Unilateral Papillary Cystadenoma of the Epididymis as a First Presentation of Von Hippel-Lindau Disease.

Association between papillary cystadenoma of the epididymis (PCE) and Von Hippel-Lindau Disease (VHLD) is well known and stronger for bilateral tumors. Unilateral PCE occurs either as a sporadic tumor without evidence of VHLD or in the context of a known diagnosis of VHLD, indeed it has never been reported as the first manifestation of VHLD. In contrast, we report the case of a boy with an apparently isolated, unilateral PCE that resulted to be the first manifestation of an unknown VHLD. Thus, we recommend screening for VHLD in patients with a new diagnosis of unilateral PCE, especially if the patients are young.

Papillary cystadenoma of the epididymis.

Papillary cystadenoma is a rare benign neoplasm of the epididymis, occurring mainly in young adult males. More than one-third of the cases reported in the literature have occurred in patients with von Hippel-Lindau disease. Conversely, epididymal nodules presumed to be papillary cystadenomas are found in one-third of males with von Hippel-Lindau disease. The association is stronger for bilateral tumors. The pathogenesis involves loss of the von Hippel-Lindau gene resulting in overexpression of the angiogenic protein "hypoxia-inducible factor." Papillary cystadenoma is of mesonephric derivation. It originates in the efferent ductules of the head of the epididymis in the form of tiny precursor lesions. Histologically, papillary cystadenoma is characterized by cystic spaces with intracystic papillary projections lined by clear cells, with a resultant resemblance to renal cell carcinoma. Immunohistochemical markers may facilitate the distinction between the 2 tumors. Treatment consists of surgical excision and the prognosis is excellent.

Bilateral papillary cystadenoma of the mesosalpinx: a rare manifestation of Von Hippel-Lindau disease.

We report a rare case of a woman with bilateral papillary cystadenomata of the broad ligament with von Hippel-Lindau disease (VHL) (other manifestations: capillary hemangioblastomas of the spinal cord). Patient surveillance is important, because in the course of VHL-associated tumors malignant lesions may arise that are relevant for the prognosis.

[Endolymphatic sac tumors]. / Tumeur du sac endolymphatique.

Tumors of the endolymphatic sac are rare and can be found in patients with Von Hippel Lindau disease. They most often develop within the intrapetrosal part of the sac but can sometimes be located in the distal part. Their growth is slow and they spread in two directions: laterally toward the external and middle ear and in the direction of the jugular foramen and medially into the ponto-cerebellar angle. The symptoms are usually auditive, with a constant neurosensorial deafness of sudden onset in 50 % of cases and sometimes associated with a tinnitus and dizziness. The varieties with medial extension result in a cerebellopontine angle syndrome. Diagnosis is made by imaging (CT scan and MRI) that reveals a heterogeneous tumor between the lateral sinus and the internal auditory meatus, hypervascularized showing contrast, with cystic zones and associated with bone lysis. Histological examination of a papillary cystadenoma is performed and the differential diagnosis is essentially made with a papillary of the choroïd plexus. They require total surgical excision in order to avoid possible recurrence and can be performed by retrosigmoïd approach, or more ideally, by transpetrosal approach.

[Papillary cystic neoplasms in the ectopic pancreas]. / Papillaris és cisztikus neoplasia ectopiás pancreasban.

The authors reported a case of a young woman. From her omentum a clinically and radiologically benign tumour was removed that had been observed for years and caused minor symptoms. Histologically the tumour proved to be a so called papillary and cystic tumour that originated from ectopic pancreatic tissue. The histological diagnosis was justified by immunohistochemical and electronmicroscopical examinations. Reports of this type of tumour evolving in an ectopic pancreas appeared only twice in the world literature to the knowledge of the authors.

Solid-cystic tumor of the pancreas. An extensive ultrastructural study of fourteen cases.

Fourteen cases of solid-cystic tumor of the pancreas have been investigated at the ultrastructural level. A heterogeneous cell population has been found, and some distinctive features have been identified: oncocytoid cells; clear cells reminiscent of ductular and centro-acinar cells; cells with exocrine and endocrine features; cells with bundles of microfilaments and dense bodies, reminiscent of myoepithelium. This varied differentiation strongly suggests the tumor histogenesis to be from multipotent cell capable of recapitulating the normal ontogenesis during its neoplastic proliferation. Furthermore, the heterogeneity in ultrastructural appearance is a characteristic and reliable feature of solid-cystic tumor of the pancreas.

Two cases of ovarian tumours in women who had undergone multiple ovarian stimulation attempts.

Concerns have been raised recently about the possible association between superovulation and ovarian cancer. In order to contribute to the limited literature on this important issue, two cases of ovarian tumours in women who had undergone multiple ovulation inductions are presented. In the first case, the patient had secondary anovulatory infertility. She was treated with human menopausal gonadotrophin (HMG) alone and in combination with clomiphene citrate or buserelin for six cycles. She then underwent ovarian stimulation with buserelin/HMG in the long protocol for in-vitro fertilization (IVF) and embryo transfer. In preparation for a new IVF/embryo transfer attempt, 8 months later, the screening ultrasound revealed a cystic formation of the left ovary and an enlargement of the right. During laparotomy, both ovaries were found to bear large tumours (approximately 6 x 5 x 4 cm) which were removed. Histological examination showed that they were epithelial tumours (serous-papillary cystadenomas) of borderline malignancy. The patient conceived spontaneously 1.5 years after the operation. In the second case, the patient presented with secondary anovulatory infertility. She underwent ovulation induction with clomiphene/HMG and with buserelin/HMG in the long protocol, and intra-uterine insemination with husband's spermatozoa and conceived (singleton pregnancy). She was delivered by Caesarean section, during which a cystic tumour of the left ovary was removed. Histological examination revealed a benign mucous cystadenoma of the ovary.(ABSTRACT TRUNCATED AT 250 WORDS)

Well-differentiated serous neoplasms of the ovary.

Serous neoplasms of the ovary, which constitute the largest subgroup of the surface epithelial tumors, cluster into three distinctly clinicopathologic groups: benign neoplasms, which are architecturally noncomplex, confined to the ovary, and composed of cytologically bland cells; carcinomatous neoplasms, which have spread beyond the ovary and are cytologically malignant; and an intermediate group, which raises serious problems in taxonomy, differential diagnosis, and prognosis. This chapter focuses on differential diagnosis, emphasizing the authors' experience as well as reports from other investigators.