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#1
09.07.2003, 10:13
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Solid-psevdopapillaris papiloma
 Здравствуйте. Уважаемые коллеги, Вас беспокоит Черкасский онкодиспансер, отделение гематологии. пожалуйсто, подскажите где можно достать информацию по Solid-psevdopapillaris papiloma? Заранее благодарны. 
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#2
09.07.2003, 10:39
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Уважаемые коллеги!
На ваш запрос приведу в оригинале текст истории болезни о плотной псевдопапилярной опухоли поджелудочной железы и обзор по таким опухолям [без референсов] (а папиллома ли она или нет, понятия не имею): вами приведенного сочетания дословно обнаружить не удалось. The Lancet Oncology Volume 4, Issue 4 , 1 April 2003, Pages 255-256 Solid pseudopapillary tumour of the pancreas. Canzonieri V, Berretta M, Buonadonna A, Libra M, Vasquez E, Barbagallo E, Bearz A, Berretta S. Division of Pathology, Istituto Nazionale Tumori, IRCCS, Aviano, Italy. [Ссылки доступны только зарегистрированным пользователям ] Solid pseudopapillary tumour (Frantz's tumour) is a rare benign or low-grade neoplasm of the pancreas with distinct clinicopathological features. The diagnosis may be difficult, but should be considered as a possibility in young women who present with a large abdominal mass involving the pancreas. In this report we describe the clinical and pathological characteristics of these lesions and discuss therapeutic options. A 27-year-old woman was admitted to our hospital in October 1999 with a 3-month history of mild asthenia, weight loss, and a feeling of heaviness localised to the left lumbar region. Routine laboratory analyses were all within the normal range; however, abdominal ultrasound revealed the presence of a mass in the superior abdomen, which was composed of high and low echoic areas. Computed tomography showed the mass to be solid, encapsulated, and about 9 cm in diameter. It was composed of high and low density areas and was located close to the splenic hilum––above the pancreatic tail, behind the stomach, and between the spleen and the aorta (Albarran-Chatelin's anatomosurgical quadrilateral). Nuclear magnetic resonance imaging revealed a posterior anastomosis between the mass drainage vessels and the diaphragmatic veins. The patient underwent a complete surgical resection of the tumour (enucleation) and a resection of the pancreatic tail. The spleen was removed because of vascular damage consisting of large patches of venous stasis and ischaemia which did not resolve over time or after application of warm, moist dressings. The pathological diagnosis was epithelial solid papillary cystic neoplasm with signs of incipient pseudocapsular invasion. Immunohistochemical studies established that the tumour cells were positive for cytokeratins and vimentin, and negative for S100 protein, CD34, chromogranin, neurone-specific enolase (NSE), and glial fibrillary acid protein. The postoperative course was uneventful and the patient was discharged from hospital on day 7 and referred to the Centro di Riferimento Oncologico di Aviano for counselling. No residual tumour or metastases were found with clinical imaging, so no adjuvant therapy was recommended. In November 2001, routine work-up identified no signs of tumour relapse. This case is a typical example of solid pseudopapillary tumour of the pancreas or Frantz's tumour. The diagnostic imaging techniques used were effective in defining the location of the tumour, although a non-functioning neoplasm of the left adrenal gland was initially suspected. In 1959, Frantz[1] described a rare form of pancreatic neoplasm, which is now referred to as Frantz's tumour or solid pseudopapillary tumour. These tumours account for 2·7% of all exocrine neoplasms of the pancreas and have specific clinicopathological features which differ from those of adenocarcinoma, cystadenocarcinoma, and insulae-originating tumours. No definite aetiological factors are known, although some morphological and immunohistological features of these tumours support the idea that they originate from omnipotent cells. The exclusive occurrence of these cancers in young women indicates a relation between tumour development and female hormones. Frantz's tumours have a mean diameter of 10 cm (range 2–20) at the time of diagnosis. They appear macroscopically well-encapsulated and totally or partially cystic in 92% of cases; microscopically, they have the features of a mixed, solid, papillary cystic tumour. The malignant potential of these tumours is low and there is a good overall prognosis. Local recurrences are rare despite the use of radical excision. Frantz's tumours may be discovered by chance during diagnostic imaging procedures or may be suspected in the presence of an asymptomatic palpable mass, or an abdominal swelling in young women. CT reveals high and low densities, central cystic degeneration, and occasional calcified margins. Depending on the tumour position (on the head, body, or tail of the pancreas), preoperative assessment may suggest that it originates from the adrenal glands or that it is a pancreatic endocrine tumour, a pseudocyst,2 a hydatid cyst of the liver, or a hepatoma. Notably, the initial symptom of acute abdomen seen in 8% of cases may actually be haemoperitoneum in the lesser sac, as a result of spontaneous or traumatic rupture of the capsule.