Background Primary breast sarcoma (PBS) is rare, comprising approximately 1% of breast malignancies. Rhabdomyosarcoma (RMS) accounts for an extremely small proportion of PBSs, often leading to delayed histologic confirmation.
Methods Upon reviewing Asan Medical Center’s pathology database between 2000 and 2018, 41 PBS cases were retrieved, including three cases of primary RMS of the breast. Their clinicopathological features were analyzed, and the literature related to PBS and primary RMS of the breast was reviewed.
Results We identified three primary breast RMS cases from our institution database, comprising 7.3% of PBS: one case each of spindle cell/sclerosing RMS (ssRMS), alveolar RMS (aRMS), and embryonal RMS (eRMS). All cases involved adolescents or young adults (14, 16, and 25 years, respectively) who underwent mastectomy or radiotherapy and were confirmed using immunohistochemical testing for myogenin, desmin, and myogenic differentiation. The ssRMS patient experienced recurrence at the operation site 4 months post-surgery despite undergoing concurrent chemoradiotherapy. The aRMS patient had multiple metastases at diagnosis and showed FAX3-FOXO1 fusion transcripts; she died 22 months after the diagnosis. The eRMS patient had enlarged axillary lymph nodes; post-radiotherapy, the lesion recurred as multiple metastases to the bone and lung. She died 18 months post-diagnosis.
Conclusions Our experience on RMS cases suggests that spindle cell or small round cell malignancy in breasts of young female should raise suspicion for the possibility of primary or secondary RMS. To our knowledge, this is the second report of primary breast ssRMS and it may help clinicians who encounter this rare disease in the future.
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So-called sclerosing hemangioma of the lung is a rare benign neoplasm which usually presents with a coin lesion detected through routine chest X-ray. We report two cases showing characteristic cytologic appearances which have been rarely reported. Both cases were young females with coin lesions in the lung. Fine needle aspiration of each case revealed unique but some different cytologic features. Case 1 showed bland-looking polygonal epithelial cells resembling bronchioloalveolar cells having predominantly papillary configurations with loosely arranged solid sheets or isolated cells. Cytoplasms were plump, cyanophilic, and finely granular, with eccentric nuclei. The nuclei were usually monotonous, round-to-ovoid, and vesicular with a small but conspicuous nucleolus. In comparison to case 1, case 2 revealed largely loose pavement-like solid sheets or clusters rather than papillary patterns in the hemorrhagic background. The size of tumor cells were a little smaller than that of case 1. Bronchiotoalveolar carcinoma and papillary adenocarcinoma of metastatic origin were considered to be one of the important differential diagnoses with these cytologic features. Histologically, both cases exhibited findings compatible with so-called sclerosing hemangioma of the lung.
Chronic sclerosing sialadenitis, Mikulicz disease or Kuttner tumor has been recently recognized as a spectrum of IgG4-related sclerosing disease. IgG4-related disease is characterized by a high serum IgG4 level and tissue infiltration of IgG4-positive plasmacytes. We report three cases of chronic sclerosing sialadenitis with variably associated systemic involvement. All patients presented with a submandibular mass or swelling, and all the resected submandibular glands showed diffuse lymphocytic infiltration, lymphoid follicles, and septal fibrosis. Two of the specimens revealed numerous IgG-positive plasma cells, most of which were IgG4-positive on immunohistochemical staining. One of them was associated with dacryoadenitis and hypophysitis. The other patient had ureterorenal lesions. Immunohistochemical study was unavailable in remaining one case, but the histologic features along with elevated IgG level and associated pancreatitis supported the diagnosis. All patients received steroid therapy postoperatively and are doing well. Salivary gland involvement in IgG4-related fibrosclerosis should be recognized in systemic medical pathology.
