Skip to main content
Erschienen in:

Open Access 01.11.2007 | Case Reports

Pulmonary Sclerosing Hemangioma Detected by Fluorodeoxyglucose Positron Emission Tomography in Familial Adenomatous Polyposis: Report of a Case

verfasst von: Daan B. de Koning, M.D., Joost P. H. Drenth, M.D., Ph.D., Wim J. G. Oyen, M.D., Ph.D., Michiel Wagenaar, M.D., Ph.D., Riena P. Aliredjo, M.D., Fokko M. Nagengast, M.D., Ph.D.

Erschienen in: Diseases of the Colon & Rectum | Ausgabe 11/2007

download
DOWNLOAD
print
DRUCKEN
insite
SUCHEN

Abstract

We present a 53-year-old female suffering from familial adenomatous polyposis, who was found to have a positive nodus, lateral to the hilus of the left lung, on routine FDG-PET scan. This lesion was found to be a sclerosing hemangioma. We found an aberrant β-catenin expression on immunohistochemical staining, suggesting that sclerosing hemangioma and familial adenomatous polyposis share the same pathophysiology. It is important to be aware of the association of familial adenomatous polyposis and sclerosing hemangioma.
Hinweise
Supported by a NWO-VIDI grant to Joost P. H. Drenth, M.D., Ph.D.
Reprints are not available.
Sclerosing hemangioma is a rare, but presumably, benign lung tumor, believed to originate from pneumocyte Type II cells,1 which was first as such described by Liebow and Hubbell in 1956.2 It is a rare lung tumor, probably accounting for approximately 3 to 5 percent of benign lung lesions. Case series suggest that females are mainly affected, with a reported median age of 46 years at presentation.3,4 Sclerosing hemangiomas often are asymptomatic and are usually an unexpected finding on routine imaging studies. The lesions are well circumscribed and do not invade the adjacent normal lung parenchyma. The diagnosis is made on the basis of histologic examination. Sclerosing hemangiomas are composed of round cells with bland nuclei and pale to clear cytoplasm, but also papillary structures, with cuboidal cells lining them, are present. Sclerosing hemangiomas can exhibit four described patterns on histologic presentation: papillary, sclerotic, hemorrhagic, and solid, and all patterns can be present or absent within lesions.4
Little is known of the natural course, prognosis, and associated risk factors of sclerosing hemangioma. A recent case described the identification of a sclerosing hemangioma in a patient with attenuated familial adenomatous polyposis (FAP).5 FAP is a rare genetic disease (OMIM #175100) characterized by the development of literally hundreds to thousands of adenomatous polyps in the colon, which, if left untreated, lead to cancer at a young age.6 We wish to report the unexpected discovery of a sclerosing hemangioma that accumulated radiolabeled fluorodeoxyglucose (FDG) on positron emission tomography (PET) in a patient suffering from FAP.

Report of A Case

Clinical Evaluation

A 53-year-old female was seen at our outpatient clinic because of follow-up of FAP. Her medical history revealed a total colectomy and insertion of an ileostoma in 1986 because of FAP. The germline mutation was localized on exon 13 of the APC gene (C1660 T; Arg 554 X). Routine endoscopy of duodenum revealed a number of adenomatous polyps, mainly located around the ampulla of Vater. Her father had died at age 60 years because of lung cancer. She complained of a persistent, dry cough, but denied smoking. No abnormalities were found on physical examination. Because of a protocol for the follow-up of duodenal adenomas, a FDG-PET scan was made. Whole-body PET, 30 minutes after the intravenous injection of 250 MBq Fluor-18-FDG, revealed a small hot spot located at the hilus of the left lung (Fig. 1A). Although the uptake of the entire mass was little over background uptake (standard uptake value (SUV) 1.6), this did not rule out a malignancy. A chest x-ray and contrast-enhanced CT revealed a single circumscriptive lesion, with a diameter of 2.1 cm, situated in the left lung close to the hilus (Fig. 1B and C). There were no enlarged lymph nodes. Because a malignant lung tumor could not be excluded on basis of the available imaging data and because of her underlying FAP, we proceeded to a left-sided thoracotomy with excision of the tumor followed.

