 |
|
|
|
|
|
|
|
|
 |
 |
 |
 |
 |
 |
 |
 |
|
Background
This is a very common tumor of the skin, usually occurring on the head and neck area. It is also known as a chondroid syringoma, a reference to the characteristic histopathological appearance of this tumor, with some glands resembling a syringoma.
OUTLINE
LABORATORY/
RADIOLOGIC/
OTHER TESTSCHARACTERIZATION RADIOLOGIC
Malignant chondroid syringoma of the skin: magnetic resonance imaging features.Nicolaou S, Dubec JJ, Munk PL, O'Connell JX, Lee MJ.
Department of Radiology, Vancouver General Hospital, Vancouver, British Columbia, Canada.
Australas Radiol 2001 May;45(2):240-3 Abstract quote Skin tumours are usually divided into melanoma and non-melanoma types. Malignancies of the adnexal structures, of which sweat gland tumours are an example, are characterized under the non-melanoma types. Sweat gland malignancies are rare tumours that are usually associated with a poor prognosis. Given the rarity of these tumours, MRI findings of such tumours have not been described previously in the literature.
We present a case report of an unusual malignant tumour of sweat gland origin known as a malignant chondroid syringoma of the skin with described MRI features. The MRI features are non-specific depicting intermediate signal intensity, changes on the proton density sequence and increased signal on the T2 and STIR-weighted sequences.
Although these imaging features are characteristic of most soft tissue masses MRI can, in most cases, accurately depict the anatomic extent and identify tissue of origin, depth of invasion and relation to adjacent structures, such as muscles and bones. Thus high-resolution MRI of the skin in the future can be extremely helpful in characterizing and staging dermal neoplasms.
GROSS APPEARANCE/
CLINICAL VARIANTSCHARACTERIZATION GENERAL
Chondroid syringoma: a diagnosis more frequent than expected.Yavuzer R, Basterzi Y, Sari A, Bir F, Sezer C.
Department of Plastic and Reconstructive Surgery, Gazi University, Medical Faculty, Ankara, Turkey.
Dermatol Surg 2003 Feb;29(2):179-81 Abstract quote BACKGROUND: Chondroid syringoma or mixed tumor of the skin is a rare subcutaneous tumor that may be confused with various skin lesions.
OBJECTIVE: To elucidate the incidence of condroid syringomas among skin lesions that were excised under local anesthesia.
METHODS: The histopathologic diagnosis of 16,200 skin lesions that had been operated between 1986 and 2002 were retrospectively evaluated. The cases with condroid syringoma were histopathologically re-examined, and confirmed cases were further analyzed for preoperative diagnosis, age, gender, and lesion location.
RESULTS: Sixteen patients were found to have histopathologic diagnosis of chondroid syringoma constituting 0.098% of the excised skin lesions in this series. All of these 16 cases were misdiagnosed preoperatively. The typical presentation was a solitary skin lesion located in the head and neck region in a middle-aged male patient.
CONCLUSION: In the evaluation of a middle-aged male patient with a small subcutaneous nodule in the head and neck region, chondroid syringoma should be also taken into consideration for differential diagnosis. For such a lesion, excisional biopsy without destroying aesthetic and functional structures is the preferred diagnostic approach.VARIANTS AXILLA
Giant chondroid syringoma of the axilla.Hardisson D, Linares MD, Nistal M.
Department of Pathology, University Hospital "La Paz", Autonomous University of Madrid, Spain.
J Cutan Med Surg 1998 Oct;3(2):115-7 Abstract quote BACKGROUND: Chondroid syringomas are benign uncommon tumours of controversial histogenesis that most often affect the head and neck region and usually measure > 3 cm in greatest dimension. Objective: To describe the clinical features, histology, and differential diagnosis of an unsually large axillary chondroid syringoma.
METHODS: A 64-year-old man presented with a painless, subcutaneous tumour measuring 8 3 7 3 6.5 cm on his right axilla, which had been growing slowly for several years. The tumour was completely excised under local anesthesia. No recurrence of the tumour has been observed 12 months after surgery.
RESULTS: Histological examination showed cords, nests, and tubuloglandular structures composed of well-differentiated tumour cells embedded in a hyalinized stroma with abundant chondroid matrix, and the diagnosis of chondroid syringoma was established. No features suggesting malignancy were observed.
