Effective surgical treatment of progressive nodular histiocytosis

Nakayashiki, Norihisa; Akita, Hirotaka; Mori, Wakako; Watanabe, Daisuke; Sakai, Yoshie; Yoshimura, Y.

doi:10.3109/2000656x.2012.748323

Cited by 6 publications

(5 citation statements)

References 10 publications

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“…However, such a bulky mass as in the present case has not been reported previously. Because surgical resection of selected nodules is thought to be the only therapeutic option in PNH (8), this option was chosen for the bulky mass for cosmetic reasons.…”

Section: Discussionmentioning

confidence: 99%

Progressive Nodular Histiocytosis with Large Nodules and a Bulky Mass

Kiyohara

Makimura²,

Miyamoto³

et al. 2019

Acta Derm Venerol

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Section: Discussionmentioning

confidence: 99%

Progressive Nodular Histiocytosis with Large Nodules and a Bulky Mass

Kiyohara

Makimura²,

Miyamoto³

et al. 2019

Acta Derm Venerol

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“…It has been proposed that spindle cell xanthogranuloma is the solitary counterpart of PNH; presenting as yellow-brown papules or nodules on the head, trunk, and extremities of younger patients (20 to 40 years of age). [416][417][418][419] Xanthoma disseminatum. XD is a rare disease in the JXG family that typically occurs in young adults with a male to female ratio of 2:1.…”

Section: Jxg Family Of Disordersmentioning

confidence: 99%

Cutaneous Hematolymphoid and Histiocytic Proliferations in Children

Gru

Dehner

2018

Pediatr Dev Pathol

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This article focuses on cutaneous hematopoietic neoplasms that are more likely to be encountered in the pediatric age-group and includes both lymphoproliferative and histiocytic disorders. The cutaneous hematologic disorders in children have a different epidemiologic profile to what is seen during adulthood. Although mycosis fungoides is the most frequent form of cutaneous lymphoma in adults, it is very rare in children. Because lymphoblastic leukemias and lymphomas are more frequent in the pediatric setting, cutaneous leukemic infiltrates are relatively common in this age-group. Similarly, histiocytic disorders are more common in children, particularly Langerhans cell histiocytosis and juvenile xanthogranuloma. Notably, the histiocytic disorders have undergone significant modifications on their nomenclature in the basis of the molecular characteristics that are present in them. A summary of the most frequent cutaneous hematopoietic disorders in children will be discussed further in this review.

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“…Current treatment for PNH is difficult and mostly unsuccessful ( 4 , 11 ). Previous attempts to treat the disease have included surgical/physical resection and chemotherapy (prednisolone, vincristine, and cyclophosphamide), but have proven to be unsuccessful ( 12 , 13 ).…”

Section: Discussionmentioning

confidence: 99%

Treatment of progressive nodular histiocytosis: a case report

Hua¹,

Jiang²,

Cheng³

et al. 2022

Ann Transl Med

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Background: Progressive nodular histiocytosis (PNH) is an extremely rare type of non-Langerhans cell histiocytosis, characterized by the emergence of hundreds small to large cutaneous papulonodules without spontaneous remission. It can be life-threatening if pharyngeal and laryngeal mucosa were involved, just as we reported in this case. Disfigurement and disabling are common, whereas current treatment options are of limited efficacy. At present, about 20 reported cases can be found through a PubMed search, fewer with treatment options. In this article, we report a unique case of a patient diagnosed with PNH, in whom regorafenib had a remarkably curative effect.Case Presentation: We present the case of a 21-year-old man who developed lesions on his face, trunk, limbs, and exceptionally in the pharyngeal and laryngeal mucosa. Immunohistochemistry showed diffuse CD68 positivity and scattered S-100 positivity, while CD1α, smooth muscle actin (SMA) and B-Raf protooncogene, serine/threonine kinase (BRAF) were negative. Histopathological and biochemical examinations confirmed the diagnosis of PNH. The patient underwent facial lesion resections; however, the lesions recurred rapidly within 1 month. In December 2019, treatment with a small multi-kinase inhibitor, regorafenib (120 mg daily for 3 weeks on, and 1 week off), was initiated, and the patient's progress was monitored. After 10 days of administration, the patient's facial lesions began to gradually alleviate, and after 1 month, the lesions in the trunk, limb, and especially the face continued to subside even further. In 2021, the regorafenib dose was subsequently adjusted to 40 mg daily, with intermittent administration. No abnormalities were observed in the routine blood tests, liver, and kidney function results during the followups to date. Presently, the patient's overall condition is good, the lesions are gradually improving, and the patient has returned to normal life and work.Conclusions: This clinical report supports the future exploration of regorafenib treatment in patients with PNH.

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Effective surgical treatment of progressive nodular histiocytosis

Cited by 6 publications

References 10 publications

Progressive Nodular Histiocytosis with Large Nodules and a Bulky Mass

Progressive Nodular Histiocytosis with Large Nodules and a Bulky Mass

Cutaneous Hematolymphoid and Histiocytic Proliferations in Children

Treatment of progressive nodular histiocytosis: a case report

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