Zystische Adventitiadegeneration

Cystic adventitial disease (CAD) as a cause of claudication or stenosis of the popliteal artery is still very rare. Publications mainly consist of case reports describing predominantly male patients of medium age. We report on a 65-year-old female patient with a significant arteriosclerotic risk profile (hypertension, hyperlipidemia, hyperuricemia, smoker), symptoms of a claudication (Fontaine classification stage IIb), and a stenosis of the left popliteal artery. Preoperatively performed ultrasound scan (duplex) and angiography showed indications of CAD, especially regarding the location of the lesion in the P2 section of the popliteal artery. Clinical presentation and local findings in the diagnostic procedures led to operative treatment. Surgery was performed via dorsal approach to the popliteal artery. The affected section of the artery was resected and replaced by an autologous, ipsilateral graft of the greater saphenous vein. Intraoperatively, the diagnosis was confirmed macroscopically and later also by histological examination. The clinical course was normal. Our case is an unusual example for the appearance of CAD in an elderly woman with arterial occlusive disease and corresponding arteriosclerotic risk profile. The importance of duplex ultrasound and angiography for the diagnosis and differential diagnosis of CAD and other causes of popliteal artery occlusive disease is emphasized. Surgery remains the standard treatment.     

Zystische Adventitiadegeneration (CAD) als Ursache einer invalidisierenden Claudicatio intermittens

Duplex sonography and angiography revealed cystic adventitial degeneration (CAD) in a 45-year-old patient with intermittent claudication of the right calf and an initial maximum walking distance of 50 m. Intraoperatively we found a cystic lesion containing gelatinous fluid in the adventitial layer of the popliteal artery. Because of the extended lesion, resection of the involved segment was performed, followed by autologous venous reconstruction. CAD is a rare disease and should be considered in young patients without systemic atherosclerosis. The surgical treatment is, depending on the case, either nonresectional by circumferential removal of adventitial cysts or resectional followed by autologous reconstruction in extended lesions or total occlusion of the artery.     

Zystische Pankreastumoren

Zusammenfassung Vor 1978 unterschied man bei den zystischen Tumoren des Pankreas nur zwischen Zystadenom und Zystadenokarzinom [2]. Inzwischen wurden von den Pathologen eine Reihe weiterer Tumorentitäten klassifiziert. Man differenziert heutzutage zwischen dem generell gutartigen serösen Zystadenom, dem potentiell maligne entartenden muzinösen Zystadenom, dem fakultativ malignen papillär-zystischen Tumor und dem muzinösen Zystadenokarzinom als obligat malignen Tumor. Weitere seltenere Tumortypen sind der solid-zystische Acinuszelltumor, der zystische Inselzelltumor sowie die muzinöse duktale Hyperplasie. Aufgrund des langsamen Wachstums mit in erster Linie verdrängendem Charakter werden alle diese Tumoren häufig erst bet schon beachtlicher Tumorgröße diagnostiziert. Bei der Diagnostik haben das CT, die Ultraschalluntersuchung sowie die ERCP einen festen Stellenwert. Dabei ist es in der Regel zwar möglich, eine Unterscheidung zur Pseudozyste zu führen, eine Differenzierung der einzelnen Tumortypen ist mit Mitteln der Diagnostik nahezu unmöglich. In unserem Krankengut sahen wir in den Jahren 1979 his 1990 10 Fälle von serösen Zystadenomen, weiterhin 5 muzinöse Zystadenome, 9 Zystadenokarzinome sowie 4 maligne papillär-zystische Tumoren. Von diesen konnten 9 der 10 serösen Zystadenomen, 4 von 5 muzindsen Zystadenomen, alle 4 papillär-zystischen Tumoren Bowie 5 von 9 Zystadenokarzinomen kurativ reseziert werden. Alle Patienten mit kurativ resezierten Adenomen blieben wie auch ein Patient mit einem RI -resezierten Zystadenom in der Nachbeobachtungszeit tumorfrei. Eine Patientin, bei der ein seröses Zystadenom nur histologisch gesichert worden war, lebt inzwischen 8 Jahre nach Diagnosestellung mit nunmehr 86 Jahren unter langsamer Progression weiterhin beschwerdenfrei. Von den 5 Patienten, bei denen ein Zystadenokarzinom kurativ reseziert worden war, verstarben 2 Patienten postoperativ. Ein dritter verstarb 4 Monate nach dem Eingriff an einem ausgedehnten Tumorrezidiv. Die beiden restlichen Patienten leben nach 16 und 28 Monaten tumorfrei. Alle 4 palliativ operierten Patienten mit muzindsen Zystadenokarzinomen verstarben nach 8 bis 28 Monaten an ihrer Tumorerkrankung. Von den 4 Patienten mit einem malignen papillär-zystischen Tumor leben 2 73 und 30 Monate nach der Operation erscheinungsfrei. Ein Patient verstarb tumorfrei 45 Monate nach dem Eingriff und der vierte ist 39 Monate nach dem Eingriff wegen eines synchronen metastasierenden Zweittumors inzwischen in einem präfinalen Zustand. Eine Unterscheidung zwischen den einzelnen Tumortypen ist durch diagnostische Maßnahmen nicht möglich. Da aber nach kurativer Resektion zystische Pankreastumoren eine sehr gute Prognose aufweisen, ist grundsätzlich eine komplette Entfernung des Tumors sowie anschließend eine vollständige histologische Aufarbeitung der gesamten Zyste zu fordern. Diese Resektion sollte nach den Grundsätzen der onkologischen Radikalität durchgeführt werden.

