renal cell carcinoma with metastasis to the · pdf filerenal cell carcinoma with metastasis to...

1
Renal Cell Carcinoma with Metastasis to the Clivus 1 Amit A. Patel, MD; 1 Arjuna B. Kuperan, MD; 1 Chirag R. Patel, MD; 2 Leroy R. Sharer, MD; 3,4 James K. Liu, MD; 1,4 Jean A. Eloy, MD Introduction Renal cell carcinoma is a uncommon malignancy which accounts for approximately 3% of all adult malignancies diagnosed per year. It has been known to metastasize to unusual distant locations. We report a case of a renal cell carcinoma with metastasis to the clivus causing cranial nerve neuropathy. Case Report A 59 year-old female with a medical history notable for hypertension and mental retardation presented to an outside hospital with progressive headaches, acute onset left ptosis, diplopia and left V2 hypoesthesia. CT and MRI scans revealed a large clival lesion with skull base destruction with extension into the sphenoid sinuses and nasopharynx. There was encasement of the left carotid artery with narrowing of the lumen of the artery. Endoscopic endonasal extended approach of this anterior skull base tumor was successfully performed with near complete resection. Intraoperative frozen section was concerning for sarcoma, however, final pathology showed renal cell carcinoma. Postoperatively the patient had complete resolution of her cranial nerve neuropathies. Follow-up CT of the abdomen revealed a large left renal mass. The patient elected to undergo palliative radiation treatment. References 1. Rini BI, Ward JF, Vogelzang NJ. Ch 128 Primary Neoplasms of the Kidney and Renal Pelvis. Diseases of the Kidney & Urinary Tract, Ed. Schrier RW, 2007. 2. Alpers CE. Ch 20 The Kidney. Pathologic Basis of Disease, 8 th edition, Ed. Kumar V, Abbas AK, Fausto N, Aster JC, 2009 3. Traynelis VC, Menezes AH. Chapter 4 Differential Diagnosis of Clival Tumors. Chordomas and Chondrosarcomas of the Skull Base and Spine, Ed. Harsh GR, 2003 4. Sagoh M, Kodaki K, Ichikizaki K, Murakami K, Oizumi T, Kawase T, Toya S, Shiga H. Skull base metastasis from renal cell carcinoma presenting as abducens nerve paresis: report of two cases. No Shinkei Geka. 1996 Sep;24(9):829-33. 5. Fumino M, Matsuura H, Hayashi N, Arima K, Yanagawa M, Kawamura J. A case of renal cell carcinoma with metastasis in clivus presenting as diplopia. Hinyokika Kiyo. 1998 May;44(5):319-21. 6. Pallini R, Sabatino G, Doglietto F, Lauretti L, Fernandez E, Maira G. Clivus metastases: report of seven patients and literature review. Acta Neurochir 2009 Apr;151(4):291-6 Discussion Renal cell carcinoma accounts for 3% of all malignancies diagnosed per year. Clear cell renal carcinoma accounts for 85- 90% of all renal carcinoma diagnosed. It typically affects middle aged to elderly men with a history of smoking. 1 In the most classic presentation, renal carcinoma causes hematuria, flank pain, and a palpable mass. However, renal carcinoma is notorious for its protean manifestations including multiple paraneoplastic syndromes and unusual locations of metastasis. 10-50% of patients present with metastasis. There are case reports of metastasis to the heart, thyroid, and even skeletal muscle. 1 Clear cell carcinomas most likely arise from proximal tubular epithelium, and usually occur as solitary unilateral lesions. Histologically, the show a growth pattern that varies from solid to trabecular or tubular. The tumor cells have a rounded or polygonal shape and abundant clear or granular cytoplasm, which contains glycogen and lipids along with delicate branching vasculature. Most tumors are well differentiated, but some show marked nuclear atypia with formation of bizarre nuclei and giant cells. 2 The most common type of clival tumor is a chordoma. Other common possibilities include chondromas, meningiomas, and chondrosarcomas. Nasopharyngeal malignancies can invade posteriorly into the clivus. Clival tumors usually present with pain, cranial nerve neuropathies, and occasionally, cerebellar dysfunction. 