Case of multiple cutaneous granular cell tumors

Granular cell tumor is an uncommon, benign tumor, which mainly occurs on the skin, tongue and oral cavity as a single nodule. Multiple granular cell tumors are rare, with the incidence reported to vary from 7–29% in adult cases of the tumor. We describe a case of multiple cutaneous granular cell tumors in the right lumber and back regions along with a brief review of the published work on multiple cutaneous granular cell tumors.     

Multiple Granular Cell Tumors in a Child with Noonan Syndrome

Abstract: Granular cell tumors are benign neurally derived neoplasms, involving cutaneous and subcutaneous tissues; and typically occur as solitary lesions. Multiple granular cell tumors occur in 10% of affected individuals, but are in children. Children with underlying somatic and genetic syndromes, including neurofibromatosis and Noonan syndrome, appear to be at higher risk of developing multiple granular cell tumors. Skin biopsy assists in diagnosis, since granular cell tumors have a similar appearance to other cutaneous nodules. Painful or rapidly growing granular cell tumors should be excised and asymptomatic non‐growing granular cell tumors may be observed. Children with multiple granular cell tumors should have a complete physical examination to rule out an underlying genetic syndrome.     

Multiple cutaneous granular cell tumors of the scrotum

A 55-year-old black man with two firm intradermal nodules of the scrotum is presented. On histopathologic examination, the nodules were determined to be cutaneous granular cell tumors. This was confirmed by strong immunohistochemical reactivity to S-100 protein. There are no previous reports of multiple granular cell tumors of the male genitalia. Only one report of a solitary granular cell tumor of the scrotum appears in the literature.     

Multiple cutaneous granular cell tumors, joint hypermobility and mild facial dysmorphism in a child

BACKGROUND: The association of multiple cutaneous granular tumors with systemic defects is extremely rare. To date, 14 cases have been described. CASE REPORT: A 14-year-old boy presented multiple nodular lesions on his arms and legs; facial dysmorphism and joint hypermobility were also present. Histopathologic examination of two nodules showed granular cell tumors. We review the literature regarding multiple granular cell cutaneous tumors in children with associated somatic defects and genetic syndromes. CONCLUSIONS: The combination of multiple cutaneous granular tumors with alterations in other organs is not only fortuitous, justifying the proposal of a distinct syndrome, termed "Bakos's syndrome" after the author who first described this association. The relation with neurofibromatosis type 1 is discussed, and an association is suggested with the range of variants of this syndrome.     

Keloidartiger Granularzelltumor

A 27-year-old man presented with multiple granular cell tumors of the skin. One tumor presented clinically as a dermatofibrosarcoma or a spontaneous keloid. Histologically, both this tumor and another cutaneous lesion showed striking peritumoral fibrosis. Granular cell tumor should be included in the differential diagnosis of keloidal lesions. The tumors should be removed completely and patients then examined regularly to detect new tumors in a timely fashion.     

Multiple cutaneous granular-cell tumors

A 54-year-old woman presented with subcutaneous nodules and a prior diagnosis of multiple, cutaneous granular-cell tumors. The first case of granular cell tumor was reported in 1854. It remains a rare tumor, which is most commonly seen in women. One-half are usually located in the head and neck region, mostly on the tongue. Approximately one third are cutaneous and 25 percent of the cases occur as multiple lesions.     

Multiple cutaneous granular cell tumors and neurofibromatosis in childhood. A case report and review of the literature

Multiple cutaneous granular cell tumors have been previously reported in only 26 children or adolescents. An association of these tumors with neurofibromatosis has never been reported previously. We describe a 12-year-old black girl with multiple cutaneous granular cell tumors and neurofibromatosis. Although the histogenesis of these tumors is not completely clear, the findings of ultrastructural and immunohistochemical evaluation of our patient's tumors and the associated neurofibromatosis support a neural crest origin for granular cell tumors.     

Primitive nonneural granular cell tumor (so-called atypical polypoid granular cell tumor). Report of 2 cases with immunohistochemical and ultrastructural correlation

Primitive nonneural granular cell tumors (so-called atypical cutaneous granular cell tumors) were first described in 1991, followed by few case reports, and 2 recent larger series. We report here 2 additional cases in 2 women aged 73 and 74, who presented with 0.6- and 0.4-cm skin nodules on the right side of the jaw and the forearm, respectively. Biopsies showed cutaneous granular cell neoplasms with epithelioid morphology. The cells exhibited nuclear pleomorphism and brisk mitotic activity with atypical mitoses. Immunohistochemically, the tumor cells expressed vimentin and PGP 9.5 but lacked S-100 and CD34 expression. Ultrastructurally, both cases showed primitive cells packed with large secondary lysosomes. Primitive nonneural granular cell tumors seem to consist of neoplastic proliferating cells that fail to break down uncharacterized cellular material within the lysosomes. They are, however, different from classic granular cell tumor by lacking neural differentiation. Despite reported worrisome cellular atypia, these rare tumors seem to pursue a favorable outcome.     

