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Pathological features of congenital fibrosarcoma and haemangiopericytoma with long term followup after local surgical resection
ANATOMICAL PATHOLOGY PATHOLOGICAL FEATURES OF CONGENITAL FIBROSARCOMA AND HAEMANGIOPERICYTOMA WITH LONG TERM FOLLOWUP AFTER LOCAL SURGICAL RESECTION
CL
James'. NH Bramwell, RB Davev, RW Bvard Depts of Pathology & Surgery, Adelaide Children's Hospital, Dept of Tissue Pathology, Institute of Medical & Veterinary Science, Adelaide. S A , Australia, Bronson Methodist Hospital Kalamazoo, Michigan, USA
The treatment of congenital neoplasms has been the subject of debate due t o their inlrequent occurrence, and difference in biological behaviour compared to adult turnours of similar hiStOlOgiCal appearance We report 2 cases of congenital fibrosarcoma and haemangiopericytoma, respectively w i t h long term followup, in which the sole method of treatment w a s local surgical rese,tion Case I A term male infant underwent excision of a 1 0 c m diameter mass that w a s overlying the left scapula Histologically the tumour w a s a fibrosarcoma which abutted the resection margin, and had up t o 3 mitoses1HPF w i t h moderate nuclear pleomorphism The patient is alive and well w i t h no evidence of recurrence or of metastasic disease at 1 8 yr followup Case II A term female infant underwent excision of a 5 c m diameter facial mass Histologically the tumour was a haemangiopericytoma which abutted the resection margin and had 1 mitosisl3 HPF, with focal areas of necrosis The patient is alive and well w i t h no evidence of recurrence or of metastasic disease at 1 5 yr followup Histologic features of frequent mitoses, cycoiogic pleomorphism and necrosis did n o t signify a poor prognosis >nthese congenital tumours Subsequent ploidy analysis of both turnours revealed predominantly diploid cell populations. and so it is possible that this rnay be Of use as an alternative prognostic parameter A s these cases both had reasonable cosmetic and functional results, local surgical resection alone w i t h careful followup may be regarded as a viable treatment option The added advantage of extensive surgery, or of adjuvant therapy with chemo or radlotherapy would have to be questioned, particularly in view of the apparently incomplete nature of the original excisions Given reports in the
PROGNOSTIC PARAMETERS IN CHlLDHOOD RHABOOMYOSARCOMA. H.S. Preston.. L. Moore. I. Toooood. J , Drew. R.W. Bvard. Lab. Med. Services, Caulfield Gen. Med. Centre, Victoria; Depts. Oncology & Histopathology. Adelaide Children's Hospital (ACH), South Australia. As there has been considerable discussion regarding the validity of various histologic and morphologic criteria in predicting the clinical outcome of rhabdomyosarcomas in the paediatric age group, the following study of histologic subtype, ploidy and clinico-pathological features of a total of 2 6 turnours taken from the files of the ACH from 1 9 7 3 - 9 2 was undertaken. Treatment consisted of chemotherapy 126). radiotherapy (231 and surgery (18) with 16 survivors (followup 1-19 yrs). The number of patients surviving related to stage were: 100%-stage I (4/4); 100%-stage II 13/31; 56%-stage Ill (9116); 0%-stage IV (0/3). Cases were divided histologically into alveolar 110) and nonalveolar (16) subgroups (the bulk of the latter being embryonall. 5 of the 1 0 patients w h o died (50%) (age range 2-1 7 yrs, ave=9.7yrs) were in the alveolar group, as were 5 (31%1 of the 1 6 survivors (age range 2-12 yrs, ave =6.3yrs). Primary sites involved the genitourinary system 1516 survivorsl, extremities 12/31, head & neck (7181 & retroperitoneumlabdomenlthorax (219). 8 turnours were D N A diploid, 1 3 aneuploid, 3 tetraploid, w i t h insufficient material for analysis in 2 . Within the alveolar group there were 5 aneuploid, 3 diploid and 2 tetraploid turnours. In the nonalveolar group 5116 were diploid and 8 / 1 6 were aneuploid. 3/13 aneuploid cases had multiple aneuploid stemlines and 6 had at least 1 stem-line w i t h DNA index > 2.1. Of the 10 patients w h o died. 5 had diploid and 4 had aneuploid tumours I 1 insufficient for analysis). Of the 8 patients with diploid turnours, 5 (63%)died, compared to Conclusions: The most significant 411 3 ( 3 1 %) w i t h aneuploid turnours. features relating t o poor prognosis in this group of patients were primary tumour site, stage a t presentation and diploid DNA content. Patients w i t h alveolar morphology also tended to have a worse outcome than those w i t h embryonal morphology. 911 1 patients with primary tumours in the extremities or head & neck survived, in spite of having 8 stage 111 turnours in this group.