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Cystic tumors of the pancreas

Before 1978, where cystic tumors of the pancreas were concerned, pathologists only differentiated between cystic adenomas and cystadenocarcinomas. Recently, however, further tumor entities have been introduced. We now differentiate between the generally benign serous cystic adenoma, the potentially malignant mucinous cystadenoma, the possibly malignant papillary cystic tumor, and the always malignant mucinous cystadenocarcinoma. Other rare tumors include the solid cystic acinous-cell tumor, the cystic islet tumor, and mucinous ductal hyperplasia. Because of their slow growth and primary displacement nature, all of these tumors can usually be detected only after they have attained considerable size. Computed tomography (CT), sonography and endoscopic retrograde cholangiopancreatography (ERCP) have an established role in diagnosis. With these methods, as a rule, it is possible to identify pseudocysts; however, differentiation between the individual tumor types is almost impossible. In our study from 1979 to 1990, we observed ten cases of serous cystic adenomas, nine cystadenocarcinomas, and four malignant papillary-cystic tumors. Of these, nine of the ten serous cystic adenomas, four of the five mucinous cystadenomas, all four papillary-cystic tumors, and five of the nine cystadeno-carcinomas were curatively resected. All patients with curatively resected adenomas and one patient with an R1-resected cystic adenoma remained free of recurrence throughout the follow-up period. One 86-year-old female patient in whom a serous cystic adenoma was histologically determined still has no symptoms 8 years after diagnosis despite slow tumor progression. Two of the five patients in whom a cystadenocarcinoma was curatively resected died postoperatively; a third patient died of tumor recurrence 4 months following resection. The two remaining patients are alive and tumor-free at 16 and 28 months postoperatively. All four patients with mucinous cystadenocarcinomas who underwent palliative operations died of their tumor 8 to 28 months later. Two of the four patients with a malignant papillary cystic tumor were alive and recurrence-free 73 and 30 months postoperatively. One patient died free of tumor 45 months postoperatively, and the fourth patient is presently at the preterminal stage following development of a synchronous metastasizing second tumor. Diagnostic techniques cannot be used to differentiate between the individual tumor types; however, because cystic tumors of the pancreas have a good prognosis following curative resection, complete removal of the tumor and a complete histological workup of the entire cyst is required. The resection should be performed in accordance with the oncological rules radical operations.

     

Die zystische Adventitiadegeneration der Venen

Cystic adventitial degeneration of veins is a very rare disease, particularly in women. In proportion to cases involving arteries, it occurs approximately 20 times less frequently. Histologic findings reveal genuine ganglia in the adventitia, which often are also connected by a branch to the joint capsule. Relatively speaking, the most frequent site is the common femoral vein, but sporadically it also develops in the popliteal vein or in superficial veins. In the large guide veins, the disease becomes apparent through swelling of the entire leg without weight bearing and a feeling of congestion. Dermatological alterations as seen in cases of chronic venous stasis syndrome have not been reported in the literature. The diagnosis is made by B-scan and color-coded duplex sonography, which demonstrates cystic formation in the venous wall and the ensuing narrowing of the vessel. In cases of more pronounced stenosis, respiratory modulation of the Doppler signals is lost. Ascending phlebography exhibits a typical pelotte-form compression effect. As a rule, computed tomography or magnetic resonance imaging is performed preoperatively. Surgical strategy consists in removal of the cyst within the adventitia. When sclerotic alterations in other wall layers are suspected, consideration can be given to venous patch plasty. This procedure results in complete recovery, but cases of relapse have been reported in the literature. We report on a case of successful management.     

