A 19-year-old man presented with a mass in his right eye that had been present since birth but had gradually increased in size. The mass caused vision defects, mild discomfort on blinking, and the intermittent sensation of the presence of a foreign body.
Ovarian tumors in reptiles are uncommonly reported in the literature and for green iguanas previously reported cases include teratomas, one adenocarcinoma and one papillary cystadenocarcinoma. The present report is the first of a malignant ovarian teratoma in a green iguana. Complete and detailed pathological features, differential diagnosis and surgical management of malignant ovarian teratoma are discussed in this paper.
Well-formed cerebellum and brainstem-like structures in a mature ovarian teratoma: Neuropathological observations
- Neuropathology : official journal of the Japanese Society of Neuropathology
- Published almost 4 years ago
In the surgical case of a mature cystic teratoma of the ovary that arose in a 16-year-old girl, a large amount of well-differentiated and highly organized cerebellar tissue was found. Three layers of the cerebellar cortex were well formed, and synaptophysin-positive “glomeruli” were found in the granule cell layer. Some Purkinje cells exhibited focal expansion and a dysmorphic appearance of the dendrites. Adjacent to the cerebellar tissue, a large space lined by the ependymal layer and a club-shaped CNS tissue mass resembling the brainstem were found, and structures reminiscent of the midbrain tectum and pontine nuclei were distinguished within this mass. The CNS tissue was surrounded by the leptomeninges and a skull-like, bony shell. Structures consistent with the supra-tentorial CNS tissue were not found. This case represents an example of infra-tentorial CNS tissue that was well-differentiated and organized to an exceptionally high degree in an ovarian mature teratoma. Various degenerative changes have been documented in CNS tissue in ovarian teratomas, but the dendritic abnormalities of Purkinje cells seen in the present case are novel findings.
Goldenhar syndrome is well known for its classical triad of epibulbar dermoids or lipodermoids, auricular appendages and pretragal fistulas. Here we report a case of Goldenhar syndrome with an unusual association of a fibroepithelial polyp attached to a limbal dermoid. A case of Goldenhar syndrome in a 5-month-old male infant presented with the features of a fibroepithelial polyp attached to a limbal dermoid, right-sided polydactylia with hypoplastic thumb, and accessory preauricular appendages on the left side. The association of a fibroepithelial polyp attached to a limbal dermoid with Goldenhar syndrome is a rare report in the literature. In these cases, regular follow-up with an ophthalmologist is important to monitor the visual development of the patient. Ideally, a multidisciplinary approach is required to manage the other associated anomalies.
Periorbital dermoid cysts are benign tumors most often seen in young children. Intraorbital location, though rare, should not be overlooked. Good quality imaging plays a major role in the etiological diagnosis, providing a precise analysis of the location of the lesion, its components, and its effects on adjacent and nearby structures, as well as in planning the surgical approach. The authors report a case of a 46-year-old male with an intraorbital dermoid cyst presenting with progressive left proptosis. Appearance on imaging (CT and MRI) was consistent with intraorbital dermoid cyst. The tumor was extirpated. Histopathologic examination confirmed the diagnosis of dermoid cyst. We also present a review of the literature.
Anti-NMDA-R encephalitis has been described as a cause of acute psychosis in young females. It is rare during pregnancy. We describe a primigravida 32-year-old woman with acute onset psychosis during the first trimester. Eight weeks after becoming pregnant, the patient became psychotic with associated catatonia and autonomic disturbance. Serum anti-NMDA-R antibodies were found. She responded to plasma exchange. At caesarean section, a healthy baby boy was born and a benign mature cystic teratoma was removed from the left ovary. Catatonia associated with psychosis may occur in pregnancy secondary to anti-NMDA-R encephalitis. Prompt and aggressive treatment can lead to a good outcome for both baby and mother.
We describe an 11-month-old boy with an unusually large presternal mass present since birth. The large size, fluctuant properties, transillumination, compressibility, and imaging of this lesion were characteristic of a lymphatic malformation. Although four treatments with sclerotherapy markedly reduced its size, it was not until definitive treatment with surgical excision and the final pathology report that we arrived at the ultimate diagnosis of dermoid cyst. Dermoid cysts, although appearing along embryologic lines of closure, are rarely presternal. They are usually small, thick walled, and filled with sebaceous or keratinous fluid, which typically allows for clinical diagnosis, and show characteristic features on magnetic resonance imaging (MRI) and ultrasound. However, this case illustrates that dermoid cysts can appear in somewhat atypical locations, and imaging is not always diagnostic, so dermoid cyst should remain a part of the differential diagnosis for any lesion presenting midsternally, regardless of the size and imaging characteristics.