literature of recurrence and metastases in similar neoplasms and the lack O f reliable histological features predicting outcome in individual cases, close and prolonged clinical followup is. however essential
EMBRYOGENESIS OF ENTERIC DUPLICATION CYSTS OF THE TONGUE. J Lcpset('. AL Soarnon. RW Bvard Department of Histopathology, Flinders University Medical Centre and Deoartments of Histopathology and Surgery, Adelaide Children s Hospital. North Adelaide. South Australia
Enteric duplications rnay be found throughout the gastrointestinal tract and rnay be multiple They appear to recapitulate the architecture of the normal gastrointestinal tract We report 2 cases of lingual enteric duplications diagnosed in childhood Both occurred in males, one of 4 months and the other of 5 years of age Both were relatively asymptomatic cystic swellings of the tongue One was lined by stratified squamous nonkeratinizing epithelium that merged w l t h gastric type epithelium, while the second w a s lined by large bowel type mucosa The differential diagnosis of a tongue mass in the paediatric age group includes dermoid cyst, epidermaid cyst, neurofibroma, haernangioma, cystic hygroma, ranula. retention cyst. hamartoma. lingual thyroid and teratoma Enteric duplication cysts are rare In the tongue 10 3%) and more frequently occur in relation to the small intestine oesophagus colon or stomach The lesions may consist of Only a mucosal h n g or may be invested by an entire multilayered wall of mucosa. submurosa and muscularis propria The rnucosal linlng may be histologically similar t o that of the segment of the gut to w h i c h it is topographically Not related or heterotopic tissue (particularly gastric) may be present infrequently more than one type of mucosa occurs Enteric duplications of the tongue may form from epithelial inclusions that are retained along the lines of fusion of the embryonic primordia that contribute t o the formation of the organ Other theories of pathogenesis of enteric duplications include their development from a1 isolated lacunae, after incomplete coalescence of the spaces that form between the cells within the solid epithelial core of the developing gut tube. b) persistent intestinal epithelial buds in the submucosal conncciwe itssue in the developing gut wall and c) strands or masses ot adherent entodermal ~ ~ 1 1caught 9 up w i t h the infolding of the notochordal plate during the formation of the notochord As not all of the theories of pathogenesis of entcric duplcation cysts can be applied to the tongue. i t appears that enteric duplications form a heterogeneous group that may share similar morphologic features, but not necessarily the same pathogenPziS
INFECTIOUS MONONUCLEOSIS TONSILLITIS
E. Donaldson'*, B. Franz' and D. Ellis 3 , Eastern Suburbs Pathology Servics' and Department of Otolaryngology , Maroondah Hospital, East Ringwood, Vic 3135 3 Department of Anatomical Pathology , St. Vincent's Hospital, Fitzroy, Vic. 3065. Three cases of acute infectious mononucleosis (TM) tonsillitis in young males are presented. Emergency tonsillectomy was performed i n e a c h patient because of oropharyngeal obstruction. The patients ages w e r e 1 5 , 17 and 2 3 y e a r s respectively. Histological examination confirmed a florid acute suppurative tonsillitis. The underlying architecture of the tonsils was effaced with virtual obliteration of follicles. Sheets of large atypical lymphoid cells were present. In one case Reed-Sternberg variant cells were n o t e d . A l t h o u g h the h i s t o l o g i c a l f e a t u r e s were alarming, the findings were consistent w i t h IM t o n s i l l i t i s . T h i s correlated w i t h clinical and other laboratory data. Lymphoid tissues are rarely biopsied in IM and often present a diagnostic challenge. The immunophenotypic and histological features which may assist in the differentiation of IM from lymphoid neoplasia are discussed.
