Ute Jänig

Solid–pseudopapillary tumor of the pancreas: its origin revisited

Abstract Solid–pseudopapillary tumor of the pancreas (SPT) has distinctive morphologic and biologic features but an unclear origin. It is classified among the pancreatic epithelial tumors, though many are reported to be negative for cytokeratin. Also unclear are its neuroendocrine differentiation, its capability to express alpha-1-antitrypsin (AAT) and, in view of the tumor’s striking prevalence in women, its relationship with the female genital tract. To clarify these issues, the immunoprofiles of 59 SPTs were defined by applying a battery of antibodies against cytokeratin, vimentin, neuron-specific enolase (NSE), synaptophysin, chromogranin A, tyrosine hydroxylase (TH), AAT, LeuM1, Ki-M1P, smooth-muscle actin, CD34, alpha-inhibin, calretinin, placental alkaline phosphatase (PLAP), and progesterone and estrogen receptors. The most consistent markers with the strongest immunoreactivity were vimentin, AAT, NSE, and the progesterone receptor, which were each found in more than 90% of the tumors. Using immunocytochemical methods involving antigen retrieval, cytokeratin was demonstrated in almost 70% of the cases. Synaptophysin was found in 22% of the tumors, while chromogranin was absent and tyrosine hydroxylase was only present in a few tumors. None of the other markers tested were expressed by SPTs. This staining pattern fails to reveal a clear phenotypic relationship with any of the defined cell lineages of the pancreas. In view of the striking female preponderance of SPTs and the known close approximation of the genital ridges to the pancreatic anlage during embryogenesis, it is, however, hypothesized that SPTs might derive from genital ridge/ovarian anlage-related cells, which were attached to the pancreatic tissue during early embryogenesis.

Germ cell tumours of childhood: Report of 170 cases including 59 pure and partial yolk-sac tumours

170 germ cell tumours of childhood and adolescence were studied by light microscopy and immunohistochemistry. The male-to-female ratio was 1:1.3. 52 (30.6%) tumours were benign (mature teratoma), 30 (17.6%) potentially malignant (immature teratoma), and 88 (51.8%) unequivocally malignant. The main locations were ovary, testis and sacrococcygeal region. 92 tumours were located in a gonad, 78 tumours in extragonadal sites (ratio: 1.2:1). Of the frankly malignant tumours 40 were yolk-sac tumours (YST) and an additional 19 tumours of more than one histological type contained a YST component. Therefore, 67% of the malignant tumours had a YST component. Children with immature teratoma and pure YST showed the lowest median age (5 and 24 months, respectively), while children with germinomas of various locations had the highest median age (153 months). A festoon pattern was the predominant histological feature in all YST and in the YST component of mixed germ cell tumours. Hyaline globules were found in 33/37 YST and in 16/17 YST components. Immunohistochemically, alpha1-fetoprotein (AFP) was demonstrated in 18/22 YST and in 6/7 YST components of mixed germ cell tumours. Hyaline globules were mostly AFP-negative (only 5 cases with AFP-positive globules in addition to many AFP-negative globules). In 3 cases beta-HCG-positive giant cells were seen. In most YST prekeratin intermediate filaments could be demonstrated in the epithelial cells. Follow-up data, available from 51 cases of YST and tumours with YST components showed disease-free survival in 37 cases (72.5 %). 10 patients (19.6 %) died of disease, and 4 patients (7.8%) are living with disease. The comparably high rate of survivors reflects the effectiveness of modern therapy, particularly polychemotherapy, in addition to surgery.

