Primary retroperitoneal mucinous cystadenoma with sarcoma-like mural nodule

Primary retroperitoneal cystadenomas are extremely rare. This is the first report in literature to describe a primary retroperitoneal cystadenoma with a sarcoma-like mural nodule. A 45-year-old woman complained of a left-sided abdominal mass. A computed tomography scan revealed a cystic mass with a mural nodule, which seemed to originate from the tail of the pancreas. At laparotomy the cyst was not adhered to the pancreas but localized retroperitoneally. Histologic examination showed a mucinous cystadenoma with only foci of borderline malignancy with a mural “sarcoma-like” nodule. In view of the surgical and histopathological findings, the mucinous cystadenoma was regarded as primary retroperitoneal. This case demonstrates that in the era of radiological preoperative refinement, pathological diagnosis remains of utmost importance, especially for rare cases.     

卵巢黏液性囊性肿瘤伴实性附壁结节

目的：探讨卵巢黏液性囊性肿瘤伴实性附壁结节的临床病理学特点。方法：对2例卵巢黏液性囊性肿瘤进行光镜观察和免疫组化染色并复习文献。结果：1例卵巢黏液性交界性乳头状囊腺瘤伴实性附壁恶性纤维组织细胞瘤结节，结节内瘤细胞呈多形性。1例卵巢黏液性囊腺瘤伴实性附壁间变性癌结节；间变性癌结节内瘤细胞体积较大，胞质丰富，嗜酸，少数胞质透明，排列呈巢或索状；免疫表型：EMA和cytokeratin阳性。恶纤组结节中肿瘤细胞AACT和vimentin阳性。结论：免疫组化有助于卵巢黏液性囊性肿瘤伴实性附壁结节的诊断及鉴别诊断。     

Ovarian mucinous cystic tumor with sarcoma-like mural nodules and multifocal anaplastic carcinoma: a case report

A 48-year-old woman presented with left abdominal pain and fullness. Computed tomography scan revealed a multicystic mass with multifocal mural nodules. Histologic examination showed a mucinous cystic tumor with cystadenoma, borderline malignant cystadenoma and cystadenocarcinoma, which were associated with sarcoma-like mural nodules (SLMNs) and multifocal anaplastic carcinoma. Mural nodules showed a positive reaction for CD56 and vimentin, but were negative for cytokeratin 7 and SMA. She underwent postoperative chemotherapy and is currently under follow-up; no recurrence or metastases were found in the first year of follow-up. Ovarian mucinous cystic tumor with SLMNs and foci of anaplastic carcinoma is extremely rare. To our knowledge, this case reports the most complex neoplastic and reactive components. Our findings shed some light on the pathogenesis of this rather rare carcinoma. We think that the formation of SLMNs may be the result of the reactive proliferation of undifferentiated mesenchymal cells, while the anaplastic carcinoma may be derived from mucinous epithelium. Moreover, because of difficulties encountered in their differential diagnosis, we think that the existence of foci of anaplastic carcinoma along with SLMNs necessitates careful histologic and immunohistochemical analysis of mural nodules for the determination of treatment and prognosis.     

Pure (non-papillary) serous cystadenoma of the epididymis: a histologic and immunohistochemical study

A 44-year-old man presented with painless right scrotal swelling of 2 years duration. A cystic tumor strictly attached to the head of the epididymis was surgically resected. The pathologic examination revealed a unilocular cyst with a thin fibrous capsule, lined by ciliated cubical or cylindrical columnar cells, mostly arranged in a single layer. No papillary projection could be detected. Immunohistochemical staining was positive for epithelial membrane antigen, low- and high molecular weight cytokeratins, progesterone receptor, vimentin, and S-100 protein, but was negative for carcinoembryonic antigen, CD10, p53 protein, and calretinin. Single MIB-1 positive cells were noted. Histologic and immunohistochemical features suggest a Müllerian origin or differentiation. The lesion was diagnosed as pure serous cystadenoma of the epididymis, possibly originating from vestigial remnants of the Müller duct in male. The differential diagnosis to spermatocele and adenomatoid tumor of the epididymis is discussed.     