[3] Jaundice, which is associated with tumours located in the pancreatic head, is very rare because of the slow and prevalently expansive nature of tumour growth. Although Chavez and colleagues[4] considered that a correct preoperative diagnosis by CT needle-guided aspiration biopsy might be feasible as well as by ultrasound, [5] diagnoses should be confirmed by histological analysis in all cases. Frozen section diagnosis can exclude usual types of pancreatic adenocarcinoma. Immunohistological studies confirm the epithelial nature of Frantz's tumours, but well-documented cases of co-expression of cytokeratins and vimentin raise the question of whether these tumours are of mesenchymal type.[6] Moreover, neoplastic cells are generally positive for 1-antitrypsin, Leu-M1, and Ki-M1P, and mostly negative for some neuroendocrine markers such as NSE, synaptophisyn, and chromogranin, but positive for other markers including CD56. [7] Therefore, the absence of a distinct immunoreactivity pattern lessens any phenotypic relation with the cell lining of the pancreas. [6] Interestingly, the preponderance of these tumours in women, along with the presence of progesterone receptors, [6] may indicate a possible genital link, substantiated by the closeness of the genital ridges to the pancreatic anlage during embryogenesis. [4] Nishihara and colleagues[8] reported that oestrogens may have an aetiological role in Frantz tumour development and emphasised the hormone dependence of these tumours. However, the search for receptors has produced discordant results. The presence of oestrogen receptors has been shown by biochemical assay, but not by immunohistochemical analysis. [9] Positive results have only been found for progesterone. [10] Sex hormones have a role in the growth of Frantz tumour and pregnancy is associated with stimulation of tumour growth. The idea that these tumours originate from pluripotent embryonal cells that are not yet differentiated into either endocrine or exocrine was proposed on the basis of immunohistochemical and electronic microscopy studies. However, this theory needs more investigation. The clinical behaviour of these tumours can be unpredictable and histological benign tumours can show local aggressiveness. Overtly malignant lesions such as pseudopapillary carcinomas exist, as do tumours that are "borderline", which show subtle or limited morphological malignant characteristics.[11] Aggressiveness is generally associated with cellular atypia, mitotic activity, and invasion of vascular spaces, perineural interstitium, or neighbouring organs. Mao and colleagues [3] reported a 14·7% incidence of malignancy in a total of 292 cases of Frantz's tumours, and Whittle and colleagues [12] defined this tumour as "an infrequent carcinoma of good prognosis". In fact, a local recurrence rate of 6·2% is reported in cases treated by radical surgical excision, and hepatic or Krukenberg-type distant metastases develop in 5·6% of cases. [13] Frantz's tumours should be managed by surgery. Conservative resection, when technically possible, is the treatment of choice. Otherwise, duodenopancreatectomy can be done, preferably with pyloric-preserving measures rather than with the Whipple technique. Distal pancreatectomy or total pancreatectomy, with or without splenectomy, may be warranted in some cases. The presence of metastasis at first diagnosis does not exclude primary surgery because of the clinical benefits associated with tumour resection.[14] Chemotherapy and radiotherapy have both proved ineffective despite the report by Fried and colleagues [15] of a single case successfully treated with radiation. The case presented here is a typical Frantz's tumour with no histological signs of high malignant potential. The early neoplastic infiltration of the inner side of the capsule suggests that the patient should be carefully monitored. Since Frantz's tumours are rare and have a variable prognosis, all such cases should be published to improve clinicopathological information.
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#5
09.07.2003, 15:40
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Одна из последних публикаций на тему.
