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Cases Journal

Open Access

Case Report

Unusual histological presentation in neurofibromas: Two case

reports

Deepti Joshi1 , Nitin Gangane*1 , Sanjeev Kishore2 and Sunita Vagha3

Address: 1 Department of Pathology, Mahatma Gandhi Institute of Medical Sciences, Sevagram, Maharashtra, India, 2 Department of Pathology,

Himalayan Institute of Medical Sciences, Dehradun, Uttaranchal, India and 3 Department of Pathology, Jawaharlal Nehru Medical College,

Sawangi Meghe Maharashtra, India

Email: Deepti Joshi - deeptim75@rediffmail.com; Nitin Gangane* - nitingangane@rediffmail.com; Sanjeev Kishore - rksent5@gmail.com;

Sunita Vagha - sunitavagha@rediffmail.com.com

* Corresponding author

Abstract

Various histological variants of neurofibroma have been described. The objective of this paper is to

discuss the unusual histological findings seen in two cases of neurofibromas associated with

neurofibromatosis type 1 Both cases presented with multiple subcutaneous nodules. Surgical

excision of the largest nodule was done in both the cases. Histological examination of case no.1

revealed a benign tumor of the peripheral nerve sheath, of neurofibroma type with presence of

mucus producing glands. The epithelial component was benign in this case. The second case

showed presence of rosettes in between areas of typical neurofibroma.

Background

Neurofibromatosis-Type1 (von Reckling hausen's dis-

ease) presents with various pathological manifestations

like histological abnormalities of epidermis (café au lait

spots), iris (Lisch nodules), skeleton (malformations),

blood vessels (mesodermal vascular dysplasias, brain

(glial tumors and hamartomas), intestine (endocrine and

gastrointestinal stromal tumors) and the peripheral nerve

sheath (neurofibromas and malignant peripheral nerve

sheath tumors) [1]. Neurofibroma is a benign peripheral

nerve sheath tumor with distinctive histological features.

Various distinct histological variants of neurofibroma

have been described. We hereby report the unusual histo-

logical presentation of neurofibroma seen in two patients

with NF Type 1.

Case presentation

Case number 1

A 35 year old male patient presented with multiple, nod-

ular swellings over back (in the region of thoracic spine)

and thigh. Freckles were noted in axillary region and

groin. Patient's mother also had nodular swellings though

none were biopsied. Swelling over thigh was excised and

submitted for histopathology. Gross examination

revealed presence of a 4 × 2.5 × 1 cm grey white tumor

which was mucoid in consistency.

Microscopic examination showed presence of tumor con-

sisting of spindle cells having slender, curved, often wavy

nuclei embedded in a collagenous stroma. Cytoplasm of

these cells was indistinct and nuclear pleomorphism or

mitotic figures were not present. These cells were also seen

arranged in interlacing bundles but no nuclear palisading

or Antoni A/B areas were demonstrated. Tumor also

showed presence of mucus producing glands lined by a

Published: 29 September 2008

Cases Journal 2008, 1 :188 doi:10.1186/1757-1626-1-188

Received: 26 August 2008

Accepted: 29 September 2008

This article is available from: http://www.casesjournal.com/content/1/1/188

© 2008 Joshi et al; licensee BioMed Central Ltd.

This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0),

which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Cases Journal 2008, 1 :188 http://www.casesjournal.com/content/1/1/188

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single layer of benign tall columnar epithelial cells with

basally located nuclei and abundant clear cytoplasm (Fig-

ure 1). A diagnosis of benign glandular peripheral nerve

sheath tumor (PNST or glandular neurofibroma) was

given.

Case number 2

A 32 year old male patient presented with swellings over

neck, thigh and upper extremity. The patient had distinc-

tive skeletal abnormalities and careful clinical examina-

tion revealed presence of café au lait spots in left posterior

axillary fold. Swelling over neck was excised and submit-

ted for histopathology. It measured 3 × 1.5 × 1 cm and the

cut surface was homogenously tan grey and glistening.

On microscopic examination, variably spaced slender,

spindle shaped cells were seen in a loose, myxoid and col-

lagenous stroma. No mitosis was observed in this spindle

cell component. Rosette like structures were seen amidst

these areas of typical neurofibroma. These rosettes dis-

played presence of a central, eosinophilic, fibrillary core

which was surrounded by round to elongated cells. The

cells bordering the rosettes were seen merging into the

adjacent stroma (Figure 2). The tumor was diagnosed as

neurofibroma and the presence of focal rosette like struc-

tures was noted.

