Roman, M Varela, D Russo, G Podesta, D Steinberg, C Schauvinhold, A Etchegaray, M T G de Dávila
{"title":"Two Synchronous Neonatal Tumors: An Extremely Rare Case.","authors":"M Rodríguez-Zubieta, K Albarenque, C Lagues, A San Roman, M Varela, D Russo, G Podesta, D Steinberg, C Schauvinhold, A Etchegaray, M T G de Dávila","doi":"10.1155/2021/6674372","DOIUrl":"https://doi.org/10.1155/2021/6674372","url":null,"abstract":"<p><p>We report a case of a newborn with two synchronous tumors-sialoblastoma and hepatoblastoma-diagnosed at 20 weeks of gestation by magnetic resonance imaging (MRI) and ultrasonography (US). The aim of this study was to describe the management of this case together with a review of the literature. Our patient had a large facial tumor associated with extremely high alpha-fetoprotein levels. Diagnosis of the tumors was made by surgical biopsy, showing typical features in both. Sialoblastoma is a potentially aggressive tumor. In our case, the Ki67 index in the sialoblastoma was between 20 and 30%, indicating a possibly unfavorable behavior. The infant underwent surgery and chemotherapy in different steps. Complete surgical resection with clean margins is considered to be the best treatment option for sialoblastoma. Only four similar cases were previously reported. Timely management by a multidisciplinary team is essential in these difficult cases. In our patient, outcome was good at the time of this report.</p>","PeriodicalId":45638,"journal":{"name":"Case Reports in Pathology","volume":null,"pages":null},"PeriodicalIF":0.6,"publicationDate":"2021-04-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8075656/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"38889435","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":"Pulmonary Alveolar Microlithiasis: A Unique Case of Familial PAM Complicated by Transplant Rejection.","authors":"Austin Helmink, Samir Atiya, Ernesto Martinez Duarte","doi":"10.1155/2021/6674173","DOIUrl":"https://doi.org/10.1155/2021/6674173","url":null,"abstract":"<p><strong>Background: </strong>Pulmonary alveolar microlithiasis (PAM) is a rare lung disease characterized by the deposition of calcium phosphate microliths or calcospherites, within the alveolar airspace. Typical imaging findings demonstrate a \"sandstorm\" appearance due to bilateral, interstitial sand-like micronodularities with basal predominance.</p><p><strong>Methods and results: </strong>We describe an unusual case of a 48-year-old male with severe, familial PAM ultimately treated with a bilateral lung transplant.</p><p><strong>Conclusions: </strong>PAM is a rare lung disease caused by a mutation in the <i>SLC34A2</i> gene, which encodes for a sodium-phosphate cotransporter in type II alveolar cells, leading to accumulation of intra-alveolar phosphate causing microlith formation. PAM has an indolent course but can progress to chronic hypoxic respiratory failure, ultimately requiring lung transplant, the only known effective treatment.</p>","PeriodicalId":45638,"journal":{"name":"Case Reports in Pathology","volume":null,"pages":null},"PeriodicalIF":0.6,"publicationDate":"2021-04-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8041554/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"38897108","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}
Steffen Gretser, Maria-Noemi Welte, Frederik Roos, Jens Köllermann
{"title":"Leydig Cell Tumor in a Patient with 46,XX Disorder of Sex Development (DSD), Ovotesticular: A Case Report and a Review of the Literature.","authors":"Steffen Gretser, Maria-Noemi Welte, Frederik Roos, Jens Köllermann","doi":"10.1155/2021/5552305","DOIUrl":"https://doi.org/10.1155/2021/5552305","url":null,"abstract":"<p><p>Disorder of sex development (DSD) is a rare condition with atypical development of chromosomal, gonadal, or anatomical sex. It is classified in different subgroups based on the patient's karyotype, gonadal dysgenesis, and the appearance of the internal and external genitalia. Within the subgroups, the risk for developing neoplasms varies a lot. Here, we report the case of a 41-year-old patient with disorder of sex development, showing a 46,XX karyotype with an ovotestis and the simultaneous manifestation of a Leydig cell tumor in the ovotestis. The patient initially presented with infertility, and a suspicious lesion of the left testicle was noted on MRI-Scan. Upon resection, a Leydig cell tumor and an ovotestis were diagnosed. Nongerm call tumors are rare in patients with DSD. We report a nongerm cell tumor in a patient with 46,XX DSD, ovotesticular. This shows that although 46,XX DSD, ovotesticular is known to have a low potential for germ cell neoplasia, nongerm cell tumors can develop and should be into account for the management of those patients.