Gastrointestinal Endoscopy
Volume 57 • Number 7 • June 2003 Copyright © 2003 American Society for Gastrointestinal Endoscopy Diagnosis of solid-pseudopapillary neoplasm of the pancreas by EUS-guided FNA Sonali S. Master MD Thomas J. Savides MD -------------------------------------------------------------------------------- Current affiliations: Division of Gastroenterology, University of California, San Diego, California. -------------------------------------------------------------------------------- Reprint requests: Thomas J. Savides, MD, UCSD Division of Gastroenterology, 200 W. Arbor Dr., San Diego, CA 92103-8413. Copyright © 2003 by the American Society for Gastrointestinal Endoscopy Solid-pseudopapillary tumors (SPT) of the pancreas account for approximately 1% of all pancreatic neoplasms. They occur predominantly in young women, often do not cause symptoms, have low malignant potential, and are most commonly located in the body or tail of the pancreas. They generally are treated by surgical resection. Two cases of SPT of the pancreas diagnosed by EUS-FNA are described. Case reports Case 1 A 51-year-old woman presented with a 1-year history of abdominal pain, maximal in the epigastrium, associated with nausea and 2 episodes of vomiting. The symptoms were thought to be most consistent with functional dyspepsia. She noted no association of the symptoms with ingestion of food, and there had been no weight loss. There was no personal or family history of pancreatic disease. Examination and laboratory studies, including serum lipase, were unremarkable. Transabdominal US revealed a 1.8 × 1.6 × 1.2-cm hypoechoic mass in the body of the pancreas. CT confirmed the presence of an ill-defined, nonenhancing 1.5-cm soft tissue mass in the body of the pancreas, with decreased attenuation compared with the adjacent pancreatic tissue. EUS with a radial echoendoscope revealed a 16 × 11-mm hypoechoic mass in the superior portion of the pancreatic genu. The mass was located within the pancreas and did not involve the pancreatic duct or any adjacent structure. At the request of the referring physicians, EUS-FNA was performed to document malignancy before subjecting the patient to a major pancreatic resection. The procedure was performed with a linear array echoendoscope (GF-UC30P, Olympus America Corp., Melville, N.Y.). A total of 2 passes were made with a 22-gauge fine needle (Wilson-Cook Medical Inc., Winston-Salem, N.C.). Evaluation of the cytologic specimens revealed a monomorphic population of small cells with scant to moderate cytoplasm and irregular nuclear membranes. Papillary fronds with fibrovascular stalks, trabecular areas, and microacinar structures were noted, as well as metachromatic hyaline globules. Immunohistochemical stains for chromogranin and synaptophysin were negative. These findings were consistent with a diagnosis of solid-pseudopapillary neoplasm. The patient underwent a central pancreatectomy with Roux-en-Y pancreaticojejunostomy. Gross examination revealed a well-circumscribed, gelatinous-appearing, heterogeneous mass measuring 1.8 × 1.4 × 1.3 cm. Histopathologic evaluation demonstrated pseudopapillary areas, fibrosis, hyalinization, slit-like cystic spaces, and hemorrhage. There were no mitoses or nuclear pleomorphism. No perineural invasion or vascular invasion was identified. The surgical resection margins were free of tumor. The histopathologic findings confirmed the previous FNA-cytologic diagnosis of solid-pseudopapillary neoplasm of the pancreas. Postoperative recovery was uneventful. At an 18-month follow-up, the patient continued to experience similar abdominal symptoms thought to be caused by nonulcer dyspepsia. There was no evidence of endocrine or exocrine pancreatic insufficiency and no evidence of tumor recurrence or metastatic disease. Case 2 A 32-year-old woman with no GI symptoms or history of pancreatic disorder underwent magnetic resonance imaging to further evaluate an enlarged right ovary. This revealed a large, complex, cystic mass posterior to the gastric body. CT revealed a 4.6 × 4.1-cm well-circumscribed mass with a cystic portion originating within the junction of the body and neck of the pancreas (Fig. 1A). Fig. 1. A, CT showing 4.6 × 4.1-cm well-circumscribed pancreatic mass with cystic component. B, Radial EUS image (7.5 MHz, range 12 cm) showing 34 × 37-mm cystic pancreatic lesion with debris/solid component. EUS was performed with a radial