Hypercalcemia Associated with a Malignant Brenner Tumor Arising from a Mature Cystic Teratoma

A 60-year-old woman presented with abdominal pain and weight loss and was found to have serum calcium of 15.0 mg/dl. Serum parathyroid hormone-related peptide (PTHrP) returned elevated. Imaging suggested bilateral mature cystic teratomas. Her hypercalcemia was treated initially with intravenous saline, as well as intramuscular and subcutaneous calcitonin. She underwent total abdominal hysterectomy with bilateral salpingo-oophorectomy, and final pathology revealed malignant Brenner tumor in association with a mature cystic teratoma. Her postoperative PTHrP returned less than assay, and her total and ionized calcium fell below normal, requiring supplemental calcium and vitamin D. At follow-up one month after discharge, her calcium had normalized. We present the first reported case of hypercalcemia occurring in association with a malignant Brenner tumor. Malignancy-associated hypercalcemia occurs via four principal mechanisms: (1) tumor production of PTHrP; (2) osteolytic bone involvement by primary tumor or metastasis; (3) ectopic activation of vitamin D to 1,25-(OH)₂ vitamin D, and (4) ectopic production of parathyroid hormone. PTHrP-mediated hypercalcemia is the most common mechanism and was responsible in this case. In patients with paraneoplastic hypercalcemia who undergo surgical treatment, close monitoring and management of serum calcium is necessary both pre- and postoperatively.Key Words: Hypercalcemia, Parathyroid hormone-related peptide, Mature cystic teratoma, Malignant Brenner tumor     

Primitive Neuroectodermal Tumor in an Ovarian Cystic Teratoma: Natural Killer and Neuroblastoma Cell Analysis

In the present study, we report an extremely rare case of a 31-year-old woman with neuroblastoma arising in an ovarian cystic teratoma. We analyzed the expression of activating receptors on natural killer (NK) cells derived from the patient''s peripheral blood and peritoneal fluid. In addition, we investigated the presence of specific ligands recognized by different NK cell receptors on tumor cells. We show that NK cells isolated from peritoneal fluid expressed certain triggering receptors including DNAM-1 (CD226) and CD16 with lower intensity as compared to peripheral blood NK cells. Remarkably, at variance with most cases of childhood neuroblastoma, the tumor cells from this patient expressed substantial amounts of HLA class-I molecules. These molecules are known to be protective against NK cell-mediated lysis. In addition, neuroblastoma cells expressed B7-H3 (CD276), another surface molecule that inhibits NK cell function. Finally, this tumor did not express the PVR (CD155) and nectin-2 (CD112) ligands for the DNAM-1 activating NK receptor, which plays a crucial role in NK/neuroblastoma interactions. Altogether, these findings indicate that the neuroblastoma cells of this patient express an NK-resistant surface phenotype, which is at least in part similar to that previously described in a fraction of childhood neuroblastoma.Key words: Activating natural killer receptors, Natural killer cells, Neuroblastoma, Ovarian cancer     

卵巢囊性成熟性畸胎瘤恶变11例分析

为了了解卵巢囊性成熟性畸胎瘤恶变临床特征及预后 ,对 11例卵巢囊性成熟性畸胎瘤恶变的临床症状、肿瘤分期、病理类型、治疗进行回顾性分析。结果 11例患者平均生存时间为 6 8 6个月 (12～ 16 8个月 ) ,5年生存率为5 7 2 %。研究结果提示 ,卵巢囊性成熟性畸胎瘤恶变发生率低、恶性度高 ,手术治疗 ,辅以化、放疗有一定疗效。     

Neuroendocrine Tumor Arising within Mature Cystic Teratoma of the Pancreas: Literature Review and Case Report

Cystic teratomas are germ cell tumors most commonly found in the ovaries and testes. The pancreas, however, is very rare as a site of occurrence. Moreover, only two cases of cystic teratoma with concomitant neuroendocrine tumor have been reported to date. We report the case of a 33-year-old female who presented with abdominal pain. Computed tomography and magnetic resonance imaging of the upper abdomen revealed an 85 mm cystic tumor in the head of the pancreas. Cystic teratoma and mucinous cystadenoma were suggested as differential diagnoses. Cytopathologic analysis of endoscopic ultrasound-guided fine needle aspiration was consistent with mucinous cystadenoma. Therefore, the patient underwent surgical resection. Histologic analysis revealed a mature cystic teratoma of the pancreas with a concomitant neuroendocrine tumor. The patient is in great condition at 8 months follow-up. Cystic teratoma of the pancreas with a concomitant neuroendocrine tumor is an extremely rare condition. Surgical resection remains the mainstay of treatment as it provides a definitive diagnosis and no recurrences have been reported to date.     