Gliomatosis Peritonei in Childhood and Adolescence: Clinicopathological Study of 13 Cases Including Immunohistochemical Findings*

Gliomatosis peritonei (GP) can be defined as the metastatic implantation of neural tumor tissue on surfaces of the peritoneum in patients with immature ovarian teratomas. Data from 13 patients with GP were evaluated. The average age at time of biopsy was 11 years (median: 11.5 years; range: 2.9 to 18.6 years); average time of follow-up was 26.6 months (median: 22 months; range: 4 to 77 months). In 11/13 patients the GP was discovered at the same time as the primary tumor, in two patients it was detected at second-look surgery. Degree of maturity according to scheme of Robboy and Scully was 8 x G 1, 4 x G 2, and 1 x G 3 for the ovarian teratomas (x = 1.46), and 11 x G 0 and 2 x G 3 for the GP implants (x = 0.46). Light microscopically, the mature glial nodules consisted almost exclusively of GFAP and S-100 protein positive astroglia. Twelve of the 13 patients are alive and clinically healthy. One patient with metachronic immature GP is receiving chemotherapy. In general, the prognosis for GP is good: it depends chiefly on the degree of maturity of the implants. In mature GP, usually no additional chemotherapy is necessary; in immature GP, chemotherapy can induce maturation of the implants.

Cemento-ossifying fibroma of the petromastoid region: case report and review of the literature

The cemento-ossifying fibroma (COF) is a mesodermal, non-odontogenic tumour of ectopic multipotential periodontal membrane blast cells. It is aggressive, locally destructive, and has a high recurrence rate. A case report of COF of the petromastoid region is presented. This location has not been described until now. Trauma may act as a trigger to sudden growth of the atopic periodontal tissue. Due to the aggressive behaviour of this tumour and its frequent recurrence radical surgery is needed.

Detection of chromosome aberrations in paraffin sections of seven gonadal yolk sac tumors of childhood

Yolk sac tumors are the most frequent kind of malignant pediatric germ cell tumor and may have a fundamentally different pathogenesis than adult germ cell tumors. Since few cytogenetic studies have been performed so far, in situ hybridization was applied to interphase cell nuclei of seven gonadal yolk sac tumors of childhood in routine paraffin-embedded tissue sections. The panel of chromosome-specific DNA probes was selected on the basis of their relevance in adult germ cell tumors and consisted of five DNA probes specific for the (peri)centromeric regions of chromosomes 1, 8, 12, 17 and/or X and/ or one DNA probe specific for the subtelomeric region of chromosome 1 (p36.3). As in adult germ cell tumors, all pediatric gonadal yolk sac tumors had an increased incidence of numerical chromosome aberrations. All tumors showed an overrepresentation of at least three chromosomes. Gains of chromosome 12, which is highly specific in adult germ cell tumors, were diagnosed in six pediatric gonadal yolk sac tumors. The DNA indices determined in the paraffin-embedded tumor material correlated well with the in situ hybridization findings. A chromosome was either over- or underrepresented, compared with the corresponding DNA indices, in only a few cases. The short arm of chromosome 1 in adult germ cell tumors is often involved in structural aberrations. In pediatric germ cell tumors, the short arm of chromosome 1 is also a nonrandom site of structural aberrations. Moreover, the presence of a deletion at 1p36.3 in four out of five tumors suggests that the loss of gene(s) in this region is an important event in the pathogenesis of gonadal yolk sac tumors of childhood.

LASER SURGICAL TREATMENT OF LARYNGEAL PARAGANGLIOMA

Paragangliomas are rare benign neoplasms arising from the neural crest-derived paraganglia of the autonomic nervous system. In the larynx three different localizations of paraganglia are known. Most laryngeal paragangliomas arise from the supraglottic paraganglia. A review of the literature shows that the treatment of choice for laryngeal paragangliomas is surgical excision. Since the implementation of CO(2) laser surgery into laryngology in 1972, no reports of endoscopic laser surgical excisions of laryngeal paragangliomas have been published so far. We present the case of a 66-year-old female patient who suffered from a large (4 x 4 x 3 cm) left supraglottic paraganglioma. The tumour was completely excised utilizing the CO(2) laser. Histopathology and immunohistochemistry of the tissue presented the typical findings of a laryngeal paraganglioma. The pre- and post-operative management as well as the treatment strategies are discussed. To our knowledge the present case demonstrates for the first time a complete transoral CO(2) laser surgical resection of an advanced laryngeal paraganglioma.