Plexiform pattern in cutaneous granular cell tumors

Granular cell tumors, considered to be of peripheral nerve sheath origin, can involve various parts of the body with skin and tongue being the most common organs involved. In the skin, the granular cell tumors usually form an ill-defined mass of round to oval cells with abundant eosinophilic granular cytoplasm and central or eccentric bland nuclei. Here we present nine cases of granular cell tumors, which display features different from the classical cutaneous granular cell tumor and have features similar to the earlier described plexiform granular cell tumors. Plexiform pattern in granular cell tumors may be more common than is reported.     

Solitary cutaneous histiocytosis with granular cell changes: a morphological variant of reticulohistiocytoma?

We first report a case of granular cell histiocytosis occurring as a solitary polypoid lesion of the nipple in a 15‐year‐old girl. Histologically, the lesion was composed of a dermal population of medium‐ to large‐sized, short spindle‐ to round‐ to epithelioid‐shaped cells with eosinophilic cytoplasm containing numerous and small diastase‐resistant periodic acid‐Schiff (PAS) positive granules. No associated inflammatory cells were observed. Immunohistochemical studies, revealing immunoreactivity exclusively to vimentin and CD68, were consistent with their histiocytic profile. Based on clinical, morphological and immunohistochemical features, the diagnosis of ‘solitary cutaneous histiocytosis with granular cell changes’ was proposed. The absence of an inflammatory cell component, such as lymphocytes and leucocytes, along with no history of a previous trauma or medical treatment, suggest that the present lesion could fit into the morphological spectrum of the so‐called solitary epithelioid histiocytoma, also known as reticulohistiocytoma. Alternatively, the possibility of a histiocytic reaction to unknown stimuli cannot be completely ruled out. Nevertheless, awareness of solitary cutaneous histiocytosis with granular cell changes is useful to avoid confusion with other dermal tumors, especially ‘granular cell tumor’ and ‘dermal non‐neural granular cell tumor’. Caltabiano R, Magro G, Vecchio GM, Lanzafame S. Solitary cutaneous histiocytosis with granular cell changes: a morphological variant of reticulohistiocytoma?     

MULTIPLE CUTANEOUS GRANULAR CELL TUMORS WITH SYSTEMIC DEFECTS: A DISTINCT ENTITY?

Background. The association between cutaneous granular cell tumors and systemic defects Is extremely rare, this being the tenth case reported in the literature. The reported defects in the literature include lentiginosis, face and skull alterations, heart defects, muscular and neural pathologies, among other sporadic defects in other organs. The patients do not present visceral granular cell tumors. Case Report. An 11year-old girl had 124 skin granular cell tumors associated with face and skull defects, pulmonary stenosis, EEG and other neurologic alterations, diffuse muscle hypotonia, and an excess of finger joint mobility. Conclusions. The combination of multiple cutaneous granular cell tumors with repeated alterations in other organs seems to be more than simply coincidental, thus suggesting a syndromic separate entity.     

Dermal non‐neural granular cell tumor in a 3‐year‐old child

Dermal non‐neural granular cell tumors, also known as primitive polypoid granular cell tumors, are a rare group of distinct cutaneous non‐neural granular cell tumors. Pediatric cases are rare, and to the best of our knowledge, we report the youngest patient with dermal non‐neural granular cell tumors.     

Multiple Granular Cell Tumors and Growth Hormone Deficiency in a Child

Granular cell tumor is an uncommon benign tumor occurring on the skin as a single nodule. Multiple tumors are very rare, particularly in children. We describe a child with multiple granular cell tumors on the skin in association with growth hormone deficiency. The occurrence of multiple granular cell tumors in association with other clinical manifestations in childhood is discussed.     

Multiple cutaneous granular cell tumors: case report of a 19-year-old African American female

BACKGROUND AND OBJECTIVE: This is a somewhat rare case of a 19-year-old African American female with multiple cutaneous granular cell tumors. Granular cell tumors are of neural origin, except in rare cases, and are considered benign, with a low incidence of malignancy. The clinical presentation varies greatly, but these tumors are most commonly painful and slow growing, with two-thirds occurring on the head and neck. Patients are most commonly in their second to fourth decades of life, two-thirds are black, and two-thirds are women. Granular cell tumors are diagnosed by the characteristic pathologic findings of polygonal cells with eosinophilic granular cytoplasm. CONCLUSION:These tumors are most commonly singular but can be multiple in 10 to 15% of patients. Older patient age, rapid growth or enlargement, and a history of local recurrence should raise concern for malignant behavior. The distribution and family history in this case are suggestive of possible mosaicism.     