CONCLUSION: As this case shows, chondroid syringoma may reach a large size, and it should be included in the differential diagnosis of slowly growing solid nodules in the skin or subcutis.EYELID
Chondroid syringoma of the eyelid.Martorina M, Capoferri C, Dessanti P.
Department of Ophthalmology, Regional Hospital, Aosta, Italy.
Int Ophthalmol 1993 Oct;17(5):285-8 Abstract quote An 81-year-old man underwent excisional biopsy of a big cylindric painless mass of the right lower eyelid, which had been present for over 23 years.
Histopathologic and immuno-histochemical examination showed a chondroid syringoma, an uncommon benign neoplasm of possible eccrine sweat gland origin, which occurs generally in the head and neck skin.
In the eyelids, it has been previously described as a small nodule in the upper lid. Surgical excision of these tumours is recommendable because malignant transformation is possible, although rare.NOSE
Chondroid syringoma of the nose: report of a case.Bhargava D, Bhusnurmath S, Daar AS.
Department of Surgery, College of Medicine, Sultan Qaboos University, Sultanate of Oman.
J Laryngol Otol 1997 Sep;111(9):862-4 Abstract quote Chondroid syringoma or mixed tumour of the skin is a rare benign tumour which can present on the face.
We present here the case of a patient who underwent an excisional biopsy of a small painless nodule in the soft triangle of the nose for cosmetic reasons.
The clinical presentation, histology and treatment, with review of the relevant literature, is discussed.
HISTOLOGICAL TYPES CHARACTERIZATION GENERAL
Chondroid syringoma (mixed tumor of the skin). A clinicopathological study of 13 cases.Kunikane H, Ishikura H, Yamaguchi J, Yoshiki T, Itoh T, Aizawa M.
Acta Pathol Jpn 1987 Apr;37(4):615-25 Abstract quote The clinicopathology of 13 cases of chondroid syringoma were examined. The ages of the patients ranged from 26 to 86 years, with an average of 48 years. There were eight males and five females. Ten tumors out of the thirteen appeared on the face.
Only one patient out of ten was suspected of recurrence in follow-up information. Histologically, all tumors consisted of epithelial cells, chondroid or myxoid matrix, and other strumal elements. The tumors were histologically classified into two types; twelve tumors had tubular and cystic lumina lined by two layers of epithelial cells, and only one case had small lumina lined by only a single layer. By an immunohistochemical study with a PAP method, positive stainings of keratin were observed in all cases, and S-100 protein in all but one.
Ultrastructurally, the tumor cells showed features of an epithelial cell. Some ultrastructural differences were noted between two types of chondroid syringoma. Type I tumor cells had many tonofilaments in cytoplasm, but cytoplasmic filaments in type II were of the intermediate-type.VARIANTS HYALINE
Hyaline cell-rich chondroid syringoma: epithelial nature of the hyaline cells.Nakayama H, Kishi A, Kajihara H.
Laboratory of Pathology, Hiroshima University School of Medicine, Japan.
Jpn J Clin Oncol 1996 Aug;26(4):237-42 Abstract quote We report a case of hyaline cell-rich chondroid syringoma arising in the skin on the left side of the neck. The tumor was composed exclusively of hyaline cells arranged in a predominantly thick trabecular pattern. Ductal structures of various sizes in the tumor were composed of eosinophilic cuboidal cells showing transition to hyaline cells.
Immunohistochemically, the hyaline cells were positive for cytokeratin, S-100, and vimentin, although the best marker for myoepithelial differentiation, alpha-smooth muscle actin, was negative.
As is the case with plasmacytoid monomorphic adenomas of the salivary gland and some reported cases of hyaline cell-rich chondroid syringoma, hyaline cells in the present tumor similarly lacked any evidence of myoepithelial differentiation. These immunohistochemical findings reveal that the hyaline cells in the present tumor were epithelial.LIPOMATOUS APOCRINE MIXED TUMOR
Lipomatous apocrine mixed tumor of the skin.Ohata C, Hanada M.
Am J Dermatopathol 2003 Apr;25(2):138-41 Abstract quote The common mesenchymal elements of apocrine mixed tumor of the skin are mucin and chondroid tissue.
An apocrine mixed tumor of the skin with extensive adipose content is reported. A 56-year-old Japanese man presented with a subcutaneous mass in the glabella region that was 1 cm in diameter and had developed within the preceding 5 years. Light microscopic examination showed a well-circumscribed and encapsulated subcutaneous tumor composed of tubular structures with apocrine secretion and mucin as well as extensive adipose tissue.