echoendoscope with imaging at both 7.5 and 12 MHz. This revealed a 45 × 35-mm mixed cystic and solid lesion at the pancreatic genu (Fig. 1B). On imaging, it was unclear whether this lesion represented a benign pseudocyst with debris versus a solid mass with a cystic component. Transgastric FNA was performed with removal of all of the fluid from the cystic portion of the lesion. FNA was then performed of the solid/debris portion of the lesion, a total of 5 passes being made with a 22-gauge fine needle (Wilson-Cook). Fluid analysis of the cystic portion revealed an amylase of 51 U/L, lipase of 32 U/L, CA 19-9 of 5 U/mL (0-37 U/mL), and CEA of less than 0.2 ng/mL (0-3.0 ng/mL). Examination of the cellular aspirate from the mass lesion revealed loosely cohesive aggregates and single monomorphic cells with moderately enlarged nuclei and a moderately increased nuclear/cytoplasmic ratio, as well as characteristic papillary structures (Fig. 2A). Fig. 2. A, Photomicrograph of EUS-FNA specimen showing characteristic papillary structures (Papanicolau stain, orig. mag. ×100). B, Photomicrograph of tissue section demonstrating solid and papillary areas (H&E, orig. mag. ×100). The chromatin was fine and evenly distributed. Immunostains showed a strong positive reaction with neuron specific enolase and a weak reaction with chromogranin in only rare cells. The tumor cells were also positive for vimentin (diffuse strong reaction) and alpha-1-antitrypsin. Synaptophysin and cytokeratin were negative. These findings were consistent with a diagnosis of solid-pseudopapillary neoplasm of the pancreas. The patient subsequently underwent a segmental resection of this lesion. Histopathologic evaluation demonstrated both solid and pseudopapillary areas (Fig. 2B). Immunocytochemical stains confirmed the findings for the aspirate, and, in addition, the tumor cells exhibited a positive reaction for progesterone receptor and a negative reaction for estrogen receptor. At a 6-month follow-up, the patient was asymptomatic from a GI standpoint, with no signs of tumor recurrence, metastases, or signs of either endocrine or exocrine insufficiency of the pancreas. Discussion SPT of the pancreas was first described in 1959.[1] There have since been many synonyms for SPT used in various publications; some of these are solid and papillary epithelial neoplasm, solid and cystic tumor, papillary-cystic neoplasm, papillary cystic tumor, and Franz's tumor. In 1996, this tumor was renamed solid-pseudopapillary tumor by the World Health Organization for the histologic classification of tumors of the exocrine pancreas.[2] Although these tumors have characteristic cytologic and histologic features, their cell of origin remains unclear. During embryogenesis, the genital ridges are in close proximity to the pancreatic anlage. Some have speculated that SPTs may come from genital ridge/ovarian anlage-related cells, which were attached to the pancreatic tissue during early embryogenesis.[3] Yet others have considered ductal, acinar, and endocrine cell origins without convincing evidence. SPTs comprise approximately 1% of all pancreatic neoplasms, and in one center they accounted for 12% of 134 resected, clinically cystic pancreatic tumors.[4] These tumors most commonly are identified in young women. In one series of 59 SPTs, 88% of the patients were women.[3] Mean age at diagnosis is approximately 26 years, with a reported range of patient's age from 8 to 70 years.[3] [5] [7] Patients with SPTs are frequently asymptomatic, and the tumor is often found incidentally on imaging studies performed for other indications. When present, symptoms are nonspecific and may include nausea, vomiting, abdominal pain, and fullness. These lesions can present at any size, with reported dimensions ranging from 1.5 to 30 cm (mean 10.5 cm).[8] They most commonly are located in the pancreatic body and tail. On CT, SPTs may appear as well-circumscribed hypodense lesions with or without obvious cystic change.[9] (Продолжение-в след.посте)
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#6
09.07.2003, 15:41
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Продолжение...
....Bondeson et al.[10] were the first to correctly diagnose SPT by preoperative transabdominal FNA. Subsequently, SPTs have also been studied by preoperative, sonographically guided, percutaneous aspiration.[11] However, SPT has not been diagnosed previously by EUS-FNA, as in the 2 cases presented.