Pathologic Examination

On gross examination, a well-circumscribed, solid, subpleural tumor was found with a greatest dimension of 2 cm. Histologically, the lesion had a mixed papillary, solid and sclerotic pattern of two cell types: cuboidal surface cells, and round stromal cells. Focal clusters of foamy macrophages and cholesterol clefts were seen. Immunohistochemical studies showed expression of cytokeratin 7 in the surface cells (Fig. 2A). Thyroid transcription factor 1 was positive in both surface cells as well as in stromal cells. The tumor was classified as a sclerosing hemangioma. Additional immunohistochemical staining for •-catenin showed an aberrant expression in the cytoplasm and in the nucleus of both the surface and round cells (Fig. 2B).

Discussion

This is a case report of a solitary pulmonary lesion discovered by FDG-PET in a patient with FAP and found to be a sclerosing hemangioma on pathologic examination. Most manifestations of FAP are restricted to the gastrointestinal tract and include duodenal and colonic cancer as well as fundic gland polyposis. In a variant of FAP, Gardner's syndrome, patients not only have colonic polyps, but also extracolonic manifestations, such as desmoid tumors, osteomas, epidermoid cysts, various soft tissue tumors, and a predisposition to thyroid cancers.7 Our patient did not have any extraintestinal manifestations of her FAP until she presented with a sclerosing hemangioma. We were able to identify a single, comparable case from the literature. This 54-year-old, asymptomatic female affected with attenuated familial adenomatous polyposis (AFAP) presented with a left lower lobe mass on chest x-ray examination on a routine health checkup. CT and magnetic resonance imaging studies revealed a sharply demarcated lesion with a maximal diameter of 3.6 cm without evidence for metastases. The lesion had a slightly increased FDG uptake ratio of 1.8 on PET. A surgical enucleation followed and pathologic examination was compatible for sclerosing hemangioma.8
In the majority of cases, a sclerosing hemangioma is asymptomatic and is usually found only after routine imaging studies for other unrelated reasons. In a clinical series, 11 percent of patients presented with hemoptysis and 9 percent with cough.3 In a retrospective series of 100 cases, there was no evidence for bilateral tumors or widespread metastasized disease, corroborating with the benign nature of the tumor.3 On the other hand, there was one patient with regional lymph node metastases, and there have been reports with multiple sclerosing hemangioma lesions and lymph node metastases implying a more malignant potential.9,10 This might corroborate with the monoclonality of the cells within the lesion.11 This finding suggests a neoplastic growth pattern, but invasion of normal lung parenchyma has not been described.12 The prognosis after surgical resection is excellent, although performing a second surgical procedure because of local recurrence is necessary in a small percentage of cases.3,4
The actual prevalence of sclerosing hemangiomas among FAP patients is unknown, and this is most likely caused by the relatively asymptomatic nature of the disease. Although the co-occurrence may have arisen by chance, a recent report reiterates its association and demonstrated an aberrant nuclear and cytoplasmic expression of β-catenin in the sclerosing hemangioma.5 β-catenin is an extracellular circulating protein that activates the Wnt-pathway. Uncontrolled activation of the Wnt-pathway leads to uncontrolled cell growth and finally to malignancy. The aberrant staining of β-catenin of the sclerosing hemangioma in FAP suggests that it is a part of the clinical phenotype of FAP.5 When abundantly available in the cell cytosol, β-catenin can induce transcription of a number of genes, primarily involved in regulating cell growth, which are thought to play a critical role in tumorigenesis. Positive staining for β-catenin, thus demonstrating accumulation of the protein in the cell cytosol, is described in a number of tumors, especially colorectal cancers. In colorectal cancer, β-catenin accumulation emerges as a result of mutation of the APC gene, which encodes for a product that is critical for β-catenin degradation. FAP probably arises because a dominant-negative effect of APC gene protein products, which suggest that it acts as a tumor-suppressor gene. APC gene mutations have been described almost exclusively in colorectal cancer. It is possible that somatic APC mutations underlie the sclerosing hemangioma, which would imply sclerosing hemangioma and FAP share the same pathophysiology. We did not pursue this possibility, but we found aberrant β-catenin staining, which has not been described in sclerosing hemangioma so far, with the exception of one other FAP case. The aberrant β-catenin is similar to those found in other FAP-associated tumors.13,14
FDG-PET is well positioned to distinguish benign from malignant lesions in the evaluation of a solitary pulmonary nodule. A recentmeta-analysis indicated that FDG-PET has an overall median sensitivity of 97 percent and a specificity of 78 percent in the evaluation of a solitary pulmonary nodule.15 In this respect, this diagnostic approach has been shown to be more accurate than CT. The relatively low SUV in our patient with FAP (1.6) and another recent FAP case from the literature (1.8) suggest that the biologic behavior of sclerosing hemangioma in FAP may be benign because malignant lesions are characterized by higher SUV values.5 We used FDG as radiopharmacon, but C-11-choline might be an alternative for identification of a sclerosing hemangioma with PET.16