This case, in light of another recently reported case, indicates that adipose tissue can be the major mesenchymal element in apocrine mixed tumor of the skin.LIPOMATOUS MIXED TUMOR WITH FOLLICULAR DIFFERENTIATION
- Lipomatous mixed tumor with follicular differentiation of the skin.
Kasashima S, Hiroshi M, Toshinori M, Yoshio O.
Pathology Section, Kanazawa University Hospital.
J Cutan Pathol. 2006 May;33(5):389-94. Abstract quote
A very rare case of cutaneous mixed tumor with numerous adipose tissue and hair follicular structures in a 67-year-old Japanese male was reported. A well-circumscribed tumor was in the subcutaneous tissue of the cheek and far from the parotid gland.
Histologically, the tumor consisted of an admixture of the adipose tissue, fibromyxoid tissue with spindle cells, and branching tubular structures. Outer layers of ductal epithelial cells and single spindle cells were often in a transition, likely as typical mixed tumor. Branching ducts connected with keratinous cysts, strands of trichoblastic basophilic cells and clear cell nests. There was a gradual transition, between small-sized adipocytes and vacuolated spindle cells. No chondroid stroma was seen.
To our knowledge, this tumor is the first case of a lipomatous mixed tumor with hair follicular differentiation. The case indicates an additional wide spectrum of histologic appearances of cutaneous mixed tumor.MALIGNANT
- Malignant Chondroid Syringoma with Bone Invasion: A Case Report and Review of the Literature.
Takahashi H, Ishiko A, Kobayashi M, Tanikawa A, Takasu H, Md MT.
*Department of Dermatology, Keio University School of Medicine, Tokyo, Japan and the daggerDepartment of Dermatology, Kitasato University School of Medicine, Kanagawa, Japan.
Am J Dermatopathol. 2004 Oct;26(5):403-406. Abstract quote
We describe a 27-year-old Japanese female with a recurrent nodule on the left big toe and local bone invasion.
Histopathologically, the tumor consisted of nests of atypical cells with few mitotic cells, which partly formed gland-like structures. Areas of myxoid degeneration, positive for Alcian blue staining and that did not stain after they were digested with hyaluronidase, were prominent in the matrix among tumor cells. Positive staining was noted in tumor cells for cytokeratin (AE1+AE3), S-100 protein, neuron specific enolase (NSE), and glial fibrillary acidic protein (GFAP).
These findings, especially positive GFAP staining were characteristic and very helpful for the diagnosis of the rare tumor—malignant chondroid syringoma. Based on the previous reports, 39% of cases were found to have metastatic lesions and 22% died of this malignant tumor.
There have been no reports reporting effectiveness of chemotherapy and radiotherapy, and an early wide excision with a broad margin may be the most reliable treatment to date.
Malignant chondroid syringoma. Report of a case with widespread metastasis and review of pertinent literature.Ishimura E, Iwamoto H, Kobashi Y, Yamabe H, Ichijima K.
Cancer 1983 Nov 15;52(10):1966-73 Abstract quote The authors report the autopsy case of a 73-year-old man with widespread, metastatic, malignant chondroid syringoma, with a long clinical history. Three years after resection of the primary tumor on the back, the tumor recurred at the same site, metastasized to the cervical lymph nodes 7 years later, and finally, after 13 years exhibited widespread metastases.
Histologically, the tumor showed cords and nests of cuboidal or polygonal tumor cells with little cellular pleomorphism and few mitoses surrounded by a mucoid matrix. Histochemically, the matrix contained hyaluronic acid and sulfated acid mucopolysaccharides, such as chondroitin sulfate A and/or C. To the knowledge of the authors there have been nine reported cases of malignant chondroid syringoma in the English literature, four of which had distant metastasis.A review of the literature reveals that malignant chondroid syringoma is more common in women and occurs most often in trunk and extremities, which is in contrast to its benign counterpart, its histologic appearances vary greatly, and it may even have benign appearances.
Malignant mixed tumor of the skin: malignant chondroid syringoma.Redono C, Rocamora A, Villoria F, Garcia M.
Cancer 1982 Apr 15;49(8):1690-6 Abstract quote Malignant tumors of sweat gland origin are rare. Probably one of the rarest types, still poorly understood, is the so-called malignant chondroid syringoma or malignant mixed tumor of the skin. A case of malignant chondroid syringoma is presented.