</p>","PeriodicalId":45638,"journal":{"name":"Case Reports in Pathology","volume":null,"pages":null},"PeriodicalIF":0.6,"publicationDate":"2021-03-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8024097/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"38883917","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":"Malignant Glandular Triton Tumor Arising in the Radial Nerve with Prolonged Survival: A Case Report and Review of the Literature.","authors":"Batool M AlAli, Samir S Amr","doi":"10.1155/2021/4614185","DOIUrl":"https://doi.org/10.1155/2021/4614185","url":null,"abstract":"<p><p>Divergent differentiation is a well-known phenomenon in malignant peripheral nerve sheath tumors (MPNST) which occurs approximately in 15% of these tumors, usually towards mesenchymal elements. Differentiation towards epithelial components, however, is quite uncommon, and even exceptionally rare is concomitant mesenchymal and glandular differentiation. To our knowledge, only 14 cases of MPNST with both mesenchymal (rhabdomyoblastic) and glandular differentiation had been reported, and only two of these tumors had frankly malignant glandular components. Herein, we report the third such case. A 26-year-old male, without any of the stigmata of NF1, presented with a 2-year history of pain in his left shoulder and an elbow swelling of six-month duration. The tumor was initially diagnosed clinically as a neurofibroma at a local hospital. The patient underwent excision of the mass there, and pathological examination at that hospital showed the tumor to be MPNST. Six months later, the patient was referred to our hospital, a tertiary care medical center, with recurrent swelling at the same location. Histopathological material from the referral hospital was reviewed, and the tumor was diagnosed as MPNST with rhabdomyoblastic differentiation or malignant triton tumor (MTT) that contained in addition foci of malignant glandular epithelium. The patient refused any surgical intervention. He received three cycles of chemotherapy followed by radiotherapy with excellent response and marked reduction in the size of the tumor. The patient had prolonged survival for 10 years following the initial resection of the tumor.</p>","PeriodicalId":45638,"journal":{"name":"Case Reports in Pathology","volume":null,"pages":null},"PeriodicalIF":0.6,"publicationDate":"2021-03-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7997754/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"25537573","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}
Daniela Cabibi, Andrea Mondello, Ada Maria Florena, Giulia Rimi, Antonino Giulio Giannone, Calogero Cipolla, Maria Rosaria Valerio, Giuseppa Graceffa
{"title":"A Case of Follicular Tumor of Uncertain Malignant Potential (FT-UMP) with Glomeruloid Features Showing Capsular Mucinous Degeneration.","authors":"Daniela Cabibi, Andrea Mondello, Ada Maria Florena, Giulia Rimi, Antonino Giulio Giannone, Calogero Cipolla, Maria Rosaria Valerio, Giuseppa Graceffa","doi":"10.1155/2021/1686025","DOIUrl":"https://doi.org/10.1155/2021/1686025","url":null,"abstract":"<p><p>The most recent revision of the <i>World Health Organization (WHO) Classification of Tumours of Endocrine Organs</i> introduced a new variant of follicular thyroid carcinoma (FTC). It is characterized by a \"glomeruloid\" architectural pattern of growth. We present a case of follicular tumor with glomeruloid features, with Alcian Blue positive mucinous stromal degeneration in foci of questionable capsular microinvasion. At our knowledge, this the second case of glomeruloid follicular tumor in the literature and the first case in which Alcian Blue staining was used to investigate capsular invasion. Moreover, RAS mutation further supports that this is a variant of follicular tumor with uncertain malignant potential.</p>","PeriodicalId":45638,"journal":{"name":"Case Reports in Pathology","volume":null,"pages":null},"PeriodicalIF":0.6,"publicationDate":"2021-03-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8016592/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"25575735","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}
Jessy Nellipudi, John Brealey, Sonja Klebe, David Lance
{"title":"Atypical Nodular Pulmonary Kappa Light-Chain Deposition.","authors":"Jessy Nellipudi, John Brealey, Sonja Klebe, David Lance","doi":"10.1155/2021/5578885","DOIUrl":"https://doi.org/10.1155/2021/5578885","url":null,"abstract":"<p><p>We report a case of an incidental positron emission tomography avid right middle lobe lesion which was increasing in size. Due to concerns regarding malignancy, the patient underwent right middle lobectomy. Microscopic examination showed a 12 × 10 × 10 mm poorly circumscribed lesion composed of eosinophilic material. The material labelled strongly for kappa light chains; however, Congo red stain was only weakly positive and without \"apple-green\" positive birefringence under polarised light. Electron microscopy revealed fibrillar amyloid-like material. The features were those of kappa light-chain deposition.</p>","PeriodicalId":45638,"journal":{"name":"Case Reports in Pathology","volume":null,"pages":null},"PeriodicalIF":0.6,"publicationDate":"2021-03-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7979308/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"25525980","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":"A Case of Low-Grade Oncocytic Tumor/Chromophobe Renal Cell Carcinoma (Oncocytic Variant) of the Kidney.","authors":"Noriyoshi Ishikawa, Nao Kimura, Toshio Yoshida, Ichiro Yoshimura, Ken Nakahara, Toyonori Tsuzuki, Osamu Tokunaga","doi":"10.1155/2021/6684777","DOIUrl":"https://doi.org/10.1155/2021/6684777","url":null,"abstract":"<p><p>The oncocytic variant of chromophobe renal cell carcinoma (oChRCC) and low-grade oncocytic tumor (LOT) is introduced as new renal disease entity. Both of these tumors are low-grade malignancies consisting of cells with eosinophilic cytoplasm. Distinguishing between eosinophilic variant of chromophobe renal cell carcinoma (eCRCC) and oncocytoma is often a diagnostic challenge in routine surgical pathology. However, oChRCC and LOT might be independent disease entities that might not fit completely into any of these categories. Histologically, these tumors have greater morphological similarity with oncocytoma than with ChRCC. However, immunohistochemically, they exhibit diffuse and dense positivity for CK7 and are negative for CD117. In the present case, we initially had difficulty distinguishing among oncocytoma, eCRCC, and type 2 papillary renal cell carcinoma (2-pRCC). However, after learning about new disease entities such as oChRCC and LOT, we were able to diagnose this tumor.