Conclusions

We described the first sclerosing hemangioma associated with true FAP, a lesion that may become increasingly more important when more patients with FAP are screened with modern imaging modalities.
Open Access This is an open access article distributed under the terms of the Creative Commons Attribution Noncommercial License ( https://​creativecommons.​org/​licenses/​by-nc/​2.​0 ), which permits any noncommercial use, distribution, and reproduction in any medium, provided the original author(s) and source are credited.

Unsere Produktempfehlungen

Die Chirurgie

Print-Titel

Das Abo mit mehr Tiefe

Mit der Zeitschrift Die Chirurgie erhalten Sie zusätzlich Online-Zugriff auf weitere 43 chirurgische Fachzeitschriften, CME-Fortbildungen, Webinare, Vorbereitungskursen zur Facharztprüfung und die digitale Enzyklopädie e.Medpedia.

Bis 19. Juli 2024 bestellen und im ersten Jahr bis zu 200 € sparen!

e.Med Interdisziplinär

Kombi-Abonnement

Jetzt e.Med zum Sonderpreis bestellen!

Für Ihren Erfolg in Klinik und Praxis - Die beste Hilfe in Ihrem Arbeitsalltag

Mit e.Med Interdisziplinär erhalten Sie Zugang zu allen CME-Fortbildungen und Fachzeitschriften auf SpringerMedizin.de.

