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Background
These rare tumors are derived from the glomus cells, specialized cells that surround small blood vessels and are important in regulating peripheral blood flow. The classic presentation is a painful nail with triad of pain, tenderness and cold sensitivity. A classic test that can be performed on physical exam places the digit in ice water which usually reproduce pain within 60 sec. Occasionally, nail bed ridging and a small blue spot at the base of the nail can be seen. This tumor may be multiple in 25% of patients. Until recently, these tumors have been thought to be benign. Recently, there have been well documented cases of malignancy and the diagnostic criteria have been elucidated by pathologists.
OUTLINE
EPIDEMIOLOGY CHARACTERIZATION AGE RANGE-MEDIAN 30-50 years Females predominate
GROSS APPEARANCE/
CLINICAL VARIANTSCHARACTERIZATION FACIAL Facial “glomangiomas”: Large facial venous malformations with glomus cells
Charbel Mounayer, MD, etal.
Paris, France, and Boston, Massachusetts
J Am Acad Dermatol 2001;45:239-45 Abstract quote
Background: “Glomangiomas” are benign cutaneous vascular lesions consisting of convoluted, abnormally formed venous channels lined by cuboidal and oval epithelioid, -actin-positive, glomus cells. Three different clinical variants of glomangioma have been recognized: solitary, multiple, and nodular, or plaquelike. Inheritable forms are common.
Objective: We describe in 7 patients (2 of them having a familial glomangiomatosis) the rare facial location of glomangiomas to differentiate this type from common facial venous malformation (VM).
Methods: We analyzed clinical data (photographs), course, investigations (computed tomographic scans in 4 patients, magnetic resonance imaging in 6, arteriography in 2, direct puncture phlebography in 4, and pathologic examinations in all 7), and outcome with treatment.
Results: Lesions were soft, composed of multiple nodules, confluent and plaquelike, deep blue or blue-to-purple, sometimes sagging, one-sided in a cheek, extending to the lips in 5 patients, to the chin in 4, and to the lower eyelid in 4. They were poorly compressible, a finding different from common facial VMs. In a young man extensive back involvement was associated. Among radiologic investigations, only magnetic resonance imaging after gadolinium enhancement offered some differential features with common VMs. However, histopathologic examination clarified the differential diagnosis: although the large tortuous venous channels were reminiscent of capillary-venous malformation, in many vessels the walls contained one or several rows of glomus cells.
Conclusion: Multiple plaquelike facial “glomangiomas” mimic a common venous malformation because of their blue hue. However, with experience, one can clinically recognize them, and their pathologic aspect is distinctive. Management should differ slightly from that for common facial VM because sclerotherapy has proven to be less effective. Therefore surgical treatment is the only helpful therapeutic option.
GASTRO-
INTESTINALGastrointestinal Glomus Tumors
A Clinicopathologic, Immunohistochemical, and Molecular Genetic Study of 32 CasesMarkku Miettinen, M.D.; Edina Paal, M.D.; Jerzy Lasota, M.D.; Leslie H. Sobin, M.D.
From the Department of Soft Tissue Pathology (M.M., E.P., J.L.) and the Division of Gastrointestinal Pathology (L.H.S.), Armed Forces Institute of Pathology, Washington, DC, U.S.A.
Am J Surg Pathol 2002;26:301-311 Abstract quote Glomus tumors usually occur in the peripheral soft tissues, but similar tumors have also been reported in the stomach and occasionally in the intestines. However, the relationship of these tumors to peripheral glomus tumors and gastrointestinal stromal tumors has not been fully clarified because previous series of gastrointestinal glomus tumors predate availability of immunohistochemistry.