The cytologic features of SPT of the pancreas are distinctive and allow differentiation from other lesions.[11] These include highly cellular aspirates composed of small, uniform epithelial cells with oval nuclei, fine chromatin, and small nucleoli. The cells are arranged as papillary structures with delicate fibrovascular cores, and there may be a layer of myxoid material between the core and lining epithelial cells. Cytologic features were diagnostic in each of these 2 cases and were confirmed by surgical resection. By immunohistochemistry, SPTs are usually positive for vimentin, alpha-1-antitrypsin, alpha-1-antichymostrypsin, and neuron specific enolase. Approximately one third are keratin positive, whereas some express other neuroendocrine markers such as synaptophysin. Chromogranin has been consistently reported as negative. Recently, SPTs have been described that also stain positive for beta catenin.[12] In both cases, there was a pattern consistent with SPT, although in the second case, there were reportedly rare cells positive for chromogranin. There is no known correlation between tumor size and metastatic potential. However, in tissue sections, the presence of venous invasion and large necrotic clusters may be indicative of an increased risk of malignancy.[13] SPTs have an excellent prognosis with a low potential for local invasion or metastasis. At presentation, approximately 85% are limited to the pancreas with metastases in only 10% to 15% of cases.[8] [14] Over 95% of SPTs limited to the pancreas may be cured by complete surgical resection. [15] Long-term survival with a 15-year follow-up has been reported after surgical resection of both primary tumors and metastatic disease that is primarily found in the liver.[16] Long-term survival has also been noted for patients with residual disease.[13] [17] A common reason for performing EUS-FNA of small or incidentally found pancreatic lesions is that most patients with such lesions are reluctant to undergo major pancreatic surgery, and most community surgeons hesitate to consider such an operation without a preoperative diagnosis of a potentially malignant lesion, as was the situation in Case 1. This is especially the case for patients who are poor candidates for surgery. In addition, EUS-FNA can guide surgical management, because a potentially benign tumor (e.g., neuroendocrine, SPT) may be treated by local excision, in contrast to an adenocarcinoma, which would require a more extensive resection. This was also the result in Case 1, as the patient underwent a central pancreatectomy. EUS features alone cannot reliably distinguish between benign and malignant cystic lesions of the pancreas, making preoperative FNA desirable.[18] There is increasing evidence that EUS-FNA may help to distinguish benign from potentially malignant pancreatic cysts through analysis of cyst fluid, not only cytologic evaluation, but also for amylase, lipase, and CEA.[19] This was the situation in Case 2 in which there was a question whether the lesion might be a pseudocyst from previous clinically silent pancreatitis. EUS-FNA is not always needed for evaluation of potentially resectable pancreatic masses. When there is an obvious large mass lesion, enlargement of a lesion demonstrated by serial imaging studies, a lesion that is clearly the cause of symptoms, or when the patient and the surgeon are comfortable with resection based on imaging characteristics alone, the diagnostic information provided by EUS-FNA ultimately may not change the need for surgical resection. Also, EUS-FNA has potential complications (1% complication rate for solid lesions, 14% for cystic lesions), which could make subsequent surgery more difficult or even impossible.[20] In summary, solid-pseudopapillary neoplasm of the pancreas should be considered in young, asymptomatic women with incidentally detected pancreatic body masses. EUS-FNA can accurately diagnose SPTs of the pancreas and may help guide surgical management. Acknowledgment Cynthia Behling, MD, PhD, for pathology and manuscript review. References 1. Frantz VK. Papillary tumors of the pancreas: benign or malignant? In: Frantz VK, editor. Tumors of the pancreas. Atlas of tumor pathology. 