scholarly journals Diagnosis of Primary Langerhans Cell Histiocytosis of the Vulva in a Postmenopausal Woman

2013 ◽  
Vol 2013 ◽  
pp. 1-3
Author(s):  
Sefa Kurt ◽  
Mehmet Tunc Canda ◽  
Aycan Kopuz ◽  
Dudu Solakoglu Kahraman ◽  
Abdullah Tasyurt
Keyword(s):  
Differential Diagnosis ◽  
Postmenopausal Woman ◽  
Rare Disease ◽  
Genital Tract ◽  
Langerhans Cell Histiocytosis ◽  
Treatment Options ◽  
Female Genital Tract ◽  
Langerhans Cell ◽  
Pathologic Diagnosis ◽  
Female Genital

Langerhans cell histiocytosis (LCH) is a very rare disease of female genital tract, most commonly seen in vulva and unusual in postmenopausal period. Herein, we report the 8th case of pure vulvar LCH in a postmenopausal woman. We pay attention to the differential diagnosis in postmenopausal state, features of pathologic diagnosis, and treatment options.

Langerhans cell histiocytosis of the female genital tract: A literature review

2003 ◽  
Vol 13 (3) ◽  
pp. 381-388 ◽  
Author(s):  
A. J. Montero ◽  
C. M. Diaz-Montero ◽  
A. Malpica ◽  
P. T. Ramirez ◽  
J. J. Kavanagh
Keyword(s):  
Literature Review ◽  
Genital Tract ◽  
Langerhans Cell Histiocytosis ◽  
Female Genital Tract ◽  
Langerhans Cell ◽  
Female Genital

scholarly journals Langerhans cell histiocytosis of the female genital tract

Cancer ◽  
1991 ◽  
Vol 67 (6) ◽  
pp. 1650-1660 ◽  
Author(s):  
Constantine A. Axiotis ◽  
Maria J. Merino ◽  
Paul H. Duray
Keyword(s):  
Genital Tract ◽  
Langerhans Cell Histiocytosis ◽  
Female Genital Tract ◽  
Langerhans Cell ◽  
Female Genital

Primary ovarian synovial sarcoma

2021 ◽  
Vol 14 (11) ◽  
pp. e244756
Author(s):  
Muhammad Amin ur Rahman ◽  
Khalid Al Wadi ◽  
Al Nuqaydan ◽  
Raghad Tallab
Keyword(s):  
Differential Diagnosis ◽  
Synovial Sarcoma ◽  
Molecular Pathology ◽  
Genital Tract ◽  
Spindle Cell ◽  
Female Genital Tract ◽  
Rare Occurrence ◽  
Spindle Cell Tumours ◽  
Spindle Cell Tumour ◽  
Female Genital

Synovial sarcoma (SS) has a rare occurrence in the female genital tract. Only three prior reports of primary ovarian sarcoma could be retrieved after a thorough literature review. We are reporting a case of primary ovarian SS in a young woman. The tumour showed monophasic spindle cell morphology, and there was a wide list of differential diagnosis to consider. We confirmed the diagnosis by cytogenetics Flourescent Insitu Hybridisation (FISH) technique to identify the classical translocation. The diagnosis of this disease can be challenging especially if the tumour is of monophasic type. Morphology and immunohistochemistry are not enough to confirm the diagnosis in many cases. A confirmatory molecular pathology test is paramount. We have discussed the differential diagnosis of spindle cell tumours in ovary. We suggest that SS should be in the differential diagnoses when facing any atypical spindle cell tumour in the ovary. Molecular pathology techniques can help to confirm the diagnosis.

Adult Extrarenal Wilms Tumor Occurring in the Uterus

2001 ◽  
Vol 125 (8) ◽  
pp. 1081-1083 ◽  
Author(s):  
Ronald S. Muc ◽  
Wayne Grayson ◽  
Johannes J. Grobbelaar
Keyword(s):  
Differential Diagnosis ◽  
Genital Tract ◽  
Wilms Tumor ◽  
Female Genital Tract ◽  
Age Group ◽  
Important Differential Diagnosis ◽  
Nephrogenic Rests ◽  
Mullerian Tumor ◽  
Glomeruloid Structures ◽  
Female Genital

Abstract Five previous cases of extrarenal Wilms tumor (EWT) occurring in the uterus have been reported. The oldest patient was 22 years. We report a case of uterine EWT occurring in a 42-year-old woman. Histologically, there was typical triphasic differentiation, including epithelial, blastemal, and mesenchymal elements. The important differential diagnosis in this age group, the malignant mixed mullerian tumor, is excluded by the absence of glomeruloid structures and primitive tubules. The exact histogenesis of EWT is unknown but most likely relates to the presence of nephrogenic rests occurring in the female genital tract.

