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SUMMARY: A case of cauda equina metastasis from ovarian serous papillary adenocarcinoma in a 51-year-old patient is reported. The clinical significance and different hypotheses on the metastatic route to the spine are discussed.
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Perineurioma (storiform perineurial fibroma) is a rarely diagnosed benign tumour of perineurial cells. Presented tumour occurred subcutaneously in the right thigh of a 53-year-old man. It was well limited and consisted of whirl like and concentric laminar bundles of spindle and oval cells in variable local density. A storiform pattern was not present. Cells with richer cytoplasm, bland nuclei and solitary intranuclear pseudoinclusions formed focal meningioma like structures. Differential diagnosis had to distinguish benign neuronal tumours, myxoid variant of dermatofibrosarcoma protuberans and especially a "low grade" fibromyxoid sarcoma. A relevant immunohistochemical marker of perineurioma (for differential diagnosis) was the expression of epithelial membrane antigen (EMA) with negativity of S-100 protein.
We report the course of a 19-year-old female who presented with a 13-year history of an occipital dermal tumor. Only five weeks after excision, the patient succumbed to cerebral dysregulation due to extensive miliary cerebral, hepatic, and pulmonary metastasis. The histologic features of the surgical specimen and the autopsy material differed suspiciously: a peculiar malignant progression of the tumor seemed to have occurred within five weeks.
It is thought that perineural invasion by pancreatic cancer takes place either by means of direct tumor invasion of the perineural space or by means of tumor invasion through the vessels perforating the perineurium. The manner of tumor progression may be divided into four types: (1) direct progression of cancer cells into the perineural space, (2) branching at the bifurcation of the nerve fascicle, (3) perineural invasion by a probing cancer tip, and (4) invasion beyond the perineurium through defects in it. Because pancreatic cancer is often followed by severe tumor invasion of surrounding organs, major blood vessels and extrapancreatic plexus, the survival rate after the conventional pancreatoduodenectomy has been unsatisfactory. Since 1977, the author has used extended pancreatectomy with a trans-lateral retroperitoneal approach, involving resection of the portal vein, extensive excision of the retroperitoneal lymph nodes and complete resection of the pancreatic head plexus. After this surgery, 11 patients survived for more than 3 years. The five-year survival rate was 25% in case where this operation was rated as being a macroscopic curative resection.
Intracranial adenoid cystic carcinoma is uncommon. We report two unusual cases of intracranial adenoid cystic carcinoma. The first patient presented with a steroid-responsive optic neuropathy from an orbital mass that simulated orbital pseudotumor, and subsequently developed intracranial involvement, presumably by contiguous perineural spread. The second patient presented with proptosis of the left eye, right facial weakness and numbness, and an intracranial mass, presumably from hematogenous metastatic spread.
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The care of patients refusing blood transfusion who require major ablative surgery for malignancy is a continuing challenge. The use of recombinant human erythropoietin is clearly efficacious in patients with renal disease and may be useful in anemic patients who refuse transfusion. Herein, we report a successful extended hemipelvectomy in a Jehovah's Witness using recombinant human erythropoietin support.
Many different types of small cell, embryonal, and poorly differentiated neoplasms originate within the central and peripheral nervous systems. Because appropriate treatment is based on a correct diagnosis, the surgical pathologist must be familiar both with basic characteristics of each of the numerous entities as well as the spectrum of morphologic features that each may display. The nosology and nomenclature of these tumors have a rich and varied history. One basic distinction is between primitive neuroectodermal tumors of the central nervous system (cPNETs) and primitive neuroectodermal tumors of the peripheral nervous system (pPNETs), which are clinicopathologically and genetically distinct. Among the cPNETs are medulloblastoma, pineoblastoma, cerebral neuroblastoma, ependymoblastoma, medulloepithelioma, primary rhabdomyosarcoma, and atypical teratoid/rhabdoid tumor, whereas the pPNETs comprise the more differentiated end of a spectrum of neoplasms that include skeletal and extraskeletal Ewing's sarcoma.
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The influence of the arachidonic acid metabolism inhibitors, indomethacin and voltaren; an inhibitor of phosphodiesterase activity, theophylline and the protease inhibitor epsilonaminocaproic acid (EACA) on N-ethyl-N-nitrosourea (ENU)-induced transplacental carcinogenesis was studied in rats. ENU was given to pregnant rats as a single i.v. exposure at a dose of 75 mg/kg body weight on the 21st day after conception. Indomethacin and voltaren (20 p.p.m. in drinking water), theophylline (0.01% in diet) and EACA (1000 p.p.m. in drinking water) were given to the offspring throughout their post-natal life until all survivors were killed at 12 months. In the ENU-only control groups, 100% of the offspring developed tumors of brain, spinal cord, peripheral nervous system or kidneys, with a total average number of 3.1 tumors per rat. The most marked inhibitory effect was exerted by theophylline, which significantly decreased the incidence and multiplicity of total tumors, and at all main sites selectively (brain, spinal cord, peripheral nerves and kidneys). It also prolonged average survival time of the offspring. Indomethacin and voltaren significantly decreased total tumor incidence and multiplicity and brain tumor incidence and multiplicity. Indomethacin also decreased kidney tumor multiplicity and voltaren diminished spinal cord tumor multiplicity. EACA decreased multiplicities of total, brain, peripheral nerve and kidney tumors, and diminished the incidence of brain tumors. These chemopreventive agents decreased tumor incidences 20-33% and tumor multiplicities 1.4-2.7 times, compared with the ENU-only controls.
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