3 On CT scan, malignant clival lesions show bony destruction and can encase vital structures such as the carotid artery and cranial nerves III, IV, V and VI. MRI appearance is dependent on the pathology of the lesions. 3 There have been three case reports of renal carcinoma presenting with clival metastasis, all in the Japanese literature. Each case presented with cranial nerve neuropathies. In addition to renal carcinoma, metastases from other malignancies to the clivus have been reported. 4,5 Pallini et. al reported a case series of 46 patients who underwent surgery for clival tumors. Seven of these tumors were diagnosed as metastatic lesions on final pathology, which included lung adenocarcinoma (two patients) prostate carcinoma (two patients), melanoma, hepatocarcinoma, and squamous cell carcinoma of the lung (one patient each). 6 In this case, we report a rare case of clear cell renal carcinoma presenting as a clival mass with acute onset cranial nerve neuropathy. Previous reports on similar casea have discussed this entity but we call attention to the importance of recognizing this pathology in the contest of endoscopic skull base surgery, in particular, the role for endoscopic biopsy and decompression of malignant masses causing cranial nerve symptoms. Abstract Background: Renal cell carcinoma is an uncommon malignancy which accounts for approximately 3% of all adult malignancies. Metastasis from renal cell carcinoma generally occurs in locoregional lymph nodes, lungs, liver, bones, and the brain. Other sites of spread have been described, but are increasingly infrequent. We describe a rare metastasis of renal cell carcinoma to the clivus causing multiple cranial nerve neuropathies treated by purely endoscopic resection with complete resolution of symptoms. This is the first report of clival metastasis due to renal cell carcinoma in the English literature. Methods: Case report and current literature review. Results: A 59 year old female presented to our institution from a long term care facility with headaches and acute onset cranial nerve neuropathies. Imaging of the head revealed a large clival based lesion with extension into the sphenoid sinus and encasement of the carotid artery. She underwent a transnasal endoscopic decompression of the large mass. Intraoperative pathology was positive for a tumor of mesenchymal origin. Final pathology revealed metastatic renal cell carcinoma. Conclusion: This case aims to increase awareness of this infrequently encountered disease of the skull base/clivus and offers insight into the diagnosis and treatment of this rare entity. Figure 2. Histopathologic slides demonstrating architecture consistent with clear cell renal carcinoma. (A) Clear cell carcinoma with cellular and nuclear pleomorphism, also a nested appearance, H&E, 10X., and (B) at higher magnification, 25X (C) Clear cell carcinoma involving bone. H&E, 10X (D) : Immunohistochemistry for PAX 8: Tumor cell nuclei are positive (considered to be a marker for renal cell carcinoma). DAB with light hematoxylin counterstain, 10X. Figure 1. (A) Preoperative CT scan with contrast, axial image demonstrating an enhancing clival lesion encasing the carotid artery. (B) Reformatted bone window of Figure 1A demonstrating bony destruction of the skull base. (C) Coronal CT scan image again demonstrating destruction of the skull base and extension into the nasopharynx. (D) Preoperative axial MRI scan image, T2 FLAIR sequence demonstrating a heterogeneous lesion of the clivus. Contact Information: Jean Anderson Eloy, MD, FACS [email protected] 1 Department of Otolaryngology Head & Neck Surgery, University of Medicine and Dentistry of New Jersey New Jersey Medical School, Newark, NJ 2 Department of Pathology, University of Medicine and Dentistry of New Jersey New Jersey Medical School, Newark, NJ 3 Department of Neurological Surgery, University of Medicine and Dentistry of New Jersey New Jersey Medical School, Newark, NJ 4 Center for Skull Base and Pituitary Surgery, Neurological Institute of New Jersey, University of Medicine and Dentistry of New Jersey New Jersey Medical School, Newark, NJ A B D A B C D C