This clinicopathologic study examined 32 gastrointestinal glomus tumors. All but one of the tumors were located in the stomach and the remaining tumor was from the cecum. The tumors occurred with a strong female predominance (23 females and 9 males) and a median age of 55 years (range 19–90 years). The gastric tumors typically presented with gastrointestinal bleeding or ulcer-like symptoms, and 14 tumors had mucosal ulceration. Five tumors were incidental findings. The tumor sizes varied from 1.1 to 7 cm (median 2 cm), and most were located in the antrum. Histologically, the tumors typically had a solid pattern of sharply demarcated, round glomus cells with prominent, mildly dilated pericytoma-like vessels. Vascular invasion and focal atypia were relatively common (seen in 11 and 13 cases, respectively), and low mitotic activity (1–4 per 50 high power fields), was seen in 10 cases. Immunohistochemically, all tumors were positive for -smooth muscle actin and calponin, and nearly all had a net-like pericellular laminin and collagen type IV positivity. All tumors were negative for desmin and S-100 protein. Three tumors had focal synaptophysin positivity, but none was positive for chromogranin. All tumors lacked KIT expression and the GIST-specific mutations in the c-kit gene. Follow-up revealed one patient death of metastatic disease to liver at 50 months; this tumor had 1 mitosis per 50 high power fields, but had spindle cell foci, mild atypia, and vascular invasion. Thirteen patients were well and alive after long-term follow-up.
Gastrointestinal glomus tumors occur almost exclusively in the stomach, and they have a good overall prognosis, but a small, unpredictable potential for malignant behavior exists. These tumors are phenotypically similar to peripheral glomus tumors and differ from epithelioid GISTs.
KIDNEY
- Glomangiomyoma
(glomus tumor) of the kidney.
Siddiqui NH, Rogalska A,
Basil IS.
Department of Pathology, University of Illinois Medical Center, Chicago, IL 60612-7335, USA.
Arch Pathol Lab Med. 2005 Sep;129(9):1172-4. Abstract quote
We report herein a case of glomus tumor arising in the kidney of a 55-year-old woman, which was found incidentally on a computed tomographic scan. Partial nephrectomy revealed a 2-cm encapsulated mass that was architecturally similar to glomus tumor.
Immunohistochemistry showed positivity of tumor cells for vimentin and smooth muscle actin. On electron microscopy, cytoplasmic thin filaments and dense bodies were seen, confirming the smooth muscle nature of the tumor.
Glomus tumors arising in visceral organs are rare, and those arising in kidney are exceedingly rare.
- Glomus tumor of renal pelvis: A case report and review of the literature.
Herawi M, Parwani AV, Edlow D, Smolev JK, Epstein JI.
Hum Pathol. 2005 Mar;36(3):299-302. Abstract quote
Summary Glomus tumors are uncommon benign perivascular neoplasms that have rarely been described outside of their usual peripheral soft tissue sites.
We report a unique case of glomus tumor of the renal pelvis in a 53-year-old woman who presented with microscopic hematuria associated with obstruction of the ureteropelvic junction and marked hydronephrosis.
At initial gross examination, the tumor mimicked a urothelial carcinoma.PULMONARY AND MEDIASTINAL
Primary pulmonary glomus tumor with contiguous spread to a peribronchial lymph node.Zhang Y, England DM.
Ann Diagn Pathol. 2003 Aug;7(4):245-8. Abstract quote Glomus tumors are uncommon soft tissue tumors. Rare occurrences in visceral organs including the respiratory tract have been reported. The vast majority of these tumors are biologically benign.
We report a case of primary pulmonary glomus tumor with atypical features characterized by mild nuclear atypia, local infiltration, and contiguous spread to a peribronchial lymph node. The current literature is reviewed.Pulmonary and Mediastinal Glomus Tumors Report of Five Cases Including a Pulmonary Glomangiosarcoma: A Clinicopathologic Study With Literature Review
Erich M. Gaertner, M.D.; David M. Steinberg, M.D.; Monica Huber, M.D.; Tomayoshi Hayashi, M.D., Ph.D.; Nobuo Tsuda, M.D.; Frederic B. Askin, M.D.; Stephen W. Bell, M.D.; Ba Nguyen, M.D.; Thomas V. Colby, M.D.; Stephen L. Nishimura, M.D., Ph.D.; Markku Miettinen, M.D.; William D. Travis, M.D.