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BACKGROUND The exact preoperative diagnosis of diffuse sclerosing papillary carcinoma (DSPC) is required for aggressive surgical treatment due to its extended involvement with thyroid and neck lymph nodes. The present study investigated the cytomorphologic characteristics of DSPC and identified cytologic features for preoperative diagnosis of DSPC. METHODS: A retrospective review of cytologic and histologic features of 16 patients diagnosed with DSPC after thyroidectomy and underwent preoperative fine needle aspiration cytology (FNAC) was performed. RESULTS Prominent psammoma bodies were observed in 16 (100%) and 10 (62.5%) cases of histology and FNAC, respectively. Lymphocytes were observed in nine (56.2%) and four (25.0%) cases, and squamous cells were noted in seven cases (43.7%) and one case (6.2%) on histology and FNAC, respectively. Nuclear grooves and inclusions, which are characteristics of papillary carcinoma, were observed in FNAC and histology slides in all 16 cases. CONCLUSIONS: DSPC displays prominent psammoma bodies and characteristic nuclear features of papillary carcinoma such as nuclear groove and inclusion in FNAC. However, the preoperative diagnosis of DSPC using only FNAC could be difficult due to the absence of other characteristic features such as lymphocytes and metaplastic squamous cells.
Primary sclerosing cholangitis (PSC) is usually progressive to the end stage liver disease and a common indication for liver transplantation (LT). We report two cases of liver explants with PSC. Case 1 was that of a 35-year-old male who was admitted for 4 weeks because he had jaundice. He had been treated several times without receiving a definite diagnosis during the last 7 years because he was excreting dark urine. Case 2 was that of a 30-year-old female who had taken a prednisolone for 6 years for treatment of ulcerative colitis. She also had stones and underwent a cholecystectomy and choledocholithotomy 2 years ago. She had complained of epigastric pain and febrile sensation for 2 months. In both cases, serum levels of aspartate aminotransferase, alanine aminotransferase, alkaline phospatase, gamma glutamyl transpeptidase, and direct builirubin were elevated.
Autoantibodies were not detected. Computerized tomography scan and endoscopic retrograde cholangiopancreatography revealed hepato-splenomegaly, stricture of intra- and extra-hepatic bile ducts, and beaded dilatation of the intrahepatic bile ducts. Despite stent insertions and medication, another episode of jaundice occurred in case 1 and cirrhosis finally developed in case 2. The histopathologic features of liver needle biopsy specimens were nonspecific, while explant livers displayed periductal fibrosis, ductopenia, and/or ductular proliferation, which are characteristic features for PSC.
Sclerosing hemangioma is a rare, benign neoplasm of the lung, usually presented as a solitary pulmonary nodule in an otherwise asymptomatic middle-aged woman.
Cytologically it shows papillary, sheet, and cyst-like arrangements representing three main histologic patterns of papillary, solid, and angiomatous ones, respectively. Herein, we report the fine needle aspiration cytology of 5 cases of sclerosing hemangioma of the lung. The most characteristic finding is cyst-like spaces intimately related to the papillary or solid cell nests. The tumor cells are relatively monotonous, round to oval, small to medium in size. They have small amount of eosinophilic cytoplasm.
The nuclei are uniform, round and have small but conspicuous nucleoli. The tumor cells in one of the presenting cases are large with abundant cytoplasm and show moderate nuclear pleomorphism. The nuclear chromatin, however, is fine and even without exception, even in the case showing nuclear pleomorphism. Major differential diagnoses based on the cytologic findings are well-differentiated papillary adenocarcinoma, bronchioloalveolar carcinoma, and carcinoid tumor.
Diffuse sclerosing papillary carcinoma(DSPC), a variant of papillary carcinoma of the thyroid, is characterized by diffuse involvement of one or both thyroid lobes, and histologic features such as prominent sclerosis, intense lymphocytic infiltrate, num erous psammoma bodies, and squamous metaplasia together with the charac teristic cytoarchitectural pattern of classical papillary carcinoma. We experienced a case of fine needle aspiration cytologic(FNAC) findings of DSPC, which was con firmed by histologic examination of the thyroidectomy specimens. The patient was 26 years old female who presented with diffuse firm enlargement of the thyroid gland with enlargement of many cervical lymph nodes. FNAC smears showed numerous psammoma bodies, many lymphocytes, metaplastic squamous cells, absence of stringy colloid, and epithelial cells showing classical features of papillary carcinoma, such as nuclear grooves, intranuclear inclusions, and ground glass chromatin pattern.
Fine needle aspiration (FNA) cytological examination is an appropriate method for the evaluation of pulmonary nodules.