Discussion

First described by Garre et al [2]. in 1892, glandular differ-

entiation is the rarest form of divergent differentiation

seen in peripheral nerve sheath tumors (PNST) which also

includes cartilage, bone, chondrosarcoma, osteosarcoma

and rhabdomyosarcoma. Around 40 cases have been

reported so far in world literature. Diagnostic criteria

includes evidence supporting a finding of nerve sheath

tumor, presence of true glandular epithelium, not

entrapped glands or pseudoepithelium. The vast majority

of PNST harbouring these glands have been malignant

(a): Section shows slender spindle shaped cells embedded in a collagenous stroma along with presence of mucus produc- ing glands Figure 1

(a): Section shows slender spindle shaped cells

embedded in a collagenous stroma along with pres-

ence of mucus producing glands. (H&E ×100) (b):

Glands are lined by goblet cells and show presence of intralu-

minal mucin. (H&E ×400).

(a): Section shows presence of rosette like structures between areas of typical neurofibroma Figure 2

(a): Section shows presence of rosette like structures

between areas of typical neurofibroma. Rosettes dis-

play central eosinophilic, fibrillary core and peripheral pal-

isading by neuronal cells. (H&E ×100) (b): Another rosette

showing presence of central capillary in the fibrillary core.

(H&E ×400).

Cases Journal 2008, 1 :188 http://www.casesjournal.com/content/1/1/188

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peripheral nerve sheath tumor (MPNST) [3]. The glandu-

lar epithelium is usually benign though there are sporadic

case reports of PNST with malignant glandular compo-

nent [4].

Glandular neurofibromas are rare and in an extensive

review by Woodruff and Christenstein, only 2 out of 25

analyzable cases of glandular peripheral nerve sheath

tumors were neurofibromas. Both of these cases occurred

in young females, were associated with NF Type 1 and

were finally diagnosed as plexiform neurofibromas [3].

No history of neurofibromatosis was present in the other

reported cases of glandular neurofibromas [5-8].

Most important differential diagnostic consideration in a

case of glandular neurofibroma is schwannoma with

entrapped adnexal structures. Schwannomas usually dis-

play typical Antoni A and B areas along with presence of

Verocay bodies. Trapped adnexal glands are generally seen

in clusters and are connected to each other. In the absence

of typical morphological features, differentiation between

these entities may be difficult and immunohistochemistry

(IHC) may be employed. Glands in true glandular PNST

are devoid of myoepithelial cell lining and are hence non

reactive for muscle common actin (HHF-35). This glandu-

lar epithelium is not only reactive for cytokeratins but also

for neuroendocrine cell markers (chromogranin, serot-

onin and somatostatin). Entrapped adnexal glands in

schwannoma show the presence of myoepithelial cell

layer and are non reactive for neural markers. Stromal

cells show S-100 positivity in both the cases [3].

Though IHC was not employed in our case (case number

1), the tumor was diagnosed as neurofibroma as charac-

teristic features of neurofibroma (spindle cells with short,

curved nuclei embedded in a collagenous stroma) were

seen on histology sections. Moreover, history of NF-type 1

in the patient corroborated the diagnosis of neurofi-

broma. The glands seen in case number 1 resembled

mucus secreting glands rather than eccrine glands of skin

adnexa.

It is interesting to note that adnexal glands have also been

reported in neurofibroma and it has been proposed that

the tumor appeared in the nerves around the eccrine

glands and grown to the subcutaneous tissue, and the

glands might have been left behind rather than entrapped

by the growing tumor [9].

The histogenesis of glandular PNST is still not clear and it

may be attributed to the metaplastic potential of Schwann

cells or the presence of primitive neural crest cells that

migrate with Schwann cells along the peripheral nerves

[4].

Neurofibroma with rosette like structures is exceedingly

rare. Enzinger reviewed a unique case of neurofibroma

showing presence of mucus secreting glands and focal

rosettes [10]. A recently described variant of neurofibroma

with rosettes is dendritic cell neurofibroma with pseudor-

osettes (DCNP) [11]. This tumor is seen in adults and has

been described mostly in patients without a history of NF-

1, [11,12]. though cases arising in association with NF-1

have also been described [13]. The lesion is well circum-

scribed and occurs in superficial dermis of head, trunk

and extremities. It is comprised of two types of cells. Type

1 cells are small lymphocyte like cells with slightly cleaved

nuclei which are concentrically arranged around larger

type 2 cells having vesicular nuclei and copious cyto-

plasm. These type 2 cells have dendritic extensions which

form the core of these pseudorosettes. On IHC, type 2

cells and most type 1 cells stain for CD57 and S-100 [11].

