Spontaneous Partial Regression of Bilateral Grade III Corneal Dermoids Associated With Fetal Valproate Spectrum Disorder.

PURPOSE: The purpose of this study was to describe a rare case of a child with bilateral central corneal dermoids (grade III) in association with fetal valproate spectrum disorder (FVSD) and to report the spontaneous regression of these tumors. METHODS: Clinical records of a 14-month-old child whose mother took sodium valproate all along her pregnancy were retrospectively reviewed. The diagnosis of FVSD was made based on phenotypic features and associated congenital malformations. Facial features included trigonocephaly, flat nose bridge and small upturned nose, cleft palate and lip, and micrognathia. Systemic anomalies included bilateral radial defects and club hands, pes equinovarus, hypospadias, secundum atrial septal defect, patent ductus arteriosus, and aortic insufficiency. Cytogenetic studies were normal. RESULTS: Ocular findings included bilateral central corneal dermoids sparing the limbus and peripheral cornea, bilateral aphakia, absence of left anterior chamber, and bilateral mass-like vitreal opacities. A computed tomography scan suggested minimal left microphthalmia. Owing to the high-risk category for general anesthesia and prioritization of other severe systemic anomalies, no ocular surgical intervention was performed. Over 5 years of follow-up, spontaneous partial regression of the corneal tumors was observed. CONCLUSIONS: The development of bilateral grade III corneal dermoids in a child with FVSD may be more than fortuitous and enlarges the list of ocular anomalies associated with FVSD. Corneal dermoids may regress spontaneously.

Malignant Transformation of Mature Cystic Teratoma: Is Squamous Cell Carcinoma Different From the Other Types of Neoplasm?

OBJECTIVES: Malignant transformation of mature cystic teratoma (MCT) is rare. Unlike squamous cell carcinoma (SCC) in MCT, the other types of neoplasm in MCT have not been discussed in publications. We analyzed the clinical characteristics and prognosis of the other types of neoplasm (non-SCC) compared with those of SCC. METHODS: A systematic literature search of literature published from 2000 to 2017 was conducted in PubMed, Web of Science, and Scopus. We reviewed case series that included all pathological types of malignant transformation. RESULTS: A total of 155 cases from 15 case series, including our cases, were included. Of the cases, 90 (58%) were SCC and 65 (42%) were non-SCC, including adenocarcinoma, carcinoid tumor, thyroid carcinoma, sarcoma, adenosquamous carcinoma, melanoma, sebaceous carcinoma, oligodendroglioma, signet ring cell carcinoma, and transitional cell carcinoma, in descending order of frequency. The mean ages of patients with SCC and non-SCC were 50.5 and 48.9 years, respectively. The mean tumor sizes were 14.7 cm in SCC and 13.9 cm in non-SCC. Surgical approaches were similar. First-line chemotherapy for epithelial ovarian cancers was the most commonly used regimen in SCC and non-SCC. Overall survival did not differ significantly, showing better prognosis in stage I and poor prognosis in stages II, III, and IV. A difference in overall survival was observed among pathological types of non-SCC. CONCLUSIONS: Clinical characteristics and outcomes did not differ significantly between SCC and non-SCC. However, chemotherapy regimens differed to some extent, and the possibility of difference in overall survival among pathological types of non-SCC was suggested.

Quiste epiglótico gigante: ¿es imprescindible la traqueotomía? / Giant epiglottic cyst: is tracheostomy essential?

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A series of malignant ovarian cancers arising from within a mature cystic teratoma: a single institution experience.

BACKGROUND: Mature cystic teratoma (MCT) is the most common germ cell tumor. It accounts for 10% to 20% of all ovarian masses. The likelihood of malignancy arising from within an MCT is low, and prognosis is poor. METHODS: A single-institution retrospective chart review was completed of all cases of MCT from 2004 to 2012. Multiple variables were examined including procedure performed, residual disease after surgery, surgical stage, histologic type, site of primary disease, date of recurrence, whether or not adjuvant chemotherapy was given, and whether or not there was death secondary to disease. RESULTS: During the study period, 1.2% of MCTs exhibited malignant transformation. The average age at presentation was 53.7 years. Mean follow-up time was 23 months. The most common presenting symptoms were bloating and abdominal pain. The average tumor size was 18 cm. Of note, 33% of cases were at least surgical stage IIIC at the time of presentation, whereas the remainder were stage IC or lower. Four (44.4%) of the 9 cases were identified as mucinous adenocarcinoma in addition to 1 case each of malignant melanoma, squamous cell carcinoma, and poorly differentiated adenocarcinoma. Only 1 patient experienced recurrence. One patient had a known MCT that was being managed expectantly and exhibited malignant transformation to a mucinous adenocarcinoma. CONCLUSIONS: A large ovarian mass that is suspected to be a mature teratoma should be managed more aggressively in older patients. Our data suggest that although malignancy arising from mature teratomas is rare, it is more likely when patients are older than 40 years, the mass is greater than 18 cm, and there is any suspicion for a mucinous tumor. Like most ovarian tumors, these tumors most often present at later stages and, thus, can be difficult to treat. It is unclear what role chemotherapy or radiation plays in the management of these tumors.