Ultrastructural study proves very useful for the differential diagnosis with other chondroid or chordoid tumors.
SPECIAL STAINS/
IMMUNOPEROXIDASE/
OTHERCHARACTERIZATION SPECIAL STAINS IMMUNOPEROXIDASE
Malignant chondroid syringoma: immunohistopathology.Metzler G, Schaumburg-Lever G, Hornstein O, Rassner G.
Department of Dermatology, University of Tubingen, Germany.
Am J Dermatopathol 1996 Feb;18(1):83-9 Abstract quote Malignant chondroid syringoma, also called malignant mixed tumor of the skin, is a rare variant of a malignant tumor derived from sweat gland cells.
Histologically the tumor is composed of an epithelial and a mesenchymal component. The epithelial structures show glandular differentiation and carcinomatous features. They are embedded in a mucinous stroma with spindle mesenchymal cells and areas of chondroid differentiation. The epithelial cells express cytokeratins. The luminal epithelial cells show binding to the lectin Ulex europaeus; intraluminal cells are carcinoembryonic antigen positive.
The stromal cells are cytokeratin negative and sporadically positive for vimentin. Chondroid areas are S-100 protein and vimentin positive. No labeling for actin is seen.
Chondroid syringoma: an immunohistochemical study using antibodies to Ca 15-3, KA-93, Ca 19-9, CD44 and BM-1.Tsuji T.
Department of Dermatology, Nagoya City University Medical School, Japan.
J Cutan Pathol 1996 Dec;23(6):530-6 Abstract quote The expression of Ca 15-3, KA-93, Ca 19-9, CD44 and BM-1 in normal skin and chondroid syringoma was investigated immunohistochemically using antibodies to these antigens. In the normal skin, the eccrine ducts and/or secretory elements were positive for all these antigens.
On the other hand, the apocrine ducts and/or secretory elements were positive for the antibodies to Ca 15-3, KA-93 and CD44. In chondroid syringoma, the luminal cells of tubuloglandular structures were positive for the antibodies to Ca 15-3 and BM-1, and partly positive for those to KA-93 and Ca 19-9. The basal cells of the tubular structures and solid nests were positive only for the antibody to CD44. Stromal cells in the myxoid area were positive for the antibodies to KA-93 and CD44, and the chondroid matrix was positive for the antibody to BM-1.
It is suggested that chondroid syringoma might originate from, or differentiate into the ducts and/or secretory elements of the eccrine sweat glands. In addition, the significance of the expression of BM-1 in the chondroid matrix is discussed.
COLLAGEN, TYPE II
- Bone morphogenetic protein-mediated type II collagen expression in pilomatricoma and cutaneous mixed tumor.
Mieno H, Kuroda K, Tajima S.
Department of Dermatology, National Defense Medical College, 3-2 Namiki, Tokorozawa, Saitama, Japan.
J Cutan Pathol. 2005 Mar;32(3):206-11. Abstract quote
Background: We have previously reported that type II collagen deposition in overlying dermo-epidermal junction (DEJ) of pilomatricoma is mediated by bone morphogenetic protein 2/4 (BMP 2/4) expressed by shadow cells (SCs) of pilomatricoma.
Objective: This time, we studied the expression of type II collagen and BMP in a large number of cases of pilomatricoma and extended our study to cutaneous mixed tumor (CMT).
Results: We found type II collagen deposition in the overlying DEJ (16 of 50 cases) and in the SCs (19/50) of pilomatricoma. The number of case of type II collagen deposition in DEJ (DEJ(+)) and in SCs (SC(+)) of pilomatricoma correlated to the chronological stage of pilomatricoma. We also found type II collagen deposition in overlying DEJ (two of 11) and in the stromal chondroid tissue (four of 11) of CMT. BMP 2 was expressed in most cases of pilomatricoma (37/50) and CMT (seven of 11).
Conclusions: The expression of type II collagen in pilomatricoma is dependent upon the chronological stage of pilomatricoma. Type II collagen expression in the overlying DEJ and chondroid matrix in CMT may be induced by BMP via the same mechanism as in pilomatricoma.
PROGNOSIS CHARACTERIZATION METASTASES
Malignant chondroid syringoma presenting as multiple pulmonary nodules.Kiely JL, Dunne B, McCabe M, McNicholas WT.
Department of Respiratory Medicine, St Vincent's Hospital, Dublin, Ireland.