Upload: trinhkhue

Post on 09-Mar-2018

215 views

Category:

Documents


1 download

TRANSCRIPT

Page 1: Renal Cell Carcinoma with Metastasis to the · PDF fileRenal Cell Carcinoma with Metastasis to the Clivus 1Amit A. Patel, MD; 1Arjuna B. Kuperan, MD; ... Yanagawa M, Kawamura J. A

Renal Cell Carcinoma with Metastasis to the Clivus 1Amit A. Patel, MD; 1Arjuna B. Kuperan, MD; 1Chirag R. Patel, MD; 2Leroy R. Sharer, MD; 3,4James K. Liu, MD; 1,4Jean A. Eloy, MD

Introduction

Renal cell carcinoma is a uncommon malignancy

which accounts for approximately 3% of all adult

malignancies diagnosed per year. It has been

known to metastasize to unusual distant locations.

We report a case of a renal cell carcinoma with

metastasis to the clivus causing cranial nerve

neuropathy.

Case Report A 59 year-old female with a medical history

notable for hypertension and mental retardation

presented to an outside hospital with progressive

headaches, acute onset left ptosis, diplopia and left

V2 hypoesthesia. CT and MRI scans revealed a

large clival lesion with skull base destruction with

extension into the sphenoid sinuses and

nasopharynx. There was encasement of the left

carotid artery with narrowing of the lumen of the

artery. Endoscopic endonasal extended approach

of this anterior skull base tumor was successfully

performed with near complete

resection. Intraoperative frozen section was

concerning for sarcoma, however, final pathology

showed renal cell carcinoma. Postoperatively the

patient had complete resolution of her cranial nerve

neuropathies. Follow-up CT of the abdomen

revealed a large left renal mass. The patient

elected to undergo palliative radiation treatment.

References 1. Rini BI, Ward JF, Vogelzang NJ. Ch 128 – Primary Neoplasms of the Kidney and

Renal Pelvis. Diseases of the Kidney & Urinary Tract, Ed. Schrier RW, 2007.

2. Alpers CE. Ch 20 – The Kidney. Pathologic Basis of Disease, 8th edition, Ed. Kumar

V, Abbas AK, Fausto N, Aster JC, 2009

3. Traynelis VC, Menezes AH. Chapter 4 – Differential Diagnosis of Clival Tumors.

Chordomas and Chondrosarcomas of the Skull Base and Spine, Ed. Harsh GR,

2003

4. Sagoh M, Kodaki K, Ichikizaki K, Murakami K, Oizumi T, Kawase T, Toya S, Shiga H.

Skull base metastasis from renal cell carcinoma presenting as abducens nerve

paresis: report of two cases. No Shinkei Geka. 1996 Sep;24(9):829-33.

5. Fumino M, Matsuura H, Hayashi N, Arima K, Yanagawa M, Kawamura J. A case of

renal cell carcinoma with metastasis in clivus presenting as diplopia. Hinyokika

Kiyo. 1998 May;44(5):319-21.

6. Pallini R, Sabatino G, Doglietto F, Lauretti L, Fernandez E, Maira G. Clivus

metastases: report of seven patients and literature review. Acta Neurochir 2009

Apr;151(4):291-6

Discussion

• Renal cell carcinoma accounts for 3% of all malignancies

diagnosed per year. Clear cell renal carcinoma accounts for 85-

90% of all renal carcinoma diagnosed. It typically affects middle

aged to elderly men with a history of smoking.1

• In the most classic presentation, renal carcinoma causes

hematuria, flank pain, and a palpable mass. However, renal

carcinoma is notorious for its protean manifestations including

multiple paraneoplastic syndromes and unusual locations of

metastasis. 10-50% of patients present with metastasis. There

are case reports of metastasis to the heart, thyroid, and even

skeletal muscle.1

• Clear cell carcinomas most likely arise from proximal tubular

epithelium, and usually occur as solitary unilateral lesions.

Histologically, the show a growth pattern that varies from solid to

trabecular or tubular. The tumor cells have a rounded or polygonal

shape and abundant clear or granular cytoplasm, which contains

glycogen and lipids along with delicate branching vasculature.