Mucinous cystadenoma of the pancreas with huge mural hematoma

A 60-year-old woman was referred for evaluation of a cystic mass in the pancreatic body that extended to the tail. Transabdominal ultrasonography demonstrated an oval cystic mass 24 cm in diameter, filled with debris. On the cyst wall there was a wide-based, smooth-surfaced, heterogeneous high-echoic protrusion that was 5 cm in diameter. On CT the protrusion showed internal enhancement. Endoscopic pancreatography showed no intraductal mucin or communication with the cyst. A distal pancreatectomy was performed under the diagnosis of mucinous cystadenocarcinoma. Grossly there was a brownish, hemispherical protrusion into the thin monolocular cyst. The cut surface of the protrusion showed a peripheral yellow-brownish area and an internal wine-colored area. Histopathologically the cyst wall consisted of tall columnar cells without atypical nuclei, ovarian-type stroma beneath the epithelium, and fibrotic tissue with abundant capillary vessels, suggestive of a mucinous cystadenoma. The protrusion was composed of peripheral organized hematoma without a covering epithelium, and internal hemorrhage and many capillary vessels, with no evidence of tumor cell necrosis. These histopathological findings appear to be similar to those of chronic expanding hematoma. The formation of a huge mural hematoma in a mucinous cystic neoplasm can occur as a repair process after the breaking of intrawall vessels.     

Prognostic significance of HLA-DR antigen in serous ovarian tumors

Abstract. The antigens encoded by the major histocompability complex (HLA-DR) are cell glycoproteins that play a fundamental role in the regulation of the immune response. The prognosis of ovarian cancer is dependent on the histological type and on the clinical stage at diagnosis. Our study reports the value of HLA-DR antigen as a prognostic marker of ovarian serous adenocarcinoma. We studied 31 cases of serous ovarian cystadenoma, 12 cases of serous ovarian borderline cystadenoma, and 39 cases of well-differentiated cystadenocarcinoma for HLA-DR monoclonal antigen. We also studied the T helper marker (CD4) in the tumor stroma of the relevant cases, given that it is now known that the dependence of immune responsiveness on the class II antigens reflects the central role of these molecules in presenting antigen to T helper cells. HLA-DR was expressed in 20 of 31 cystadenomas (64.5%), 4 of 12 borderline tumors (33.3%), and in 10 of 39 invasive carcinomas (25.6%). CD4 was expressed in 9 of 31 cystadenomas (29%), 5 of 12 borderline tumors (42%), and in 26 of 39 invasive carcinomas (67%). There was a statistically significant difference for the two examined antigens in cystadenomas (p<0.001) and invasive carcinomas (p<0.001), whereas there was no statistical difference in borderline tumors (p<0.5). The results showed decreased expression of HLA-DR and increased expression of CD4 as the lesion progressed to malignancy. The aberrant expression of HLA-DR by epithelial cells of cystadenomas, of borderline tumors, and of invasive adenocarcinomas agrees with the hypothesis of the adenoma/adenocarcinoma sequence. The immune attraction mechanism by low HLADR signaling seems to be of minor importance in the malignant and metastatic potential of serous ovarian tumors.     

Solid variant of serous cystadenoma of the pancreas

We describe a case of a solid variant of serous cystadenoma of the pancreas. The preoperative examination results led to a diagnosis of a nonfunctional pancreatic islet cell tumour, and the patient underwent a pylorus-preserving pancreaticoduodenectomy. The tumour was diagnosed as a solid variant of serous cystadenoma by histopathological examination. Solid variant of serous cystadenoma of the pancreas is difficult to diagnose preoperatively. More cases must be accumulated and investigated to obtain clues for accurate diagnosis.     