CL
James'. NH Bramwell, RB Davev, RW Bvard Depts of Pathology & Surgery, Adelaide Children's Hospital, Dept of Tissue Pathology, Institute of Medical & Veterinary Science, Adelaide. S A , Australia, Bronson Methodist Hospital Kalamazoo, Michigan, USA
The treatment of congenital neoplasms has been the subject of debate due t o their inlrequent occurrence, and difference in biological behaviour compared to adult turnours of similar hiStOlOgiCal appearance We report 2 cases of congenital fibrosarcoma and haemangiopericytoma, respectively w i t h long term followup, in which the sole method of treatment w a s local surgical rese,tion Case I A term male infant underwent excision of a 1 0 c m diameter mass that w a s overlying the left scapula Histologically the tumour w a s a fibrosarcoma which abutted the resection margin, and had up t o 3 mitoses1HPF w i t h moderate nuclear pleomorphism The patient is alive and well w i t h no evidence of recurrence or of metastasic disease at 1 8 yr followup Case II A term female infant underwent excision of a 5 c m diameter facial mass Histologically the tumour was a haemangiopericytoma which abutted the resection margin and had 1 mitosisl3 HPF, with focal areas of necrosis The patient is alive and well w i t h no evidence of recurrence or of metastasic disease at 1 5 yr followup Histologic features of frequent mitoses, cycoiogic pleomorphism and necrosis did n o t signify a poor prognosis >nthese congenital tumours Subsequent ploidy analysis of both turnours revealed predominantly diploid cell populations. and so it is possible that this rnay be Of use as an alternative prognostic parameter A s these cases both had reasonable cosmetic and functional results, local surgical resection alone w i t h careful followup may be regarded as a viable treatment option The added advantage of extensive surgery, or of adjuvant therapy with chemo or radlotherapy would have to be questioned, particularly in view of the apparently incomplete nature of the original excisions Given reports in the
PROGNOSTIC PARAMETERS IN CHlLDHOOD RHABOOMYOSARCOMA. H.S. Preston.. L. Moore. I. Toooood. J , Drew. R.W. Bvard. Lab. Med. Services, Caulfield Gen. Med. Centre, Victoria; Depts. Oncology & Histopathology. Adelaide Children's Hospital (ACH), South Australia. As there has been considerable discussion regarding the validity of various histologic and morphologic criteria in predicting the clinical outcome of rhabdomyosarcomas in the paediatric age group, the following study of histologic subtype, ploidy and clinico-pathological features of a total of 2 6 turnours taken from the files of the ACH from 1 9 7 3 - 9 2 was undertaken. Treatment consisted of chemotherapy 126). radiotherapy (231 and surgery (18) with 16 survivors (followup 1-19 yrs). The number of patients surviving related to stage were: 100%-stage I (4/4); 100%-stage II 13/31; 56%-stage Ill (9116); 0%-stage IV (0/3). Cases were divided histologically into alveolar 110) and nonalveolar (16) subgroups (the bulk of the latter being embryonall. 5 of the 1 0 patients w h o died (50%) (age range 2-1 7 yrs, ave=9.7yrs) were in the alveolar group, as were 5 (31%1 of the 1 6 survivors (age range 2-12 yrs, ave =6.3yrs). Primary sites involved the genitourinary system 1516 survivorsl, extremities 12/31, head & neck (7181 & retroperitoneumlabdomenlthorax (219). 8 turnours were D N A diploid, 1 3 aneuploid, 3 tetraploid, w i t h insufficient material for analysis in 2 . Within the alveolar group there were 5 aneuploid, 3 diploid and 2 tetraploid turnours. In the nonalveolar group 5116 were diploid and 8 / 1 6 were aneuploid. 3/13 aneuploid cases had multiple aneuploid stemlines and 6 had at least 1 stem-line w i t h DNA index > 2.1. Of the 10 patients w h o died. 5 had diploid and 4 had aneuploid tumours I 1 insufficient for analysis). Of the 8 patients with diploid turnours, 5 (63%)died, compared to Conclusions: The most significant 411 3 ( 3 1 %) w i t h aneuploid turnours. features relating t o poor prognosis in this group of patients were primary tumour site, stage a t presentation and diploid DNA content. Patients w i t h alveolar morphology also tended to have a worse outcome than those w i t h embryonal morphology. 911 1 patients with primary tumours in the extremities or head & neck survived, in spite of having 8 stage 111 turnours in this group.
