Langerhans cell histiocytosis on the penis: a case report

Background

Protein S deficiency is an inherited cause of thrombophilia. We present the second reported case in the literature of a man developing testicular infarction secondary to protein S deficiency.

Case presentation

A 63 year old man presented with sudden onset of pain in his left hemi-scrotum. Despite oral warfarin therapy the plasma INR was only 1.4 at presentation. Doppler ultrasound scan of the scrotum confirmed absent blood flow to the left testis with increased echogenicity. Orchidectomy was performed to remove the necrotic testis. Post-operatively the patient did well and was referred to the Regional Haemophilia and Thrombosis Centre for further management.

Conclusion

The case highlights a rare but potential complication of protein S deficiency and demonstrates the importance of adequate anticoagulation in these patients.     

Solitary Langerhans cell histiocytosis arising from sternum: a case report

Langerhans cell histiocytosis (LCH) is a rare idiopathic benign disease characterized by proliferation of Langerhans cells, most commonly in the skull. In extremely rare cases, the tumor can occur in the sternum. A 4-year-old girl presented to our institution with a firm, nontender mass in her anterior chest that had indolently enlarged to approximately 2 cm in diameter over the previous several months. Computed tomography, magnetic resonance imaging, and F18-flurodeoxy-glucose positron emission tomography computed tomography were performed for preoperative diagnosis, but the findings were inconclusive. Therefore, we performed surgical excision under general anesthesia, followed by microscopic and immunohistochemical analysis of the excised specimen. The mass was eventually diagnosed as a LCH arising from the sternum. No postoperative oncological treatment was given, and follow-up has continued for 1 year until the time of writing without any tumor recurrence. To our knowledge, only 10 cases of LCH arising from the sternum have been reported in the medical literature. Among them, surgical approaches including curettage and partial sternotomy were performed in only 6 cases. Without exception, all patients experienced excellent clinical outcomes. Therefore, additional clinical experiences are required. No standard treatment of choice for this disease currently exists. In our experience, curettage of the involved soft tissue mass and bone followed by appropriate reconstruction of the defect is considered a good option for the treatment of solitary LCH of the sternum. In addition, LCH should be considered in the differential diagnoses when a sharp delineated osteolytic mass is detected in the sternum.     

Recurrent torticollis secondary to Langerhans cell histiocytosis: a case report

Torticollis is a common clinical sign encountered by pediatricians and orthopaedic surgeons in a wide spectrum of childhood conditions ranging from benign to life-threatening. We report the case of a child with recurrent torticollis caused by Langerhans Cell Histiocytosis (LCH). The patient was a 1-year-old boy with recurrent torticollis, followed by a painless swelling over the right temporal bone. The diagnosis was confirmed by an open biopsy of the calvarial lesion. As LCH is a very rare cause of torticollis it was not considered in the initial differential by the primary care physicians and the diagnosis was delayed about 4 months. The patient received chemotherapy with steroids and etoposide for 52 weeks. He showed complete regression of the sign and imaging tests at the end of treatment were normal. No relapse of symptoms occurred during a follow-up period of 2 years. The rarity of this disease as well as the site and form of presentation are emphasised to alert physicians for an early diagnostic evaluation, which is important to prevent neurological lesions and other late complications.     

Resection of a Langerhans cell histiocytosis granuloma of the hypothalamus: case report

The natural course and optimal treatment for isolated hypothalamic Langerhans cell histiocytosis (LCH) are unknown. We describe an adult female in whom total resection of a hypothalamic LCH granuloma was performed 12 years after transphenoidal resection of a pituitary adenoma. A retrospective review of the histological specimen of the first operation revealed CD1a positive cells characteristic of LCH along with a plurihormonal adenoma 12 years earlier. No other manifestations of LCH were found and MRI of the brain at the last follow-up 4 years after surgery did not show any recurrent or additional lesion. The diagnosis of isolated hypothalamic LCH is only possible by biopsy and our case demonstrates the feasability of a gross total resection in certain cases.     

Langerhans cell histiocytosis revealed by painful hip: A case report

Langerhans cell histiocytosis is a rare disease in childhood. It has a very polymorphous clinical expression, ranging from a single bony disease to a multisystemic disease involving vital organs. Prognosis has been improved by use of chemotherapy. We report a 2-year-old girl with multifocal Langerhans cell histiocytosis of bone and skin, revealed by a pain of the left hip and a limp trailing from 2 months. The diagnosis has been established by histological exam. The patient received chemotherapy and steroids. Outcome was favourable over 6-month follow-up. Trailing limp should evoke diagnosis of Langerhans cell histiocytosis. Skin biopsy should be preferred to a bone biopsy because of its safety.     

