jiafm, 2010-32(2) april-june. - forensic medicine

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ISSN 0971-0973 J Indian Acad Forensic Med, 32(2)Case ReportAdult Choroid Plexus Papilloma: Cause of Sudden Death*Dr. S. Ranjan Bajpai, MD, **Dr. D. S. Badkur, MD, DFM, FIAFM, ***Dr. Reeni Malik, MD,****Dr. Arneet Arora, MD, DNB, *****Dr. Jayanthi Yadav, MD,Abstract:Choroid plexus papilloma (CPP) is a rare, benign neoplasm, relatively more common in childhood. It isassociated with signs and symptoms of increased intracranial pressure, frequently in association with obstructivehydrocephalus. CT and MRI are the investigations of choice and are diagnostic. Sudden deaths have been reported,but are very unusual. A 41 year old male was brought for medico-legal autopsy examination on ground of suddendeath. He was reported to have headaches over a long period of time. On autopsy examination, massive subarachnoidhemorrhage was seen on both the cerebral hemispheres and cerebellum. A cyst measuring about 1cmdiameter was found in choroid plexus of right lateral ventricle.On histopathological examination, it was found to be a choroid plexus papilloma. Calcification was alsoevident in the papilloma. From medico-legal aspect, the present case reveals an unusual cause for sudden death in anadult male. The pathology could have been diagnosed easily by CT scan or MRI. When diagnosed, it has goodsurvival rate, the morbidity depending on the extent of pathological effects. The present case was likely to havesurvived having minimal effects with appropriate treatment had he been diagnosed. The pathology is rare and asuspicion for this pathology in the adult male was not expected, but a CT scan to investigate chronic headache waswarranted. Absence of such a suggestion leading to death, which could have been preventable, is sufficient groundfor charge of professional negligence.Key Words: Sub-Arachnoid Haemorrhage, Intracranial Tumours, Choroid Plexus PapillomaIntroduction:Choroid plexus papillomas are rare,accounting for less than 1% of all intracranial tumorsin adults. They are relatively more common inchildhood and constitute 1.5 to 4% of intracranialtumors. [1] They are neuroectodermal in origin andsimilar in structure to a normal choroid plexus in theform of multiple papillary fronds mounted on a wellvascularized connective tissue stroma.CPPs are often associated with a vascularstalk connected to the choroids plexus, allowingmobility within the ventricular system.Corresponding author:*Dr, S.Ranjan BajpaiAssociate Professor, Deptt. of Forensic Medicine,Gandhi Medical College,Bhopal. 462001E mail: arneetrajan@yahoo.co.in*Assistant Professor, Forensic Medicine,A.J. College of Medical Sciences, Mangalore,Karnataka**Director, Medico-legal Institute, Bhopal***Associate Professor, Pathology****Associate Professor,*****Associate ProfessorGrossly, they may appear as reddishcauliflower like growths, which often become heavilycalcified. [2] CPPs are not malignant; however,malignant evolution may occur, with an incidence of10-30%. In a recent series by McEvoy et al, the fiveyearsurvival rate was 100 %. [3]The tumor‟s presence is often heralded bynon-specific signs and symptoms of increasedintracranial pressure. In adults, headache is the mostcommon presenting symptom, which may be relatedto an alteration in head position. CPPs are amenableto complete surgical excision and surgery should beperformed before complications set in. The tumor isliable to spontaneous hemorrhage, resulting in bloodstainedor xanthochromic CSF. Hydrocephalus is acommon complication which may result from directtumor obstruction of the outlet of CSF or due toexcessive production of CSF. [2] CT and MRI arethe investigative procedures of choice in theevaluation of CPPs. Because of the relatively noninvasivenature, high reproducibility and greatcontrast resolution of CT and MRI, they havesupplanted all other methods of neuroimaging.Clinical summary:A 41 years old average built male wasbrought for medico-legal autopsy examination onground of sudden death. He was reported to havecome back from work the previous evening at about 8160