Another important differential diagnostic consideration is

Schwannoma with neuroblastoma like rosettes. In this

tumor, small round to oval cells are layered around a cen-

tral eosinophilic fibrillary material. These cells may also

show presence of intranuclear cytoplasmic inclusions

[14].

Other spindle cell tumors which may show presence of

rosettes are Low grade fibromyxoid sarcoma (LGFMS) and

the closely related Hyalinising spindle cell tumor with

giant rosettes (HSCT). These tumors are comprised of fusi-

form or spindled cells enmeshed in a heavily collagenized

stroma showing abrupt transitions to myxoid zones.

Mitotic activity is typically low. Cases diagnosed as "hya-

linizing spindle cell tumor with giant rosettes" contain a

variable number of collagen rosettes [15]. though rosettes

are also seen in upto 40% of cases of LGFMS [14]. Inter-

estingly, LGFMS like areas have been described in relation

with low grade MPNST [16]. But both LGFMS and HSCT

are tumors of fibroblasts rather than schwann cells and

show much weaker expression of S-100 protein [14]. The

stroma of case number 2 was Shwannian and it lacked the

presence of fibroblastic cells arranged in whorls and cur-

vilinear blood vessels which are the characteristic features

of LGFMS,[14]. hence the tumor was suspected to be neu-

rofibroma.

Conclusion

Glandular benign PNST and neurofibromas with rosette

like structures are extremely rare and reflect the histologi-

cal diversity seen in PNST. It is important to document

these entities as these need to be differentiated from other

potentially malignant tumors.

Abbreviations

NF: Neurofibromatosis 1; PNST: Peripheral nerve sheath

tumor; MPNST: Malignant peripheral nerve sheath tumor;

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Cases Journal 2008, 1 :188 http://www.casesjournal.com/content/1/1/188

Page 4 of 4

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IHC: Immunohistochemistry; DCNP: Dendritic cell neu-

rofibroma with pseudorosettes; LGFMS: Low grade fibro-

myxoid sarcoma; HSCT: Hyalinising spindle cell tumor

with giant rosettes.

Competing interests

The authors declare that they have no competing interests.

Authors' contributions

DJ confirmed the diagnosis of the cases, and wrote the

first draft of the manuscript. NG confirmed the diagnosis

of the cases, and critically reviewed the manuscript. SK

reviewed the case slides of case number 1 and verified the

diagnosis. SV reviewed the case slides of case number 2

and verified the diagnosis.

Consent

A written consent has been obtained from both patients.

No personal identifiers are used in this case report.

Acknowledgements

None.

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ResearchGate has not been able to resolve any citations for this publication.

  • James M. Woodruff

The two main peripheral nerve sheath tumors found in patients with neurofibromatosis, type 1 (NF1), are neurofibroma, a benign tumor, and malignant peripheral nerve sheath tumor (MPNST). The tumors are related in that most MPNSTs are thought to arise by malignant transformation of neurofibromas. Such an event occurs in about 2% of NF1 patients. There are five forms of neurofibroma; three of them—localized cutaneous neurofibroma when multiple, plexiform neurofibroma, and massive soft-tissue neurofibroma—are highly specific for NF1. Only two forms of neurofibroma, plexiform and localized intraneural neurofibroma, are significant precursors of MPNST. Massive soft-tissue neurofibromas are worrisome in that they may mask MPNST arising from one of the mentioned neurofibromas. The vast majority of MPNSTs are high-grade malignant tumors with a high rate of distant metastasis. The overall 5-year survival rate for patients with MPNSTs ranges from 34% to 52%. MPNSTs generally are solitary, deep-seated globoid or fusiform tumors. They are firm, fleshy, tan, and often focally to extensively necrotic, and they invade surrounding soft tissue. On histological examination, MPNSTs are most often hypercellular, hyperchromatic, fasciculated, and mitotically active tumors. Low-grade tumors account for only about 10–15% of cases. Twenty percent of MPNSTs have unusual and potentially misleading histological features, such as epithelioid cells and divergent mesenchymal or glandular differentiation. Am. J. Med. Genet. (Semin. Med. Genet.) 89:23–30, 1999.