From the Walter Reed Army Medical Center (E.G.), Washington, DC, U.S.A.; the Armed Forces Institute of Pathology (W.T., M.M.), Washington, DC, U.S.A.; Johns Hopkins University (D.S., F.A., B.N.), Baltimore, Maryland, U.S.A.; Pathologisch–
Bakteriologisches Institut (M.H.), Vienna, Austria; Nagasaki University Hospital (T.H., N.T.), Japan; St. John's Hospital (S.B.), Springfield, Illinois, U.S.A.; Mayo Clinic Scottsdale (T.C.), AZ, U.S.A.; and the University of California at San Francisco (S.L.N.), CaliforniaAm J Surg Pathol 2000;24:1105-1114 Abstract quote
Pulmonary and mediastinal glomus tumors are rare lesions, with four previously reported primary pulmonary cases and three mediastinal cases.
The authors report one mediastinal glomus tumor, a locally infiltrative type, and four pulmonary glomus tumors, including the first case of primary pulmonary glomangiosarcoma.
These tumors show a variety of clinical and pathologic differences from the more common cutaneous variety, including later age at presentation, larger size, and more frequent atypical/malignant features.
Mediastinal and pulmonary glomus tumors both have an average patient age at presentation of 45 years. However, compared with their pulmonary counterparts, mediastinal glomus tumors are less common, more often symptomatic, and are larger (average size, 5.4 cm). Additionally, mediastinal glomus tumors more often demonstrate malignant or atypical features. Pulmonary glomus tumors average 3.3 cm in greatest dimension, with the majority measuring less than 2.5 cm. The pulmonary glomangiosarcoma presented was large, measuring 9.5 cm, and showed increased mitotic count (9 mitoses/10 high-power fields), necrosis, cytologic atypia, and was associated with disseminated disease.
Regardless of clinical symptoms, histologic features, and even metastases, the vast majority of all benign and malignant glomus tumors are indolent and cured surgically, with adjuvant therapy needed only for occasional patients with more advanced disease. The four patients with glomus tumors reported are currently alive and free of disease as of last follow up. The patient with the glomangiosarcoma developed widespread metastases and died of disease 68 weeks after initial therapy.
HISTOLOGICAL TYPES CHARACTERIZATION General Am J Surg Pathol 2001;25:1-12 VARIANTS MALIGNANT glomus tumor Deeply located tumors with a size of >2cm or atypical mitotic figures or moderate to high nuclear grade AND >/=5 mitotic figures/50 hpf
- Malignant glomus tumor: a case report and review of literature, focusing on its clinicopathologic features and immunohistochemical profile.
Khoury T, Balos L, McGrath B, Wong MK, Cheney RT, Tan D.
Department of Pathology, Roswell Park Cancer Institute, State University of New York at Buffalo, Buffalo, New York 14263, USA.
Am J Dermatopathol. 2005 Oct;27(5):428-31. Abstract quote
Malignant glomus tumor (MGT) is a rare, recently described neoplasm that recapitulates the appearance of the modified smooth cells of the normal glomus body.
We report a case of MGT of the hand of a 48-year-old woman. Magnetic resonance imaging (MRI) showed a 2.8-cm, well circumscribed, enhancing mass on the volar aspect of the thenar region of the right hand in immediate continuity with the ulnar artery and nerve. Computed tomography scan (CT-Scan) of the chest was normal.
Histologic evaluation revealed a multilobular lesion with prominent branching capillary vasculature and perivascular arrangement of sheets of tumor cells. The tumor cells were round, relatively uniform in size with distinct cell borders and perinuclear cytoplasmic clearing. They were of intermediate to high nuclear grade and showed significant mitotic activity. A wide local excision with negative margins was performed. Multiple lung metastases were evident at 8-month follow-up. To date, forty-five cases of MGT of skin and soft issue have been reported in the literature. Twelve of the forty-five cases developed metastasis.
In this report, we emphasize the differential diagnosis of MGT in the skin and deep soft tissue.SYMPLASTIC glomus tumor Tumors with high nuclear grade in the absence of any other malignant feature Glomus tumor of uncertain malignant potential Tumors that lack criterai for malignant glomus tumor or symplastic glomus tumor but have high mitotic activity and superficial location only, or large size only, or deep location only GLOMANGIOMA
- Glomuvenous malformation (glomangioma) and venous malformation: distinct clinicopathologic and genetic entities.
Boon LM, Mulliken JB, Enjolras O, Vikkula M.