Literatur
1.
Zurück zum Zitat Nagata N, Dairaku M, Sueishi K, Tanaka K. Sclerosing hemangioma of the lung. An epithelial tumor composed of immunohistochemically heterogeneous cells. Am J Clin Pathol 1987;88:552-560.PubMed Nagata N, Dairaku M, Sueishi K, Tanaka K. Sclerosing hemangioma of the lung. An epithelial tumor composed of immunohistochemically heterogeneous cells. Am J Clin Pathol 1987;88:552-560.PubMed
2.
Zurück zum Zitat Liebow A, Hubell D. Sclerosing hemangioma (histiocytoma, xanthoma) of the lung. Cancer 1956;9:53–75.CrossRefPubMed Liebow A, Hubell D. Sclerosing hemangioma (histiocytoma, xanthoma) of the lung. Cancer 1956;9:53–75.CrossRefPubMed
3.
Zurück zum Zitat Devouassoux-Shisheboran M, Hayashi T, Linnoila RI, Koss MN, Travis WD. A clinicopathologic study of 100 cases of pulmonary sclerosing hemangioma with immunohistochemical studies: TTF-1 is expressed in both round and surface cells, suggesting an origin from primitive respiratory epithelium. Am J Surg Pathol 2000;24:906-6.CrossRefPubMed Devouassoux-Shisheboran M, Hayashi T, Linnoila RI, Koss MN, Travis WD. A clinicopathologic study of 100 cases of pulmonary sclerosing hemangioma with immunohistochemical studies: TTF-1 is expressed in both round and surface cells, suggesting an origin from primitive respiratory epithelium. Am J Surg Pathol 2000;24:906-6.CrossRefPubMed
4.
Zurück zum Zitat Iyoda A, Hiroshima K, Shiba M, et al. Clinicopathological analysis of pulmonary sclerosing hemangioma. Ann Thorac Surg 2004;78:1928-1.CrossRefPubMed Iyoda A, Hiroshima K, Shiba M, et al. Clinicopathological analysis of pulmonary sclerosing hemangioma. Ann Thorac Surg 2004;78:1928-1.CrossRefPubMed
5.
Zurück zum Zitat Hosaka N, Sasaki T, Adachi K, et al. Pulmonary sclerosing hemangioma associated with familial adenomatous polyposis. Hum Pathol 2004;35:764-.CrossRefPubMed Hosaka N, Sasaki T, Adachi K, et al. Pulmonary sclerosing hemangioma associated with familial adenomatous polyposis. Hum Pathol 2004;35:764-.CrossRefPubMed
6.
Zurück zum Zitat Camp ER, Hochwald SN, Liu C. FAP with concurrent duodenal adenomatous polyposis and carcinoid tumor. J Surg Oncol 2004;87:187-0.CrossRefPubMed Camp ER, Hochwald SN, Liu C. FAP with concurrent duodenal adenomatous polyposis and carcinoid tumor. J Surg Oncol 2004;87:187-0.CrossRefPubMed
7.
Zurück zum Zitat Nandakumar G, Morgan JA, Silverberg D, Steinhagen RM. Familial polyposis coli: clinical manifestations, evaluation, management and treatment. Mt Sinai J Med 2004;71:384-1.PubMed Nandakumar G, Morgan JA, Silverberg D, Steinhagen RM. Familial polyposis coli: clinical manifestations, evaluation, management and treatment. Mt Sinai J Med 2004;71:384-1.PubMed
8.
Zurück zum Zitat Hara M, Iida A, Tohyama J, et al. FDG-PET findings in sclerosing hemangioma of the lung: a case report. Radiat Med 2001;19:215-.PubMed Hara M, Iida A, Tohyama J, et al. FDG-PET findings in sclerosing hemangioma of the lung: a case report. Radiat Med 2001;19:215-.PubMed
9.
Zurück zum Zitat Katzenstein AL, Gmelich JT, Carrington CB. Sclerosing hemangioma of the lung: a clinicopathologic study of 51 cases. Am J Surg Pathol 1980;4:343-6.PubMedCrossRef Katzenstein AL, Gmelich JT, Carrington CB. Sclerosing hemangioma of the lung: a clinicopathologic study of 51 cases. Am J Surg Pathol 1980;4:343-6.PubMedCrossRef