Multiple lesions of granular cell basal cell carcinoma: a case report

Granular cell change in basal cell carcinoma (BCC) occurs rarely. Only 11 such cases have been reported; all of them were solitary nodular BCC. We report herein a case of multiple granular cell BCC with infundibulocystic features. The tumors presented as papules on the anterior neck of a 44‐year‐old female with a prior history of a well‐differentiated squamous cell carcinoma (SCC) of the tongue and radiation involving the area in which BCC developed. Microscopically, the tumors were circumscribed small dermal nodules composed of epithelial cords with granular eosinophilic cytoplasm and entrapped infundibular keratocysts. Given the eosinophilic appearance of the tumor, history of SCC and the lesions multiplicity, the initial biopsy was first interpreted as metastatic SCC. The correct diagnosis of granular cell BCC was established upon rereview of the slides at a cancer center. Given the diagnostic controversy, immunohistochemical stains were performed. The tumor cells expressed Ber‐EP4, CD63 (NKI/C3) and CD68. The tumors were compared to the prior SCC finding different morphologies. Extensive clinical evaluation showed no evidence of recurrent SCC. This report expands the clinicopathologic spectrum of granular cell variant of BCC and documents for the first time eruption of multiple such tumors in a localized area.     

Cutaneous plasmacytomas. A review and presentation of an unusual case.

Recent reviews separate four types of plasma cell tumor: multiple myeloma, extramedullary plasmacytoma (without multiple myeloma), solitary myeloma of bone, and plasma cell leukemia. Cutaneous plasma cell tumors may arise from lymphatic or vascular spread of tumor (metastatic cutaneous plasmacytoma) or by direct extension from bone lesions. The former, metastatic cutaneous plasmacytomas, are quite rare. Specific malignant plasmacyte cutaneous tumors can also be seen in extramedullary plasmacytoma, solitary myeloma of bone, and plasma cell leukemia. We present a patient with multiple myeloma and lymphedema of the right arm, who developed a pathologic fracture of the right humerus and subsequently developed numerous metastatic cutaneous plasmacytomas localized to the lymphedematous arm. Direct immunofluorescence of frozen sections and enzymatically released cells from tumor nodule failed to reveal cell-associated immunoglobulins.     

An infiltrative variant of non-neural granular cell tumor: a case report

Dermal non-neural granular cell tumors are rare tumors of indeterminate lineage that typically present as well-circumscribed tumors with nuclear pleomorphism and mitotic activity. We describe a dermal non-neural granular cell tumor with a distinctive growth pattern with granular cells interspersed between collagen bundles. This asymptomatic papule arose on the scapula of a 46-year-old woman and consisted of a mixture of epithelioid and spindled granular cells. The immunohistochemical characteristics were similar to those of previously reported dermal non-neural granular cell tumors. Despite mild nuclear pleomorphism and dispersion of lesional cells among collagen bundles, mitoses were not present and Ki-67 staining indicated a low proliferative rate. In addition to being S-100 protein negative and NKI/C3 positive, our case was positive for PGP9.5 and weakly positive for neuron-specific enolase, a staining pattern similar to what has been observed for cellular neurothekeomas. Our case could represent a dermal non-neural granular cell tumor with unique architecture, a granular cellular neurothekeoma or a granular cell dermatofibroma. As both dermal non-neural granular cell tumor and cellular neurothekeoma are of indeterminate lineage, our case with features characteristic of both entities may suggest a common precursor or lineage for dermal non-neural granular cell tumor and cellular neurothekeoma.     

New or unusual dermatopathology tumors: a review

As experience is acquired, there is a constant evolution in both terminology and understanding of various relatively newly described tumors in the realm of dermatopathology. Several mesenchymal tumors of the lower extremity have undergone various changes in nomenclature, molecular discoveries, and histologic grading. Examples include hemosiderotic fibrohistiocytic lipomatous lesion/pleomorphic hyalinizing angiectatic tumor; superficial acral fibromyxoma; and myxoinflammatory fibroblastic sarcoma. Primary cutaneous myoepithelioma is also a relatively newly described entity for which grading and classification continue to evolve. Finally, even our understanding of the classic granular cell tumor has expanded to include a non-neural variant. This article reviews the current nomenclature, emerging concepts, and differential diagnosis of these evolving entities.     

Tumor de células granulares. Estudio clínico de 81 pacientes

IntroductionAlthough a granular cell tumor (GCT) usually develops on the skin or oral mucosa, it has been described in many other organs. GCT typically presents as a solitary tumor, but multiple lesions can occur. It has also been described in association with other diseases.ObjectivesTo describe the clinical characteristics of cutaneous and oral mucosal GCTs and explore potential associations with other diseases.Material and methodsRetrospective study of patients diagnosed with GCT at our hospital between 1995 and 2019. The following information was collected from the patients’ medical records: age, sex, number of GCTs, location, diameter, time to diagnosis, tentative clinical diagnosis, surgical margin status, recurrence, follow-up time, and associated diseases.ResultsWe detected 89 cutaneous or oral mucosal GCTs in 81 patients (43 women, 38 men) with a mean age of 40.21 years. The mean tumor diameter was 1.34 cm. Five of the 81 patients (6.2%) had multiple GCTs, including noncutaneous tumors. Patients with multiple GCTs were on average younger than those with a single tumor (P = .004). There was only a single case of local recurrence and no cases of distant metastasis. None of the patients had associated diseases.ConclusionsMost GCTs are benign and local recurrence is uncommon, even in patients with positive margins. Nevertheless, the possibility of multiple tumors affecting the skin, oral mucosa, or internal organs should be borne in mind, especially in young patients.