Langerhans cell histiocytosis of the spine causing cord compression: case report.

A case of Langerhans cell histiocytosis of the spine causing myeloradiculopathy is reported. Almost complete regression of symptoms and signs was observed in the patient after surgical treatment and radiotherapy. The clinical and histological features of the disease are discussed. The literature dealing with the previously reported cases of spinal histiocytosis involving neural structures is reviewed.     

Langerhans cell histiocytosis of the temporal bones with infiltration of temporal muscle: a case report

A 9-year-old boy presented a painful right painful parietal mass that rapidly enlarged within a month. He had no other symptom of infection or malignancy. Laboratory findings were normal. Plain skull X ray demonstrated a "punched-out" lesion in the right parietal bone. Magnetic resonance (MR) imaging revealed a 4 x 4 x 3 cm mass centered in the right parietal bone which was enhanced after administration of a contrast material. In addition, as well as the mass region, the pericranial soft tissue was densely enhanced. The parietal bone mass was soft and successfully removed. Histological examination disclosed Langerhans cell histiocytosis (LCH) infiltrating the temporal muscle. MR imaging is useful for diagnosis of LCH of the skull with soft tissue involvement.     

Spontaneous regression of lung metastasis of renal cell carcinoma: a case report

A chest X-ray tomography revealed a metastatic shadow in the left lung of a 56-year-old man with pathologically established right renal cell carcinoma. The shadow was found to be regressed to a fibrous lesion on the 18th day after radical nephrectomy. Because of non-A, non-B, hepatitis, the anticancer treatment with alpha-interferon and 1-(2-tetrahydrofuryl-5-fluorouracil) was started 40 days after the nephrectomy. At that time, only a fibrous lesion was noted at the site of lung metastatic shadow. At present, the patient remains free of disease for 21 months after nephrectomy.     

髂骨朗格汉斯细胞组织细胞增生症1例

正患者,男,24岁,因右髋关节疼痛3个月入院。患者自诉3个月前无明显诱因出现右髋关节疼痛,疼痛呈锐性,沿右大腿后侧向膝关节放射痛,间断性发作,活动后加重,休息可缓解。曾就诊于当地医院,拍片后考虑"右髂骨骨肿瘤",因症状较轻,患者未接受系统治疗。近3个月患者自觉疼痛逐渐加重,为求进一步诊治于2016年5月收住我院。入院查体:右侧髋关节周围皮肤无发红,局部无肿胀,皮温正常。右侧腹股沟中点处压痛阴性,右侧股骨大转子处叩击痛阴性,Faber试验阴性,Thomas征阴性。右侧髋关节活动范围:伸0°,屈曲100°,内收35°,外展45°,     

Langerhans cell histiocytosis of the vulva: two case reports

This report describes the histopathologic results of Langerhans cell histiocytosis of the vulva and options for treatment. We present two new cases demonstrating vulvar manifestations of disease and their course of treatment with a review of the literature. Langerhans cell histiocytosis of the female genital tract is rare. The disease cannot be diagnosed without biopsy of cutaneous lesions. Langerhans cell histiocytosis of the vulva is a rare disorder with few options for treatment.     

Incidental finding of Langerhans cell histiocytosis of temporoparietal bone - A case report

Introduction and importanceLangerhans cell histiocytosis (LCH) is a rare haematological disorder affecting infants and young children and has an estimated incidence of 2-5 cases per million people per year. LCH invades the reticuloendothelial system and causes the proliferation of Langerhans cells and mature eosinophils. LCH involving the temporoparietal bone has rarely been reported in the literature.Presentation of caseA ten-year-old boy presented to the Neurosurgical outpatient clinic with a swelling on the right temporoparietal region following a fall from his bicycle. Local examination revealed a single, 3 × 3 cm, non-tender, cystic, immobile swelling in the right temporoparietal region. On evaluation for recent head trauma, an incidental finding of eosinophilic granuloma was discovered on a CT scan. The FNAC was suggestive of a histiocytic lesion pertaining to a diagnosis of LCH. The patient underwent wide excision of the mass and cranioplasty. A one-month follow-up CT scan of the head had no evidence of residual or recurrent disease.DiscussionEosinophilic granuloma is one of the three variants of LCH and has a relatively better prognosis. Clinical diagnosis can be challenging and mandates tissue sampling for histopathological examination. Treatment modalities including surgery, radiotherapy, chemotherapy, and steroid injection are used alone, or in combination, depending on the extent and severity of the disease.ConclusionExamining a swelling in the temporoparietal region with no other characteristic symptoms could be a case of LCH. The timely diagnosis and surgical excision with other adjuvant treatment options of this rare pediatric disease would help in a better outcome.     