A 30 years old nullipara with a diagnosis of papillary thyroid carcinoma with nodal metastases had undergone total thyroidectomy and nodal dissection for the same. In the postoperative work-up for radioiodine-131 ((131)) ablation of the residual tissue, she was found to have on (131)I whole-body scan multifocal uptake in the neck and a solitary focus in the mediastinum. In addition, there was a relatively intense focal uptake in the right pelvis above the urinary bladder. Ultrasonography (USG) of the pelvis demonstrated a 2.3×2.0cm mostly echogenic mass with some sound attenuation in the right ovary. No color uptake to suggest internal vascularity was observed within this mass. Axial plain CT without contrast, undertaken in order to discard iodine interference, demonstrated in the 2.0×1.54cm right ovarian mass cavity with fat attenuation. Magnetic resonance imaging (MRI) of the pelvis with diffusion weighted imaging (DWI) was undertaken to further characterize the right ovarian lesion noninvasively. The axial T1-weighted spin-echo MR image showed a high-signal-intensity mass of the right ovary . On the fat-saturated T2-weighted fast spin-echo MR, the mass demonstrated saturation of the cystic contents with punctate high signal intensity of size 2.0×1.8cm. On diffusion weighted MRI the cystic contents of the lesion showed high signal on DWI, which had low apparent diffusion coefficient (ADC) value, attributable to fat and keratinoid substance. The ADC value of the cystic contents was 0.570×10(-3)mm(2)/s, while the cystic component presented values of 1.77×10(-3)mm(2)/s. The above findings were commensurate with the diagnosis of an ovarian dermoid cyst. Dermoid cysts are cystic teratomta that contain developmentally mature skin, complete with hair follicles and sweat glands and can differentiate into a variety of tissues (e.g. sebum, blood, fat, bone, nails, teeth, eyes, cartilage) including thyroid tissue. The incidence of thyroid tissue in the ovarian dermoids ranges from 11% to 18% though in one series it was 7.3%. Though mostly benign, they may undergo malignant transformation in one of its elements, usually develop squamous cell carcinoma. Malignant transformation of the thyroid tissue is extremely rare, usually being papillary thyroid carcinoma. A struma ovarii is a rare form of mature teratoma that contains mostly thyroid tissue. They can present with intriguing findings on functional scans, an understanding of which is important to obviate errors. Noting the age and nulliparous status of the patient, treatment with (131)I was postponed so that the dermoid cyst could be surgically removed before high dose administration of (131)I, so as to reduce the absorbed radiation dose to the right ovary. In conclusion, it is important to recognize the physiological and pathological aetiologies (unrelated to thyroid) that demonstrates radioiodine uptake and may lead to false positive radioiodine scan in patients of differentiated thyroid carcinoma. They require to be differentiated from uncommon metastatic sites, which also show radioiodine uptake and warrants for radioiodine therapy for disease control. An appreciation is essential for proper management and will obviously prevent administration of inappropriate radioiodine therapy doses.
An 8.5-year-old girl presented with breast development, irregular uterine bleeding, and a spurt in height during the previous 9 months. She also complained of rapid increase of abdominal girth and body weight (4 kg). No remarkable past history or family history was noted. Her height (143 cm), breast development (Tanner stage 3), and pubic hair (Tanner stage 2) were compatible with precocious puberty. Laboratory examinations showed microcytic hypochromic anemia (Hb 9.2 g/dL), and serum estradiol was 138 pg/mL. LH and FSH were in the undetectable level even after a LH-RH (100 μg) injection. Ovarian tumor markers including CEA, CA19-9, CA125, α-fetoprotein, and hCG were within the normal range. Magnetic resonance imaging revealed a giant multilocular cystic tumor (25 × 14 × 10 cm) in the lower abdomen.Tumor resection was performed. The tumor originated from the right ovary and contained 3500 × mL of yellowish serous fluid. The left ovary was grossly normal. The resected right ovarian tumor was multilocular cystic, measuring 16.0 × cm in the greatest dimension, with occasional foci of whitish yellow elevated components in the cystic wall.
Protocols for pediatric germ cell tumors (GCT) allow for chemotherapy (CHT) initiation without histological diagnosis, based on typical clinical and radiological picture and increased alphafetoprotein (AFP) or beta-human chorionic gonadotropin serum levels. Such strategy may result in misdiagnoses in rare cases. We present two patients with abdominal tumors and high serum AFP levels, diagnosed as GCT. In both, no tumor shrinkage and increasing AFP was observed after first cycles of multidrug CHT for pediatric GCT. Histological examination of biopsied tumor tissues revealed metastatic cholangiocarcinoma in patient 1 and pancreatoblastoma in patient 2, which implicated immediate change of therapy. Presented cases support the necessity to consider the tumor biopsy when patients diagnosed with GCT based on typical clinical presentation and elevated AFP do not respond to CHT with AFP decrease and tumor size reduction.