Treatment of recurrent Langerhans cell histiocytosis of the vulva with lenalidomide.

2013 ◽  
Vol 31 (15_suppl) ◽  
pp. e16555-e16555 ◽  
Author(s):  
Ibrahim Fuad Ibrahim ◽  
Harris V. K. Naina
Keyword(s):  
Rare Disease ◽  
Langerhans Cell Histiocytosis ◽  
Symptom Control ◽  
Female Genital Tract ◽  
Disease Recurrence ◽  
Langerhans Cell ◽  
Effective Treatment Option ◽  
Distant Disease ◽  
First Line Therapy

e16555 Background: Langerhans cell histiocytosis (LCH) of the female genital tract is an extremely rare disease. We report a 14 year follow up of a patient with LCH successfully treated with lenalidomide in the relapsed setting. Methods: N/A. Results: A 43 year old Ethiopian woman initially presented to another institution with a nodular lesion on her left vulva with biopsy consistent with LCH. Metastatic workup at the time did not reveal evidence of distant disease. The patient was initially treated with radiotherapy to the vulva and 2 years later she was diagnosed with recurrent disease in the vulva and underwent a wide local excision. Six months afterwards she developed a lesion on her right labium majus consistent with disease recurrence. She was treated with radiotherapy again and underwent a wide radical vulvar excision. She recurred only 3 months later and was started on salvage therapy with thalidomide, 3 years after the initial diagnosis. Within 2 months of starting thalidomide therapy, the patient experienced resolution of vulvar lesions and symptoms. She remained symptom free for 8 years while on thalidomide but then presented to our institution with a new central vulvar lesion after being off therapy for 4 months. Vulvar biopsy confirmed the presence of LCH. She was subsequently restarted on thalidomide and achieved symptom control for an additional 18 months before developing worsening pain and neuropathy associated with therapy. Imaging revealed no evidence of metastatic disease. We then started lenalidomide therapy at dose of 10 mg daily for 21 days in a 28 day cycle and subsequently increased the dose to 25 mg daily continuously. She achieved marked improvement in vulvar symptoms within a month of initiation of lenalidomide. She has tolerated therapy well with no neuropathy or cytopenias. Conclusions: Primary vulvar LCH is a rare disease with no standard therapies. Thalidomide has been described in the literature as an effective treatment option. We describe the use of lenalidomide as an alternative and well tolerated therapy in the relapsed setting. Further investigation is required to determine whether lenalidomide is a viable first line therapy for this rare disease.

Pulmonary Langerhans’ cell histiocytosis

2020 ◽  
pp. 4256-4257
Author(s):  
S. J. Bourke
Keyword(s):  
Bone Marrow ◽  
Dendritic Cells ◽  
Genital Tract ◽  
Langerhans Cells ◽  
Langerhans Cell Histiocytosis ◽  
Female Genital Tract ◽  
Cyst Formation ◽  
Langerhans Cell ◽  
Pulmonary Langerhans Cell Histiocytosis ◽  
Systemic Symptoms

Pulmonary Langerhans’ cell histiocytosis is characterized by a reactive monoclonal proliferation of activated histiocytes in the distal bronchioles, resulting in inflammatory nodules, cyst formation, and fibrosis. Langerhans’ cells are a particular type of histiocyte derived from dendritic cells in the bone marrow. They normally migrate in the blood to the squamous epithelium of the skin, lungs, gastrointestinal, and female genital tract, where they are involved in antigen presentation to T cells. It presents with cough, breathlessness, and (sometimes) systemic symptoms. Chest radiography and CT typically show nodules which then cavitate and may rupture, causing pneumothorax. Corticosteroids and/or cytotoxic drugs are of some benefit, and lung transplantation is an option for progressive disease.

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