Ganglioneuroma of the Adrenal Gland: A Rare Tumor in a Rare Location

A 62-year-old man presented to his general practitioner complaining of non-specific back pain. He underwent a computerized tomography scan and magnetic resonance imaging that revealed a large left adrenal mass. A thorough investigation of this mass revealed it to be a non-secreting tumor. At surgery, a large tumor of the left adrenal was found. The final pathology report revealed a ganglioneuroma of the adrenal gland.Key Words: Ganglioneuroma, Adrenal, Sympathetic chain, Retroperitoneum, Neuroblastoma     

Congenital Ranula in a Newborn: A Rare Presentation

Ranulas are cystic lesions in the floor of the mouth. Case reports published worldwide have been very few. They are formed either as retention cyst or as pseudocyst due to extravasation of mucus in the surrounding tissue. We report the case of a full term female neonate with a congenital ranula in the floor of mouth on left side. The swelling caused no discomfort or complication and hence no immediate intervention was required. The ranula was treated by aspiration using a wide bore needle and did not recur on 4 months follow up. Other methods of treatment include excision of ranula, marsupialization, cryosurgery, sclerotherapy. As many congenital cysts resolve or rupture spontaneously, they should be observed for potential resolution for several months in uncomplicated cases.     

Rare mature B‐cell lymphomas in children and adolescents

Pediatric‐type follicular lymphoma (PTFL), pediatric nodal marginal zone lymphoma (pnMZL), and large B‐cell lymphoma (LBCL) with IRF4 rearrangement have been introduced into the current World Health Organization (WHO) classification. They account for 5% to 10% of mature B‐cell lymphomas in children and adolescents. Both PTFL and pnMZL predominantly affect male adolescents and usually present with localized lymphadenopathy in the head and neck region. The cells within the follicles of PTFL typically show high‐grade cytology, IGH monoclonality and lack the t(14;18) chromosomal alteration. In contrast, pnMZL is characterized by progressive transformation of germinal center (PTGC)‐like features and interfollicular proliferation of the cells with expansion of the marginal zones with diffuse areas. Watch and wait after complete resection seems an adequate therapy with chemotherapy restricted to incompletely resected disease. All children with PTFL and pnMZL reported, so far, survived. B‐cell lymphomas presenting in the Waldeyer's ring are characterized by the expression of IRF4/MUM1 and often associated with IRF4 rearrangements. Because of the frequent diffuse component, treatment often follows current protocols for mature B‐NHL. The prognosis is excellent.     

Growing Teratoma Syndrome with Synchronous Gliomatosis Peritonei during Chemotherapy in Ovarian Immature Teratoma: A Case Report and Literature Review

Coexistent growing teratoma syndrome (GTS) and gliomatosis peritonei (GP) arising during chemotherapy of ovarian immature teratoma (IMT) is extremely rare and can be misdiagnosed as recurrent or progressive disease. We present a 33-year-old woman diagnosed with GTS with synchronous GP during chemotherapy of IMT. She underwent ovarian cystectomy due to ovarian immature teratoma and chemotherapy were administered. The α-fetoprotein (AFP) concentration decreased from 28.7 ng/mL to normal after the second cycle. Four days after the third cycle of chemotherapy, ultrasound and CT revealed an 8-cm mass with negative tumor markers in the pouch of Douglas. An exploratory laparotomy was conducted, and a smooth round cystic-solid 8-cm mass was noted in the pouch of Douglas. Extensive peritoneal seeding glial nodules were also observed on the surface of the uterus, peritoneum, and omentum. The patient underwent a partial omentectomy, intact resection of the tumor, and resection of most of the glial nodules. Postoperative pathology demonstrated a pure mature cystic teratoma component in the mass, as well as diffuse GP involving the uterine serosa, peritoneum, and omentum; this diagnosis of GTS with synchorous GP should be considered in IMT patients with mass newly identified during chemotherapy while tumor markers are normal after treatment.     

Malignant Transformation Rate and P53, and P16 Expression in Teratomatous Skin of Ovarian Mature Cystic Teratoma

Objective: To investigate the incidence of malignant transformation and P53 and P16 expression interatomatous skin of ovarian mature cystic teratoma. Materials and Methods: Data on ovarian teratomaspecimens in nearly 10 years were reviewed. P53 and P16 expression were detected by immunohistochemistryin 25 cases of teratomatous skin of ovarian mature cystic teratoma, 20 cases of squamous cell carcinoma and 2cases of squamous cell carcinoma originated from teratomatous skin. Results: Of 1913 cases of ovarian maturecystic teratoma in nearly 10 years, only two cases of squamous cell carcinoma were found in teratomatous skin,with malignant transformation rate of 0.1045%. P53 expression was detected in 2 cases squamous cell carcinomaoriginated from teratomatous skin and P16 overexpression in one. There were no expressions of P53 and P16 in25 cases of teratomatous skin of ovarian mature cystic teratoma. Of 20 cases of squamous cell carcinoma P53overexpression (positive rate of 55%) was detected in 11 cases, P16 overexpression (positive rate of 35%) in 7cases. The positive rates of P53 and P16 expression in squamous cell carcinomas were significantly higher thanthat in the teratomatous skins (p< 0.001, p= 0.002). Conclusions: There was low risk of malignant transformationin teratomatous skin of ovarian mature cystic teratoma which can be explained by lower P53 and P16 expressioninteratomas than that in squamous cell carcinoma.     

Giant Epignathus with Intracranial Teratoma in a Newborn Infant

A male newborn infant with a giant epignathus associated with intracranial teratoma and obstructive hydrocephalus detected antenatally is reported. Only 4 similar cases have been reported in the literature. This disorder is uniformly lethal. We believe this is the first reported case of epignathus with congenital intracranial teratoma diagnosed antenatally by ultrasonography.