10.
Zurück zum Zitat Noguchi M, Kodama T, Morinaga S, Shimosato Y, Saito T, Tsuboi E. Multiple sclerosing hemangiomas of the lung. Am J Surg Pathol 1986;10:429-5.CrossRefPubMed Noguchi M, Kodama T, Morinaga S, Shimosato Y, Saito T, Tsuboi E. Multiple sclerosing hemangiomas of the lung. Am J Surg Pathol 1986;10:429-5.CrossRefPubMed
11.
Zurück zum Zitat Niho S, Suzuki K, Yokose T, Kodama T, Nishiwaki Y, Esumi H. Monoclonality of both pale cells and cuboidal cells of sclerosing hemangioma of the lung. Am J Pathol 1998;152:1065-.PubMed Niho S, Suzuki K, Yokose T, Kodama T, Nishiwaki Y, Esumi H. Monoclonality of both pale cells and cuboidal cells of sclerosing hemangioma of the lung. Am J Pathol 1998;152:1065-.PubMed
12.
Zurück zum Zitat Kim GY, Kim J, Choi YS, Kim HJ, Ahn G, Han J. Sixteen cases of sclerosing hemangioma of the lung including unusual presentations. J Korean Med Sci 2004;19:352-.PubMed Kim GY, Kim J, Choi YS, Kim HJ, Ahn G, Han J. Sixteen cases of sclerosing hemangioma of the lung including unusual presentations. J Korean Med Sci 2004;19:352-.PubMed
13.
Zurück zum Zitat Lee S, Hong SW, Shin SJ, et al. Papillary thyroid carcinoma associated with familial adenomatous polyposis: molecular analysis of pathogenesis in a family and review of the literature. Endocr J 2004;51:317-3.CrossRefPubMed Lee S, Hong SW, Shin SJ, et al. Papillary thyroid carcinoma associated with familial adenomatous polyposis: molecular analysis of pathogenesis in a family and review of the literature. Endocr J 2004;51:317-3.CrossRefPubMed
14.
Zurück zum Zitat Tejpar S, Michils G, Denys H, et al. Analysis of Wnt/Beta catenin signalling in desmoid tumors. Acta Gastroenterol Belg 2005;68:5–9.PubMed Tejpar S, Michils G, Denys H, et al. Analysis of Wnt/Beta catenin signalling in desmoid tumors. Acta Gastroenterol Belg 2005;68:5–9.PubMed
15.
Zurück zum Zitat Gould MK, Maclean CC, Kuschner WG, Rydzak CE, Owens DK. Accuracy of positron emission tomography for diagnosis of pulmonary nodules and mass lesions: a meta-analysis. JAMA 2001;285:914-4.CrossRefPubMed Gould MK, Maclean CC, Kuschner WG, Rydzak CE, Owens DK. Accuracy of positron emission tomography for diagnosis of pulmonary nodules and mass lesions: a meta-analysis. JAMA 2001;285:914-4.CrossRefPubMed
16.
Zurück zum Zitat Tsuduki E, Kawana A, Takeda Y, et al. The usefulness of 11C-Choline-positron emission tomography in the diagnosis of sclerosing hemangioma of the lung-a case report [In Japanese]. Nihon Kokyuki Gakkai Zasshi 2002;40:402-.PubMed Tsuduki E, Kawana A, Takeda Y, et al. The usefulness of 11C-Choline-positron emission tomography in the diagnosis of sclerosing hemangioma of the lung-a case report [In Japanese]. Nihon Kokyuki Gakkai Zasshi 2002;40:402-.PubMed
Metadaten
Titel
Pulmonary Sclerosing Hemangioma Detected by Fluorodeoxyglucose Positron Emission Tomography in Familial Adenomatous Polyposis: Report of a Case
verfasst von
Daan B. de Koning, M.D.
Joost P. H. Drenth, M.D., Ph.D.
Wim J. G. Oyen, M.D., Ph.D.
Michiel Wagenaar, M.D., Ph.D.
Riena P. Aliredjo, M.D.
Fokko M. Nagengast, M.D., Ph.D.
Publikationsdatum
01.11.2007
Verlag
Springer-Verlag
Erschienen in
Diseases of the Colon & Rectum / Ausgabe 11/2007
Print ISSN: 0012-3706
Elektronische ISSN: 1530-0358
DOI
https://doi.org/10.1007/s10350-006-0862-1