Spontaneous regression of testicular seminoma: case report

A 33-year-old man had a 1-year history of a painless testicular mass and a 2-month history of an enlarging left supraclavicular mass. Biopsy of the supraclavicular mass showed classical seminoma. Massive abdominal lymphadenopathy was demonstrated by computerized tomography. Radical orchiectomy was performed and examination of the specimen showed only fibrous scar tissue consistent with regression of a primary tumor in the testis. While complete regression of a primary testicular tumor with progressive growth of metastases is well recognized in cases of nonseminomatous germ cell tumors, this phenomenon is reported rarely with seminoma.     

Vertebral Langerhans cell histiocytosis in an adult patient: case report and review of the literature

Vertebral Langerhans cell histiocytosis, formerly called Histiocytosis X, is rarely seen in adults: a systematic non-quantitative review of the literature yielded only 27 cases. Vertebra plana is often associated in children, but this is not a feature in the adult population. The authors report the case of a 29-year-old woman with a two-month history of lumbar pain. Osteolysis of the right pedicle of L2 was noted on CT and MRI. Transpedicular curettage and fusion L1L3 were performed. Histopathological examination revealed Langerhans cell histiocytosis. Although rare, Langerhans cell histiocytosis should be included in the differential diagnosis of the solitary lytic vertebral lesion in the adult. Good results have been reported, in the absence of systemic manifestations, with the most conservative approach possible.     

Spontaneous regression of thymoma; report of a case

A 44-year-old woman was admitted to our hospital with chest pain. Chest roentgenograms and computed tomography (CT) scan revealed an anterior mediastinal tumor and bilateral pleural effusion. However, CT scan 3 days after magnetic resonance imaging (MRI) revealed regression of the tumor. Extended thymo-thymectomy was performed via median sternotomy. The tumor was in the right lobe of the thymus. Pathologically the tumor was diagnosed as a mixed type non-invasive thymoma, but some parts of the tumor cells were necrotic. This is the 10th case of spontaneous regression of thymoma reported in the Japanese literature.     

Spontaneous regression of extramedullary plasmacytoma--a case report

A case of extramedullary plasmacytoma in the soft tissues of the posterior chest wall of an 80-year old man is reported herein. Immunofluorescence study showed that the tumor cells produced IgG lambda. An M-component was also detected in the patient's serum by paper electrophoresis. Two months following the open biopsy done to establish diagnosis, the tumor underwent spontaneous regression and the M-component in the serum also disappeared. This is the first case report of spontaneous regression of an extramedullary plasmacytoma and the probable reasons for this spontaneous regression are discussed herein.     

Langerhans cell histiocytosis limited to the pituitary-hypothalamic axis--two case reports

Langerhans cell histiocytosis rarely presents as a solitary lesion in the pituitary-hypothalamic region, and is indistinguishable from germinoma, which occurs much more frequently, especially in Japanese. A 14-year-old girl and a 9-year-old girl presented with polydipsia and polyuria as the initial symptoms. Magnetic resonance (MR) imaging demonstrated a round mass at the pituitary stalk appearing as isointense on T1-weighted imaging and hyperintense on T2-weighted imaging. Endocrinological examination revealed mild hypopituitarism with central diabetes insipidus. Both patients underwent open craniotomy. Histological examination revealed granulomatous tissue with eosinophil infiltration and frequent Langerhans histiocyte clustering, compatible with the diagnosis of Langerhans cell histiocytosis. Low-dose local irradiation of 20 Gy was administered. First patient was followed up for 8 years, and her hypopituitarism gradually improved to a minimal level with only amenorrhea as the residual symptom. Recent MR imaging showed no residual mass at the region. Second patient was followed up for 15 months, and her diabetes insipidus is stable. MR imaging performed 5 months after the treatment showed marked reduction of the mass. These cases reemphasize the importance of histological diagnosis for lesions with similar neuroimaging appearances. Biopsy and low-dose irradiation are an effective treatment for this rare and essentially benign lesion, as opposed to attempting total removal of the mass.