Zystische Tumoren des Pankreas

Cysts of the pancreas most often develop after chronic or acute inflammation of the pancreas. Cystic neoplasia of the pancreas have been increasingly recognized in clinical practice and 90% are represented by four types: serous microcystic (SCN), mucinous cystic (MCN), intraductal papillary-mucinous (IPMN) and solid pseudopapillary (SPN) neoplasia. IPMN is the most common form nowadays and main duct and branch duct types can be differentiated by morphology. This classification is of prognostic and therapeutic relevance. While main duct IPMNs have a high risk of malignant progression and resection is therefore recommended, branch duct IPMNs have a much lower risk of harboring malignancy. Small branch duct IPMNs (<2 cm) without symptoms or mural nodules can be managed by periodic surveillance. Recently, it has become clear that IPMN constitutes a heterogeneous group with at least four subtypes. Their stratification reveals that the various subtypes of IPMN have different biological properties with different prognostic implications, but the subclassification is usually not known prior to surgery. Moreover, even differentiation between inflammatory and neoplastic cysts can be challenging. Clear indications for resection are local complications (jaundice or gastric outlet obstruction), large and increasing tumurs, symptoms or secretion of mucinous fluid from the papilla of Vater.     

Zystische Raumforderung im hinteren Mediastinum

We present the case of a 52-year-old male who underwent thoracotomy for resection of a suspected bronchogenic cyst in the right posterior mediastinum. The size of the tumor had increased over years, according to repeated X-rays, and the cyst became symptomatic with obstruction of the right subclavian and jugular veins. To our surprise, histopathology revealed a hydatid cyst.     

Zystische Adnextumoren beim Mann (Spermatozelen) und die Pseudorezidiv-Hydrozele

Zusammenfassung überblick über die zystischen Adnextumoren beim Mann. Sie müssen von Hydrozelen differenziert werden, um mittels geeigneter Operationsverfahren sogenannte “Rezidivhydrozelen” zu vermeiden, die es gar nicht geben kann.

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Summary Survey of the cystic tumours of the human scrotal organs. Their differentiation from hydroceles ist important for avoiding recurring “pseudo-hydroceies” after surgery.

     

Zystische knochenveränderungen Ätiologie, diagnostik, therapeutische Prinzipien und eigene behandlungsergebnisse

Various bone disorders become manifest as cystic lesions. The differential diagnosis must include benign and malignant tumors and also non-tumorous lesions, such as osteomyelitis. The most important and most frequent types of genuine bone cyst are juvenile bone cyst and aneurysmal bone cyst. When juvenile bone cysts occur in adults they are called solitary bone cysts. Despite intensive research the pathogenesis of bone cysts is still unknown to this day, so that successful causal therapy is impossible. The main problem in the treatment of bone cysts is their high rate of recurrence, rates ranging between 20% and 50% having been cited in the international literature. A critical review of the literature reveals few publications with helpful follow-up results. Most of the publications are case reports, and they frequently merely describe various forms of treatment. More recent reports are mainly concerned with such methods as curettage, steroid injections, and continuous decompression with perforated screws. Until the early 1980s, segmental bone resection was the treatment of choice. Because of its high complication rate it has since been abandoned. In the last analysis, the only well-established method for which long-term results obtained in studies of any size have been published, is curettage of the cyst and grafting with cancellous bone from the iliac crest. In our series, 41 patients were treated with this method, and we recorded a recurrence rate of 17.1 %. Complications were rare. The risk of recurrence depended on the age of the patient. A higher recurrence rate must be expected in children under the age of 10 years. For this reason, operative treatment should not be performed until after that age if possible. Newer methods, such as steorid injections and continuous decompression by means of perforated screws, had better results in some studies, but only according to a few authors. Further research is needed to show whether our method will yield good results in the long term when applied in larger patient collectives.

