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               Path Quiz Case-6  Diagnosis: Ancient Schwannoma

                                Schwannoma               

                                                       Dr Sampurna Roy MD

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                    Path Quiz Case 6: Case history and histopathology images:

January 2014

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Reactive and hamartomatous lesions:

Traumatic neuroma

Morton's neuroma

Digital Pacinian neuroma

Nerve Sheath Ganglion

Fibrolipomatous hamartoma of nerve

Benign tumours:

Solitary circumscribed neuroma

Schwannoma 
and variants                          

Neurofibroma and variants 

Perineurioma   

Dermal nerve sheath myxoma

Cellular neurothekeoma

Granular cell tumour

Malignant tumour:

Malignant peripheral nerve sheath tumour
  

Neuroendocrine Carcinoma:

Merkel cell   carcinoma

Malignant primitive neuroectodermal tumour   

Miscellaneous neuroectodermal tumours presenting in soft tissue

Subcutaneous Myxopapillary Ependymoma  

Heterotopic Glial Nodule

Heterotopic Meningeal Lesions

Abstracts:

The pathobiologic spectrum of Schwannomas. Histol Histopathol. 2003 Jul;18 (3): 925-34

Perineurial cells and nerve axons in gastrointestinal schwannomas: a similarity with neurofibromas. An immunohistochemical study of eight cases.
Cesk Patol. 2004 Oct;40(4):150-3

Epithelioid Schwannoma:

Benign cutaneous epithelioid Schwannoma: case report and review of the literature.Am J Dermatopathol. 2005 Feb;27(1):45-7.

Benign Epithelioid Peripheral Nerve Sheath Tumors of the Soft Tissues: Clinicopathologic Spectrum of 33 Cases. Am J Surg Pathol. 2005 Jan;29(1):39-51.

Expanding the Spectrum of Malignant Change in Schwannomas Epithelioid Malignant Change, Epithelioid Malignant Peripheral Nerve Sheath Tumor, and Epithelioid Angiosarcoma: A Study of 17 Cases Am J Surg Pathol 2001;25:13-25.

Cutaneous epithelioid schwannomas: a rare variant of a benign peripheral nerve sheath tumor.J Cutan Pathol. 1998 Jan;25(1):50-5

Glandular Schwannoma:

Benign glandular schwannoma and Recklinghausen disease. Report of a case. Ann Pathol 1992; 12 (2) : 114-120.

Benign glandular schwannoma. Am J Clin Pathol. 1993 Aug;100(2):167-70.

Benign glandular schwannoma.Br J Dermatol. 2001 Nov;145(5):834-7

Benign glandular schwannoma. Arch Pathol Lab Med. 1992;116(2):192-5

Schwannoma Perineuroma-

Hybrid peripheral nerve sheath tumors: Schwannoma-perineurioma and neurofibroma-perineurioma. A report of three cases in extradigital locations. Ann Diagn Pathol. 2005 Feb;9(1):16-23

A benign neoplasm with histopathological features of both schwannoma and retiform perineurioma (benign schwannoma-perineurioma): a report of six cases of a distinctive soft tissue tumor with a predilection for the fingers.Virchows Arch. 2004 Oct;445(4):347-53. Epub 2004 Aug 20

Nerve sheath tumours with hybrid features of neurofibroma and schwannoma: a conceptual challenge.Histopathology. 1998 May;32(5):405-10

 

Schwannoma are slow growing benign  nerve sheath tumours.

These are usually solitary lesions. Multiple lesions are rare and may occur in the following clinical settings: -

i)  multiple localized tumours.

ii)  In association with neurofibroma in von Recklinghausen's disease.

iii) In schwannomatosis,  a non hereditary disease characterized by multiple subcutaneous  and intradermal schwannomas together with

tumours of internal organs.

Age: Common in patients  between the ages of 20 and 50. 

Site: These are usually located on the head and neck and flexor surface of upper and lower extremities.

The spinal roots, cervical, sympathetic, vagus and ulnar nerves are commonly  involved.

The deeply located  lesions are situated  in the retroperitoneum and the posterior mediastinum.

Visit: Pancreatic Schwannoma ; Neurogenic Tumours of the Lung

Gross: The tumour is surrounded by true capsule consisting of the epineurium.

Tumour arising in a small nerve resemble a neurofibroma and often  obliterate the nerve of origin.

Tumours arising in larger nerves present as  eccentric masses.

Cut surface reveals smooth, glistening , grey-white appearance.

There may be  cystic areas with areas of hemorrhage and calcification.

Microscopic features:   Schwannomas are usually solitary, well circumscribed, encapsulated lesions, mostly confined to the subcutis.

Multinodular lesions may be present in the dermis, this is known as 'plexiform neurilemmoma'.

The characteristic features include presence of alternating  Antoni A and Antoni B areas

Antoni A area is composed  of spindle shaped Schwann cells arranged in interlacing fascicles.

There may be nuclear palisading. In between two compact rows of well aligned nuclei , the  cell processes form eosinophilic Verocay bodies.