Division of Plastic Surgery, Center for Vascular Anomalies, Cliniques Universitaires Saint-Luc.
Arch Dermatol. 2004 Aug;140(8):971-6. Abstract quote
Objectives To develop clinical criteria that permit clinical distinction between inherited glomuvenous malformation (GVM), known as glomangioma, and inherited cutaneomucosal venous malformation and to test these criteria on sporadic lesions.
DESIGN: Clinical data were compiled for 1685 patients with inherited or sporadic cutaneous venous anomalies. Based on a cohort of patients with a mutation in the TIE2 or glomulin gene or a histologic diagnosis, we defined clinical criteria for inherited GVM and cutaneomucosal venous malformation. We then applied these criteria to sporadic cases in a blinded manner and genetically or histologically confirmed this clinical diagnosis whenever possible.
RESULTS: Glomuvenous malformations accounted for 5.1% of venous anomalies and were frequently inherited (63.8%), whereas venous malformations were rarely familial (1.2%). Glomuvenous malformations were nodular and scattered, or plaque-like and segmental, with color varying from pink to purplish dark blue, whereas most venous malformations (VMs) were soft, blue, and often localized vascular lesions. Glomuvenous malformations were mainly located on the extremities and involved skin and subcutis, whereas VMs commonly affected muscles and joints (P<.001). Glomuvenous malformations had a distinct raised, often hyperkeratotic cobblestone-like appearance and could not be completely emptied by compression, unlike VMs. Glomuvenous malformations were painful by compression, whereas VMs were painful on awakening, after activity, or with hormonal changes. Elastic compressive garments aggravated pain in GVMs, in contrast to VMs.
CONCLUSIONS: This large series of patients with superficial venous anomalies established clinical features that distinguish VMs and GVMs. This differential diagnosis is essential, as the outcome and the treatment for GVMs differ.GLOMANGIO-
MATOSISTumors with histologic features of diffuse angiomatosis and excess glomus cells Glomangiomatosis.
Jalali M, Netscher DT, Connelly JH.
Department of Pathology and Laboratory Medicine, University of Texas-Houston Medical School, Houston; the Division of Plastic Surgery, Baylor College of Medicine, Houston; and the Department of Pathology, St Luke's Episcopal Hospital, Houston, TX.
Ann Diagn Pathol 2002 Oct;6(5):326-8 Abstractq quote Neoplasms containing glomus cells are uncommon. Glomus cells within an angiomatosis, so called glomangiomatosis, is exceedingly rare with only three previously reported cases.
We are describing the fourth case from a 17-year-old boy which involved his left hand, fingers, and distal forearm. We will review the previously reported cases.
SCLEROTIC
Sclerotic glomus tumor.
Vigovich FA, Hurt MA, Santa Cruz DJ.British Hospital of Buenos Aires, Buenos Aires, Argentina.
Am J Dermatopathol. 2010 Feb;32(1):76-8. Abstract quote
We report an unusual histopathological variant of a glomus tumor that arose in a peculiar topographic site, a sclerotic glomus tumor.
Unlike conventional glomus tumors or glomangiomas that have a loose fibrous stroma with variable hyaline and myxoid changes, the case reported herein had a diffuse, hyalinized, sclerotic stroma. A further difference was that the majority of glomus tumors and glomangiomas occur in the subungual area, trunk, or extremities, whereas the present tumor occurred on the ear. Due to the peculiar histological features and location, other tumors were considered in the differential diagnosis to include Merkel cell carcinoma, primitive neuroectodermal tumor, and small cell melanoma.
This article illustrates a unique variant of a glomus tumor, which to our knowledge has not been previously described.
DIFFERENTIAL DIAGNOSIS KEY DIFFERENTIATING FEATURES GLOMUS COCCYGEUM
- Glomus Coccygeum: Report of a Case and Review of the Literature.
Rahemtullah A, Szyfelbein K, Zembowicz A.
From the *Dermatopathology Unit, Department of Pathology, Massachusetts General Hospital and Massachusetts Eye and Ear Infirmary, Boston, Massachusetts; and daggerHarvard Medical School, Boston, Massachusetts.