</p>","PeriodicalId":45638,"journal":{"name":"Case Reports in Pathology","volume":null,"pages":null},"PeriodicalIF":0.6,"publicationDate":"2021-02-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7906815/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"25454135","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":"Endometriosis Associated Striated Muscle Changes: Simulating Tumor","authors":"R. Mirza, J. Abdulsattar, J. Cotelingam","doi":"10.1155/2021/6666283","DOIUrl":"https://doi.org/10.1155/2021/6666283","url":null,"abstract":"Endometriosis in the abdominal wall can be a diagnostic challenge, both clinically and pathologically. We are describing a case of abdominal wall endometriosis where regenerative muscle cells are florid. The cells are small and polygonal in shape, arranged in groups, and intersecting through differentiated skeletal muscle. Cells have pink cytoplasm and a centrally located nucleus. Immunohistochemistry demonstrates diffuse positivity with S100 and CD56, and they are negative with Ae1/Ae3, Pax8, inhibin, calretinin, and CD68. Cells are also focally positive with actin, vimentin, and myogenin. Electron microscopy reveals the presence of intracytoplasmic myofibrils. These groups of small cells are compatible with early to intermediately differentiated skeletal muscle cells as demonstrated by histomorphology, immunohistochemical pattern, and ultrastructure analysis. Muscle undergoes a regenerative process following injury. Endometriosis associated regenerative changes in muscle are very rare as documented in the English literature. The presence of florid regenerative muscle cells mimics a neoplastic process. In this report, we describe the histomorphological and immunohistochemical pattern of regenerative muscle groups and discuss differential diagnoses. We found electron microscopy to be an important diagnostic tool in this case.","PeriodicalId":45638,"journal":{"name":"Case Reports in Pathology","volume":null,"pages":null},"PeriodicalIF":0.6,"publicationDate":"2021-02-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"80581011","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Cutaneous Coinfection of Cytomegalovirus and <i>Mycobacterium chelonae</i> Accelerated by Immunosuppression.","authors":"Yutaka Tsutsumi, Kentaro Odani, Yasuhito Kaneko, Hideo Hashizume, Mitsuhiro Tachibana","doi":"10.1155/2021/8819560","DOIUrl":"https://doi.org/10.1155/2021/8819560","url":null,"abstract":"<p><p>A mildly diabetic 58-year-old male had traumatic ulceration on the left popliteal fossa, and the lesion progressed to a painful 6 cm deep ulcer. After surgical debridement and skin grafting, ulceration recurred. Pyoderma gangrenosum was clinically diagnosed after the first biopsy, indicating a noninfective ulcer. Immunosuppressive therapy (prednisolone and cyclosporine A) induced complete epithelialization in three months. Four months later, subcutaneous nonulcerated nodules appeared on the anterior area of the left lower leg. Subcutaneous induration progressed and ulceration recurred, so that immunosuppressive therapy continued for one year. Cytomegalovirus (CMV) viremia was detected, and the second biopsy demonstrated CMV inclusions of endothelial and perivascular cells in fibrosing septolobular panniculitis. Cyclosporine A was cancelled, prednisolone was tapered, and ganciclovir started. Viremia soon disappeared, but the lesion progressed to large induration with multiple ulcers measuring up to 3 cm. The third biopsy disclosed infection of Gram-positive mycobacteria, accompanying fat droplet-centered suppurative granulomas without CMV infection. Microbial culture identified <i>Mycobacterium chelonae</i>. Clarithromycin with thermotherapy was effective. A review of the second biopsy confirmed coinfection of CMV and Gram-positive mycobacteria. Immunostaining using a panel of anti-bacterial antibodies visualized the mycobacteria in the lesion. Positive findings were obtained with antibodies to Bacillus Calmette-Guérin, <i>Bacillus cereus</i>, MPT64 (<i>Mycobacterium tuberculosis</i>-specific 24 kDa secretory antigen), LAM (<i>Mycobacterium tuberculosis</i>-related lipoarabinomannan), and PAB (<i>Propionibacterium acnes</i>-specific lipoteichoic acid).</p>","PeriodicalId":45638,"journal":{"name":"Case Reports in Pathology","volume":null,"pages":null},"PeriodicalIF":0.6,"publicationDate":"2021-01-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7867456/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"25354223","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}