  • B Bednár

A 21-year-old man had a delimited tumour of a size of a nut, which grew expansively in the subcutaneous region of the thigh. It had the structure of neurofibroma with myofibrils and with a glandular component which on electronoptic examination was cylindromatous nature. This case is the eighth one in literature and the first one documented electronoptically. A similar tumour in a 26-year-old woman was located in the subcutaneous layer of the calf but, apart from this electronoptic structure revealed a multitude of tumours cells of a tonofibril corresponding to the spinocellular epithelium. The possibility is discussed whether even this tumour may not be of neurofibromatous origin.

  • Dmitry E Matsko Dmitry E Matsko
  • A A Nikonov

A rare observation of glandular differentiation in neurofibroma of the right orbit is described. Data on another 7 similar tumors reported in the literature are presented. The problems of histogenesis of such neoplasias and difficulties of their diagnosis are discussed. Glandular neurinomas and neurofibromas are characterized by a malignant course and trend for relapses favouring high lethality in this disease.

  • Y Oda
  • H Hashimoto
  • Masazumi Tsuneyoshi
  • Y Iwata

The glandular peripheral nerve sheath tumor is a rare variant of nerve sheath neoplasms in which the focally occurring glands are lined by cells showing divergent differentiation. The vast majority of the reported nerve sheath tumors harboring these glands have been malignant. We herein present a case of benign glandular peripheral nerve sheath tumor in a 43-year-old woman who had no evidence of von Recklinghausen's disease. Histologically, the tumor is composed of spindle cell component and collections of glandular component. The glandular component occupied the central two-thirds of the lesion and was lined by a single layer of nonciliated cuboidal or columnar cells. No mitotic figures were recognized in the spindle cell area. This spindle cell area had neurofibroma-like features rather than schwannoma. Many of the spindle cells had positive reaction products for S-100 protein. The glandular lining epithelium were positive for cytokeratins (CAM 5.2, AE1/AE3, PKK1) and EMA. Some epithelial cells were immunoreactive for CEA, chromogranin, somatostatin and Leu-7. These immunohistochemical findings support the neuroendocrine differentiation of the epithelial element from the schwannian component.

  • James M. Woodruff
  • W N Christensen

Peripheral nerve tumors (PNT) containing glands are uncommon. Which types of PNT contain glands is a matter of controversy, a factor bearing on the prognosis of these tumors. The authors reviewed the files of 11 patients with glandular PNT seen in their laboratory and 27 patients reported in the literature. The 11 instances of glandular PNT seen in their laboratory affected male and female patients equally; patients ages ranged from 8 to 68 years (mean, 28 years). Six patients had neurofibromatosis-1 (NF-1). Eleven of the tumors were histologically malignant PNT, and one was a benign neurofibroma. There were no schwannomas (neurilemomas). The glands were discrete, usually localized to a few areas and in every patient were lined by a keratin-positive epithelium, which in two patients was malignant. One tumor also contained areas of rhabdomyosarcoma, chondrosarcoma, and osteosarcoma (a pluridirectional malignant PNT). Treatment in all patients was some form of surgical resection, followed by radiation in three and chemotherapy in two. Follow-up data were available for nine patients; six of eight patients died with disease. Review of the literature revealed two purported glandular schwannomas (neurilemomas). The authors think these patients had schwannomas containing trapped skin adnexa. Overall, 74% of the patients had NF-1. Ninety-two percent of the tumors were histologically malignant, and 23% of the malignant tumors were pluridirectional malignant PNT. Of the 21 patients for whom follow-up was available, 71% died with tumor. Most glandular PNT are histologically malignant and often are fatal.

Most tumors of peripheral nerve sheaths containing glands are malignant tumors associated with Von Recklinghausen's disease. A 39 year old man consulted with a tumour on a finger of the right hand, which was not painful, and was slow growing. There was no past history of neurofibromatosis. Histological study showed a tumour of the peripheral nerve sheath, a benign type of neurofibroma containing glands. Immunohistochemical techniques confirmed that it had the stroma of a Schwannoma with well-defined glandular epithelial elements. A neurofibroma with glands is considered to be a rare type of divergent differentiation, and of considerable interest to pathologists, since it must be differentiated from other tumoral lesions. Immunohistochemical study is very useful for this.