J Indian Acad Forensic Med, 32(2) ISSN 0971-0973pm, a little later than usual and complained ofheadache. He was known to complain of headachequite often. A couple of hours later, he took somemedication for his headache and then slept in thesitting posture in a chair. He was found dead the nextmorning in the chair itself and nothing suspicious wasfound around him. The police indicated theirsuspicion of cardiac arrest in the post-mortemrequisition. On examination, no injuries were seen onthe body. Heart was 350gms in weight with leftventricular wall being 2.5 cms thick and rightventricular wall about 4mm thick. Coronaries werenormal except for left main coronary artery whichshowed 10 – 20% stenosis. Other abdominal andthoracic organs were normal and healthy. On openingthe cranial cavity, duramater was markedly tense andcongested. Massive subarachnoid hemorrhage waspresent all over on both cerebral hemispheres andcerebellum. A cystic structure 1 cm in diameter wasseen in the right lateral ventricle attached to telachoroidea. Brain was congested and edematous. Thecyst like growth was then examined.Pathological findings:Gross findings: A bluish cystic mass, about1 cm in diameter in right lateral ventricle, wasattached to the choroid plexus with a short stalk. Theouter wall appeared to be smooth. On cut section, theinner wall was granular and contained clear fluid.Massive sub-arachnoid hemorrhage waspresent on both the cerebral hemispheres.Microscopic findings: Histologically, thetumor simulates the normal architecture of thechoroids plexus. The papillary fronds showed a fibrovascular core. The epithelial cells lining the frondswere crowded and mildly pleomorphic. Occasionally,calcified psammoma bodies were also seen. Thehistological diagnosis was choroid plexus papilloma.Discussion:There is a regional difference between adultand childhood tumors with most choroid plexustumors in children arising in the lateral ventricles andthose in the adult more common in the fourthventricle with a tendency to grow through theforamen of Luschka into the cerebello-pontine angle.[4] In the present case, CPP was seen in an adult malein the lateral ventricle.The median duration of symptoms isreported to be about one month with approximatelyone-third of patients presenting within two weeks. [3]In the present case, the history as obtained fromfriends and neighbors, extended over a period ofmonths. The tumor is reported to be associated withnon-specific signs of increased intracranial pressure.In adults, headache is the most common presentingsymptom. In the present case headache was the onlysymptom reported for months. The primarily intraventriculargrowth is responsible for the paucity ofsymptoms in the early stages of the disease. [5]Grossly, choroid plexus tumors are generallydescribed as a well-circumscribed, brownish-red,cauliflower-like mass, the carcinoma being invasive,appearing hemorrhagic or necrotic. Histologically,there is delicate connective tissue fronds covered by asingle layer of cuboidal epithelium. The papillomaresembles normal choroid plexus but the cells aremore crowded and elongated. The carcinoma is farmore cellular and displays signs of anaplasia.Rarely, these tumors can exhibit mucinousdegeneration, melanization, tubular glandulararchitecture or osseous and cartilage metaplasia. [6]In adults, most CPP are heterogeneous secondary tocystic and /or calcific degenerations. The present casealso showed calcification on microscopicexamination.There has been a case report of sudden deathin which the tumor involved the third ventricle andcaused acute ventricular obstruction. [7] In thepresent case, there was massive sub-arachnoidhemorrhage leading to sudden death. WHO classifieschoroid plexus tumors into either papillomas (GradeI) or carcinomas (Grade III). [8] In the present case,the tumor was designated as Grade I and was apapilloma. The case is being presented for its rarityand some features being distinct from expectedfindings in such a case.References:1. Erman T, Gocer AL, Erdogan S, Tuna M, Ildan F,Zorludemir S. Choroid plexus papilloma of bilaterallateral ventricle. Acta Neurochir (Wien) 2003;145(2):139-43.2. Lantos PL. The Nervous System. James O‟ D McGee,Peter G. Isaacson, Nicholas A Wright, Oxfordtextbook of Pathology. Vol II. Ed.Oxford UniversityPress, 1992:1890.3. McEvoy AW, Harding BN, Phipps KP et al.Management of choroid plexus tumors in children: 20years experience at a single centre. Pediatr. Neurosurg2000; 32(4):192-9. [Medline].4. Janisch W, Staneczek W. Primary tumors of thechoroid plexus. Frequency, localization and age.Zentralbl Allg Pathol 1989; 135:235-240.5. Juan Rosai. Neuromuscular system/ Central NervousSystem and peripheral nerves. Ackerman‟s SurgicalPathology. Vol. 2. 6 th edition. The C.V. MosbyCompany. 1981.6. Kleihues P, Cavenee WK. Tumors of the NervousSystem, International Agency for Research on Cancer:Paris 1997.7. Gradin WC, Taylon C, Fruin AH. Choroid plexuspapilloma of the third ventricle: case report and reviewof the literature. Neurosu 12(2):217-20. [Medline]8. Kleihues P, Burger P, Scheithauer B. The new WHOclassification of brain tumors. Brain Pathology 1993;3:255-68.161
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