Surgical management of corneal limbal dermoids: retrospective study of different techniques and use of Mitomycin C.

PURPOSE: We retrospectively studied the efficacy and safety of different surgical approaches to treating pediatric limbal dermoids with regard to intra and postoperative complications. METHODS: The data of 12 consecutive patients (14 eyes), who underwent monocentric surgery of a limbal demoid in the past 9 years, were retrospectively analyzed for intra and postoperative complications. Group one consists of eleven eyes: seven eyes with a bare-sclera deep lamellar excision of the dermoid and four eyes with an additional amniotic membrane transplantation subsequent to excision. Group two consists of three eyes: two eyes treated with Mitomycin C 0.02% over 2 min following the excision, and one eye treated with Mitomycin C and amniotic membrane transplantation after removal of the dermoid. Follow-up ranged from 2 to 53 months (median 17 months). RESULTS: Intraoperative complications did not occur in any of the patients. Postoperatively, all patients showed corneal reepithelialization within a week. Limbal stem cell deficiency with a pseudopterygium developed in four eyes, one treated with bare-sclera excision and the others with amniotic membrane transplantation. One pseudopterygium had to be removed surgically because of visual acuity deterioration. Not a single eye treated with Mitomycin C developed a pseudopterygium. CONCLUSIONS: The transplantation of amniotic membrane following removal of a limbal dermoid cannot prevent the occurrence of a pseudopterygium. However, the use of Mitomycin C seems to have a protective effect.

Dermoid cysts: a report of 75 pediatric patients.

Dermoid cysts (DCs) are benign cutaneous tumors that tend to persist and grow. The aim of this study was to examine the clinicopathologic features of congenital DCs. We present a case series of 75 children with a clinicopathologic diagnosis of DC. Seventy-two cysts were located on the head, one on the neck, and two on the trunk. Six cysts were located along the midline. Eight patients had symptoms other than changes in cyst size. Imaging studies were performed on 15 patients. Surgical excision was the primary treatment in all 75 cases. Neurosurgery and ophthalmology services were involved in the care of some patients. Histopathologic studies reported a foreign body giant cell reaction in 17 of the cysts. No recurrence was documented. DCs can remain stable for years, but they can become symptomatic as a result of enlargement and rupture or, more rarely, as a result of extension into surrounding tissues. Physicians should be aware that certain locations have a higher risk of DC extension, and adequate diagnostic investigations should be performed before their complete resection.

Presternal dermoid cyst mimicking lymphatic malformation: a case report and review of the literature.

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.

Heightened seizure susceptibility associated with brain dermoid cyst and the administration of human chorionic gonadotropin (hCG).

It is known that the intramuscular injection of human chorionic gonadotropin (hCG) lowers the threshold for motor evoked responses (MEPs) in the first dorsal interosseous (FDI) muscle to transcranial magnetic stimulation (TMS) in humans. We describe the case of a patient with a clinically silent left-sided nasofrontal dermoid cyst who, while being treated with hCG/LH for hypogonadotropic hypogonadism, presented with simple partial seizures, ipsilateral to the cyst, with secondary generalization. Motor cortex excitability was studied by single and paired TMS and MEPs were recorded from FDI. Resting motor threshold (RMT), active motor threshold (AMT), MEP size, intracortical inhibition (ICI) and intracortical facilitation (ICF) were tested during and after suspension of hormonal therapy. RMT and AMT were lower, MEP size was larger, ICI was decreased while ICF was slightly diminished during treatment. Overall, this indicated a reduced intracortical inhibition during hormonal therapy. It is concluded that treatment with hCG/LH may favour seizure onset in the presence of potentially epileptogenic lesions such as an intracranial dermoid cyst.

TNF-based isolated limb perfusion in unresectable extremity desmoid tumours.

BACKGROUND: Desmoid tumours are soft tissue sarcomas with local aggressive behaviour and a high rate of local recurrence after treatment. Although they do not tend to metastasise systemically, the local aggressiveness can lead to situations in which limb-preserving surgery cannot be performed without severe disability. As isolated limb perfusion (ILP) with TNF and melphalan has proven to be extremely effective in the treatment of soft tissue sarcoma, we studied its potential in locally advanced extremity desmoid tumours. METHODS: Prospectively maintained database in a tertiary referral centre. Between 1991 and 2003, 12 ILP procedures were performed in 11 patients for locally advanced desmoid tumours. Local surgical therapy with preservation of limb function was impossible in all patients due to large or multifocal tumours, multiple recurrences or extensive previous treatment. Perfusions were performed with 4-3mg TNF and 10-13 mg/l limb volume melphalan form leg and arm perfusions, respectively. RESULTS: Overall response rate was 75%: Two complete responses were recorded (17%) and seven patients had a partial response (58%). Amputation could be avoided in all cases. Local control was obtained after 10/12 ILPs and in the other two patients through repeat ILP and systemic chemotherapy, thus leading to an overall local control rate of 100%. Local toxicity was mild and systemic toxicity was absent in all patients. CONCLUSION: ILP is a very effective treatment option in the multimodality treatment of limb desmoid tumours. It should be considered in patients with aggressive and disabling disease where resection without important functional sacrifice is impossible.