Thorax 1997 Apr;52(4):395-6 Abstract quote Malignant chondroid syringoma, a very rare tumour, presenting with multiple pulmonary metastases in a 50 year old woman is described. Initial diagnostic confusion with pulmonary hamartoma occurred due to histopathological similarities.
However, re-examination of a skin biopsy specimen taken 17 years previously from a hand lesion yielded the necessary information to identify the pulmonary lesions definitively as metastases from the original skin lesion.
The features of this very rare indolent tumour are described.
TREATMENT CHARACTERIZATION GENERAL RADIATION THERAPY
Role of radiation therapy in the management of malignant chondroid syringoma.Hong JJ, Elmore JF, Drachenberg CI, Jacobs MC, Salazar OM.
Department of Radiation Oncology, University of Maryland Hospital, Baltimore, USA.
Dermatol Surg 1995 Sep;21(9):781-5 Abstract quote BACKGROUND. Malignant sweat gland neoplasms are exceedingly rare tumors. Malignant chondroid syringoma (MCS) is one of the rarest subtypes, and as such, still poorly understood. It lacks distinctive clinical features, often delaying initial diagnosis and therapeutic management.
OBJECTIVE. A current case and the available literature are reviewed to determine the overall clinical course of the MCS and the potential role of adjuvant therapy.
METHODS. A case of MCS was studied by light microscope, immunohistochemistry, and electron microscopy. The clinical data of this case and of other reported cases are summarized and compared.
RESULTS. This tumor recurred locally after initial local excision. Subsequent re-excision and radiation therapy rendered the patient without evidence of disease. This case study and the literature review of the 20 reported cases indicate that MCS is highly recurrent with tendency toward metastasis.
CONCLUSION. MCS appears to behave in an aggressive manner. An initial treatment modality is aggressive surgery. Adjuvant radiation therapy with or without chemotherapy should be tried in future cases.
SURGERY
Chondroid syringoma of the head and neck: clinical management and literature review.Chen AH, Moreano EH, Houston B, Funk GF.
Department of Otolaryngology-Head and Neck Surgery, University of Iowa College of Medicine, Iowa City 52242-1078, USA.
Ear Nose Throat J 1996 Feb;75(2):104-8 Abstract quote This report describes the case of a chondroid syringoma occurring in the nasofacial groove of a 60-year-old woman.
This benign, mixed epithelial tumor is infrequently seen by the otolaryngologist-head and neck surgeon, and therefore may not be included in the differential diagnosis of a nodular lesion on the skin of the head and neck. However, the most frequent site of occurrence for these relatively rare cutaneous lesions is the head and neck region.
Histologically, these tumors are quite similar to pleomorphic adenomas of salivary gland origin, and optimal surgical management similarly requires removal of a cuff of normal tissue, rather than a simple shelling out of the lesion.
The clinical presentation, pathology and recommended management of this rare tumor are discussed.Henry JB. Clinical Diagnosis and Management by Laboratory Methods. Twentieth Edition. WB Saunders. 2001.
Rosai J. Ackerman's Surgical Pathology. Ninth Edition. Mosby 2004.
Sternberg S. Diagnostic Surgical Pathology. Fourth Edition. Lipincott Williams and Wilkins 2004.
Robbins Pathologic Basis of Disease. Seventh Edition. WB Saunders 2005.
DeMay RM. The Art and Science of Cytopathology. Volume 1 and 2. ASCP Press. 1996.
Weedon D. Weedon's Skin Pathology Second Edition. Churchill Livingstone. 2002
Fitzpatrick's Dermatology in General Medicine. 6th Edition. McGraw-Hill. 2003.
Weiss SW and Goldblum JR. Enzinger and Weiss's Soft Tissue Tumors. Fourth Edition. Mosby 2001.
Basic Principles of Disease
Learn the basic disease classifications of cancers, infections, and inflammation
Commonly Used Terms
This is a glossary of terms often found in a pathology report.Diagnostic Process
Learn how a pathologist makes a diagnosis using a microscopeSurgical Pathology Report
Examine an actual biopsy report to understand what each section meansSpecial Stains
Understand the tools the pathologist utilizes to aid in the diagnosisHow Accurate is My Report?
Pathologists actively oversee every area of the laboratory to ensure your report is accurateGot Path?
Recent teaching cases and lectures presented in conferences
Last Updated May 24, 2006
Send mail to The Doctor's Doctor with questions or comments about this web site.
Read the Medical Disclaimer.
Copyright © The Doctor's Doctor