Ovarian mucinous cystadenocarcinoma with malignant mural nodules

A case of ovarian muclnous cystadenocarcinoma with malignant mural nodules is reported. The patient was a 28-yearold Japanese female (gravida 0, para 0) with a 2 year history of increasing abdominal fullness and edema of the lower extremity. Physical examination showed a large mass in the abdomen. An abdominal ultrasound and computed tomography demonstrated a multilocular cyst with a solid component. Lymph node and distant metastases were not found. These tests were followed by surgery. The resected right ovarian tumor measured 25 × 22 × 18 cm. On cut sectioning, it was multilocular with multiple mural nodules. Microscopically, the cyst wall was lined with papillary in-foldings of atypical mucinous epithelium (intestinal type). Nuclear stratification, crlbrlform and back-to-back glandular patterns and stromal invasion were observed. In addition, the solid area of the mural nodules showed spindle or polygonal cells with Increased mitotic activity Including atypical mitoses. Nuclear pleomorphism was marked. Necrosis and hemorrhage were also present. Reticulin stain showed these pleomorphic cell clusters circumscribed by reticulin fibers and these cells were immunoreacthre for vimentln and p53.     

Ovarian mucinous cystadenoma with mural nodule of anaplastic carcinoma.

The occurrence of malignant mural nodule in benign cystic common epithelial tumor of the ovary have been reported in only three cases; the case one was mucinous cystadenoma with a mural nodule of fibrosarcoma and the others were of carcinomas. Our case was another rare case of ovarian mucinous cystadenoma with mural nodule of anaplastic carcinoma in a 42-year-old woman. The cystadenoma had an unilocular cystic cavity and a mural nodule with thick multinodular solid wall. The internal cystic wall was lined with mucinous cystadenoma without any malignant features. The mural nodule showed anaplastic carcinomatous differentiation and its nature was confirmed by immunohistochemistry and electron microscopy. This tumor had metastasized to the right salpinx, uterus, cul-de-sac, periureter and mesentery.     

原发性卵巢血管肉瘤伴浆液性囊腺瘤1例及文献复习

目的探讨原发性卵巢血管肉瘤伴浆液性囊腺瘤的临床病理特征及鉴别诊断。方法对病例进行病理组织学和免疫组织化学观察,复习文献。结果该例卵巢血管肉瘤伴浆液性囊腺瘤临床表现为下腹隐痛、腹部肿块。眼观见肿块呈囊性,囊壁增厚,切面见红褐色出血性肿块。镜下以充满红细胞的大小不等的囊腔、相互沟通的不规则管道、大部分由梭形细胞构成的实性区域为特征,伴有浆液性囊腺瘤形成。免疫表型：CD31、CD34、FⅧRAg、PCNA呈广泛性强阳性,SMA在中等大小的血管及部分肿瘤细胞呈强阳性,Ki-67为散在阳性,CK（AE1／AE3）为阴性。结论原发性卵巢血管肉瘤十分罕见,必需注意其鉴别诊断。     

Peritoneal melanosis combined with serous cystadenoma of the ovary: a case report and literature review

Benign peritoneal melanosis is extremely rare and traditionally occurs in association with ovarian dermoid cysts, but rarely with peritoneal cyst, enteric duplication cyst or gastric triplication. The pathogenesis of peritoneal melanosis, in particular, the origin of the pigment-producing cells is unclear. We describe a case of peritoneal melanosis that was associated with ovarian serous cystadenoma in a young woman. Ovarian serous cystadenoma has not been previously described as a combined lesion of peritoneal melanosis. Based on the extremely rare incidence of this lesion and heterogeneous combined lesions, the possibility of an incidentally found, coexisting lesion couldn't be excluded. Here, we suggest that peritoneal mesothelial cells pinched off during the developmental period might be a source of pigment-producing cells.     