Most tumors are well differentiated, but some show marked

nuclear atypia with formation of bizarre nuclei and giant cells.2

• The most common type of clival tumor is a chordoma. Other

common possibilities include chondromas, meningiomas, and

chondrosarcomas. Nasopharyngeal malignancies can invade

posteriorly into the clivus. Clival tumors usually present with pain,

cranial nerve neuropathies, and occasionally, cerebellar

dysfunction.3

• On CT scan, malignant clival lesions show bony destruction

and can encase vital structures such as the carotid artery and

cranial nerves III, IV, V and VI. MRI appearance is dependent on

the pathology of the lesions.3

• There have been three case reports of renal carcinoma

presenting with clival metastasis, all in the Japanese literature.

Each case presented with cranial nerve neuropathies. In addition

to renal carcinoma, metastases from other malignancies to the

clivus have been reported.4,5 Pallini et. al reported a case series

of 46 patients who underwent surgery for clival tumors. Seven of

these tumors were diagnosed as metastatic lesions on final

pathology, which included lung adenocarcinoma (two patients)

prostate carcinoma (two patients), melanoma, hepatocarcinoma,

and squamous cell carcinoma of the lung (one patient each).6

• In this case, we report a rare case of clear cell renal

carcinoma presenting as a clival mass with acute onset cranial

nerve neuropathy. Previous reports on similar casea have

discussed this entity but we call attention to the importance of

recognizing this pathology in the contest of endoscopic skull base

surgery, in particular, the role for endoscopic biopsy and

decompression of malignant masses causing cranial nerve

symptoms.

Abstract

Background: Renal cell carcinoma is an

uncommon malignancy which accounts for

approximately 3% of all adult

malignancies. Metastasis from renal cell

carcinoma generally occurs in locoregional lymph

nodes, lungs, liver, bones, and the brain. Other

sites of spread have been described, but are

increasingly infrequent. We describe a rare

metastasis of renal cell carcinoma to the clivus

causing multiple cranial nerve neuropathies treated

by purely endoscopic resection with complete

resolution of symptoms. This is the first report of

clival metastasis due to renal cell carcinoma in the

English literature.

Methods: Case report and current literature

review.

Results: A 59 year old female presented to our

institution from a long term care facility with

headaches and acute onset cranial nerve

neuropathies. Imaging of the head revealed a

large clival based lesion with extension into the

sphenoid sinus and encasement of the carotid

artery. She underwent a transnasal endoscopic

decompression of the large mass. Intraoperative

pathology was positive for a tumor of

mesenchymal origin. Final pathology revealed

metastatic renal cell carcinoma.

Conclusion: This case aims to increase

awareness of this infrequently encountered

disease of the skull base/clivus and offers insight

into the diagnosis and treatment of this rare

entity.

Figure 2. Histopathologic slides demonstrating architecture consistent with clear cell renal carcinoma. (A) Clear cell carcinoma with cellular and nuclear

pleomorphism, also a nested appearance, H&E, 10X., and (B) at higher magnification, 25X (C) Clear cell carcinoma involving bone. H&E, 10X (D) :

Immunohistochemistry for PAX 8: Tumor cell nuclei are positive (considered to be a marker for renal cell carcinoma). DAB with light hematoxylin

counterstain, 10X.

Figure 1. (A) Preoperative CT scan with contrast, axial image demonstrating an enhancing clival lesion encasing the carotid

artery. (B) Reformatted bone window of Figure 1A demonstrating bony destruction of the skull base. (C) Coronal CT scan

image again demonstrating destruction of the skull base and extension into the nasopharynx. (D) Preoperative axial MRI scan

image, T2 FLAIR sequence demonstrating a heterogeneous lesion of the clivus.

Contact Information:

Jean Anderson Eloy, MD, FACS

[email protected]

1Department of Otolaryngology – Head & Neck Surgery, University of Medicine and Dentistry of New Jersey – New Jersey

Medical School, Newark, NJ 2Department of Pathology, University of Medicine and Dentistry of New Jersey – New Jersey Medical School, Newark, NJ 3Department of Neurological Surgery, University of Medicine and Dentistry of New Jersey – New Jersey Medical School,

Newark, NJ 4Center for Skull Base and Pituitary Surgery, Neurological Institute of New Jersey, University of Medicine and Dentistry of

New Jersey – New Jersey Medical School, Newark, NJ

A B

D

A B

C D

C