Interphase cytogenetics on paraffin sections of paediatric extragonadal yolk sac tumours

Germ cell tumours in children are more often extragonadal than in adults and the most frequent type is the yolk sac tumour. Limited cytogenetic data exist on extragonadal yolk sac tumours in children. We applied in situ hybridization (ISH) to interphase cell nuclei of four paediatric extragonadal pure yolk sac tumours and one yolk sac tumour component of a mixed germ cell tumour using paraffin-embedded tissue sections. The panel of chromosome-specific DNA probes was selected on the basis of their relevance in adult germ cell tumours and consisted of five DNA probes specific for the (peri)centromeric regions of chromosomes 1, 8, 12, and/or 17, X and/or one DNA probe specific for the subtelomeric region of chromosome 1 (p36.3). Only one tumour failed to show numerical and structural chromosome aberrations with the DNA probes used. The other four had an increased incidence of numerical chromosome aberrations with an over-representation of at least one chromosome. The DNA indices determined in the paraffin-embedded tumour material correlated well with the in situ hybridization findings. In only a few cases were chromosomes over-represented, when compared with the corresponding DNA indices. Recently, we have shown that the short arm of chromosome 1 is a non-random site of deletion in paediatric gonadal pure yolk sac tumours. The occurrence of similar deletions in one extragonadal pure yolk sac tumour and in one yolk sac tumour component, in conjunction with two further ISH reports, suggests that the loss of gene(s) in this region is an important event in the pathogenesis of paediatric malignant germ cell tumours of nearly all sites.

Metastasizing malignant oncocytoma of the submandibular gland

Malignant oncocytomas are extremely rare tumours of the salivary glands. Fewer than 50 cases have been reported in the world literature so far, 34 of which were located in the parotid gland. Only three of these tumours have been located in the submandibular gland. We report one further case of a malignant oncocytoma of the submandibular gland in a 47-year-old man. Since a definite histological diagnosis of malignant oncocytoma can rarely be made both clinical and histopathological findings are essential in establishing the diagnosis. Treatment consists of wide surgical excision, neck dissection and post-operative radiotherapy. The prognosis with regard to five-year survival is poor because of metastatic disease.

Primary Papillary Carcinoma of the Thyroglossal Duct: Case Report and Review of the Literature

Primary malignancies of the thyroglossal duct are rare. Around 150 cases are described in the world literature, most of them being papillary thyroid carcinomas. Other types of tumors are squamous cell carcinomas, mixed follicular-papillary carcinomas, or adenocarcinomas. Women are affected more often than men, the ratio being 2:1. Preoperative diagnosis of primary malignancies of the thyroglossal duct is uncommon. Initial treatment of primary malignancies of the thyroglossal duct is usually sufficiently done operatively by the so-called Sistrunks procedure which, however, was first described in 1893 by Schlange. Some patients may need further treatment such as wider excision, thyroidectomy, radioiodine therapy, or neck dissection. In this report the case of a papillary carcinoma of the thyroglossal duct in a 63-year-old man is presented. It is intended to remind the reader of this pathology which is often forgotten because of its rarity. The problems that occur during the process of evaluation regarding ideal treatment of the individual case are discussed. The literature is reviewed.

Oral mature teratoma containing epididymal tissue in a female neonate

We describe a female neonate with an oral teratoma showing bone, teeth, and epidermis, but also epididymal (male) tissue. PCR amplification of Y-chromosomal DNA clearly showed male DNA from paraffin-embedded tumour tissue. The girl had a normal female karyotype without abnormalities of the genital organs. There are at least three hypotheses for the origin of teratomas: parthenogenesis, incomplete twinning, and totipotent somatic-cell origin. This case supports the hypothesis of an included dizygotic twin, and might contribute to the elucidation of the pathogenesis of extragonadal teratomas.