literature of recurrence and metastases in similar neoplasms and the lack O f reliable histological features predicting outcome in individual cases, close and prolonged clinical followup is. however essential
EMBRYOGENESIS OF ENTERIC DUPLICATION CYSTS OF THE TONGUE. J Lcpset('. AL Soarnon. RW Bvard Department of Histopathology, Flinders University Medical Centre and Deoartments of Histopathology and Surgery, Adelaide Children s Hospital. North Adelaide. South Australia
Enteric duplications rnay be found throughout the gastrointestinal tract and rnay be multiple They appear to recapitulate the architecture of the normal gastrointestinal tract We report 2 cases of lingual enteric duplications diagnosed in childhood Both occurred in males, one of 4 months and the other of 5 years of age Both were relatively asymptomatic cystic swellings of the tongue One was lined by stratified squamous nonkeratinizing epithelium that merged w l t h gastric type epithelium, while the second w a s lined by large bowel type mucosa The differential diagnosis of a tongue mass in the paediatric age group includes dermoid cyst, epidermaid cyst, neurofibroma, haernangioma, cystic hygroma, ranula. retention cyst. hamartoma. lingual thyroid and teratoma Enteric duplication cysts are rare In the tongue 10 3%) and more frequently occur in relation to the small intestine oesophagus colon or stomach The lesions may consist of Only a mucosal h n g or may be invested by an entire multilayered wall of mucosa. submurosa and muscularis propria The rnucosal linlng may be histologically similar t o that of the segment of the gut to w h i c h it is topographically Not related or heterotopic tissue (particularly gastric) may be present infrequently more than one type of mucosa occurs Enteric duplications of the tongue may form from epithelial inclusions that are retained along the lines of fusion of the embryonic primordia that contribute t o the formation of the organ Other theories of pathogenesis of enteric duplications include their development from a1 isolated lacunae, after incomplete coalescence of the spaces that form between the cells within the solid epithelial core of the developing gut tube. b) persistent intestinal epithelial buds in the submucosal conncciwe itssue in the developing gut wall and c) strands or masses ot adherent entodermal ~ ~ 1 1caught 9 up w i t h the infolding of the notochordal plate during the formation of the notochord As not all of the theories of pathogenesis of entcric duplcation cysts can be applied to the tongue. i t appears that enteric duplications form a heterogeneous group that may share similar morphologic features, but not necessarily the same pathogenPziS
INFECTIOUS MONONUCLEOSIS TONSILLITIS
E. Donaldson'*, B. Franz' and D. Ellis 3 , Eastern Suburbs Pathology Servics' and Department of Otolaryngology , Maroondah Hospital, East Ringwood, Vic 3135 3 Department of Anatomical Pathology , St. Vincent's Hospital, Fitzroy, Vic. 3065. Three cases of acute infectious mononucleosis (TM) tonsillitis in young males are presented. Emergency tonsillectomy was performed i n e a c h patient because of oropharyngeal obstruction. The patients ages w e r e 1 5 , 17 and 2 3 y e a r s respectively. Histological examination confirmed a florid acute suppurative tonsillitis. The underlying architecture of the tonsils was effaced with virtual obliteration of follicles. Sheets of large atypical lymphoid cells were present. In one case Reed-Sternberg variant cells were n o t e d . A l t h o u g h the h i s t o l o g i c a l f e a t u r e s were alarming, the findings were consistent w i t h IM t o n s i l l i t i s . T h i s correlated w i t h clinical and other laboratory data. Lymphoid tissues are rarely biopsied in IM and often present a diagnostic challenge. The immunophenotypic and histological features which may assist in the differentiation of IM from lymphoid neoplasia are discussed.