Cytology of a giant adult-type rhabdomyoma of the tongue

Adult rhabdomyoma is a rare primary benign tumour of striated muscle origin that almost exclusively presents in the head and neck region with predilection for male. We herein report a case of an adult rhabdomyoma in a 67-year-old male with cytologic features. The lesions was located in the tongue extended to the left parapharyngeal region. We discuss the cytological findings with a brief review of the literature on this entity.     

Ovarian serous cystadenoma associated with Sertoli-Leydig cell tumor--a case report.

We Describe a case of ovarian serous cystadenoma having Sertoli-Leydig cell tumor, well differentiated, in the cystic septum. Well differentiated Sertoli-Leydig cell tumor coexisting with other tumor, including serous tumor, has not yet been described. In all cases of Sertoli-Leydig cell tumor with heterologous components or other tumors, the androblastomatous components are intermediately or poorly differentiated. The present case revealed a well differentiated Sertoli-Leydig cell tumor arising in a septum of serous cystadenoma, as a circumscribed nodule. With these findings, we discuss the possibility of this Sertoli-Leydig cell tumor, considered a mural nodule, which is well established in cystic common epithelial tumors of the ovary.     

Intratesticular serous cystadenoma of borderline malignancy. A pathological,histochemical and DNA content study of a case with long-term follow-up

A 61-year-old man presented with a painless right testicular swelling of 6 months duration. A right orchiectomy was performed and pathological examination showed an intratesticular serous borderline tumour (SBT). Immunohistochemical staining was positive for carcinoembryonic antigen, LeuM1, B72.3, S100-protein, Ca125, cytokeratins AE1/AE3 and vimentin, suggesting a Müllerian origin or differentiation. DNA image analysis revealed an aneuploid histogram. The favorable outcome of the patient confirms that testicular SBTs behave as non-aggressive tumours, even when characterized by aneuploid DNA content.     

Presence of satellite cells in a cardiac rhabdomyoma

Cardiac rhabdomyoma is the most common tumour of the heart in infancy and childhood. The clinical presentation, diagnosis and histopathological characteristics have been extensively studied; however, reports on the ultrastructure and histogenesis of this lesion are scanty and inconclusive. The case to be discussed is that of a 10-year-old male who presented with a cardiac rhabdomyoma occupying almost the entire ventricular apex. Ultrastructurally, the rhabdomyoma cells have a central, deeply-indented nucleus surrounded by an admixture of mitochondria and sarcomeres. The remainder of the cytoplasm is occupied by pools of glycogen granules, randomly-orientated myofibrils and small mitochondria. Intercellular junctions are numerous and consist of alternating zonula occludens and macula adherens. Typical satellite cells, sharing a common basement lamina are seen apposed to the rhabdomyoma cells. It is tempting to postulate that the proliferation of the rhabdomyoma cells is accomplished by differentiation of satellite cells, a process known to occur in skeletal muscle. Ultrastructurally, the rhabdomyoma cells are indistinguishable from Purkinje cells. The presence of Purkinje-like cells in ectopic locations within the heart and their association with satellite cells is likely a form of embryological atavism.     

Serous cystic neoplasms of the pancreas: Clinicopathologic and molecular characteristics