1st Series. Section VII. Fascicle 27. Washington DC: U.S. Armed Forces Institute of Pathology; 1959. p. 32-3. 2. Kloppel G, Solcia E, Longnecker DS, Capella C, Sobin LH. Histological typing of tumours of the exocrine pancreas. In: World Health Organization international classification of tumours. New York: Springer; 1996. p. 8452. 3. Kosmahl M, Seada LS, Janig U, Harms D, Kloppel G. Solid-pseudopapillary tumor of the pancreas: its origin revisited. Virchows Arch 2000;436:473-80. Abstract 4. Klimstra DS, Wenig BM, Heffess CS. Solid-pseudopapillary tumor of the pancreas: a typically cystic carcinoma of low malignant potential. Semin Diagn Pathol 2000;17:66-80. Abstract 5. Pettinato G, Manivel C, Ravetto C, Terracciano L, Gould E, Di Tuoro A, et al. Papillary cystic tumor of the pancreas. Am J Clin Pathol 1992;98:478-88. Abstract 6. Matsunou H, Konishi F. Papillary-cystic neoplasm of the pancreas. A clinicopathologic study concerning the tumor aging and malignancy of nine cases. Cancer 1990;65:283-91. Abstract 7. Cubilla AL, Fitzgerald PJ. Tumors of the exocrine pancreas. In: Hartmann WH, Sobin LH, editors. Atlas of tumor pathology. Series 2. Fascicle 19. Washington DC: Armed Forces Institute of Pathology; 1984. p. 201-7. 8. Mao C, Guvendi M, Domenico DR, Kim K, Thomford NR, Howard JM. Papillary cystic and solid tumors of the pancreas: a pancreatic embryonic tumor? Studies of three cases and cumulative review of the world's literature. Surgery 1995;118:821-8. Abstract 9. Dong PR, Lu DS, Degregario F, Fell SC, Au A, Kadell BM. Solid and papillary neoplasm of the pancreas: radiological-pathological study of five cases and review of the literature. Clin Radiol 1996;51:702-5. Abstract 10. Bondeson L, Bondeson AG, Genell S, Lindholm K, Thorstenson S. Aspiration cytology of the rare solid and papillary epithelial neoplasm of the pancreas: light and electron microscopic study of a case. Acta Cytol 1984;28:605-9. Abstract 11. Pelosi G, Iannucci A, Zamboni G, Bresaola E, Iacono C, Giovanni S. Solid and cystic papillary neoplasm of the pancreas: a clinico-cytopathologic and immunocytochemical study of five new cases diagnosed by fine-needle aspiration cytology and a review of the literature. Diagn Cytopathol 1995;13:233-46. Abstract 12. Tanaka Y, Notohara K, Kato K, Ijiri R, Nishimata S, Miyake T, et al. Usefulness of beta-catenin immunostaining for the differential diagnosis of solid-pseudopapillary neoplasm of the pancreas. Am J Surg Pathol 2002;26:818-20. Citation 13. Nishihara K, Nagoshi M, Tsuneyoshi M, Yamaguchi K, Hayasshi I. Papillary cystic tumors of the pancreas: assessment of their malignant potential. Cancer 1993;71:82-92. Abstract 14. Adair CF, Wenig BM, Heffess CS. Solid and papillary cystic carcinoma of the pancreas: a tumor of low malignant potential [abstract]. Int J Surg Pathol 1995;2:326. 15. Jeng LB, Chen MF, Tang RP. Solid and papillary neoplasm of the pancreas. Emphasis on surgical treatment. Arch Surg 1993;128:433-6. Abstract 16. Martin RCG, Klimstra DS, Brennan MF, Conlon KC. Solid-pseudopapillary tumor of the pancreas: a surgical enigma? Ann Surg Oncol 2002;9:35-40. Abstract 17. Zinner MJ, Shurbaji MS, Cameron JL. Solid and papillary epithelial neoplasms of the pancreas. Surgery 1990;108:475-80. 18. Ahmad NA, Kochman ML, Lewis JD, Ginsberg GG. Can EUS alone differentiate between malignant and benign cystic lesions of the pancreas? Am J Gastroenterol 2001;96:3229-30. 19. Brugge WR, Saltzman JR, Scheiman JM, Wallace MB, Jowell PS, Pochapin MB, et al. Diagnosis of cystic neoplasms of the pancreas by EUS; The report of the cooperative pancreatic cyst study [abstract]. Gastrointest Endosc 2001;53:AB71. 20. Wiersema MJ, Vilmann P, Giovannini M, Chang KJ, Wiersema LM. Enodsonography-guided fine-needle aspiration biopsy: diagnostic accuracy and complication assessment. Gastroenterology 1997;112:1087-95. Abstract
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Линейный вид