  • T Nagasaka
  • Raymond Lai Raymond Lai
  • Michihiko Sone
  • N Nakashima

In this report, we describe a highly unusual case of glandular malignant peripheral nerve sheath tumor presenting as a neck mass in a previously healthy 29-year-old man. Grossly, the tumor was found to arise from a swollen peripheral nerve trunk. The tumor was largely composed of spindle cells that demonstrated marked nuclear pleomorphism and numerous abnormal mitotic figures. In addition, histologically malignant glandular structures lined by simple nonciliated columnar cells with goblet cells were found clustered in the center of the tumor. Examination of the swollen peripheral nerve trunk revealed the presence of a plexiform neurofibroma. The spindle cells were positive for S100. The glands were negative for S100 but positive for keratin, epithelial membrane antigen, and neuroendocrine markers (somatostatin, chromogranin, Leu-7, and calcitonin). This patient was subsequently diagnosed as having von Recklinghausen disease and died of tumor metastasis to the lungs 34 months after the presentation. To our knowledge, only 3 similar cases have been previously described in the literature.

  • Andrew L Folpe
  • Kathryn L. Lane
  • Gerson Paull
  • Sharon W. Weiss

Low-grade fibromyxoid sarcoma (LGFMS) is a rare sarcoma characterized by bland histologic features and a paradoxically aggressive clinical course. The hyalinizing spindle cell tumor with giant rosettes (HSCT) is a closely related tumor characterized by the presence of giant collagen rosettes. Only a single example of a metastasizing HSCT has been reported. A small subset of both LGFMS and HSCT display areas of increased cellularity and atypia which qualify as intermediate- to high-grade sarcoma; the significance of these features has not been definitively assessed. We present the clinicopathologic features of 77 cases of LGFMS and HSCT to determine the degree of overlap of these two lesions, their biologic behavior, and the significance of the occasional presence of intermediate- to high-grade sarcoma within both. The patients (33 female, 40 male) ranged from 3 to 78 years of age (median, 34 yrs). Fourteen cases occurred in patients less than 18 years of age. The tumors measured from 1 to 23 cm (median, 4.5 cm) and occurred predominantly in the trunk and lower extremities in both the deep (66 cases) and superficial (7 cases) soft tissues. In 15 cases, the tumor was present > 1 year before diagnosis. All tumors showed predominantly the typical hypocellularity and bland cytologic features of typical LGFMS; however, areas of hypercellularity and nuclear enlargement and hyperchromatism were identified in 12 of 73 (16%) and 7 of 73 (10%), respectively. Necrosis and mitotic activity >5/50 high-powered fields (HPF) were present in 6 of 73 (8%) and 5 of 73 (7%), respectively. Epithelioid areas were present in 33 of 73 (45%) and rosettes in 22 of 73 (30%). Follow up (54 cases; range, 2-192 mos; median, 24 mos; mean, 38 mos) showed 5 recurrences, 3 metastases, and 1 death. The diagnosis of LGFMS or HSCT was made prospectively in 51 patients; none had metastatic disease. Two of the metastatic tumors were LGFMS and one was a HSCT. LGFMS may occur more often in the pediatric population and show a much wider histologic spectrum than previously thought. A significant number of LGFMS possess inconspicuous collagen rosettes characteristic of HSCT, indicating that these two tumors are ends of a common spectrum rather than distinct entities. HSCT, like LGFMS, are low-grade sarcomas with metastatic potential. The presence of focal areas of intermediate- to high-grade sarcoma does not portend a worse outcome in the short term. The better prognosis reflected in this study compared with previous ones might reflect the fact that all were initially diagnosed as sarcomas and treated with aggressive surgery. The fact that the only three patients to develop metastatic disease were patients whose LGFMS or HSCT was identified retrospectively supports this concept.

  • Tomomi Kusumi
  • Manobu M Tanaka Manobu M Tanaka
  • Takeaki Kurita
  • Hajime Kudo

It is a recognized fact that glandular structures sometimes occur in peripheral nerve sheath tumors (PNST). Reports indicate that epithelial potential could be expressed in malignant PNST, while the glands in most benign PNST could be trapped skin adnexa. We present a case of spindle cell tumor with glandular structures. The patient was a 55-year-old man who had a subcutaneous tumor excised. The spindle cell tumor had histological characteristics of neurofibroma. The glandular structures had a pattern of immunohistochemical staining that was similar to that of the secretory coils and excretory ducts of normal eccrine glands. Therefore, the glands were thought to be naturally existing eccrine glands. The glands were completely enclosed within the tumor and not connected to one another; a cluster formation was not observed. The upper portion of tumor included some glands of skin adnexa. The spindle cell tumor may have originated from the nerves distributed around the skin adnexa, and grown to the subcutaneous tissue. The glands may have been left behind rather than have been trapped in the tumor.