We herein summarize the pathology and most recent advances in the molecular genetics of serous cystic neoplasms of the pancreas. They typically present as relatively large, well-demarcated tumors (mean size, 6 cm), predominantly occurring in females. Pre-operative diagnosis remains challenging; imaging findings and cyst fluid analysis often prove non-specific and fine-needle aspiration often does not yield diagnostic cells. Pathologically, they are characterized by a distinctive cytology referred to as “serous.” Although they have ductal differentiation, they distinctly lack the mucin production that characterizes most other pancreatic ductal tumors, including ductal adenocarcinoma and its variants, intraductal papillary mucinous neoplasm (IPMN) and mucinous cystic neoplasm (MCN). They instead produce abundant glycogen (glycogen-rich adenoma). Serous cystadenomas also lack the molecular alterations that characterize ductal neoplasms, such as mutation of KRAS (high prevalence in most mucinous ductal neoplasms), inactivation of SMAD4 (seen in ductal adenocarcinomas), and mutations in GNAS (seen in some IPMNs) and RNF43 (detected in MCNs and IPMNs). Instead, new molecular and immunohistochemical observations place serous pancreatic tumors closer to “clear cell neoplasms” seen in various other organs that are associated with the von Hippel–Lindau (VHL) pathway, such as clear cell renal cell carcinomas and capillary hemangioblastomas. Patients with VHL syndrome have an increased risk of developing serous pancreatic tumors and somatic mutations of the VHL gene are common in these tumors along with modification of its downstream effectors including hypoxia-inducible factor (HIF1), glucose uptake and transporter-1 (GLUT-1), a common factor in clear cell (glycogen-rich) tumors, as well as expression of vascular endothelial growth factor (VEGF), thought to be a factor in the striking capillarization of serous cystadenomas and other non-pancreatic clear cell tumors. VEGF may prove to be of significant diagnostic value since its elevation in cyst fluid has recently been found highly sensitive and specific for serous neoplasms. These molecular alterations establish serous tumors as prototypes of clear cell tumorigenesis and angiogenesis and may prove helpful both as diagnostic and non-surgical therapeutic targets.     

Fine-needle aspiration of adult rhabdomyoma: a case report with review of the literature

The cytologic features of adult rhabdomyoma, a rare benign tumor of skeletal muscle origin, have been infrequently reported in the literature. We present here a case of a rhabdomyoma involving the floor of the mouth of a 78-year-old man initially diagnosed by fine-needle aspiration cytology. Cytologic features seen on Papanicolaou-stained smear preparation included cohesive clusters of skeletal muscle cells having abundant eosinophilic cytoplasm and often peripherally located nuclei. Although cross-striations and elongated intracytoplasmic inclusions were not identified in the smears, they were noted in the cell block preparation of the aspirated specimen and in the touch preparation and histologic sections of the surgically resected specimen. The cytologic differential diagnosis of this tumor is discussed.     

Mucinous cystadenoma of the testis

A 35-year-old man complained of a painless enlargement of the right testis. Imaging diagnostic procedures demonstrated a multiloculated cystic tumor, 9 cm in maximal diameter, in the right testis with hydrocele. Orchidectomy specimen showed that the tumor was confined within the testis and separated from the epididymis. The locules of the tumor were lined by single-layered columnar epithelium, intermingled with MUC2 immunopositive goblet and chromogranin-A immunopositive neuroendocrine cells, exhibiting intestinal differentiation. No ciliated cell, teratomatous element or intratubular germ cell neoplasia were seen. Channels of rete testis were compressed peripherally by the tumor but there was no connection with the tumor locules. The tumor was diagnosed as mucinous cystadenoma of the testis. This seems to be the first published case of benign mucinous cystadenoma occurring within the testis. This intratesticular tumor with intestinal differentiation may represent a benign monodermal teratoma.     

Genital rhabdomyoma of the urethra in an infant girl

Extracardiac rhabdomyomas are rare benign entities that usually occur in the head and neck region. Although genital rhabdomyoma is known to occur in the lower genital tract of young and middle-aged women, involvement of the anatomically adjacent urethra by rhabdomyoma is exceedingly rare. We present a case of genital rhabdomyoma arising from the urethra of an infant girl. The tumor was characterized by the submucosal presence of mature-appearing rhabdomyoblastic cells containing conspicuous cross-striations, with the cells set in a collagenous stroma. Necrosis and mitoses were absent. Skeletal muscle differentiation of the tumor cells was supported by positive immunohistochemical staining for desmin and myogenin. To our knowledge, this is the first case of urethral genital-type rhabdomyoma in a child.