Lesiones quísticas y pseudoquísticas del suelo de la boca con afectación submentoniana / Cyst and pseudocyst lesions of the floor of the mouth with submental affectation

Pretendemos revisar los métodos diagnósticos que pueden ayudamos a diferenciar las lesiones quísticas de suelo de boca con afectación submentoniana, así como las peculiaridades de la técnica quirúrgica indicada en cada una de ellas.Presentamos dos casos que fueron diagnosticados de quistes epidermopides. En el diagnóstico diferencial deben ser considerados las ránulas, los lipomas y los linfangiomas. Se revisan el diagnóstico por la imágen, la punción-aspiración con aguja fina y el tratamiento de cada tumor. Ambos casos fueron intervenidos mediante un abordaje intraoral. Actualmente se encuentran libres de enfermedad después de al menos un año. El diagnóstico diferencial de las lesiones quísticas del suelo de la boca es importante porque la técnica quirúrgica recomendada no es exactamente la misma en todas ellas. La firmeza de la pared de los quistes dermoides permite su exéresis vía intraoral aún cuando presenten un gran tamaño. Para el tratamiento de las ránulas la mayoría de autores recomienda la exéresis de la glándula sublingual, a pesar de la morbilidad de dicha técnica

There are several lesions which can present as a cyst or pseudocyst of the floor of the mouth with submental repercussion. The aim of this paper is to review the diagnosis methods which can help us to differentiate these lesions such as the surgical peculiarities of every tumour. We are reporting two cases of cystic/pseudocystic lesions ofthe floor of the mouth with submental repercussion. Both of them were epidermoid cysts. Ranulas, lipomas and lymphangiomas should be considered in the differential diagnosis. Imaging diagnosis, fine needle aspiration and adequate treatment in all of the tumours are reviewed. Both cases were operated via intraoral. They are now free of disease after at least one year. Differential diagnosis of cystic lesions of the floor of the mouth is important because the recommended surgery technique is not exactly the same in all of them. The firmness of the wall of dermoid cysts let their exeresis via intraoral even when they are of a big size. Exeresis of sublingual gland is recommended by most authors to treat ranulas, although it is followed by an important percentage of morbidity

[A case of synchronous double cancer responding to UFT--dermoid cyst with secondary malignant transformation and uterine endometrial adenocarcinoma].

We report here a case of synchronous dermoid cyst with secondary malignant tumor and uterine endometrial adenocarcinoma that responded to UFT. A 35-year-old female complained of abdominal fullness and visited our hospital. She had an abdominal mass which was newborn-head size. We performed right salpingo-oophorectomy and partial omentectomy. The pathological findings were dermoid cyst with secondary malignant transformation. After the operation she had underwent cyclic chemotherapy with CDDP, CPA, THP and 5-FU. After three cycles of chemotherapy, a uterine recurrence was suspected from her uterine endocervical smear test. Then we performed a second operation, but radical surgery was impossible due to the presence of multiple metastases to pelvic lymph nodes. The pathological findings were primary uterine endometrial adenocarcinoma, not metastasis from dermoid cyst with secondary malignant tumor. After the second operation, she was treated with oral UFT (400 mg/day), as she refused chemotherapy and radiotherapy. Two months after the start of UFT, the tumor markers were reduced remarkably, and the patient maintained good QOL throughout the treatment without serious adverse events. We conclude that UFT might be benefical in the treatment of advanced gynecologic cancer.

[Neoplasms of the genitalia in young girls]. / Novoobrazovaniia polovykh organov u devochek-podrostkov.

The results of an analysis of 197 clinical examinations of young girls aged from 8 to 19 years with neoplasms of the genitals have shown that ovarian tumors made 98.5% of the cases. The benign forms prevailed over malignant ones. The distribution of the ovarian tumors in the girls by the histotype considerably differs from that in adult women with the same tumors. Only 24.7% of the true ovarian tumors had the epithelial forms, while 75.3% were nonepithelial and 80% of them represented germinal tumors. An analysis of literature data and personal observations prove high sensitivity of malignant tumors of the genitals in puberty girls to chemotherapy which allows the organ-saving operations to be performed. The timely diagnosis of genital tumors in girls based on the well organized children and adolescent gynecological service can promise the optimistic prognosis.

[Mature teratomas of testicular origin. Discussion of 12 cases]. / Les tératomes matures d'origine testiculaire. Réflexions à propos de douze cas.

Between 1979 and 1989, 135 patients with a non-seminomatous germ cell tumour of the tests were treated in the Val de Grâce Military Hospital in Paris. In twelve patients, histological examination of a residual mass after chemotherapy revealed the presence of mature teratoma. Although this tumour, considered to be benign, generally represents a favourable form of the initial tumour, it nevertheless has a potential for progression. The possibility of long-term recurrences, which are sometimes difficult to eradicate, justifies very careful long-term surveillance of these patients. The authors report three particularly demonstrative cases and present a review of the literature.