Am J Dermatopathol. 2005 Dec;27(6):497-499. Abstract quote
The glomus coccygeum is a vestigial structure related to the canals of Sucquet-Hoyer, an arteriovenous anastomosis surrounded by glomus cells derived from modified smooth muscle and involved in thermoregulation. It is an incidental finding in specimens from the sacral area and may represent a diagnostic challenge to the unaware observer.
We present a case of a glomus coccygeum, presenting as a 1.5-mm structure adjacent to a typical pilonidal cyst excised from a 7-month-old boy, which was the subject of a second opinion consultation. The lesion showed small to medium sized clusters of predominantly epithelioid cells with moderate amounts of clear to eosinophilic cytoplasm, intercellular borders, and plump, round nuclei with fine chromatin. These cells were closely associated with small vascular channels and nerves. Immunohistochemistry revealed that the epithelioid cells expressed vimentin, muscle-specific actin, neuron-specific enolase, and S-100 protein, were weakly positive for smooth muscle actin, and negative for desmin, synaptophysin, and chromogranin. The endothelial cells of the vascular channels were antibody CD31 positive.
Recognition of the histologic features of glomus coccygeum is important to avoid confusion with glomus tumor and neural or smooth muscle neoplasms in the sacral area.HEMANGIOPERI-CYTOMA
Myofibromatosis in adults, glomangiopericytoma, and myopericytoma: a spectrum of tumors showing perivascular myoid differentiation.Granter SR, Badizadegan K, Fletcher CD.
Department of Pathology, Brigham & Women's Hospital and Harvard Medical School, Boston, Massachusetts 02115, USA.
Am J Surg Pathol 1998 May;22(5):513-25 Abstract quote The clinicopathologic features of 24 tumors showing perivascular myoid differentiation are described. These included tumors with histologic features of "infantile-type" myofibromatosis occurring in adult patients (8 cases), tumors with composite features of "hemangiopericytoma" and glomus tumor (9 cases), and tumors with a distinctive concentric perivascular proliferation of spindle cells (7 cases).
Evidence of morphologic overlap among these groups suggests they are closely related neoplasms that form a single spectrum. Age of patients with lesions resembling infantile-type myofibromatosis ranged from 23 to 67 years (median, 37 years).
Clinicopathologic manifestations of this disease included multicentricity (4 cases), local recurrence (3 cases), persistence of congenital lesions into adulthood (4 cases), and tumors that were multifocal within the confines of one anatomic region (7 cases). Histologically, all cases showed a biphasic pattern that consisted of fascicles of spindle cells with abundant eosinophilic cytoplasm that resembled smooth muscle, in addition to a population of more primitive spindled cells associated with a hemangiopericytomalike vascular pattern. Six cases showed reversal of the typical zonation seen in pediatric cases in that the primitive component surrounded the more mature fascicular areas. Also described are nine tumors with features that are intermediate between glomus tumor and hemangiopericytoma, which we have designated glomangiopericytoma.
These tumors are characterized by prominent branching vessels lined by a single row of endothelial cells surrounded by epithelioid cells with a glomoid appearance. In other areas, the tumors showed typical hemangiopericytomatous foci similar to those in the myofibromatosis cases. The principal points of distinction were a lack of myoid nodules and an absence of small primitive cells with basophilic cytoplasm. Ages of these patients ranged from 17 to 78 years (median, 35 years). All tumors were located in the subcutaneous tissue and the superficial soft tissue of the extremities. Recurrence developed in one of six patients with follow-up information. The recurrent tumor had features of angiomatoid malignant fibrous histiocytoma.
Finally, we describe a subset of tumors characterized by concentric periluminal proliferation of bland, round to ovoid cells, which we have designated as myopericytoma. Patient age ranged from 10 to 66 years (median, 40 years). All were located in subcutaneous and superficial soft tissue of distal extremities. One patient had two recurrences in 3 years after initial excision.
Our study suggests that these three lesional groups comprise a histologic continuum of tumors that share clinical similarities and that, perhaps, are designated more appropriately as perivascular myomas. The relationship of this family of tumors to so-called hemangiopericytoma is discussed.
Leiomyosarcoma with epithelioid change Rhabdomyosarcoma Ewing's sarcoma/PNET Nodular hidradenoma
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