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Zystische knochenveränderungen Ätiologie, diagnostik, therapeutische Prinzipien und eigene behandlungsergebnisse

Zusammenfassung Eine ganze Reihe verschiedener Knochenläsionen manifestieren sich unter dem Bild einer zystischen Veränderung. Differentialdiagnostisch abzugrenzen sind gutartige und maligne Tumoren sowie nichttumoröse Erkrankungen wie die Osteomyelitis. Die wichtigsten und häufigsten Vertreter der echten KZ sind die juvenile (JKZ) sowie die aneurysmatische Knochenzyste (AKZ). Im Erwachsenenalter auftretende JKZ werden als solitäre Knochenzysten (SKZ) bezeichnet. Trotz intensiver Forschung ist die Pathogenese von KZ bis zum heutigen Zeitpunkt unklar geblieben, eine erfolgversprechende kausale Therapie somit nicht möglich. Die Hauptproblematik der Behandlung von KZ liegt in der hohen Rezidivrate. In der Literatur wird in der Regel auf Werte zwischen 20–50% verwiesen. Bei kritischer Durchsicht der Literatur wurden jedoch nur in wenigen Fällen aussagekräftige Nachuntersuchungsergebnisse veröffentlicht. In der Regel handelt es sich bei den meisten Publikationen um Kasuistiken, die sich überwiegend auf die Beschreibung verschiedener Behandlungsverfahren beschränken. In neueren Publikationen werden hauptsächlich die Methoden der Kürettage, der Steroidinjektion sowie der kontinuierlichen Dekompression mittels Lochschrauben erwähnt. Die noch bis Anfang der 80er Jahre teilweise als Verfahren der Wahl bezeichnete Segmentresektion ist aufgrund der nicht unerheblichen Komplikationsrate zunehmend verlassen worden. Letztlich muß als einzig etablierte Methode mit publizierten Langzeitergebnissen auch größerer Fallzahlen die Zystenkürettage mit Spongiosa-plastik angesehen werden. Bei unseren 41 nachuntersuchten Patienten, die mit dieser Methode behandelt wurden, konnte eine Rezidivrate von 17,l% erreicht werden. Die Komplikationsrate war gering. Das Rezidivrisiko war vom

     

Zystische nebenschilddrüsenadenome : pathologisch-anatomische variante von epithelkörperchenadenomen oder krankheitsbild mit eigener bedeutung?

Zusammenfassung Die klinisch relevanten Daten von 126 Patienten, die sich zwischen dem 1.1.1987 und dem 31.12.1990 wegen eines Nebenschilddrüsenadenoms an der Klinik für Allgemeinchirurgie der Philipps-Universität Marburg einer Erstoperation unterzogen, wurden prospektiv dokumentiert. Bei 6 Patienten (4,8 %) wurden insgesamt 7 zystische Adenome gefunden. Bei gleicher Alters- and Geschlechtsverteilung wiesen Patienten mit zystischen Adenomen deutlich höhere Serumspiegel für Parathormon and alkalische Phosphatase auf als die mit soliden Adenomen. Der Serumkalziumspiegel in den beiden Gruppen war nicht unterschiedlich. Zystische Adenome waren deutlich schwerer als solide Adenome. Ein erheblicher Anteil der zystischen Adenome war in das hintere Mediastinum verlagert. Die Eigenschaft zystischer Nebenschilddrüsenadenome, häufig nicht an den normalen anatomischen Positionen zu liegen, kann bei ihrer intraoperativen Suche zu Schwierigkeiten führen.

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Cystic parathyroid adenomas: pathoanatomical variant of parathyroid adenomas or separate disease entity?

Summary Between 01. 01.1987 and 31.12.1990 a primary operation was performed on 126 patients in the Dept. of General Surgery, University of Marburg Hospital for primary hyperparathyroidism due to a parathyroid adenoma. Their clinically relevant data were prospectively documented. In 6 patients (4.8%) a total of 7 cystic parathyroid adenomas was found. While there was no difference in age- and sex-distribution, patients with cystic adenomas were found to have markedly higher serum parathormone and alkaline phosphatase levels than patients with solid adenomas. Calcium levels were similar in both groups. Cystic adenomas were much heavier than solid adenomas. A significant number of cystic adenomas was found to be displaced into the posterior mediastinum. The property of cystic parathyroid adenomas to be frequently located away from their usual anatomical position can make the intraoperative search for them difficult.