Weitere Artikel der Ausgabe 11/2007

Diseases of the Colon & Rectum 11/2007 Zur Ausgabe

Selected Abstracts

Selected Abstracts

Neu im Fachgebiet Chirurgie

„Wir brauchen eine indikationsgerechte Adipositastherapie“

03.07.2024 Adipositas Nachrichten

Die Adipositas ist eine häufige Erkrankung, mit der sich Patienten in der Hausarztpraxis vorstellen. Für die Behandlung stehen Ernährungs- und Bewegungstherapie, medikamentöse Ansätze und Chirurgie zur Verfügung. Die Therapie muss indikationsgerecht erfolgen.

Fuchsbandwurm wütet in Leber von jungem Mann

03.07.2024 Echinokokkose Nachrichten

Mit leichten Oberbauchbeschwerden in die Klinik, mit auto-transplantierter Restleber wieder heraus: Bei einem jungen Mann mit Fuchsbandwurmbefall waren das Organ und er selbst nur durch eine drastische Maßnahme zu retten.

Die Ausbildung in Notfallchirurgie ist selbst ein Notfall

02.07.2024 Chirurgische Notfälle Nachrichten

Notfälle und ihre Versorgung gehören zu den chirurgischen Kernkompetenzen. Mit der Ausbildung hapert es jedoch offenbar, wie eine Umfrage unter Mitgliedern der Deutschen Gesellschaft für Allgemein- und Viszeralchirurgie (DGAV) vermuten lässt.

Sechs Monate E-Rezept – Erfolgsgeschichte mit Schattenseiten

IT für Ärzte Nachrichten

244 Millionen E-Rezepte sind in sechs Monaten eingelöst worden: Nach einem halben Jahr zieht die Betriebsgesellschaft gematik eine positive Zwischenbilanz. Doch noch sind nicht alle Akteure begeistert.

Update Chirurgie

Bestellen Sie unseren Fach-Newsletter und bleiben Sie gut informiert.

S3-Leitlinie „Diagnostik und Therapie des Karpaltunnelsyndroms“

Karpaltunnelsyndrom BDC Leitlinien Webinare
CME: 2 Punkte

Das Karpaltunnelsyndrom ist die häufigste Kompressionsneuropathie peripherer Nerven. Obwohl die Anamnese mit dem nächtlichen Einschlafen der Hand (Brachialgia parästhetica nocturna) sehr typisch ist, ist eine klinisch-neurologische Untersuchung und Elektroneurografie in manchen Fällen auch eine Neurosonografie erforderlich. Im Anfangsstadium sind konservative Maßnahmen (Handgelenksschiene, Ergotherapie) empfehlenswert. Bei nicht Ansprechen der konservativen Therapie oder Auftreten von neurologischen Ausfällen ist eine Dekompression des N. medianus am Karpaltunnel indiziert.

Prof. Dr. med. Gregor Antoniadis
Berufsverband der Deutschen Chirurgie e.V.

S2e-Leitlinie „Distale Radiusfraktur“

Radiusfraktur BDC Leitlinien Webinare
CME: 2 Punkte

Das Webinar beschäftigt sich mit Fragen und Antworten zu Diagnostik und Klassifikation sowie Möglichkeiten des Ausschlusses von Zusatzverletzungen. Die Referenten erläutern, welche Frakturen konservativ behandelt werden können und wie. Das Webinar beantwortet die Frage nach aktuellen operativen Therapiekonzepten: Welcher Zugang, welches Osteosynthesematerial? Auf was muss bei der Nachbehandlung der distalen Radiusfraktur geachtet werden?

PD Dr. med. Oliver Pieske
Dr. med. Benjamin Meyknecht
Berufsverband der Deutschen Chirurgie e.V.

S1-Leitlinie „Empfehlungen zur Therapie der akuten Appendizitis bei Erwachsenen“

Appendizitis BDC Leitlinien Webinare
CME: 2 Punkte

Inhalte des Webinars zur S1-Leitlinie „Empfehlungen zur Therapie der akuten Appendizitis bei Erwachsenen“ sind die Darstellung des Projektes und des Erstellungswegs zur S1-Leitlinie, die Erläuterung der klinischen Relevanz der Klassifikation EAES 2015, die wissenschaftliche Begründung der wichtigsten Empfehlungen und die Darstellung stadiengerechter Therapieoptionen.

Dr. med. Mihailo Andric
Berufsverband der Deutschen Chirurgie e.V.