Case Report
Rare Founding in Peripheral Nerve Surgery: An Unicentric Castleman Disease Presenting as Median Nerve Tumor: Case Report
Anne Carolus1*, Roland Schroers2, Iris Tischoff3, Kirsten Schmieder1 and Christopher Brenke1
1Department of Neurosurgery, University Hospital Knappschaftskrankenhaus Bochum, Ruhr-University Bochum, Germany
2Department of Hematology and Oncology, University Hospital Knappschaftskrankenhaus Bochum, Ruhr-University Bochum, Germany
3Department of Pathology, University Hospital Bergmannsheil Bochum, Ruhr-University Bochum, Germany
*Corresponding author: Anne Elisabeth Carolus, Department of Neurosurgery, University Hospital Knappschaftskrankenhaus Bochum, In der Schornau 23-35, 44892 Bochum, Germany
Published: 18 Jul, 2018
Cite this article as: Carolus A, Schroers R, Tischoff
I, Schmieder K, Brenke C. Rare
Founding in Peripheral Nerve Surgery:
An Unicentric Castleman Disease
Presenting as Median Nerve Tumor:
Case Report. Clin Surg. 2018; 3: 2035.
Abstract
A 51 year old man presented with progressive swelling in the upper arm. MRI revealed a solitary
mass extending from the median nerve. Intraoperative finding was a tumor extending within the
nerve in its proximal fibers. The histological result showed typical criteria of a Castleman disease.
Postoperative staging did not reveal any involvement of other organs. In conclusion, an unicentric
Castleman disease could be diagnosed. To our knowledge such an entity appearing at a peripheral
nerve has not been described in literature so far.
Keywords: Peripheral nerve tumor; Unicentric Castleman disease; Contrast enhancing mass; Infiltrative growth; Surgery
Introduction
Tumors of the peripheral nerve system represent a broad and inhomogeneous group. They can be subdivided into two categories according to their intrinsic or extrinsic growth: Peripheral nerve sheath tumors (PNST) and Peripheral non-neural sheath tumors (PNNST). Each entity includes benign and malignant tumors [1,2] (Table 1). Diseases arising from lymphoid cells which are associated to the peripheral nerve system are extremely rare. A few cases of primary lymphoma [3], neurolymphomatosis [4] and lymph node metastasis [5] have been described previously. We present another entity of lymphoid tissue found in a peripheral nerve. It is known as Castleman diseaese (CM) or angiofollicular lymph node hyperplasia [6].
Case Presentation
A 51 year old man presented to our outpatient department with an increasing swelling in the
right distal upper arm. He reported about local pain without radiation. There was no loss of sensory
or motor function. The patient´s medical history was empty, no previous infections, surgeries or
other diseases. The mass in the arm presented solid and relocatable.
MRI of the upper arm showed a spindle-shaped homogeneously contrast enhancing mass. Its
diameter was about 11 cm × 4 cm. It was located in the course of the median nerve respectively
seemed to originate from part of its fibers (Figure 1a and 1b). The primary diagnosis from the
radiologist was schwannoma.
Surgical extirpation was indicated and performed. In its middle part the exposed tumor had a
smooth capsule which was opened (Figure 2a). In its equator the surface had a good boundary to
the surrounding tissue (Figure 2b). It did not extend to the muscles or tendons. In its distal and
especially in its proximal ending the tumor showed a more infiltrative growth (Figure 2c). A feeding
fascicle could be identified and was cut after ensuring by electric stimulation that it had no motor
function. But with the intention to set no damage at the main nerve trunk approximately twenty
percent residual tumor was left (Figure 2d).
The postoperative course was uneventful. The patient suffered a light hypoesthesia in the
forearm. This did not match to the supply territory of the median nerve which is the palmar hand. It
rather corresponded to another skin nerve, possibly damaged by the approach. The local upper arm
The final histological examination of the tumor showed typical
criteria of the Castleman disease with an effaced architecture of
a lymph node with regressed germinal centers and typical high
endothelial venules (Figure 3a and 3b). Immunohistochemistry
demonstrated regressed atrophic germinal centers (Figure 3c)
and aberrant network of follicular dendritic cells (Figure 3d). The
combination of these features ensured the diagnosis.
To exclude a multicentric disease the patient was admitted to the
internal medical department. Entire virus tests including HIV were
negative. A bone marrow biopsy showed a normal hematopoiesis
without evidence for an infiltration by pathologic cells. A staging
PET-CT showed no further organ manifestations. An unicentric form
was approved in synopsis of all findings. In regard to the tumor rest
and the curative approach of a unicentric M. Castleman the patient
finally underwent a selective radiation of the upper arm [7]. In a 6
month follow-up the patient reported no nerve related problems or
restrictions in everyday life.
Table 1
Figure 1
Figure 1
MRI of the upper arm showing a spindle shaped contrast enhancing
mass in the median nerve course. a) Coronar view. b) Axial view.
Figure 2
Figure 2
Intraoperative views of tumour dissection and removal. a) Opening
the capsule of the tumour and electric stimulation of fascicle-like structures.
b) Exposed tumour with a smooth surface in its middle part. c) Exposed
tumour, infiltrative growing in its proximal ending (arrow). d) Tumorbed after
removal of the median part, rest tumour embedding a nerve branch (arrow).
Figure 3
Figure 3
Histological stain sections. a) Immunhistochemical staining for
T-cells within the interfollicular zone. b) In 10x HE stain section areas with high
endothelial venules are demonstrated. c) Immunhistochemical expression of
CD20 demonstrating regressed germinal centers. d) Immunhistochemical
expression of CD23 shows extended network of follicular dendritic cells.
Discussion
Retrospectively one can discuss what would have been the best
neurosurgical management for our patient. Would he have benefit
from a more radical tumor resection? Or contrariwise, would a
frozen section have been helpful in such a case and a biopsy been the
consequence? Should staging have had priority?
According to the literature unicentric Castleman disease has a
good prognosis concerning the overall survival if treated by either
complete surgical removal or a combination of surgery and radiation
[7]. In this respect we think that an extirpation should out value a sole
biopsy in such a case. But peripheral nerve function preservation has
priority, especially if a major nerve of the upper or lower extremity
is involved.
The histological dignity and growth behavior of a peripheral nerve
tumor is irremissible information in this benefit-risk-assessment
between complete removal on the one side and preservation of
sensorimotor function on the other side. The features from Castleman
disease distinguish clearly from the characteristic of tumors
originating from the nerve sheath which is neoplastic proliferation of
Schwann cell differentiation [8]. Nevertheless there is an increasing
literature of MPNST mimics. Concerning the MRI finding and
the infiltrative growth our case represents not a microscopic, but a
macroscopic differential diagnosis of MPNST.
In our opinion the fact of its rareness, the challenging MRI, the
malignant histological features and the necessity of interdisciplinary
treatment justify that unicentric Castleman disease is registered in the
itemization of peripheral nerve-associated malignant neoplasms.
Conclusion
Castleman disease is a group of lymph proliferative disorders including special histological characteristics of lymph node. It is rare and may affect any body region. A manifestation at a solitary peripheral nerve has not been described so far. We present a case in which an unicentric Castleman disease mimicked a MPNST if dealing with the radiological and neurosurgical features. The pathological examination resolved the challenging case. An interdisciplinary treatment approach seems to be essential.
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