Mitotic figures may be present (usually less than 5 per 10 high power field).

Antoni  B area consists of loose meshwork of gelatinous and microcystic tissue. 

Large, irregularly spaced, thick walled blood vessels  are noted in Antoni B area. These may contain thrombus material in the lumina.

Immunohistochemistry:  S100 is strongly expressed by most cells of schwannoma.The cells also express Vimentin and myelin basic protein.

Differential diagnosis:  Neurofibroma Palisaded encapsulated neuroma (more number of axons and Schwann cells in interlacing fascicles together with cleft like spaces.;   Cutaneous leiomyoma  (smooth muscle actin and desmin positive) ;    Palisaded myofibroblastoma ( involve lymph nodes, contain fibroblastic and myofibroblastic cells and are S100 protein negative).   

Ancient Schwannoma :  Image Link1 ; Image Link2 ; Image Link3 ; Image Link4 ; Image Link5

This is a variant of Schwannoma displaying prominent degenerative changes.

The degenerative changes include cyst formation, calcification, hemorrhage and hyalinization.

Macroscopically these are  large tumours which are usually deeply located (eg.  retroperitoneum).

There is prominent cellular atypia  characterized by hyperchromatic nuclei and coarse clumping of chromatin.

These changes are purely degenerative.

No mitotic figures are identified. These tumours behave as ordinary schwannomas.

Abstracts:

Degenerative (ancient) changes in benign cutaneous schwannoma. A light  microscopic, histochemical and immunohistochemical study.

Fine needle aspiration cytology of ' ancient ' schwannoma.

Degenerative changes in parapharyngeal schwannoma ancient schwannoma. Case report and review of literature.

Malignant peripheral nerve sheath tumour arising in benign ancient schwannoma : a case reprt with an immunohistochemical study.

Cellular Schwannoma:  Image

This variant  is usually found in the deeper tissues (retroperitoneum or mediastinum). 

Histologically ,there are compact spindle shaped cells arranged in a fascicular or whorled growth pattern.

Verocay bodies and Antoni B areas are  not conspicuous. Mitotic figures are present (more that 10 per10 high power fields).

The thick fibrous capsule around the tumour  shows a dense lymphocytic infiltrate.

Differential diagnosis include low grade malignant peripheral nerve seath tumour and leiomyosarcoma.

Immunohistochemistry reveals strong and diffuse positivity with S100 protein. Desmin is negative.

ABSTRACTS:

Cellular schwannoma. A clinicopathologic, DNA flow cytometric, and proliferation marker study of 70 patients.Cancer. 1995 Mar 1;75(5):1109-19

Cellular schwannoma: a distinct pseudosarcomatous entity.Histopathology. 1987 Jan;11(1):21-35.

Melanotic Schwannoma: 

This rare variant usually occurs in middle aged adults and  commonly arises from  the posterior spinal nerve roots.

Melanotic schwannomas are well circumscribed, partly encapsulated lesions characterized by polygonal and vesicular cells with grooved nuclei.

These cells contain abundant melanin pigment.

In psammomatous melanotic schwannoma , varying numbers of psammoma bodies are present.

Some of these cases are associated with Carney's complex (myxoma, spotty pigmentation and endocrinopathy).

Malignant melanotic schwannoma usually  arises from the sympathetic chain and is characterized by brisk mitosis and prominent nucleoli.

Immunohistochemistry reveals that tumour cells stain positively for S100, HMB45, MART-1, synaptophysin and vimentin.

 
Abstracts:

Cutaneous psammomatous melanotic schwannoma: non-recurrence with surgical excision. Am J Clin Dermatol. 2003;4(11):799-802.

Multiple melanotic schwannoma. Ann Diagn Pathol. 2003 Aug;7(4):254-8

Melanotic schwannoma, a tumor with a unpredictable prognosis: case report and review of the literature.Neurochirurgie. 2003 Mar;49(1):31-8

                                                  

Plexiform Schwannoma:

Neuroblastoma - Like Schwannoma :

 Image Link1 ; Image Link2

Benign schwannomas may contain rosette-like structures mimicking neuroblastoma/PNET.

Tumour cells are positive for S100 protein and negative for neuronal markers synaptophysins and neurofilament protein.

MIC2 is negative (rules out PNET type differentiation). Individual cells were surrounded by type IV collagen.

 This is a feature characteristically seen in schwannian but not in neuronal tumours.

Abstracts:

Neuroblastoma-like neurilemoma. Am J Surg Pathol. 1994 Mar;18(3):266-73

Schwannoma with Neuroblastoma-Like Rosettes: An Unusual Morphologic Variant. Am J Dermatopathol. 2005 Jun;27(3):243-246

Neuroblastoma-like schwannoma: a case report and review of the literature.Am J Dermatopathol 2003 Feb;25(1):32-4

Schwannoma with features mimicking neuroblastoma: report of two cases with immunohistochemical and ultrastructural findings.J Clin Pathol. 1998 Nov;51(11):842-5 

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