In major types of lung cancer, its diagnostic accuracy is quite high. However, it is sometimes difficult, using this technique, to differentiate between some unusual phenotypes including adenosquamous carcinoma, bronchioloalveolar carcinoma (BAC), neuroendocrine tumor, mucoepidermoid carcinoma, and sclerosing hemangioma. Here, we present a case involving extremely well differentiated adenosquamous carcinoma, mimicking benign lesions, such as pulmonary scar and adenomatoid malformation with squamous metaplasia. The patient was a 68-year-old man presenting with a solitary pulmonary nodule (1.6x1.6 cm), which was incidentally found at the periphery of the right lower lobe.
FNA revealed some clusters of glandular cells with minimal atypia, in addition to squamous cells at a nearly full maturational state. Histological examination verified the cytological diagnosis on a lobectomy specimen. The tumor exhibited a well differentiated adenocarcinoma component, mimicking the bronchioles in scarred lung tissue, and a well differentiated squamous cell carcinoma component, mimicking the squamous cell nests of adenoacanthoma, in the other organs. In the present case, the possibility of adenosquamous carcinoma should have been considered if squamous cells were seen in the FNA from the peripheral pulmonary nodule, even though they appeared to be benign.
Chronic sclerosing sialadenitis, also known as Kuttner tumor, is a benign chronic inflammatory lesion of the salivary gland. Here, we describe a case of chronic sclerosing sialadenitis with mucinous ductal metaplasia in a parotid gland, which was confused with low-grade mucoepidermoid carcinoma on aspiration cytology.
Liposclerosing myxofibrous tumor (LSMFT) is a benign fibro-osseous lesion that is characterized by a complex mixture of histologic elements, including its fibrous dysplasia-like features and its lipoma, myxofibroma, xanthoma and pseudo-Paget's bone patterns. However, this lesion is considered by some researchers as a variant of fibrous dysplasia or as the non-specific end result of degenerative change, while it is considered by others as a definite clinicopathologic entity. Here, we report on a case of LSMFT occurring in tibia, which is a very uncommon location for this tumor, and we review the related literatures. The case presented here shares features with those described for LSMFT, except for the location of this tumor. We believe that more studies on a larger scale that compare LSMFT with other benign bone lesions, including fibrous dysplasia, are required to clarify the origin and behavior of this lesion.
Sclerosing hepatic carcinoma (SHC) is composed of slender cords or small nests of tumor cells with peripheral palisading, and abundant intervening sclerosis. The tumor seems to have the histologic features of both hepatocellular carcinoma (HCC) and cholangiocarcinoma. To evaluate the phenotypic expression of SHC and to investigate its cellular origin, immunohistochemical studies on three cases of SHC were performed. In all cases, the tumor cells showed positive staining for cytokeratins AE1, AE3 and 19, carcinoembryonic antigen (CEA) and epithelial membrane antigen (EMA). The expressions of cytokeratins AE1 and 19 were stronger in the palisading cells than the interior of the cords and nests. Conversely, CEA and EMA were expressed mainly in the inner portion. Alpha-fetoprotein was expressed in only one case, mainly in the palisading cells. In summary, SHC has the histological as well as the immunohistochemical profiles intermediate between HCC and cholangiocarcinoma, and the immunohistochemical profile suggests that SHC arises from primitive hepatoblast with a tendency of differentiation to the bile duct epithelium.
A complex syndrome, later called as Prader-Willi syndrome, was first described in 1956 by Prader et al, and Zellweger and Schneider characterized this syndrome as hypogonadism, hypotonia, hypomentia and boesty. It is not rare in western countries and more than 400 cases have been reported until 1983. But our interest arose because of our recent experience of diffuse noncirrhotic fibrosis of the liver in a 6 year-old boy who had the clinical features of Prader-Willi syndrome. The core of liver showed destruction of most of the hepatic lobules, particularly of the acinar zone 3, and replacement bt diffuse fibrosis. The remaining liver cells underwent fatty change, and the overall changes resembled chronic sclerosing hyaline disease of the alcoholic type. Inflammation was negligible. This particular case suggests that the severe fatty change of liver could result in irreversible damage to the hepatocytes and progressive fibrosis.